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Home , Abdomen, Abdominal cavity, Abdominal wall, Aggressive fibromatosis, Gastrointestinal tract, Greater omentum

and

2 DISEASES OF THE AND RETROPERITONEUM

Amanda K. Arrington, MD, and Joseph Kim, MD

Anatomy and Physiology: Peritoneum transverse mesocolon, on the other , is the of the and suspends this structure from the posterior . The root of the transverse The word peritoneum is derived from the Greek terms peri mesocolon extends across the descending and (“around”) and tonos (“stretching”). The peritoneum, which the head of the and continues along the inferior lines the innermost surface of the abdominal wall and the border of the body and tail of the pancreas. The transverse majority of the abdominal organs, consists of a layer of mesocolon is continuous with the duodenocolic on dense stroma covered on its inner surface by a single sheet the right and with the phrenicocolic and splenorenal liga- of mesothelial cells. In men, the peritoneum is completely ments on the left. Finally, the sigmoid mesocolon attaches enclosed, whereas in women, the peritoneum is open to the the to the posterior pelvic wall. This mesen- exterior only at the ostia of the fallopian tubes. The perito- tery, which has an inverted V-shape confi guration, with its neum is divided into two components: the parietal and the apex lying anterior to the bifurcation of the left common ilia c visceral peritoneum [see Figure 1]. The parietal peritoneum , contains both sigmoid and hemorrhoidal vessels, covers the innermost surface of the abdominal walls, the nodes, , and abundant fat .3 inferior surface of the diaphragm, and the . The vis- ceral peritoneum, on the other hand, covers the majority physiology of the intraperitoneal organs [see Table 1] and the anterior The supply and innervation of the peritoneum aspect (only) of the retroperitoneal organs [see Table 1]. As depend on whether it is visceral peritoneum or parietal peri- shown in Figure 1, the intraperitoneal organs are suspended toneum. The blood fl ow to the visceral peritoneum is sup- by of peritoneum within the abdomen that have plied by the splanchnic blood vessels, whereas the parietal previously been identifi ed by Meyers and colleagues [see peritoneum is supplied by intercostal, subcostal, , Figure 1 and Table 1].1 The spread of , as well as the and iliac vessels. Correspondingly, the innervation of the spread of a primary tumor or metastatic disease, can be pre- two components is divided: the visceral peritoneum is sup- dicted based on subdivisions of the abdominal compart- plied by nonsomatic nerves, whereas the parietal peritone- ments created by these peritoneal ligamentous structures of um is supplied by somatic nerves. This detail is important the abdomen. when evaluating for pain. Visceral pain is poorly localized The omentum (greater and lesser omentum) is a well- and vague and is generally caused by stretching, distention, vascularized double fold of peritoneum with fat that torsion, and twisting . On the other hand, parietal contributes to the control of intra-abdominal infection and pain is caused by direct stimulation of fi bers and is infl ammation. The omentum aids in sealing off perforations described as sharp and well localized. (as in perforated or perforated ) and controls infl ammation (i.e., unruptured appendicitis). The peritoneum is a bidirectional, semipermanent bio- Additionally, the omentum delivers phagocytes that destroy membrane that controls the amount of fl uid in the perito- unopsonized . Thus, the omentum has multiple neal cavity. The typically contains less than functions in the body’s defense system against infection. 100 mL of sterile peritoneal fl uid. This peritoneal fl uid may The small bowel mesentery and transverse mesocolon are act as both part of the local defense system and a lubricant also complex peritoneal folds that originated from the dorsal for intraperitoneal organs. In certain disease states, such as and ventral midline early in development and , congestive failure, , or are considered part of the peritoneum. The small bowel , the amount of peritoneal fl uid may mesentery suspends the and from the poste- increase from the normal less than 100 cc protective amount rior abdominal wall. This mesentery contains the superior to several liters in volume [see , below]. mesenteric vessels, lymph nodes, nerves, and fat tissue. Its Circulation of peritoneal fl uid is driven in part by the root originates at the duodenojejunal junction and extends movement of the diaphragm. The peritoneal cavity can downward in an oblique direction across the , inferior absorb peritoneal fl uid through two different systems. vena cava, right , and psoas muscle to the right iliac Substances smaller than 2 kDa are absorbed through the at the ileocecal junction. Across the mesenteric root, peritoneal mesothelial venous pores directly into the portal the small bowel mesentery is in anatomic continuity with circulation.4 Conversely, larger particles (> 2 kDa) are the extraperitoneal anterior pararenal space, thus providing absorbed through the peritoneal mesothelial lymphatics a channel for the spread of disease.2 As a result of its shorter and enter the via the thoracic duct. This length (15 cm) in comparison with the much longer length particular route of absorption plays an important role in of the small bowel itself (6 to 8 m), the small bowel mesen- controlling abdominal and has been shown to tery has a pleated appearance along its intestinal border. The propagate the metastatic spread of certain .

ACS © 2014 Decker Intellectual Properties Inc DOI 10.2310/7800.2257

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Liver Visceral Peritoneum

Parietal Peritoneum Pancreas

Mesocolon

Duodenum

Colon

Small Bowel Mesentery

Small Intestine

Douglas Pouch

Bladder

Figure 1 Peritoneal components. The peritoneum consists of parietal peritoneum and visceral peritoneum. Visceral peritoneal covers the surface of intraperitoneal organs. The omentum and mesentery are also peritoneal structures.

Anatomy and Physiology: Retroperitoneum inferiorly. Organs are classifi ed as retroperitoneal The retroperitoneum is defi ned as the space between the in location if they have no peritoneal lining whatsoever or posterior parietal peritoneum and the posterior body wall. have peritoneum on their anterior surface only [see Table 1]. Although it has no specifi c delineating anatomic structures, Furthermore, structures that are not suspended by mesen- the retroperitoneal space is bound anteriorly by the poste- tery in the and that lie between the pari- rior refl ection of the peritoneum, superiorly by the dia- etal peritoneum and the abdominal wall are also classifi ed phragm, and inferiorly by the muscles. This as retroperitoneal in location; this includes the duodenum, space also includes the lumbar fossa, extending from the , and [see Figure 2]. 12th thoracic spine superiorly to the base of the and Because of the compliance of the anterior border of the

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Table 1 Abdominal Components Retroperitoneal operative approaches may be benefi cial for a number of reasons. First, it avoids entrance in to the Intraperitoneal organs peritoneum and, thus, decreases manipulation and the risk Gastrointestinal Stomach of to intra-abdominal organs. Minimal manipulation of the small intestine, for instance, signifi cantly decreases Transverse colon complications such as postoperative . Given successful avoidance of entering the peritoneum, intra-abdominal Endocrine 7 adhesions are also minimized. Retroperitoneal structures Gastrointestinal Diseases of the Peritoneum Duodenum Ascending colon ascites Descending colon Pancreas Pathophysiology and Etiology Vascular Inferior vena cava Ascites is the pathologic accumulation of fl uid within the Aorta peritoneal cavity. The primary cause of nonmalignant asci- Lymphatics tes accumulation is (cirrhosis and portal hyper- Iliac Renal tension), which accounts for 85% of cases of ascites in the Kidneys United States. Other causes of ascites are listed in Table 2 [see Table 2]. In cirrhotic patients, the onset of ascites is an Bladder overall poor prognostic factor as patients are more likely Endocrine Adrenals to develop spontaneous bacterial (SBP), renal Sexual (hepatorenal) failure, decreased quality of life, and decreased overall survival.8 Vas deferens Accumulation of ascites in patients with cirrhosis is secondary to the combination of renal sodium and water Other retention with portal hypertension [see Figure 3]. Driven Nerves by activation of the renin-angiotensin-aldosterone system, Peritoneal ligaments and mesenteries renal sodium retention is the result of proximal and distal Ligaments renal tubule sodium reabsorption. The renin system is acti- Splenorenal vated in response to decreased circulating blood volume Gastrohepatic Coronary secondary to the redistribution of fl uid and accumulation Hepatoduodenal of ascites within the abdomen. Furthermore, in cirrhotic Falciform patients, the accumulation and deposition of lead Gastrocolic to postsinusoidal vascular obstruction, which eventually Duodenocolic Gastric splenic results in portal hypertension [search the publication for infor- Phrenocolic mation on portal hypertension]. Increased hydrostatic pressure Mesenteries within the hepatic sinusoids and the splanchnic vasculature Transverse mesocolon Small bowel mesentery drives the extravasation of fl uid from the microvasculature into the extracellular compartment. Ascites then results when the capacity of the lymphatic system to return this accumulation of fl uid within the extracellular compartment to the systemic circulation is overloaded.9 retroperitoneum (the posterior parietal peritoneum), retro- Although cirrhosis is, by far, the most common cause of peritoneal tumors tend to expand anteriorly toward the ascites, Table 2 lists a number of other etiologies of ascites peritoneal cavity. The retroperitoneal space is divided into three spaces: the [see Table 2]. Of note, patients with develop anterior, the posterior, and the retrofascial spaces. The ante- ascites by one of three mechanisms. First, occlusion of the rior space consists of the second and third segments of the portal venous system by compression from malignancies, as duodenum, the pancreas, and the ascending and descending in liver metastases from colorectal , can cause portal branches of the colon, whereas the kidneys, the adrenal hypertension with resultant ascites. Next, diffuse peritoneal , the aorta, and the inferior vena cava are located in malignancies or metastases (carcinomatosis) can release the posterior space.5,6 Finally, the retrofascial space, which -rich fl uid into the peritoneal cavity, which accumu- extends between the transversalis and posterior pari- lates as ascites. Finally, when tumor burden obstructs the etal wall, contains the and paravertebral lymphatic drainage system (thoracic duct), resulting in rup- musculature. ture of major lymphatic channels, the leakage of chyle into Structures contained in the retroperitoneal space, in the peritoneal cavity results in an accumulation of a chylous particular the aorta, the inferior vena cava, the iliac vessels, ascites. This can be seen in cancers such as the kidneys, and the adrenals, can be operatively approached and retroperitoneal tumors, including sarcomas, or due to transabdominally or via the retroperitoneal approach. iatrogenic injury after extensive retroperitoneal .

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Inferior Vena Cava

Spleen Right Adrenal

Pancreas

Right

Left Kidney

Duodenum

Ureter

Abdominal Aorta

Cecum

Rectum

Inguinal Ligament

Figure 2 Retroperitoneal structures.

Diagnosis and/or pain secondary to ascites. Perhaps more importantl y, The diagnosis of ascites is made on the basis of the medi- for ascitic fl uid analysis is the most rapid and cal history and the appearance of the abdomen. Approxi- cost-effective method of determining the etiology of ascites. mately 1.5 L of fl uid must be present before dullness can Paracentesis can be performed safely in most patients, be detected by . Because cirrhosis is the most including those with cirrhosis and mild coagulopathy. Only common cause of ascites, evaluation of any physical evi- clinically evident disseminated intravascular coagulation dence of cirrhosis is important, such as palmar erythema, and fi brinolysis are contraindications to paracentesis in dilated abdominal wall collateral (varicosities), and patients with ascites.10 multiple angiomas. Other systemic causes of ascites, The serum-ascites albumin gradient (SAAG) is the most such as congestive heart failure, may also have signs on reliable method for quickly categorizing the various causes physical examination (i.e., jugular venous distention). of ascites [see Table 3]. This gradient is the difference between Paracentesis is benefi cial in both diagnosis and treatment. serum albumin concentration and ascitic fl uid albumin First, paracentesis allows for symptomatic relief of pressure concentration. SAAG values greater than 1.1 g/dL represent

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Table 2 Principal Causes of Ascites (suprahepatic) space and the subhepatic space. Possible eti- ologies of abscesses in this compartment include GI sources Portal hypertension (, perforated ulcer), trauma, postopera- Cirrhosis Secondary to tive complications, and biliary causes. The right subdia- Secondary to phragmatic space consists of the concavity between the right Secondary to primary sclerosing cholangitis hemidiaphragm and the dome of the liver, with the inferior Noncirrhotic causes limits of this space being the attachments of the coronary Portal venous obstruction and triangular ligaments of the liver. The left subdiaphrag- Multiple hepatic metastases causing obstruction Budd-Chiari syndrome matic space, on the other hand, is the space between the left hemidaphragm and the spleen. Trauma, postoperative com- Malignancies plications after upper abdominal or colon surgery, and gross Peritoneal carcinomatosis Primary peritoneal malignancies GI perforations can cause abscesses in this location. The Peritoneal right subhepatic space is located between the undersurfaces Metastatic disease of the liver and superiorly and the right kidney and mesocolon inferiorly. Perforated appendicitis and gall- Gastrointestinal cancers (gastric, colon, pancreatic) stone or other biliary disease are frequent causes of abscesse s Retroperitoneal obstruction of lymphatic system in this space. The left subhepatic space is essentially the lesser sac and is situated behind the lesser omentum and metastases stomach, lying anterior to the pancreas, duodenum, trans- Compression from retroperitoneal tumors verse mesocolon, and left kidney. Perforated peptic ulcers Cardiac and can cause abscesses in the left subphrenic Congestive heart failure space. The infracolic compartment is located below the Chronic pericardial tamponade transverse mesocolon and includes the pericolic and pelvic Infections cavity. Each lateral paracolic gutter and lower quadrant Tuberculous peritonitis area communicates freely with the . However, Other common causes whereas right paracolic collections may track upward Nephrotic syndrome into the subhepatic and subdiaphragmatic spaces, the ascites hinders fl uid migration along the left Pancreatic ascites paracolic gutter into the left subdiaphragmatic area. Pancreatitis Chylous ascites An intraperitoneal abscess should be suspected in any patient with a predisposing condition. The typical present- Rare causes ing symptoms of fever, tachycardia, and pain may be mild Meigs syndrome Vasculitis or absent, especially in patients receiving . Hereditary Prolonged ileus in a patient who has had recent abdominal surgery or peritoneal , worsening leukocytosis, or nonspecifi c radiologic abnormality may provide the initial or only sign of an intraperitoneal abscess. Treatment for an intraperitoneal abscess consists of ascites in patients with portal hypertension. In addition to immediate recognition, initiation of broad-spectrum anti- analyzing the ascitic fl uid albumin level, routine tests sent biotics, prompt drainage of the abscess, and identifi cation on ascitic fl uid from a paracentesis include cell count, total and control of the primary cause of the abscess (such as GI protein, glucose, Gram stain, and fl uid culture [see Table 3]. perforation). Percutaneous drainage is the preferred route In the case of suspected , ascitic fl uid should be for localized intraperitoneal abscesses that are approachable sent for cytologic examination. Other studies to consider via image guidance. Operative drainage is reserved for sending on ascitic fl uid are shown in Table 3 and are based intraperitoneal abscesses for which percutaneous drainage on the individual case [see Table 3]. is inappropriate or unsuccessful. Drainage may be inappro- priate or unsuccessful if there is not a safe radiographic peritoneal infections window through which to approach the abscess. Operative drainage may be indicated in cases where there is a persis- Intraperitoneal Abscesses tent focus of infection (e.g., diverticulitis or anastomotic An intraperitoneal abscess is a contained pocket of infec- dehiscence) after attempted percutaneous drainage. In cases tion within the abdominal cavity. Intraperitoneal abscess without evidence of continued soiling, the percutaneous may be caused by gastrointestinal (GI) perforations, postop- route has the advantage of establishing dependent drainage erative complications, , and genitouri- without contaminating the rest of the peritoneal cavity. 3 nary (GU) infections [see Table 4]. The areas in which abscesses commonly occur are defi ned by the confi guration Primary Peritonitis of the peritoneal cavity as compartmentalized by the perito- SBP is the most common cause of primary peritonitis neal ligaments, small bowel mesentery, and transverse and typically occurs in patients with underlying cirrhosis and sigmoid mesocolons. The supracolic compartment, or portal hypertension and resultant ascites. SBP is defi ned located above the transverse mesocolon, broadly defi nes the as an infection of previously sterile ascitic fl uid without an subphrenic spaces, consisting of both the subdiaphragmatic apparent intra-abdominal source of infection, in particular,

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CIRRHOSIS

Collagen deposition and fibrosis

PORTAL HYPERTENSION

SPLANCHNIC

INCREASED DECREASED ARTERIAL SPLANCHNIC PRESSURE FILLING

Increased Vasocontrictors and Antinatriuretic factors Lymphatic Fluid Accumulation Water retention Sodium Retention

PLASMA VOLUME EXPANSION

ASCITES

Figure 3 Development of ascites in cirrhotic patients.

without obvious GI perforation. Patients with ascites be started and then tailored once peritoneal fl uid culture unrelated to cirrhosis, such as nephrotic syndrome or con- results reveal the specifi c organism causing SBP. Surgical gestive heart failure, may also develop SBP. SBP results intervention is rarely indicated. from bacterial, chlamydial, fungal, or mycobacterial infec- tion in the absence of a perforation of the GI or GU tract. Secondary or Surgical Peritonitis This bacterial translocation is facilitated by depression of Secondary (surgical) peritonitis is a result of an infl amma- the hepatic reticuloendothelial system, commonly seen in tory process in the peritoneal cavity due to infl ammation, cirrhotic patients. perforation, or gangrene of the GI or GU tract. It is defi ned SBP can occur in patients with asymptomatic ascites; as the presence of pus or frank GI contents in the peritoneal therefore, the diagnosis should be suspected in all hospital- cavity. Diagnosis and early treatment of certain GI diseases, ized cirrhotic patients with ascites exhibiting any symptoms such as appendicitis or gangrenous , prior to the of or abdominal tenderness. SBP should development of secondary peritonitis signifi cantly decreases also be considered in patients with fever, leukocytosis, signs morbidity. Once peritonitis develops, surgical intervention of sepsis, renal insuffi ciency, or a history of hepatic enceph- is typically required because in most cases, secondary peri- alopathy. Diagnosis is made by diagnostic paracentesis and tonitis will lead to septic and death if left untreated. is established by an elevated neutrophil count of the ascitic Diagnosis of secondary peritonitis is based on the history, fl uid greater than 250 cells/mL. Peritoneal fl uid from the physical examination, and radiologic fi ndings [see Figure 4]. paracentesis should be sent for Gram stain and cultures Secondary peritonitis has a number of potential causes at the same time. If the neutrophil count is greater than [see Table 5], including perforated peptic ulcer, perforated 250 cells/mL, broad-spectrum antibiotics including cover- appendicitis, perforated diverticulitis, acute cholecystitis, age of gram-negative aerobes and anaerobes (particularly and/or postsurgical complications. Of note, blood within coli and Klebsiella) should be initiated. the peritoneum can cause symptoms similar to those of SBP. coverage, such as a third-generation cephalosporin, should Blood is locally highly irritating to the peritoneum and, thus,

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Table 3 Ascitic Fluid Analysis: Typical Labora- Table 4 Causes of Abscesses tory Studies to Order on Ascitic Fluid Obtained Intraperitoneal by Paracentesis Renal disease Postoperative Always ordered Trauma Cell count Cancer Albumin GI causes Total protein Appendicitis Diverticulitis Routinely ordered for definitive diagnosis lesions (cholecystitis) Gram stain Peptic ulcer perforation Fluid culture Inflammatory bowel disease (Crohn disease) Glucose Pancreatitis LDH Other (pancreatic ascites) Retroperitoneal Ordered in difficult cases Renal disease (chylous ascites) Pyelonephritis Cytology (malignancy) Urinary tract infection Bilirubin (bile ascites) Trauma TB culture, PCR test Spinal column infections Unhelpful tests Osteomyelitis Lactate Disk space infections pH Cancer GI causes Serum-ascites albumin gradient (SAAG) Pancreatitis High (transudate) (> 1.1 g/dL) Duodenal injury/perforation Cirrhosis Peptic ulcer perforation Appendicitis Congestive heart failure Diverticulitis Obstructive liver metastases Inflammatory bowel disease (Crohn disease) Fulminant Primary psoas abscesses Budd-Chiari syndrome Hematogenous origin (Staphylococcus aureus) Portal venous of GI = gastrointestinal. Myxedema Low (exudate) (< 1.1g/dL) Nephrotic syndrome Peritoneal carcinomatosis Broad-spectrum antibiotic therapy is required preopera- Tuberculous peritonitis tively, during surgery, and postoperatively. The microorgan- Chylous ascites ism causing secondary peritonitis varies by the perforation Pancreatic ascites site and underlying source of sepsis. In general, colonic per- Bile ascites forations necessitate coverage for gram-negative aerobic and Bowel anaerobic bacteria, whereas a peptic ulcer perforation may Obstruction//perforation Serositis require additional coverage. Although antibiotic coverage is necessary in generalized SBP, the condition will LDH = lactate dehydrogenase; PCR = polymerase chain reaction; TB = tuberculosis. not be resolved without operative intervention. Operative intervention should occur as soon as possible after the patient is stabilized and antibiotics have been initiated. mimics the of bacterial peritonitis. remains the standard of care in the treatment of Therefore, sources that introduce blood into the peritoneal SBP. Source control is the ultimate goal of any surgical inter- cavity, such as gynecologic causes, including rupture of an vention and may require repair or resection of the perforated or ruptured , can also lead to and/or débridement of necrotic or infected tissues. peritoneal signs and should be included in the differential Patients may benefi t from staged surgical procedures, with diagnosis. the fi rst surgery geared only toward source control and sub- As with SBP, secondary peritonitis can be caused by a sequent geared at verifi cation of source control number of bacterial organisms but is generally the result of and defi nitive surgical repair or plan of the diseased organ. 4 gram-negative and Enterococcus bacterial species. Because of If the omentum is able to contain and wall off the source the specifi c anatomy of the peritoneal cavity, patients with of generalized bacterial peritonitis, antibiotics alone will generalized secondary peritonitis may become very ill. The typically fail without further intervention, but operative large surface area of the peritoneal cavity can lead to mas- exploration may not be necessary. In this situation, an intra- sive fl uid loss as well as the sequestration or accumulation peritoneal abscess develops and can be treated as described of peritoneal fl uid secondary to bacteremia. Furthermore, above with localized drainage and antibiotic therapy. peritoneal circulation provides a route for rapid absorption The response rate to prompt and adequate treatment of bacteria, endotoxin (in the case of gram-negative (antibiotic administration and defi nitive source control) bacteria), and infl ammatory cytokines into the systemic for bacterial peritonitis is now approaching 70% in the lit- circulation, which can lead to sepsis. erature, which is a signifi cant improvement from historical

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b a

Figure 4 Representative radiologic fi ndings that can result in bacterial peritonitis. (a) Free air can be evaluated by chest x-ray and found as between the right hemidiaphragm and liver. (b) When the is ruptured, appendicitis can lead to peritonitis.

response rates. Despite advances in the management, recog- TB and is the sixth most common site of extrapulmonary TB nition, and subsequent treatment of SBP, morbidity and just after lymphatic, GU, and , miliary, and mortality remain particularly high in the elderly and in the meningeal TB. diabetic population. In fact, peritoneally related mortality Tuberculous peritonitis presents as a primary infection ranges anywhere from 14% in low-risk patients with without active pulmonary, renal, or intestinal involvement. isolated perforations to greater than 50% in patients with Nonspecifi c chronic symptoms can be present for weeks multiple comorbidities.11 to months prior to recognition and can include low-grade fever, night sweats, weight loss, anorexia, and malaise. Tuberculous Peritonitis Abdominal tenderness is present in only half of patients Although less common in the United States than in third with TB peritonitis.14 A positive tuberculin test is world countries, tuberculosis (TB) remains prevalent in the typically present, and ascitic fl uid will have an elevated United States, with an incidence of 3.2 per 100,000 people white blood cell count with a lymphocytic predominance.15 (approximately 10,000 new cases) in 2012.12 One of the major Microscopic examination of ascitic fl uid may be unhelpful as challenges in the treatment of TB, particularly in the United acid-fast bacilli are identifi ed in less than 3% of the cases. States, is the development of multiresistant strains of Myco- Lymphocytic ascites is not necessarily pathognomonic of bacterium tuberculosis, especially in vulnerable (homeless), TB peritonitis as patients with lymphocytic ascites without confi ned (incarcerated), and immunocompromised (HIV/ fever typically have a malignancy-related ascites.16 In fact, 13 AIDS) populations. In these at-risk populations, therefore, cancer is the underlying cause of lymphocytic ascites extrapulmonary TB should be given particular consider- 10 times more often than TB. However, if carcinomatosis ation. Tuberculous peritonitis is seen in 0.5% of new cases of is present, cytology from a paracentesis should be positive for malignancy, differentiating it from TB peritonitis in the majority of cases.17 In cases where peritoneal cytology is negative and cancer Table 5 Causes of Secondary Bacterial is not the etiology, a diagnostic can confi rm TB Peritonitis peritonitis with the specifi c fi ndings of scattered whitish peritoneal nodules [see Figure 5]. Any nodules visualized Estimated Mortality Rate should be biopsied. Histologic examination of these nodules Severity Causes (%) will demonstrate caseating granulomas in more than 90% of Mild Appendicitis < 10 cases. Additionally, laparoscopy will typically reveal multi- Perforated gastroduodenal ulcers ple adhesions between the abdominal organs and parietal Acute salpingitis peritoneum. Moderate Diverticulitis < 20 Treatment of TB peritonitis consists of at least a 6-month Small bowel perforation course of anti-TB starting with an 8-week course Cholecystitis (gangrenous) of isoniazid, rifampin, and pyrazinamide followed by iso- Severe Colonic perforations 20–50 niazid and rifampin for at least another 4 months.14 Depend- Ischemic bowel ing on susceptibilities, a longer course of antituberculous Necrotizing pancreatitis may be necessary.

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patients present with urinary symptoms and 25% present with GI symptoms (most commonly ). Computed tomography (CT) is often the best imaging study for diagnosing lipomatosis and readily demonstrates the abundance of pelvic fat as well as any extrinsic com- pression of surrounding pelvic organs (rectum, bladder). Furthermore, CT is helpful in differentiating between pelvic lipomatosis and liposarcomas. Evaluation should also include as 75% of patients with lipomatosis will have some degree of proliferative cystitis and nearly 40% will have cystitis glandularis.18 Medical treatment for pelvic lipomatosis has ranged from steroids to to radiation. However, these treatment strategies rarely improve the symptoms or the Figure 5 Tuberculosis peritonitis on laparoscopy. Nodules should overall condition. Complete surgical resection of pelvic be biopsied and sent for and culture. lipomatosis is diffi cult, and as such, surgery is not routinely recommended. Improvement in symptoms after surgery is rare. Fortunately, the disease is not progressive in most patients. Surgical intervention in patients with urinary obstruction (ureteral stenting, percutaneous nephrostomy, benign peritoneal tumors urinary diversion) may be required to alleviate obstructive Peritoneal Cysts symptoms. Peritoneal cysts are extremely rare but can arise in many Torsed Omentum forms. For example, benign cystic occur in Torsion of the omentum is a condition in which the omen- adult women and are typically manifested by pain. These tum twists on its long axis to the extent that its vascularity cystic lesions generally recur after resection. On the other is compromised. First described in 1899 by Eitel, few case 5 hand, peritoneal inclusion cysts, also referred to as perito- reports exist. Although rarely diagnosed preoperatively, neal , are cystlike structures that result from a knowledge of the entity is important because its symptoms nonneoplastic reactive mesothelial proliferation. Peritoneal may mimic the common causes of acute surgical abdomen. inclusion cysts occur almost exclusively in premenopausal Omental torsion is usually discovered during laparotomy women who have had a history of pelvic/abdominal for presumed appendicitis or another surgical cause of peri- surgery, pelvic/, or pelvic infl ammatory tonitis. When identifi ed at the time of laparotomy, the torsed disease. The primary presentation includes or a segment should be resected. pelvic mass that is secondary to the accumulation of ovarian fl uid contained within peritoneal adhesions. Thus, the Desmoids development of peritoneal inclusion cysts depends on the presence of an active and peritoneal adhesions. The Desmoid tumors are tumors that arise from the cysts can range in size from several millimeters to centime- and present as a single, slow-growing ters in diameter. Conservative treatment includes the use mass. Desmoids are benign, and their fi brous tissue appears of gonadotropin-releasing (GnRH) antagonists much like scar tissue. Despite their benign nature, desmoids or oral contraceptives to suppress ovulation and pain can be locally invasive into surrounding tissue. Most des- medication for symptomatic relief. Surgical resection is moid tumors are sporadic and, therefore, not inherited. recommended only in select cases and has a 30 to 50% risk Their incidence in the general population is two to four of recurrence. cases per million people per year in the United States, but the incidence is much higher in certain disease states, such Lipomatosis as familial adenomatous polyposis (FAP), in which the risk Lipomatosis is a rare disease involving an overgrowth of of developing a desmoid tumor is increased 1,000-fold com- 20 pelvic fat found in the perirectal and perivesical spaces. It pared with the general population. Although most patients occurs predominantly in African-American men between present with an asymptomatic mass, symptoms (most the ages of 40 and 60 years.18,19 The underlying etiology commonly pain) are related to mass effect from the tumor. remains unclear. Fogg and Smyth initially suggested that Other signs and symptoms are often caused by growth lipomatosis was comparable to Weber-Christian disease, of the tumor into the surrounding tissue or neighboring a generalized mesenteric panniculitis characterized by organs. increased tissue density and fi brosis within the mesentery.19 of the mass is required for defi nitive diagnosis of and fat overproduction by an infl ammatory process desmoid and will demonstrate spindle cells in a bed of such as proliferative cystitis, urinary tract obstruction, or a abundant fi brous stroma on histologic examination. On fur- GI infl ammatory process are postulated to be possible mech- ther histologic workup, desmoids stain positive for b-catenin, anisms of pelvic lipomatosis.18 The symptoms are variable , and vimentin. Treatment for desmoid tumors is widely and generally related to differing degrees of fi brous reaction variable and may include systemic therapy, radiation secondary to abnormal proliferation. Approximately 50% of therapy, and/or surgery. Surgery is typically the mainstay

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of therapy, with the goal of surgery being negative micro- and, in rare instances, gastric cancers. Patients with dissem- scopic (R0) margins. For large lesions, neoadjuvant radio- inated carcinomatosis should not be considered for this therapy may be given to shrink the tumor prior to surgery. procedure. Instead, eligible patients have limited peritoneal Radiation is typically administered postoperatively to disease that can be safely resected [see Cytoreductive Surger y reduce the risk of recurrence, especially in cases of positive and Hyperthermic Intraperitoneal Chemotherapy, below]. margins. alone is an acceptable treatment Pseudomyxoma Peritonei option in the case of surgically unresectable tumors. Medical management is usually reserved for those Pseudomyxoma peritonei (PMP) is characterized by the desmoids that have failed surgical and radiation treatment intraperitoneal dissemination of a large volume of mucinous or those that are deemed initially unresectable. Medical ascites. Unlike peritoneal carcinomatosis, in which the peri- therapy typically involves combinations of antiestrogenic toneum is coated in metastatic cancer, in PMP, the perito- agents ( or an aromatase inhibitor), nonsteroidal neum becomes coated with cells that secrete that antiinfl ammatory medications (ibuprofen or ), or can fi ll the peritoneal cavity with semisolid mucin. The targeted chemotherapy such as imatinib.21 mucinous material may arise from diffuse peritoneal adeno- mucinosis (DPAM), which consists of lesions composed of malignant peritoneal tumors scant simple to focally proliferative mucinous with little cytologic atypia or mitotic activity. On the other Peritoneal Metastases and Peritoneal Carcinomatosis hand, PMP can also arise from more aggressive peritoneal Primary malignancies of the peritoneum are rare. Instead, lesions composed of more abundant mucinous epithelium most cancers that involve the peritoneum are transperito- with the architectural and cytologic features of carcinoma, neal metastases originating from intra-abdominal carcino- typically seen in a ruptured appendiceal or mas. Peritoneal carcinomatosis occurs when metastatic ovarian cancer. PMP is a rare condition with an incidence nodules diffusely coat the peritoneum. Carcinomatosis is of approximately one in 1,000,000 per year.25 The degree of commonly associated with GI cancer metastases (colorectal malignant potential is variable in PMP. The of the or stomach) or the GU tract (ovarian). In fact, carcinomatosis cells causing PMP is an important predictor of survival, with can be the primary presentation in patients with malignan- adenomucinosis (DPAM) having the best survival rate (75% 6 cies such as ovarian cancer; therefore, in the setting of carci- at 5 years) and peritoneal mucinous carcinomatosis (PMCA) nomatosis in females, ovarian cancer should be high on the worst (14% at 5 years).26 the differential diagnosis. Other cancers that are known to Due to the underlying normal peritoneal circulation, neo- cause peritoneal carcinomatosis include , pancreatic, plastic cells and the resultant mucinous ascites spread freely 27 colon, and cancer. Noncarcinoma malignancies such to two main areas in the abdominal cavity. The fi rst area of as sarcomas and subsets of lymphomas can also diffusely accumulation is the upper abdomen at the typical peritoneal metastasize to peritoneal surfaces. fl uid absorption sites: the undersurface of the diaphragm The signs and symptoms of peritoneal metastatic disease (especially the right upper abdomen) and the omentum. The vary widely and can include weight loss, abdominal pain, second area of accumulation includes the dependent perito- and early satiety. Although ascites can also be present, this neal areas (the pelvic and lateral abdominal gutters). Distant metastases and extraperitoneal spread of PMP are rare. is usually seen in patients with large tumor burden and PMP is most often diagnosed in women between the ages not in the setting of initial primary manifestation of carcino- of 40 and 60 years. The degree of malignant potential is vari- matosis. Compared with non–malignancy-related ascites, able and can range from a low-grade PMP (DPAM, grade 1) malignant ascites is not well tolerated and can cause signifi - to the malignant variant, high-grade PMP (DMCA, grade 3). cant pain and symptoms even at low volumes. This intoler- Low-grade PMP is a benign disease at a microscopic level; ance may be due to decreased compliance of the abdominal however, even this slow-growing disease can cause signifi - wall secondary to the metastatic peritoneal deposits. These cant accumulation of mucin if not adequately treated. The patients may benefi t from repeated paracenteses for symp- outcome is eventually fatal without treatment. tomatic palliation. In addition, malignant ascites with high PMP patients are often asymptomatic until the develop- serum-ascites albumin ratios (> 1.1 g/dL) may respond ment of signifi cant mucin accumulation that occurs late in 22 well to medications. Unfortunately, patients with the course of the disease process. In retrospect, patients malignant ascites develop progressive symptoms with often elicit a chronic deterioration in their health status accumulation of fl uid; the ascitic fl uid can also be replaced months to years before the actual diagnosis. Presenting by a solid tumor component, which can lead to a malignant symptoms include abdominal pain and abdominal disten- obstruction. tion. Although not diagnostic, a CT scan may provide tell- Given the inherent metastatic nature of the underlying tale signs, such as low-attenuating, and often loculated, primary cancer, the response to routine therapies (chemo- fl uid throughout the peritoneum and omentum, scalloping therapy and/or radiation) is generally poor, with a median of the visceral surfaces (particularly the liver surface), survival without treatment on the order of 5 to 8 months (in and scattered calcifi cations throughout the abdomen [see the case of colon cancer).23,24 Therefore, newer treatments Figure 6]. Defi nitive diagnosis is made with percutaneous have been suggested, such as peritonectomy and cytoreduc- biopsy of the gelatinous material or, more frequently, at the tive surgery (CRS) combined with hyperthermic intraperito- time of laparotomy. neal chemotherapy (HIPEC). CRS-HIPEC has been approved The standard treatment of PMP is surgery. CRS alone was for peritoneal metastases secondary to colorectal, ovarian, the initial mainstay of treatment; however, relapses occur in

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than the degree of abdominal distention would suggest. The ascites, abdominal distention, and pain associated with peritoneal mesothelioma are typically intractable. Further- more, cytologic studies of ascitic fl uid are rarely positive. Radiologic fi ndings can include the presence of ascites, peri- toneal and mesenteric thickening, pleural plaques, and soft tissue masses involving the omentum and peritoneum on CT scan. Percutaneous or laparoscopy can establish the diagnosis. The traditional treatment strategy for peritoneal mesothe- lioma is CRS. Unlike PMP, peritoneal mesothelioma can locally invade surrounding tissues and organs, which may lead to unresectibility of disease. Encasement of the small bowel mesentery and the small bowel itself can lead to a malignant . Given this, complete tumor debulking may not be possible. However, in those cases of resectable disease, peritoneal mesothelioma is now treated with CRS-HIPEC, with signifi cant improvements in survival compared with CRS alone. In multiple studies, the 5-year overall survival approaches 50%, and the median overall Figure 6 Scalloping of liver surface secondary to mucin accumula- survival is now reported at 30 to 60 months.32,33 tion in pseudomyxoma peritonei. Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy With proper patient selection and the use of standard most cases and require repeated debulking surgeries. Cur- surgical techniques and HIPEC administration, the complex rently, the optimal treatment for PMP is now CRS-HIPEC CRS and HIPEC procedure can be performed with accept- [see Cytoreductive Surgery and Hyperthermic Intraperito- able morbidity and mortality [see Figure 7]. Complete cyto- neal Chemotherapy, below].28 A literature review of patients reductive resection of all tumor burden to macroscopically undergoing CRS-HIPEC reported an operative mortality of negative disease is the mainstay of this treatment modality. 2% and a morbidity of 40%, with a mean survival of 13 The disease burden may be estimated prior to surgical years.29 Historically, patients with PMP had 10-year survival consideration by determining the peritoneal cancer index rates of up to 20 to 30% only with the addition of debulking surgeries with each subsequent recurrence of disease. In (PCI). The PCI is a scoring system evaluating the extent of cases of PMP treated with optimal CRS-HIPEC, 10-year metastasis in the peritoneal cavity. The abdomen and pelvis overall survival has been reported up to 85%.25,30 In cases in are divided into 13 distinct regions [see Table 6] in which a which the appendix is the primary PMP site, a right hemico- lesion size (LS) score is determined for the largest tumor in lectomy is generally performed, although there is now that area. The signifi cance of the PCI will vary with the type debate on whether this is necessary given an adequate of peritoneal surface malignancy treated. In a study of negative resection margin with .25 sarcomatosis by Berhet and colleagues, a PCI less than 13 7 was associated with a 74% 5-year survival, whereas a PCI of Mesothelioma 13 or greater was associated with an 11% 5-year survival.34 Although rare, with an incidence of 300 to 500 cases/year Sugarbaker reported in colorectal carcinomatosis that a in the United States, peritoneal mesothelioma is the most PCI of 10 or less was associated with a 50% 5-year survival, common primary peritoneal tumor.31 Most mesotheliomas a PCI of 11 to 20 was associated with a 20% 5-year survival, are malignant and are directly related to asbestos exposure. and an index greater than 20 was associated with a 0% Development of peritoneal mesothelioma typically occurs 35 5-year survival.35 to 40 years after initial asbestos exposure. The differentiation At the time of laparotomy, CRS should include omentec- of malignant mesotheliomas from cystic mesotheliomas and tomy, stripping of the involved peritoneum (peritonectomy), well-differentiated papillary mesotheliomas in women is resection of any involved organs, and appendectomy if the essential because the latter two disease states are generally appendix is still present. No residual tumor nodules greater considered benign and carry a better prognosis. Malignant than 2 mm in diameter should be present after adequate peritoneal mesothelioma is more often seen in men, with the tumor resection. CRS is then followed by administration of median age at presentation in the mid-50s. HIPEC, typically in the form of mitomycin C or a platin- The typical presenting symptoms include weight loss, based chemotherapy [see Figure 8]. The rationale for applica- crampy abdominal pain, a large mass or distention second- tion of intraperitoneal chemotherapy is that it allows for ary to ascites, and a history of asbestos exposure. Less than larger local concentrations of the chemotherapeutic half of patients with peritoneal mesothelioma have asbesto- to directly reach tumor cells, whereas the hyperthermic sis of the demonstrated on plain chest fi lms. In temperature is cytotoxic itself and also potentiates the che- contrast to peritoneal carcinomatosis, malignant peritoneal motherapeutic agents. Furthermore, intraperitonal adminis- mesotheliomas are associated with a lower volume ascites tration decreases the overall systemic effects and side effects

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PERITONEAL CARCINOMATOSIS

DISSEMINATED DISEASE??

No Yes

Completeness of Cytoreduction Assessment

Laparotomy

Cytoreductive Cytoreductive Surgery Surgery Possible NOT Possible

Palliative Surgery

HIPEC Best Systemic Therapy

Figure 7 Evaluation for cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC).

of the chemotherapy. Mitomycin C is the chemotherapeutic of peritoneal carcinomatosis secondary to agent now recommended by the American Society of Perito- but also reported signifi cant improvement in survival, as neal Surface Malignancies (ASPSM), with oxaliplatin being noted by multiple studies.36–40 the next most common for colorectal cancer. A recent ASPSM HIPEC can be performed using an open technique, in consensus statement not only recommended standardiza- which the abdomen is left open to ensure adequate chemo- tion of patient selection and delivery of HIPEC in the setting therapy distribution throughout the abdominal cavity. The more common technique, however, is a closed HIPEC procedure, in which infl ow and outfl ow cannulas attached to temperature probes are placed in the abdomen, which is Table 6 Peritoneal Cancer Index Scoring to then closed prior to administration of the chemotherapy [see Determine Eligibility for HIPEC Figure 9, Figure 10, Figure 11, Figure 12, and Figure 13]. A com- Peritoneal cancer index (PCI) parison of the benefi ts of the open technique to the closed technique is shown in Table 7 [see Table 7]. Once closed, the Abdomen divided into 13 regions For each region, a lesion size (LS) score is calculated from abdomen is warmed with hyperthermic saline until the largest lesion intra-abdominal temperature approaches 40°C, and then LS-0 No tumor nodules hyperthermic chemotherapy is infused into the abdomen LS-1 Tumor nodules < 0.5 cm and circulated for up to 90 minutes. The closed technique LS-2 Tumor nodules 0.5–5.0 cm in size allows for easier maintenance of a hyperthermic tempera- LS-3 Tumor nodules > 5.0 cm in size ture (40°C). Next, the hyperthermic chemotherapeutic fl uid Total PCI score is drained from the abdomen and the procedure is completed PCI < 10 Resection recommended by performing any necessary anastomoses and/or ostomies, PCI 11–20 Resection may be considered followed by defi nitive abdominal closure. Some institutions PCI > 20 Resection should not be considered are evaluating laparoscopic HIPEC administration, although HIPEC = hyperthermic intraperitoneal chemotherapy. the results are mixed.41–43

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Thermistor Drugs

Reservoir

Roller Pumps

Heat Exchanger

Water Heater

Figure 8 Diagram of the closed hyperthermic intraperitoneal chemotherapy (HIPEC) system.

Figure 9 Cytoreductive surgery and hyperthermic intraperitoneal Figure 10 Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy: placement of cannulas for the closed abdominal hyper- chemotherapy (HIPEC): placement of cannulas and temperature thermic intraperitoneal chemotherapy (HIPEC) procedure. Both infl ow probes for the closed abdominal HIPEC procedure. and outfl ow cannulas have temperature probes to adequately deter- mine intraperitoneal temperature and maintain it at least as 40°C.

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Diseases of the Retroperitoneum

retroperitoneal abscesses Retroperitoneal infections are typically contained abscesse s and may be caused by hematogenous spread (primary) or, more typically, are due to infection from a neighboring organ (secondary). Abscesses occur less commonly in the retroperitoneum than in the peritoneal cavity. Specifi c examples include retrocecal appendicitis, perforated duode- nal ulcers, pancreatitis, perinephric abscesses (secondary to pyelonephritis or urinary tract infection), and diverticulitis [see Table 4]. The underlying etiology of the bacterial organ- ism is related to the offending organ. For example, cultures of a retroperitoneal abscess secondary to GI disease may grow a combination of gram-negative anaerobes or aerobes (Enterobacter, E. coli, or Bacteroides), whereas abscesses caused Figure 11 Both infl ow and outfl ow cannulas are placed intra- by a GU process are often secondary to a single organism abdominally through the midline incision. (Proteus or E. coli). Specifi c cases of intrapsoas abscesses may be the result of hematogenous spread, typically of Staphylo- coccus aureus. Other causes of intrapsoas abscesses can include osteomyelitis or disk space infections of the thoracic or lumbar spine. Typical symptoms of retroperitoneal abscesses include pain, fevers, chills, and malaise that have usually been pres- ent for a prolonged period of time. Given that this infection is contained within the retroperitoneal space, large abscesses can develop prior to the development of any symptoms. The location of the pain may vary depending on the location of the abscess and the offending organ. Laboratory values will include a leukocytosis. The diagnostic imaging of choice, CT, will show the location of the abscess, size, and involve- ment of surrounding tissue. As with abscesses in other sites of the body, treatment includes broad-spectrum antimi- crobial coverage and adequate percutaneous drainage. If percutaneous drainage is not feasible by image guidance, operative drainage may be necessary and should be done through a retroperitoneal approach to avoid peritoneal con- Figure 12 A watertight skin closure is required in the closed hyper- tamination. Operative drainage may also be necessary in a thermic intraperitoneal chemotherapy (HIPEC) technique to maintain multiloculated retroperitoneal abscess. Antibiotic coverage intra-abdominal hyperthermia. should be tailored to the offending organisms once cultures provide defi nitive results. retroperitoneal fibrosis Retroperitoneal fi brosis is a relatively uncommon disease characterized by extensive fi brotic encasement of retroperi- toneal structures secondary to hyperproliferation of fi brous tissue.44,45 Primary idiopathic retroperitoneal fi brosis, com- monly referred to as Ormond disease,46 affects approximatel y one in 200,000 people annually. Ormond disease is the underlying diagnosis in 70% of patients presenting with retroperitoneal fi brosis, whereas the other 30% of cases are secondary to medications, infections (most commonly GU infections), trauma, malignancies, retroperitoneal hemor- rhage, or iatrogenic causes (radiation therapy or surgical interventions).47 This disease affects men more than women (2:1) and is usually diagnosed in individuals 40 to 60 years old. Many cases of Ormond disease are associated with infl ammatory abdominal aortic aneurysms and, in these 44 Figure 13 The watertight skin closure includes the around infl ow cases, may be considered a form of chronic periaortitis. and outfl ow cannulas to maintain intraperitoneal hyperthermia and a Retroperitoneal fi brosis is usually confi ned to the central closed chemotherapy circulation system. and paravertebral spaces between the renal arteries and

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Table 7 Hyperthermic Intraperitoneal Chemotherapy (HIPEC): Open versus Closed Technique Comparisons 10 Open Closed Abdominal pressure Normal Raised Continued surgery Yes No Aerosol contamination Yes Reduced Distribution of heat and chemotherapy agent Uniform Variable Simultaneous perfusion of Yes No Management of technical problems Easy May be difficult Detection and management of occult bowel perforation Easier Difficult

tends to lead to encasement of the aorta, inferior vena cava, The goals of treatment of retroperitoneal fi brosis are and ureters. The underlying pathogenesis is likely multi- to stop the progression of the fi brous hyperproliferation, to factorial. There is considerable evidence to suggest that inhibit or relieve any obstructive symptoms, and to prevent Ormond disease is a manifestation of an overall systemic recurrence. The fi rst-line medical treatment is corticoste- autoimmune disease.44,48 roids, which work by suppressing acute-phase reactants Secondary retroperitoneal fi brosis is caused by a broad and decreasing the infl ammatory reaction.56 In the case of range of factors. The most common cause is use of certain steroid-resistant disease or intolerance of steroid therapy, drugs, although in these cases, the underlying reasons are immunosuppressants (, azathioprine, still debated.47,49–52 Some medications, such as methysergide methotrexate, or cyclosporine) have also been used com- and ergot alkaloids, are known to cause fi brotic reactions monly, with marginal results.57 Antiestrogenic agents, in that can affect not only the retroperitoneum but also other particular tamoxifen, have also been employed as second- 53 anatomic structures (, lungs) as well. Second- line therapy.58,59 Surgical intervention is reserved for those ary retroperitoneal fi brosis due to malignancy results from cases of retroperitoneal fi brosis that lead to severe ureteral the desmoplastic reaction to either metastases in the retro- obstruction as surgery does not address or prevent the peritoneum or to the primary cancer itself. For example, progression of disease.60 via a -mediated mechanism can induce a desmoplastic reaction that could involve the retroperito- retroperitoneal sarcoma 54 neum. On the other hand, radiation therapy may lead Soft tissue sarcomas are rare and unusual to a sclerosing fi brosis that is isolated to the radiation accounting only for 1% of adult cancers, with approximately fi eld itself. 10,000 sarcomas diagnosed in the United States each year. The macroscopic appearance of retroperitoneal fi brosis is Soft tissue sarcomas consist of a wide range of diseases with a white, hard retroperitoneal plaque of varying thickness. over 50 histologic types and subtypes that can develop in Again, this plaque develops between the origin of the renal any anatomic site. Thus, diagnosis, management, and subse- arteries and the . Microscopically, this process is quent treatment of sarcomas are extremely challenging. seen as sclerotic tissue infi ltrated by a mixture of mononu- With an incidence of 1,000 cases per year, retroperitoneal clear cells. Symptoms of retroperitoneal fi brosis are nonspe- sarcomas account for 10 to 15% of all soft tissue sarcomas. cifi c. Localized symptoms may include back or abdominal pain that is dull and constant in nature and not exacerbated Most cases are thought to be sporadic, although a history by movement. Constitutional symptoms may include of remote radiation therapy (> 10 years after exposure) and 61,62 fatigue, low-grade fever, , anorexia, and malaise. genetic factors have been shown to play a role. A few Physical examination is unhelpful in diagnosis for most cases have been shown to be caused by genetically inherited circumstances. diseases, including Li-Fraumeni syndrome, neurofi bromatosi s 63 Diagnostic studies, including laboratory studies, are con- type 1, and hereditary retinoblastoma. sistent with infl ammatory disease. For example, 80 to 100% In contrast to sarcomas of other anatomic sites, retroperi- of patients will have both an elevated erythrocyte sedimen- toneal sarcomas often present as large masses (50% are tation rate (ESR) and elevated C-reactive protein level.55 greater than 20 cm in diameter) as they are typically asymp- Of the autoimmune disease studies, positive antinuclear tomatic until compressive symptoms of the surrounding are most frequently found and are seen in structures develop. Abdominal pain is present in 50% of approximately 60% of patients. Imaging studies are essential patients diagnosed with retroperitoneal sarcoma. CT of the in diagnosis. Ultrasonography is the best fi rst-line noninva- abdomen and pelvis can provide vital details regarding the sive study as most patients will have urologic obstruction tumor size, the extent of the tumor, and its relationship to secondary to the underlying fi brotic reaction. If available, surrounding structures. MRI may be benefi cial to delineate magnetic resonance imaging (MRI) can also be used and nerve involvement or details regarding local organ invasion. provides better defi nition of surrounding soft tissues Percutaneous tissue biopsy and defi nitive diagnosis can and organs. typically be performed by CT guidance.

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Histologically, the most common subtypes of retroperito- Unfortunately, neoadjuvant or adjuvant chemotherapy neal sarcoma are liposarcoma, malignant fi brous histiocy- regimens have not been proven effective in soft tissue sarco- toma (MFH), and leiomyosarcoma. Given the nearly 50 mas. Most studies have failed to show a survival advantage subtypes of sarcoma, the diagnosis and staging of sarcomas of any chemotherapeutic regimen evaluated.66 Due to sig- are diffi cult at best. The 7th edition of the American Joint nifi cant local recurrence rates, radiation therapy has been Commission on Cancer (AJCC) includes grade and depth to proposed as a potential means of treating microscopic posi- the fascia as part of the staging system for some soft tissue tive surgical margins. There are no consensus guidelines sarcomas [see Table 8]. In particular, grade is the main deter- for the usual radiation dose or timing in patients with retro- minant for staging for retroperitoneal sarcomas as these peritoneal sarcomas. The role for external-beam radiation is malignancies are by defi nition deep to the fascia. Prognostic often limited by radiation tolerance and location of adjacent factors associated with poor survival include higher organs. Another factor that complicates radiation therapy of stage, higher histologic grade, and either unresectability or retroperitoneal sarcomas is the size of the tumor as larger positive surgical margins.64 tumors require much larger radiation fi elds. The timing When possible, complete surgical resection is the most of radiation therapy has also been evaluated. Preoperative effective treatment for retroperitoneal sarcomas. En bloc radiation may be benefi cial; however, it has not been well resections often include resection of neighboring organs due studied. For high-risk disease, 50 Gy preoperative radiation to local invasion of tumor. Lymph node dissection is gener- therapy followed by en bloc surgical resection has been ally not required as lymph node metastases for sarcomas suggested.67 are reported in less than 5% of cases. The essential part of Regardless of treatment strategy, 5-year recurrence rates surgical intervention is to achieve a negative margin (R0) of retroperitoneal sarcomas are high (nearly 75%), whereas resection. In a study of 500 patients with retroperitoneal overall 5-year survival is less than 50%.65,68 Retroperitoneal sarcoma, median survival times were improved by over sarcomas can recur locally or diffusely within the peritoneal 7 years (85 months) with complete R0 resection.65 cavity. Furthermore, retroperitoneal sarcomas can metasta- size, with the most common sites being the lungs and the liver. If recurrent disease is resectable, surgery is once again the treatment modality of choice.

Table 8 AJCC 7th Edition Staging System for Financial Disclosures: Amanda K. Arrington, MD, and Joseph Kim, MD, have no Sarcoma relevant fi nancial relationships to disclose. 8 T stage T0 No evidence of primary tumor References T1 Tumor < 5 cm T1a Superficial tumor 1. Meyers MA, Oliphant M, Berne AS, Feldberg MA. The T1b Deep tumor (below fasica, retroperitoneal) peritoneal ligaments and mesenteries: pathways of intraab- T2 Tumor > 5 cm dominal spread of disease. 1987;163:593–604. T2a Superficial tumor 2. Oliphant M, Berne AS. Computed tomography of the sub- T2b Deep tumor (below fasica, retroperitoneal) peritoneal space: demonstration of direct spread of intraab- N stage dominal disease. J Comput Assist Tomogr 1982;6:1127–37. NX Lymph nodes cannot be assessed 3. Healy JC, Reznek RH. The peritoneum, mesenteries and N0 No regional lymph node metastases omenta: normal anatomy and pathological processes. Eur N1 Regional lymph node metastases Radiol 1998;8:886–900. Grade 4. Dunn DL, Barke RA, Knight NB, et al. Role of resident GX Grade cannot be assessed , peripheral neutrophils, and translymphatic G1 Grade 1 absorption in bacterial clearance from the peritoneal cavity. G2 Grade 2 Infect Immun 1985;49:257–64. G3 Grade 3 5. Harris LF, Sparks JE. Retroperitoneal abscess. Case report M stage and review of the literature. Dig Dis Sci 1980;25:392–5. M0 No distant metastases 6. Ioannidis O, Kakoutis E, Katsifa H, et al. Streptococcus M1 Distant metastases mutans: a rare cause of retroperitoneal abscess. Adv Med Stage Sci 2011;56:113–8. 7. Gardner GP, Josephs LG, Rosca M, et al. The retroperitoneal IA T1a N0 M0 G1, GX T1b N0 M0 G1, GX incision. An evaluation of postoperative fl ank ‘bulge.’ Arch IB T2a N0 M0 G1, GX Surg 1994;129:753–6. T2b N0 M0 G1, GX 8. Sandhu BS, Sanyal AJ. Management of ascites in cirrhosis. IIA T1a N0 M0 G2, G3 Clin Liver Dis 2005;9:715–32, viii. T1b N0 M0 G2, G3 9. Rosner MH, Gupta R, Ellison D, Okusa MD. Management IIB T2a N0 M0 G2 T2b N0 M0 G2 of cirrhotic ascites: physiological basis of diuretic action. III T2a, T2b N0 M0 G3 Eur J Intern Med 2006;17:8–19. Any T N1 M0 Any G 10. Runyon BA, Canawati HN, Akriviadis EA. Optimization IV Any T Any N M1 Any G of ascitic fl uid culture technique. 1988;95: AJCC = American Joint Committee on Cancer. 1351–5.

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11. Seiler CA, Brugger L, Forssmann U, et al. Conservative 30. Vaira M, Cioppa T, De Marco G, et al. Management of pseu- surgical treatment of diffuse peritonitis. Surgery 2000;127: domyxoma peritonei by cytoreduction+HIPEC (hyperther- 178–84. mic intraperitoneal chemotherapy): results analysis of a 12. Centers for Disease Control and Prevention. Tuberculosis twelve-year experience. In Vivo 2009;23:639–44. incidence. 2012. Available at: http://www.cdc.gov/tb/ 31. Boffetta P. Epidemiology of peritoneal mesothelioma: a 9 statistics/tbcases.htm. review. Ann Oncol 2007;18:985–90. 13. Hopewell PC. Impact of immunodefi ciency virus 32. Yan TD, Yoo D, Sugarbaker PH. Signifi cance of lymph node infection on the epidemiology, clinical features, manage- metastasis in patients with diffuse malignant peritoneal ment, and control of tuberculosis. Clin Infect Dis 1992;15: mesothelioma. Eur J Surg Oncol 2006;32:948–53. 540–7. 33. Yan TD, Deraco M, Baratti D, et al. Cytoreductive 14. Sanai FM, Bzeizi KI. Systematic review: tuberculous surgery and hyperthermic intraperitoneal chemotherapy peritonitis—presenting features, diagnostic strategies and for malignant peritoneal mesothelioma: multi-institutional treatment. Aliment Pharmacol Ther 2005;22:685–700. experience. J Clin Oncol 2009;27:6237–42. 15. Runyon BA. Care of patients with ascites. N Engl J Med 34. Berthet B, Sugarbaker TA, Chang D, Sugarbaker PH. 1994;330:337–42. Quantitative methodologies for selection of patients with 16. Runyon BA. Malignancy-related ascites and ascitic fl uid recurrent abdominopelvic sarcoma for treatment. In: 8th “humoral tests of malignancy.” J Clin Gastroenterol 1994; World Congress of the International Gastro-Surgical Club. 18:94–8. Bologna: Monduzzi Editore; 1998. p. 899–903. 17. Runyon BA, Hoefs JC, Morgan TR. Ascitic fl uid analysis in 35. Sugarbaker PH. Successful management of microscopic malignancy-related ascites. 1988;8:1104–9. residual disease in large bowel cancer. 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AMANDA K. ARRINGTON, MD cr, Assistant Professor, Division of , Department of Surgery, University of South Carolina, Columbia, SC

JOSEPH KIM, MD, Associate Professor of Surgical Oncology, Department of Surgery, City of Hope Cancer Center, Duarte, 1 CA

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