Mucinous Epithelial Neoplasms of the Appendix and Pseudomyxoma Peritonei Joseph Misdraji
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Hyperthermic Intrathoracic Chemotherapy for Malignant Pleural Mesothelioma: the Forefront of Surgery-Based Multimodality Treatment
Journal of Clinical Medicine Review Hyperthermic Intrathoracic Chemotherapy for Malignant Pleural Mesothelioma: The Forefront of Surgery-Based Multimodality Treatment Vittorio Aprile 1,†, Alessandra Lenzini 1,†, Filippo Lococo 2, Diana Bacchin 1,* , Stylianos Korasidis 1, Maria Giovanna Mastromarino 1, Giovanni Guglielmi 3, Gerardo Palmiero 4, Marcello Carlo Ambrogi 1 and Marco Lucchi 1 1 Unit of Thoracic Surgery, Department of Critical Area and Surgical, Medical and Molecular Pathology, University of Pisa, 56122 Pisa, Italy; [email protected] (V.A.); [email protected] (A.L.); [email protected] (S.K.); [email protected] (M.G.M.); [email protected] (M.C.A.); [email protected] (M.L.) 2 Thoracic Surgery Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Rome, Italy; fi[email protected] 3 Occupational Health Department, U.O. Medicina Preventiva del Lavoro, Azienda Ospedaliero-Universitaria Pisana, 56122 Pisa, Italy; [email protected] 4 Pneumology Unit, Versilia Hospital, 55049 Camaiore, Italy; [email protected] * Correspondence: [email protected]; Tel.: +39-0-5099-5230 † These authors contributed equally to this work. Abstract: Introduction: Malignant Pleural Mesothelioma (MPM) is characterized by an aggressive Citation: Aprile, V.; Lenzini, A.; behavior and an inevitably fatal prognosis, whose treatment is still far from being standardized. The Lococo, F.; Bacchin, D.; Korasidis, S.; role of surgery is questionable since a radical resection is unattainable in most cases. Hyperthermic Mastromarino, M.G.; Guglielmi, G.; IntraTHOracic Chemotherapy (HITHOC) combines the advantages of antitumoral effects together Palmiero, G.; Ambrogi, M.C.; Lucchi, with those of high temperature on the exposed tissues with the aim to improve surgical radicality. -
Pseudo-Pseudomyxoma Peritonei from Peritoneal Sarcomatosis
http://crcp.sciedupress.com Case Reports in Clinical Pathology, 2015, Vol. 2, No. 4 CASE REPORT Pseudo-pseudomyxoma peritonei from peritoneal sarcomatosis Shuja Ahmed1, Ling Guo2, Shadi A. Qasem2, Edward A. Levine1 1. Surgical Oncology Service, Department of General Surgery, Wake Forest University School of Medicine, Winston Salem, NC, USA. 2. Department of Pathology, Wake Forest University School of Medicine, Winston Salem, NC, USA. Correspondence: Edward A. Levine, MD. Address: Surgical Oncology Service, Medical Center Blvd, Winston-Salem, North Carolina, USA. E-mail: [email protected] Received: February 12, 2015 Accepted: April 12, 2015 Online Published: June 3, 2015 DOI: 10.5430/crcp.v2n4p14 URL: http://dx.doi.org/10.5430/crcp.v2n4p14 Abstract Background: Pseudomyxoma peritonei (PMP) is a rare clinical entity of mucinous ascites, most commonly associated with appendiceal mucinous neoplasms. Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) remains the current standard of care for PMP. Peritoneal sarcomatosis (PS) is an exceptionally rare disease with a poor prognosis. PMP associated with PS has not been previously described. The role of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for PS with or without PMP is not well-defined. PS manifesting like PMP has not been previously described. Case presentation: A 74-year-old patient with several weeks history of vague abdominal pain and increased abdominal girth was referred to our facility after incidental finding of PMP during laparoscopic inguinal hernia repair. After complete work-up, he was advised to undergo CRS/HIPEC. Intra-operatively, he was noted to have extensive mucinous ascites and underwent aggressive CRS and HIPEC Result: Final pathology revealed myxoid liposarcoma with associated intraperitoneal mucin dissemination, which was confirmed with cytogenetic analysis. -
Appendix Cancer and Pseudomyxoma Peritonei (PMP) a Guide for People Affected by Cancer
Cancer information fact sheet Understanding Appendix Cancer and Pseudomyxoma Peritonei (PMP) A guide for people affected by cancer This fact sheet has been prepared What is appendix cancer? to help you understand more about Appendix cancer occurs when cells in the appendix appendix cancer and pseudomyxoma become abnormal and keep growing and form a peritonei (PMP). mass or lump called a tumour. Many people look for support after The type of cancer is defined by the particular being diagnosed with a cancer that cells that are affected and can be benign (non- is rare or less common than other cancerous) or malignant (cancerous). Malignant cancer types. This fact sheet includes tumours have the potential to spread to other information about how these cancers parts of the body through the blood stream or are diagnosed and treated, as well as lymph vessels and form another tumour at a where to go for additional information new site. This new tumour is known as secondary and support services. cancer or metastasis. Many people feel shocked and upset when told they have cancer. You may experience strong emotions The abdomen after a cancer diagnosis, especially if your cancer is rare or less common like appendix cancer or PMP. A feeling of being alone is usual with rare cancers, and you might be worried about how much is known about your type of cancer as well as how it will be managed. You may also be concerned about the cancer coming back after treatment. Linking into local support services (see last page) can help overcome feelings of isolation and will give you information that you may find useful. -
Pleural and Peritoneal Cavities 95
94 Maher, Daly Management of bleomycin lung toxicity is immunosuppressive and known steroid spar- frequently difficult, though steroids are wide- ing effects. ly recommended and evidence supporting We believe that all patients with bleomycin their role comes from both animal studies6 lung toxicity should receive a trial of cortico- Thorax: first published as 10.1136/thx.48.1.94 on 1 January 1993. Downloaded from and clinical reports on a few patients.5 steroids. The dose used should depend on the Less settled, however, is the value of these severity of the pneumonitis. agents in advanced bleomycin lung toxicity. Samuels and coworkers7 described a series of 1 Umezawa H, Maeda K, Takeuchi T, Okami Y. New five such patients, all of whom received pred- antibiotics, bleomycin A and B. Journal of Antibiotics (Tokyo) 1966;19:200-9. nisolone in doses of 60-100 mg daily. All five 2 Kuo MT, Haidle CW. Characterization of chain breakage died of acute respiratory failure despite this in DNA induced by bleomycin. Biochem Biophys Acta 1974;335:109-14. treatment. Gilson and Sahn8 reported a 3 Chandler DB. Possible mechanisms of bleomycin-induced patient with bleomycin lung toxicity who fibrosis. In: Cooper J Allen D, ed. Clinics in chest medi- cine. Philadelphia: Saunders, 1990:21-30. developed the adult respiratory distress syn- 4 Holoye PY, Luna MA, Mackay B, Bedrossian CWM. drome after surgery and ultimately responded Bleomycin hypersensitivity pneumonitis. Ann Intern Med 1978; 88:47-9. to a combination of antibiotics and methyl- 5 Jules-Elysee K, White DA. Bleomycin-induced pulmonary prednisolone 500 mg a day. -
Ovarian Carcinomas, Including Secondary Tumors: Diagnostically Challenging Areas
Modern Pathology (2005) 18, S99–S111 & 2005 USCAP, Inc All rights reserved 0893-3952/05 $30.00 www.modernpathology.org Ovarian carcinomas, including secondary tumors: diagnostically challenging areas Jaime Prat Department of Pathology, Hospital de la Santa Creu i Sant Pau, Autonomous University of Barcelona, Spain The differential diagnosis of ovarian carcinomas, including secondary tumors, remains a challenging task. Mucinous carcinomas of the ovary are rare and can be easily confused with metastatic mucinous carcinomas that may present clinically as a primary ovarian tumor. Most of these originate in the gastrointestinal tract and pancreas. International Federation of Gynecology and Obstetrics (FIGO) stage is the single most important prognostic factor, and stage I carcinomas have an excellent prognosis; FIGO stage is largely related to the histologic features of the ovarian tumors. Infiltrative stromal invasion proved to be biologically more aggressive than expansile invasion. Metastatic colon cancer is frequent and often simulates ovarian endometrioid adenocarcinoma. Although immunostains for cytokeratins 7 and 20 can be helpful in the differential diagnosis, they should always be interpreted in the light of all clinical information. Occasionally, endometrioid carcinomas may exhibit a microglandular pattern simulating sex cord-stromal tumors. However, typical endometrioid glands, squamous differentiation, or an adenofibroma component are each present in 75% of these tumors whereas immunostains for calretinin and alpha-inhibin are negative. Endometrioid carcinoma of the ovary is associated in 15–20% of the cases with carcinoma of the endometrium. Most of these tumors have a favorable outcome and they most likely represent independent primary carcinomas arising as a result of a Mu¨ llerian field effect. -
Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy for Peritoneal Surface Malignancies: Learning Curve Based on Surgical and Oncological Outcomes
cancers Article Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy for Peritoneal Surface Malignancies: Learning Curve Based on Surgical and Oncological Outcomes Jerzy Mielko, Karol Rawicz-Pruszy ´nski* , Katarzyna S˛edłak,Katarzyna G˛eca, Magdalena Kwietniewska and Wojciech P. Polkowski Department of Surgical Oncology, Medical University of Lublin, Radziwiłłowska 13 St., 20-080 Lublin, Poland; [email protected] (J.M.); [email protected] (K.S.); [email protected] (K.G.); [email protected] (M.K.); [email protected] (W.P.) * Correspondence: [email protected]; Tel.: +48-881318964 Received: 29 June 2020; Accepted: 21 August 2020; Published: 23 August 2020 Keywords: CRS + HIPEC; peritoneal surface malignancies; learning curve 1. Introduction Cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) is a complex, highly specialized procedure used to treat peritoneal surface malignancies (PSM) [1–4]. The PSM includes both rare diseases of the peritoneum (pseudomyxoma peritonei, peritoneal mesothelioma) as well as metastases from various primary cancers (ovarian, colorectal, gastric, etc.) to the surface of peritoneum. Despite initial skepticism, CRS + HIPEC has become a widely accepted procedure in the treatment of pseudomyxoma peritonei, appendiceal cancer, metastatic colorectal cancer, and peritoneal mesothelioma [5–10]. Significant improvement in oncological results has also been reported in peritoneal dissemination from gastric and ovarian cancers compared to palliative treatment [11,12]. Achieving complete cytoreduction often requires extensive surgical procedure with multiple anastomoses, associated with relatively high complication rates (14–70%) [13]. In reference centers, perioperative mortality reaches 8%. This high rate has been attributed to the learning curve (LC), and it decreases after about 100–140 operations [14–18]. -
A Rare Cause of Painless Haematuria- Adenocarcinoma of Appendix
Ju ry [ rnal e ul rg d u e S C f h o i l r u a Journal of Surgery r n g r i u e o ] J ISSN: 1584-9341 [Jurnalul de Chirurgie] Case Report Open Access A Rare Cause of Painless Haematuria- Adenocarcinoma of Appendix Shantanu Kumar Sahu1*, Shikhar Agarwal2, Sanjay Agrawal3, Shailendra Raghuvanshi4, Nadia Shirazi5, Saurabh Agrawal1 and Uma Sharma1 1Department of General Surgery, Himalayan Institute of Medical Sciences, Swami Rama Himalayan University, Uttarakhand, India 2Department of Urology, Himalayan Institute of Medical Sciences, Swami Rama Himalayan University, Uttarakhand, India 3Department of Anesthesia, Himalayan Institute of Medical Sciences, Swami Rama Himalayan University, Uttarakhand, India 4Department of Radiodiagnosis, Himalayan Institute of Medical Sciences, Swami Rama Himalayan University, Uttarakhand, India 5Department of Pathology, Himalayan Institute of Medical Sciences, Swami Rama Himalayan University, Uttarakhand, India Abstract Neoplasms of the appendix are rare, accounting for less than 0.5% of all gastrointestinal malignancies and found incidentally in approximately 1% of appendectomy specimen. Carcinoids are the most common appendicular tumors, accounting for approximately 66%, with cystadenocarcinoma accounting for 20% and adenocarcinoma accounting for 10%. Appendiceal adenocarcinomas fall into one of three separate histologic types. The most common mucinous type produces abundant mucin, the less common intestinal or colonic type closely mimics adenocarcinomas found in the colon, and the least common, signet ring cell adenocarcinoma, is quite virulent and associated with a poor prognosis. Adenocarcinoma of appendix is most frequently perforating tumour of gastrointestinal tract due to anatomical peculiarity of appendix which has an extremely thin subserosal and peritoneal coat and the thinnest muscle layer of the whole gastrointestinal tract. -
Hyperthermic Intrathoracic Chemotherapy (HITHOC) for Pleural Malignancies—Experience from Indian Centers
Indian Journal of Surgical Oncology (February 2019) 10 (Suppl 1):S91–S98 https://doi.org/10.1007/s13193-018-0859-y ORIGINAL ARTICLE Hyperthermic Intrathoracic Chemotherapy (HITHOC) for Pleural Malignancies—Experience from Indian Centers Mahesh D. Patel1 & Dileep Damodaran2 & Ashvin Rangole3 & Sakina Shaikh1 & Kairav Shah4 & Rajendra Bagwade4 & Aditi Bhatt1 Received: 14 November 2018 /Accepted: 4 December 2018 /Published online: 11 December 2018 # Indian Association of Surgical Oncology 2018 Abstract Hyperthermic intrathoracic chemotherapy (HITHOC) has been used in addition to radical surgery for primary and secondary pleural malignancies to improve local control, prolong survival, and improve the quality of life. This study was performed to study the indications, methodology, perioperative outcomes, and survival in patients undergoing HITHOC at Indian centers. A retrospective analysis of prospectively collected demographic and clinical data, perioperative and survival data of patients undergoing surgery with or without HITHOC was performed. From January 2011 to May 2018, seven patients underwent pleurectomy/decortication (P/D) or extrapleural pneumonectomy (EPP) with HITHOC and four had P/D or EPP alone at three Indian centers. P/D was performed in two and EPP in nine patients. The primary tumor was pleural mesothelioma in eight, metastases from thymoma in one, germ cell tumor in one, and solitary fibrous tumor of the pleura in one. HITHOC was performed using cisplatin. Grade 3–4 complications were seen in one patient in the HITHOC group and none in the non- HITHOC group, and one patient in the non-HITHOC group died of complications. At a median follow-up of 9 months, five patients of the HITHOC group were alive, four without recurrence, and one with recurrence. -
Carcinoid Tumor of Appendix: Review of Consecutive 5131 Appendectomy
J Surg Med. 2018;2(2):134-136. Research article DOI: 10.28982/josam.414055 Araştırma makalesi Carcinoid tumor of appendix: Review of consecutive 5131 appendectomy Apendiksin karsinoid tümörü: Ardışık 5131 apendektominin incelemesi İlyas Kudaş 1, Olgun Erdem 2, Ahmet Topçu 2, Abdullah Şişik 2 1 Department of General Surgery, Abstract Tekman State Hospital, Erzurum, Aim: Appendiceal carcinoid tumor (neuroendocrine tumor) is rarely seen, and it is frequently found incidentally Turkey after evaluation of appendectomy specimen. Histopathologically, the appendiceal carcinoid tumor is usually the 2 Department of General Surgery, University of Health Science, Umraniye type of enterochromafine cell and stems from a sub-epithelial cell population that differs from the Education and Research Hospital, neuroendocrine tumor in other regions. Although it is often detected in appendectomy, appendiceal one is the Istanbul, Turkey most common type of primary malignant lesion and is detected in 0.3-0.9% of patients with appendectomy. In this study, it is intended to present an appendix carcinoid tumor series that is diagnosed incidentally after appendectomy. Methods: 5131 appendectomy that was performed between years of 2009 and 2017 constituted the study universe. 35 (0.68%) patients diagnosed with carcinoid tumors were evaluated in the histopathological examination. The patients were recorded in terms of demographic data, clinical status, histopathology and surgical reports. Additional operations and follow-up data were noted. Results: 21 of the 35 patients with appendiceal carcinoid tumors were males, and 14 were women. Male/Female ratio was 1.5. The mean age of the patients was 27.3±11.0. There was no difference in terms of gender and age with other appendectomy patients who diagnosed non-tumor (p=0.476 and p=0.413, respectively). -
Integrated Clinico-Molecular Profiling of Appendiceal Adenocarcinoma
www.nature.com/bjc ARTICLE Molecular Diagnostics Integrated clinico-molecular profiling of appendiceal adenocarcinoma reveals a unique grade-driven entity distinct from colorectal cancer Kanwal Raghav 1, John P. Shen1, Alexandre A. Jácome1, Jennifer L. Guerra1, Christopher P. Scally2, Melissa W. Taggart3, Wai C. Foo3, Aurelio Matamoros4, Kenna R. Shaw5, Keith Fournier2, Michael J. Overman1 and Cathy Eng1 BACKGROUND: Appendiceal adenocarcinoma (AA) is an orphan disease with unique clinical attributes but often treated as colorectal cancer (CRC). Understanding key molecular differences between AA and CRC is critical. METHODS: We performed retrospective analyses of AA patients (N = 266) with tumour and/or blood next-generation sequencing (NGS) (2013–2018) with in-depth clinicopathological annotation. Overall survival (OS) was examined. For comparison, CRC cohorts annotated for sidedness, consensus molecular subtypes (CMS) and mutations (N = 3283) were used. RESULTS: Blood-NGS identified less RAS/GNAS mutations compared to tissue-NGS (4.2% vs. 60.9%, P < 0.0001) and showed poor concordance with tissue for well-/moderately differentiated tumours. RAS (56.2%), GNAS (28.1%) and TP53 (26.9%) were most frequent mutations. Well/moderately differentiated tumours harboured more RAS (69.2%/64.0% vs. 40.5%) and GNAS (48.7%/32.0% vs. 10.1%) while moderate/poorly differentiated tumours had more TP53 (26.0%/27.8% vs. 7.7%) mutations. Appendiceal adenocarcinoma (compared to CRC) harboured significantly fewer APC (9.1% vs. 55.4%) and TP53 (26.9% vs. 67.5%) and more GNAS mutations (28.1% vs. 2.0%) (P < 0.0001). Appendiceal adenocarcinoma mutation profile did not resemble either right-sided CRC or any of the four CMS in CRC. -
In Vivo Model of Pseudomyxoma Peritonei for Novel Candidate Drug Discovery
ANTICANCER RESEARCH 29: 4051-4056 (2009) In Vivo Model of Pseudomyxoma Peritonei for Novel Candidate Drug Discovery TERENCE C. CHUA, JAVED AKTHER, PENG YAO and DAVID L. MORRIS Department of Surgery, University of New South Wales, St. George Hospital, Kogarah, Sydney, NSW 2217, Australia Abstract. Pseudomyxoma peritonei (PMP) is a clinical mucinous implants accumulating in the pelvis and condition characterized by diffuse intraabdominal spread of subphrenic regions are often daunting to general surgeons mucinous tumor implants that form on peritoneal surfaces. To (2). Traditional treatments include repeated abdominal complement the efficacy of cytoreductive surgery, an effective lavage of mucin, limited debulking of tumor and systemic cytotoxic drug is required as an intraperitoneal agent for chemotherapy (3). However, these treatments are unable to microscopic cytoreduction locoregionally. To develop a model adequately combat the reaccumulation of mucin that for drug discovery, human pseudomyxoma peritonei tumor characterizes the failure of these treatments. implants consisting of a mixture of mucin and epithelial cells Over the last two decades, cytoreductive surgery and were injected into the peritoneal cavity of CBH/rnu/rnu rats. perioperative intraperitoneal chemotherapy comprising of A fixed quantity of tumor (2 ml) was implanted in each of the hyperthermic intraperitoneal chemotherapy (HIPEC) using 9 male rats. Over 90 days, all rats developed PMP indicating mitomycin C with or without postoperative intraperitoneal that this in vivo animal model may become useful platform in chemotherapy (EPIC) using 5-fluorouracil has emerged to a drug discovery program to allow drug efficacy testing become the standard of care in specialized centers (4, 5). A against human PMP. -
An Uncommon Cause of Isolated Ascites: Pseudomyxoma Peritonei ISSN 2640-2750 Louly Hady1*, I Nassar2, K Znati3 and N Kabbaj1
Open Access Annals of Clinical Gastroenterology and Hepatology Case Report An uncommon cause of isolated ascites: Pseudomyxoma peritonei ISSN 2640-2750 Louly Hady1*, I Nassar2, K Znati3 and N Kabbaj1 1EFD-HGE Department, Ibn Sina Hospital, Rabat, Morocco 2Central Radiology Department, Ibn Sina Hospital, Rabat, Morocco 3Central Anatomical Pathology Department, Ibn Sina Hospital, Rabat, Morocco *Address for Correspondence: Louly Hady, Summary EFD-HGE Department, Ibn Sina Hospital, Rabat, Morocco, Email: [email protected] Pseudomyxoma peritonei or Gelatinous Peritoneal Disease is a rare disease. We report a Submitted: 15 April 2019 case treated in the department of Hepato-Gastroenterology at Ibn Sina Hospital in Rabat, of a Approved: 25 April 2019 64-year-old male who presented with an abdominal pain and an increased volume of the abdomen Published: 26 April 2019 corresponding to ascites. Imaging and anatomopathological study made it possible to diagnose the disease. However, given the general state of the patient, he is under palliative care. Copyright: © 2019 Hady L, et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction Introduction in any medium, provided the original work is properly cited Pseudomyxoma peritonei (PMP) or Gelatinous Peritoneal Disease is a rare condition that refers to an anatomo-clinical entity characterized by ascites of variable abundance Keywords: Ascites; Appendix mucocele; Pseudomyxoma peritonei in the peritoneal cavity, viscous or mucinous, associated or not with neoplastic epithelial cells. It predominates in women. Diagnosis is guided by imaging and conirmed by histology. Prognosis is good in case of early management.