Peritoneal Malignancy in Children Is Broad

PeritonealPeritoneal MalignancyMalignancy inin Children:Children: AA PictorialPictorial ReviewReview Conor O. Bogue1, Amanda Murphy2, Oscar M. Navarro1 Department of Diagnostic Imaging1 and Division of Pathology, Pediatric Laboratory Medicine2, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada Introduction Burkitt Lymphoma Desmoplastic Small Round Cell Tumor Dendritic Cell Sarcoma of Pancreas Peritoneal malignancy is significantly less common in children than in adults, commensurate with the infrequency of neoplasms such American Burkitt lymphoma is a rapidly growing malignancy of B-lymphocyte origin that occurs in non-endemic form in North America. Desmoplastic small round cell tumor has recently been described as a distinctive member of the small round blue cell group of tumors. Follicular dendritic cells, interdigitating dendritic cells and Langerhans cells are nonlymphoid, nonphagocytic accessory cells of the as gastrointestinal, ovarian and uterine tumors, that are associated with carcinomatosis in the adult population. When it does occur, it Sporadic Burkitt lymphoma accounts for 30 to 50% of pediatric lymphomas. In contrast to endemic or African Burkitt lymphoma, It is a highly aggressive neoplasm with a mean survival of 2-3 years. It is rare, occurring primarily in adolescents and young adults, and lymphoid system. Malignant neoplasms showing dendritic cell differentiation are uncommon. Most reported cases involve lymph nodes. is most commonly metastatic, usually after the neoplasm breaks through the organ capsule or through peritoneal spill at the time of the sporadic form is rarely associated with Epstein-Barr virus. Children presenting with sporadic Burkitt lymphoma are often boys with a male predominance. The clinical presentation may include abdominal distension, a palpable abdominal mass, urinary disorders, Approximately 30% of cases are located in extranodal sites. These neoplasms usually occur in young or middle aged adults and there is surgery. Primary neoplasms of the peritoneum, such as mesothelioma, are exceptionally rare. between the ages of 5 and 10 years. American Burkitt Lymphoma usually produces bulky extranodal tumors in the abdomen and differs fever, weight loss, adenopathy or hepatosplenomegaly. The most common site of origin of the tumor is the peritoneum, followed by the no sex predilection. The clinical presentation is usually that of a solitary mass. Intraperitoneal tumors have resulted in abdominal pain clinically and radiologically from African Burkitt lymphoma, which most often affects the head and neck. The common presenting tunica vaginalis of the testis. Peritoneal dissemination is the commonest pattern of metastatic spread. Direct organ invasion, adenopathy The findings on imaging may include ascites, mesenteric or peritoneal nodules (<3cm) or masses (>3cm), calcification, bowel wall and weight loss. Local recurrence occurs in 36% and distant metastases in 28%. The most common sites of metastases are lung, liver, symptoms include abdominal pain, nausea, vomiting, abdominal distention, gastrointestinal bleeding, weight loss, right lower quadrant and liver metastases may also be seen. The typical radiological appearance is of a dominant abdominal or pelvic mass with peritoneal thickening and omental caking (thickening >2mm). A pseudomyxoma-like appearance with scalloping of the solid organs has been peritoneum and lymph nodes. Adverse clinical outcome correlates positively with intra-abdominal location, large tumor size (> 6 cm), mass, and, uncommonly, bowel perforation. Intraabdominal disease can be bulky and enlarge rapidly and has been reported to cause seeding. Calcification may be seen and contrast enhancement is weak. described in rhabdomyosarcoma, Wilms and Burkitt lymphoma. high mitotic count, coagulative necrosis and cellular atypia. abdominal compartment syndrome. Peritoneal infiltration has been described in up to 24.2% of patients and ascites in 39.3%. When The spread of intraperitoneal malignant cells follows the natural circulation of peritoneal fluid. This is influenced by gravity, negative Burkitt lymphoma is the cause of diffuse peritoneal disease, it usually is accompanied by focal gastrointestinal masses and/or significant Fig. 9a Fig. 9b Fig. 9c Fig. 12a Fig. 12b Fig. 12c subdiaphragmatic pressures and the anatomical features of the peritoneum. These mechanisms account for the most common location lymphadenopathy. of tumor seeding being in the pouch of Douglas (>50%). A dominant pelvic mass has been reported in up to 82% of cases of desmoplastic small round cell tumor. Other commonly involved locations are the small bowel mesentery (40%), the sigmoid mesocolon (20%) and the Fig. 6a Fig. 6b Fig. 6c Fig. 6d right paracolic gutter (20%). The purpose of this exhibit is to review those pathologies that may present with peritoneal involvement in children and to illustrate the imaging findings of such entities with different imaging modalities, including ultrasonography, computed tomography and magnetic resonance imaging. Figure 9. Imaging at presentation in a 10 year old male with desmoplastic small round cell tumor. (a) Axial CT image demonstrates a Figure 12. 16 year old male with pancreatic dendritic cell sarcoma. (a) Axial contrast-enhanced fat-suppressed T1-weighted image of subdiaphragmatic peritoneal mass (arrow) with central calcification.(b) Axial CT image demonstrates a dominant pelvic soft tissue mass the abdomen demonstrates a large, poorly-enhancing mass (arrows) in the head of the pancreas. (b) CT of abdomen three years later (arrow), also with central calcification (arrowhead). (c) Axial CT image six months later demonstrates a peritoneal soft tissue deposit demonstrates a subdiaphragmatic peritoneal deposit (yellow arrows) and a splenic metastasis (black arrow). (c) CT image of the pelvis Figure 6. 15 year old male with Burkitt lymphoma. (a) Axial CT at diagnosis demonstrates ascites, peritoneal thickening (yellow arrows) indenting the right lobe of the liver (yellow arrow) and mesenteric lymphadenopathy (black arrows). from the same study demonstrates a hypodense, multilobulated pelvic peritoneal metastatic lesion (arrows). Rhabdomyosarcoma and omental infiltration (black arrows). (b) A lower axial CT image demonstrates peritoneal enhancement (arrowheads), omental caking (arrow) and small bowel wall thickening (black arrows). (c) Axial CT at the level of the mesentery demonstrates peritoneal enhancement Rhabdomyosarcoma is a soft tissue malignancy of childhood that is thought to arise from primitive mesenchymal cells. It accounts for (black arrows), omental thickening (yellow arrow) and mesenteric lymphadenopathy (arrowheads). (d) Axial CT image through the pelvis 5-15% of all solid malignancies in children. At diagnosis, 70% of children are younger than 10 years old. It may present as a focal mass demonstrates marked thickening of a small bowel loop (yellow arrows), ascites and peritoneal thickening (black arrows). or, rarely, with fever, generalized malaise and bone pain if there is diffuse metastatic disease. Rhabdomyosarcoma may arise from, and metastasize to, nearly all body organs. However, the head and neck (40%) and the genitourinary region (20%) are the commonest primary Duodenal Adenocarcinoma sites. Diffuse metastatic spread is more often seen with primary tumors in the extremities and with alveolar histology. It may be present Paravaginal Wolffian Tumor in up to 7% of cases at diagnosis or 11% over the course of the disease. Ascites has been reported to be present in most but not all cases Primary duodenal adenocarcinoma is a rare tumor, representing <1% of all gastrointestinal tumors and 25-35% of malignant tumors of This rare neoplasm usually arises in the leaves of the broad ligament or in the fallopian tube. Clinical presentation may include a palpable with peritoneal spread. Other imaging findings reported include enhancing nodules, masses, omental caking and a pseudomyxoma Wilms Tumor the small intestine. The overall 5-year survival is 23-62% but has been reported to be as high as 75% if fully resected. The median age of mass and lower abdominal pain. These tumors can arise anywhere along the trajectory of the wolffian duct, which extends from the peritonei-like appearance. diagnosis is in the sixth decade of life. In children, intestinal cancers are particularly rare and have been observed to be associated with Wilms tumor, or nephroblastoma, arises from the metanephros, the mesodermal precursor of the renal parenchyma. Occasionally, hilum of the ovary, along the mesosalpinx and the lateral aspects of the uterus, to the outer third of the vagina. Female adnexal tumors functional deficiencies of the mismatch repair system causing a cancer-predisposition syndrome such as Lynch syndrome or hereditary it is found to arise in the extrarenal retroperitoneum, presumably within mesonephric remnants. Wilms tumor accounts for 87% of of probable wolffian origin are a distinctive epithelial neoplasm arising from the remnants of the mesonephric duct. Although generally Fig. 1a Fig. 1b Fig. 1c Fig. 1d non-polyposis colorectal cancer. The clinical presentation may be non-specific and may mimic benign conditions. The most frequent pediatric renal masses and occurs in approximately 1:10,000 children. It accounts for 7% of all childhood cancers. The peak incidence considered a tumor of low malignant potential, these tumors can recur. In a review of the literature, recurrence was documented in 8/71 symptoms found are

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