Abdominal Plexiform Neurofibromatosis Simulating Pseudomyxoma Peritonei on Computed Tomography

Mirich, David R.; Gray, Robin; Grosman, H

doi:10.1097/00004728-198907000-00034

Cited by 16 publications

(3 citation statements)

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“…Plexiform neurofibroma in neurofibromatosis type 1 has been described involving the mesentery (64)(65)(66). Histopathologically, the nerve is infiltrated by wavy Schwann cells in a mucopolysaccharide and collagenous matrix.…”

Section: Benign Mesenchymal Massesmentioning

confidence: 99%

Solid Tumors of the Peritoneum, Omentum, and Mesentery in Children: Radiologic-Pathologic Correlation:From the Radiologic Pathology Archives

Em¹,

Dm²,

Am³

et al. 2015

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Intraperitoneal solid tumors are far less common in children than in adults, and the histologic spectrum of neoplasms of the peritoneum and its specialized folds in young patients differs from that in older patients. Localized masses may be caused by inflammatory myofibroblastic tumor, Castleman disease, mesenteric fibromatosis, or other mesenchymal masses. Inflammatory myofibroblastic tumor is a mesenchymal tumor of borderline biologic potential that appears as a solitary circumscribed mass, possibly with central calcification. Castleman disease is an idiopathic lymphoproliferative disorder that appears as a circumscribed, intensely enhancing mass in the mesentery. Mesenteric fibromatosis, or intra-abdominal desmoid tumor, is a benign tumor of mesenchymal origin associated with familial adenomatous polyposis. Mesenteric fibromatosis appears as a mildly enhancing, circumscribed solitary mass without metastases. Diffuse peritoneal disease may be due to desmoplastic small round cell tumor (DSRCT), non-Hodgkin lymphoma, or rhabdomyosarcoma. DSRCT is a rare member of the small round blue cell tumor family that causes diffuse peritoneal masses without a visible primary tumor. A dominant mass is typically found in the retrovesical space. Burkitt lymphoma is a pediatric tumor that manifests with extensive disease because of its short doubling time. The bowel and adjacent mesentery are commonly involved. Rhabdomyosarcoma may arise as a primary tumor of the omentum or may spread from a primary tumor in the bladder, prostate, or scrotum. Knowledge of this spectrum of disease allows the radiologist to provide an appropriate differential diagnosis and suggest proper patient management.

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Section: Benign Mesenchymal Massesmentioning

confidence: 99%

Solid Tumors of the Peritoneum, Omentum, and Mesentery in Children: Radiologic-Pathologic Correlation:From the Radiologic Pathology Archives

Em¹,

Dm²,

Am³

et al. 2015

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“…[3] Soliter nörofibromlar (SN) genellikle boyunda, pelviste ve ekstremitelerde görülmektedir. [7,8] SN'ler nadiren retroperitonda görülür. [9] Retroperitoneal SN'ler genellikle bilgisayarlı tomografilerde, parapsosas veya presakaral yerleşimli, iki taraflı, simetrik, düşük yoğunluklu kitleler olarak tespit edilir.…”

Section: Discussionunclassified

Solitary neurofibroma in the retroperitoneum without neurofibromatosis: case report

Kayilioglu¹

2014

TJ Oncol

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Neurofibroma is a common form of benign peripheral nerve sheath neoplasms. Neurofibromas can be seen multiple or as a solitary tumor. We present a case of solitary neurofibroma located in retroperitoneum without von Recklinghausen's disease. A 38-year-old female, suffering from abdominal pain is presented. Ultrasonography and computerized tomography revealed a retroperitoneal mass. Complete resection was performed. Histopathological evidences were consistent with neurofibroma. After an uneventful recovery; six-month follow-up computerized tomography scan revealed no signs of relapse, residue tumor or any mass. Retroperitoneal localization of solitary neurofibroma, is an extremely rare entity. Our case represents a unilateral, symptomatic neurofibroma located in the retroperitoneum, which is treated surgically with success.

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“…Other differential diagnoses reported in the literature include endometriosis with myxoid change [62], melioidosis (a lethal infectious disease caused by Burkholderia pseudomallei ) [63] and those with abdominal CT resemblance, e.g. extensive abdominal plexiform neurofibromatosis [64]. …”

Section: Introductionmentioning

confidence: 99%

Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects

Amini

Masoumi-Moghaddam

Ehteda

et al. 2014

Orphanet J Rare Dis

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Pseudomyxoma peritonei (PMP, ORPHA26790) is a clinical syndrome characterized by progressive dissemination of mucinous tumors and mucinous ascites in the abdomen and pelvis. PMP is a rare disease with an estimated incidence of 1–2 out of a million. Clinically, PMP usually presents with a variety of unspecific signs and symptoms, including abdominal pain and distention, ascites or even bowel obstruction. It is also diagnosed incidentally at surgical or non-surgical investigations of the abdominopelvic viscera. PMP is a neoplastic disease originating from a primary mucinous tumor of the appendix with a distinctive pattern of the peritoneal spread. Computed tomography and histopathology are the most reliable diagnostic modalities. The differential diagnosis of the disease includes secondary peritoneal carcinomatoses and some rare peritoneal conditions. Optimal elimination of mucin and the mucin-secreting tumor comprises the current standard of care for PMP offered in specialized centers as visceral resections and peritonectomy combined with intraperitoneal chemotherapy. This multidisciplinary approach has reportedly provided a median survival rate of 16.3 years, a median progression-free survival rate of 8.2 years and 10- and 15-year survival rates of 63% and 59%, respectively. Despite its indolent, bland nature as a neoplasm, PMP is a debilitating condition that severely impacts quality of life. It tends to be diagnosed at advanced stages and frequently recurs after treatment. Being ignored in research, however, PMP remains a challenging, enigmatic entity. Clinicopathological features of the PMP syndrome and its morbid complications closely correspond with the multifocal distribution of the secreted mucin collections and mucin-secreting implants. Novel strategies are thus required to facilitate macroscopic, as well as microscopic, elimination of mucin and its source as the key components of the disease. In this regard, MUC2, MUC5AC and MUC5B have been found as the secreted mucins of relevance in PMP. Development of mucin-targeted therapies could be a promising avenue for future research which is addressed in this article.

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Abdominal Plexiform Neurofibromatosis Simulating Pseudomyxoma Peritonei on Computed Tomography

Cited by 16 publications

References 0 publications

Solid Tumors of the Peritoneum, Omentum, and Mesentery in Children: Radiologic-Pathologic Correlation:From the Radiologic Pathology Archives

Solid Tumors of the Peritoneum, Omentum, and Mesentery in Children: Radiologic-Pathologic Correlation:From the Radiologic Pathology Archives

Solitary neurofibroma in the retroperitoneum without neurofibromatosis: case report

Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects

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