Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The fibromatoses represent a broad group of fibroblastic proliferations characterized by infiltrative growth and a tendency toward recurrence but, unlike malignant fibrous tumors, they do not metastasize. Mesenteric fibromatosis arising from the mesentery of the small bowel is rare. It may be sporadic or may occur in association with polyposis coli and other soft tissue tumors as a component of Gardner's syndrome. We report a case of mesenteric fibromatosis in a 52-year-old man with no evidence of Gardner's syndrome. The neoplasm occupied the whole abdominal cavity, weighing 12 kg, with the greatest diameter being 50 cm. Histological findings (i.e moderate degree of cellularity, lack of nuclear pleomorphism and mitotic figures) allowed to rule out malignancy. Surgical removal is actually the only effective treatment for mesenteric fibromatosis. Excision must be as wide as possible in order to prevent local recurrence. Until now, no satisfactory results have been obtained with external radiotherapy. More recently, anti-inflammatory drugs have been used in the management of this tumor.
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PMID:[Giant mesenteric fibromatosis: a case report]. 868 6

Mesenteric fibromatosis is a rare, benign fibrous lesion found in the bowel mesentery or the retroperitoneum. Its biological behavior is intermediate between benign fibrous tissue proliferation and fibrosarcoma. Fibromatosis characteristically is locally invasive and tends to recur but does not metastasize. Most reported cases have been in older individuals, and there is a frequent association with familial polyposis coli, previous trauma, and hormonal imbalance. The authors report a case of mesenteric fibromatosis in a 32-month-old girl with a 1-month history of abdominal pain who was discovered to have an abdominal mass. After appropriate investigations, the mass was excised. The pathology report confirmed the diagnosis of fibromatosis. Mesenteric fibromatosis in children, as in adults, presents a management challenge for the surgeon.
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PMID:Mesenteric fibromatosis: case report and literature review. 1044 7

Mesenteric fibromatosis, sclerosing mesenteritis, inflammatory pseudotumor, and extrapleural solitary fibrous tumor constitute a loosely associated group of benign fibrous tumors and tumorlike lesions of the mesentery. These lesions are linked histologically by the presence of fibroblasts or fibrosis and anatomically by their location within the mesentery. Although rare, and distinctly different in pathogenesis and biologic behavior, these fibrous lesions have pathologic and radiologic features that overlap with one another and with more common neoplastic and nonneoplastic lesions of the mesentery. Mesenteric fibromatosis is a locally aggressive, benign proliferative process that may occur sporadically or in association with familial adenomatous polyposis. It most frequently manifests as a focal mesenteric mass and may simulate lymphoma, metastatic disease, or a soft-tissue sarcoma. Sclerosing mesenteritis is a rare idiopathic disorder that most commonly produces a stellate mass within the mesentery and should be differentiated from metastatic disease, specifically metastatic carcinoid, because it frequently responds to conservative or medical management. Inflammatory pseudotumor (inflammatory myofibroblastic tumor) is a benign, chronic inflammatory disorder of unknown cause that manifests as a solid mesenteric mass, indistinguishable from malignancy. Extrapleural solitary fibrous tumor is a tumor of submesothelial origin that is identical to the solitary fibrous tumor of the pleura. When located in the mesentery or peritoneal cavity, extrapleural solitary fibrous tumor has an imaging pattern that must be differentiated from metastatic disease, soft-tissue sarcomas, and other benign and malignant neoplasms of the mesentery and peritoneum. Knowledge of this group of benign fibrous tumors and tumorlike lesions of the mesentery is important in the preoperative evaluation of a mesenteric mass.
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PMID:From the archives of the AFIP: benign fibrous tumors and tumorlike lesions of the mesentery: radiologic-pathologic correlation. 1641 55

Desmoid tumor is a monoclonal, fibroblastic proliferation arising in musculoaponeurotic structures. This connective tissue hyperplasia infiltrates locally, recurs frequently after resection but does not metastasize. Abdominal desmoid occurs sporadically, in association with some familial syndromes and often represents a clinical dilemma for surgeons. The enigmatic biology and anatomical location of abdominal desmoids make treatment recommendations difficult. This distinct pathological entity is reviewed with a specific focus on aetiology and management.
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PMID:Updates on abdominal desmoid tumors. 1802 87

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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PMID:Solid tumors of the peritoneum, omentum, and mesentery in children: radiologic-pathologic correlation: from the radiologic pathology archives. 2576 37

Fibromatosis are rare, accounting for 0.03% of all tumours. Mesenteric fibromatosis is a very rare (8% of all desmoid neoplasm). Aggressive fibromatosis of mesentery is a rare surgical problem affecting 2-4 per million people. Females are more commonly affected than males (Estrogen acts as a growth factor). It is locally invasive and tends to recur but never metastasize. Here, we are discussing about 24-year-old male presented with progressive abdomen distension associated with pain since one month. Abdominal examination showed a firm non-tender intra-abdominal mass, measuring around 15x14 cm size, with intrinsic mobility, which was perpendicular to mesenteric line, all borders were well-made out. CECT abdomen showed features suggestive of GIST . Elective Laparotomy was done and a giant mass arising from mesentery without any infiltration to the surrounding structures was noted. The entire mass was excised and mesentery repaired. Histopathology showed uniform band of spindle shaped cells arranged in fascicles admixed with blood vessels in a collagenous stroma. Immunohistochemistry showed Beta Catenin +ve, CD 117-ve, CD 34 -ve and SMA-ve, which is confirmative of Fibromatosis. Postoperative period was uneventful.
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PMID:Giant aggressive mesenteric fibromatosis- a case report. 2585 91