Desmoid tumours, also known as aggressive fibromatosis, are rare lesions with an intermediate biological behaviour between benign fibrous lesions and fibrosarcomas. Although abdominal desmoids have an increased incidence in Gardner's syndrome, they are rarely found in an isolated form. We report the findings of a barium study, ultrasound, computed tomography and magnetic resonance imaging in a nine-year-old boy with intermittent nausea and vomiting, diagnosed as having a desmoid tumour. Although intra-abdominal desmoids are usually detected as a solitary lesion in sporadic cases, the presented case had two mesenteric lesions in the left upper quadrant. There was intestinal obstruction and the masses had invaded the colon wall.
[Show abstract][Hide abstract] ABSTRACT: Intraabdominal or mesenteric fibromatosis is a rare benign nonmetastatic neoplasm that appears as a sporadic lesion or in patients with familiar polyposis. Its evolution is unpredictable. About 10% of the cases shaw a very aggressive growth.
We presented a 22-year-old woman in whom an aggressive intraabdominal fibromatosis had appeared during the first pregnancy as a well circumscribed ovoid tumor, involving the terminal ileum, the caecum, the ascending colon, the right kidney, the ureter, and the right common iliac artery. The tumor was excised with right colectomy, nephroureterectomy and resection of the involved artery using arterial reconstruction with graft interposition. Two years after the surgery the patient developed an inoperable tumor recurrency with a fatal outcome.
In spite of a successful surgical excision during the original surgery intraabdominal or mesenteric fibromatosis might have an aggressive evolution leading to an inoperable tumor recurrency and a fatal outcome.
[Show abstract][Hide abstract] ABSTRACT: Abdominal desmoid tumor is one of the forms of deep (musculoaponeurotic) fibromatosis. It occurs more often as a desmoid tumor in the abdominal wall, less often in various intra-abdominal locations. In this work, we performed retrospective study concerning diagnostic problems of this disease with the use of imaging techniques.
Four patients (three females and one male) were examined by postcontrast CT (computed tomography) and one of them also by MR (magnetic resonance). All findings were correlated with the operational findings and histologic examination.
The findings were typical only in the case with the lesion located in the abdominal wall, three described cases of the intra-abdominal desmoid provided a broad range of differentially diagnostic possibilities (metastases, GIT tumors, lymphomas, etc.). In particular, the findings in infiltrative processes in intra-abdominal location and retroperitoneal involvement were less typical. Ultimately, the histological findings were decisive.
The possibility of the occurrence of intra-abdominal desmoid tumor must be considered particularly in younger individuals with rapidly growing tumorous process, which does not immediately arise from the surrounding organs (digestive tract, internal genitalia, etc.), and is located in the abdominal wall or in the abdominal cavity.
European Journal of Radiology 06/2007; 62(2):180-5. DOI:10.1016/j.ejrad.2007.01.034 · 2.37 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Desmoid tumor is a rare benign tumor derived from fibrous sheath or musculoaponeurotic structure. The tumor is benign histologically but considered as malignant clinically because it has high propensity on infiltrative growth with local invasion and tendency to recurrence after local excision. Especially, when this tumor happens to be in the intra-abdomen, the prognosis is worse because it can cause intestinal obstruction, ureter obstruction and, fistula formation. It also can invade major vessels in abdomen. This tumor occurs more frequently in patients with familial adenomatous polyposis (FAP), in post-partume women, and at old surgical incision site. However, in this case, the patient had neither previous surgery nor a FAP history. We report a rare case of the young male patient who presented with an acute abdomen and underwent laparotomy and was found to have an intra-abdominal desmoid tumor with abscess formation.
The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chi 06/2009; 53(5):315-9. DOI:10.4166/kjg.2009.53.5.315
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