Cases reported "Fibromatosis, Aggressive"

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1/176. Hereditary desmoid disease in a family with a germline Alu I repeat mutation of the APC gene.

    Two families with autosomal dominantly inherited desmoid tumors have recently been shown to have germline mutations at the 3' end of the APC gene. We subsequently identified an amish family with autosomal dominantly inherited desmoid tumors. Genetic analysis performed on one family member, a 47-year-old man with multiple desmoid tumors and no colon polyps, revealed a protein truncating mutation in the middle of the APC gene. The truncating mutation is the result of a 337-bp insertion of an Alu I sequence into codon 1526 of the APC gene. The presence of a poly(A) tail at the 3' end of the insertion suggests that the Alu I sequence was inserted by a retrotranspositional event. Germline insertions of Alu I sequences have occasionally been reported to cause other genetic diseases including type I neurofibromatosis, hereditary site-specific breast cancer (BRCA2), and hemophilia b. However, this is the first report of a germline mutation of the APC gene resulting from an Alu I insertion. ( info)

2/176. Desmoid tumor of the larynx complicating pregnancy: a case report.

    Desmoid tumors are locally invasive fibrous neoplasms that arise from musculoaponeurotic structures. We report the first case of a desmoid tumor of the larynx complicating pregnancy. At 21 weeks' gestation fiberoptic and indirect otolaryngologic examination of the patient's larynx revealed a submucosal tumor involving the left true vocal cord, ventricle of the larynx, and false vocal cord. Histopathologic examination revealed areas of extensive fibrosis intermixed with degenerated vocal cord skeletal muscle. Despite a subtotal excisional biopsy, growth of the desmoid tumor continued during pregnancy. Endoscopic evaluation 9 weeks post partum revealed complete regression of the tumor. ( info)

3/176. Infantile fibromatosis of the neck with intracranial involvement: MR and CT findings.

    CT and MR imaging studies were performed in a 3-year-old boy with infantile fibromatosis arising from the infratemporal fossa and extending into the middle cranial fossa. On CT scans, the lesion was hyperattenuating (44-49 Hounsfield units [HU]), enhancing significantly after application of contrast material (63-66 HU). The MR images showed a multilobulated lesion of heterogeneous signal intensity. The tumor was markedly hypointense on T2-weighted images and slightly hypointense on T1-weighted images relative to brain tissue, iso- or slightly hyperintense relative to tongue muscle on both T2- and T1-weighted images, and enhanced strongly after administration of gadopentetate dimeglumine. ( info)

4/176. Desmoid fibromatosis of the shoulder and of the upper chest wall following a clavicular fracture.

    A desmoid tumor of the shoulder girdle infiltrating the upper chest wall and weighing 1500 g was almost completely removed in an 18-year-old man, 27 months after a bifocal fracture of the clavicule. Thirteen years later, the patient was free of recurrence. The interval time between trauma and diagnosis, as the particular characteristics of aggressive fibromatosis, strongly support a major causal role of the clavicular fracture in the occurrence of this tumor. ( info)

5/176. Development of postoperative fibromatosis after resection of an intraspinal meningioma. Case report.

    The authors report the case of an adult female patient who developed a paraspinous thoracic fibromatosis (desmoid tumor) after undergoing resection of an intraspinal thoracic meningioma that was complicated by postoperative wound infection. To the best of the authors' knowledge, this is the first report of such a tumor occurring after resection of a spinal meningioma. awareness of the development of postoperative fibromatosis and recognition of its association with wound sepsis is important. Although rare, this distinctive lesion should be considered in the differential diagnosis of the apparent rapid regrowth of otherwise indolent lesions including meningioma. ( info)

6/176. A case report on aggressive fibromatosis with bone involvement.

    Aggressive fibromatosis is a locally infiltrative fibroblastic tumour that arises from fascial planes of soft tissue but does not metastasize. It is known to invade muscle, subcutaneous tissue and neurovascular structures. However, bone involvement is very rare and there has been few reports of bone involvement. We present a case of a young man with aggressive fibromatosis of the right lower leg with fibula involvement. ( info)

7/176. Extra-abdominal desmoid mimicking malignant male breast tumor.

    A rare case of extra-abdominal desmoid tumor is reported. A palpable mass was detected in the right breast of a 47-year-old man. mammography showed a stellate mass without calcification, and breast ultrasound examination revealed a solid, inhomogeneous, non-calcified lesion. The result of cytological examination of the fine-needle aspiration biopsy specimen was equivocal. histology of the surgical specimen showed extra-abdominal desmoid tumor. Extra-abdominal presentation of this semimalignant tumor is rare and may mimic malignant breast tumor. Differential diagnosis is difficult and usually based on the result of the histological examination. ( info)

8/176. Development of a desmoid tumor at the site of a total hip replacement.

    Extra-abdominal desmoid tumors are locally aggressive benign soft tissue tumors arising from the connective tissue of muscle and overlying fascia or aponeuroses. Location around the hip occurs in about 10% of all desmoid tumors. The authors report the development of a desmoid tumor around the hip 45 months after implantation of a total hip prosthesis. Endocrine and physical factors seem to play an important role in the development of the disease. Moreover, an association has been reported between trauma and desmoid tumor. Desmoid tumors developing around silicon implants have also been described. However, no association between hip prostheses and desmoid tumors has been published in the world literature. Although soft tissue tumors induced by metallic implants have been observed clinically, we still seek a coherent explanation for the exact pathogenesis. ( info)

9/176. Desmoid tumor of the vulva. A case report.

    BACKGROUND: Extraabdominal desmoid tumors are very rare; primary vulvar desmoid tumors are even rarer. CASE: A desmoid tumor involving the right labium majus, extending upward along the vaginal wall, was excised completely. CONCLUSION: These rare tumors, which may enlarge rapidly during pregnancy, are difficult to diagnose before surgery. ( info)

10/176. Desmoid tumor arising in a cesarean section scar during pregnancy: monitoring and management.

    Desmoid tumors (DTs) are frequently associated with either surgical trauma, like scars, or physiologic trauma, like pregnancy. A DT arising in a cesarean scar during pregnancy shows both correlations. A case of DT that grew gradually in a cesarean scar during the first trimester of a subsequent pregnancy is presented. After initial MR imaging, the lesion was biopsied and carefully monitored; immediate surgical removal was considered at any time if indicated by the follow-up results. No increase in the tumor size was observed during the following 6 months of gestation, and the lesion was extirpated with mesh replacement of the abdominal defect at the time of cesarean section. ( info)
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