1/8. Familial adenomatous polyposis complicated by an intrahepatic desmoid tumor: report of a case.Desmoids are uncommon proliferations of fibroblasts that occur with disproportionate frequency in patients with familial adenomatous polyposis. They do not metastasize and are histologically benign. Despite this, the unpredictable and often aggressive nature of familial adenomatous polyposis-associated desmoids and their tendency to occur in intra-abdominal sites means that they present a difficult management problem, and they are a leading cause of death in patients with familial adenomatous polyposis who have undergone colectomy. We report a case of a patient with familial adenomatous polyposis who had extensive and aggressive desmoid disease and whose management was further complicated by a large intrahepatic desmoid. There are no previous reports of desmoids occurring in the liver.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/8. Extra-abdominal desmoid tumor presenting as an intrathoracic tumor: case report and literature review.A case of an extra-abdominal desmoid tumor presenting as an intrathoracic tumor (intrathoracic desmoid tumor) in a 46-year-old woman is reported. The tumor originated in the left chest wall and protruded into the left pleural cavity. Simple resection was carried out. The tumor, measuring 13 x 9 x 7 cm, was solid, gray-tan in color, and covered with parietal pleura. Histologically, the tumor was composed of a hypocellular arrangement of spindle-shaped cells with a fibromyxoid background. In some areas, keloid-like hyalinized collagen fibers proliferated, and a perivascular hypercellular area was seen. Immunohistochemical analysis showed that the cytoplasms of the tumor cells were strongly positive for vimentin, and some tumor cells were positive for alpha-smooth muscle actin, but all tumor cells were negative for CD34. These findings were consistent with the characteristics of an intrathoracic desmoid tumor. The differential diagnoses, in particular solitary fibrous tumor and tumors with a myofibroblastic nature, are discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/8. Aggressive intracranial fibromatosis: case report.Fibromatosis is a locally aggressive, proliferative fibroblastic lesion affecting musculoaponeurotic structures, most often in the limbs and trunk. Intracranial fibromatosis is extremely rare and requires aggressive treatment to prevent recurrence. We present the case of a 48 year old woman with aggressive skull base fibromatosis. The lesion extended through the sphenoid sinus, into both pterygoid recesses, destroying the right lateral wall of the sphenoid sinus and invading the cavernous sinus. There was also involvement of the floor of the sella, the clivus, the right petrous temporal bone and the right mastoid. The patient underwent partial resection of the lesion via an extended trans-sphenoidal approach. Postoperative MRI showed residual tissue. A review of the literature shows that intracranial fibromatosis usually appears in the first or second decade. Complete resection is often impossible because of its widely infiltrative nature. radiotherapy and chemotherapy are often required to improve local control of the lesion.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/8. Management of an esophagogastric fibromatosis in a child: a case report.Intraabdominal fibromatosis is an uncommon neoplasm. The aggressive nature of these tumors and the potential for major morbidity secondary to resection can present a difficult surgical dilemma. A 9-year-old boy presented with anemia and vomiting. He had esophagogastric fibromatosis diagnosed and underwent distal esophagectomy, total gastrectomy, Hunt-Lawrence jejunal pouch, and Roux-en-Y esophagojejunostomy. This report describes the successful surgical management of an esophagogastric fibromatosis for the first time in the English-language literature. The management of this tumor is discussed with particular regard to the very unusual clinical presentation.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/8. Calcified fibromatosis of the neck in 4-year old girl: rapid growth, rapid therapy.Fibromatosis is a rare soft tissue disease typical for infants and characterized by fibroblastic proliferation, which may appear similar to fibrosarcoma. An unusual case of 4-year old girl presenting large tumor of the neck with massive calcification is described. The growth of the tumor was rapid and mediastinal involvement was observed. The final diagnosis showed benign nature of tumor with microscopic features of fibromatosis calcificans. After surgical resection of neck tumor, residual mass persisted both in the neck and in the mediastinum in 6 years of follow-up without signs of progression.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/8. Recurrent bilateral mammary fibromatosis (desmoid tumor) imaged with technetium-99m pentavalent dimercaptosuccinic acid [99mTc-(V)DMSA] scintimammography.BACKGROUND: breast fibromatosis is a rare, benign, recurring, locally destructive entity. CASE: A 35-year-old woman underwent right-sided lumpectomy, revealing fibromatosis with epithelial hyperplasia. Two years later, she was re-evaluated due to a three times as large mass recurrence on X-ray mammography. (99m)Tc-(V)DMSA scintimammography revealed diffuse increased radiotracer uptake occupying the entire breast. A less widespread similar uptake was observed in the contralateral breast. mammography did identify neither the posterior margins of the mass nor the left-sided lesion. The patient underwent mastectomy and left-sided open biopsy, revealing bilateral fibromatosis. Ki-67 expression was moderate. CONCLUSIONS: (99m)Tc-(V)DMSA scintimammography can provide information regarding the nature of the lesion, its extent and bilateral involvement (especially in dense breasts) and could prove useful as a predictor of its potential for recurrence.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/8. Fibromatosis of the remnant pancreas after pylorus-preserving pancreaticoduodenectomy.Intra-abdominal fibromatosis or desmoid tumors are rare forms of connective tissue cellular dysplasia characterized by proliferation of fibroblasts and abundant collagen. Most often these tumors associated with familial adenomatous polyposis or Gardner's syndrome. Those tumors not associated with polyposis are termed sporadic desmoids and tend to be locally aggressive in nature. Sporadic intra-abdominal desmoids involving the pancreas are quite rare, as only six previously reported cases exist. In this report we present a seventh case of a sporadic intraabdominal desmoid involving the pancreas. The patient, a 63-year-old white man, developed the desmoid tumor following a pylorus-preserving pancreaticoduodenectomy for an insulinoma. The patient was managed via further pancreatectomy, consisting of a distal pancreatectomy with en bloc splenectomy, sparing a 6-cm portion of pancreatic neck and proximal body. Finally, we present a complete review of the six previous cases of sporadic pancreatic fibromatosis.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/8. Desmoid fibromatosis is a clonal process.Desmoid fibromatosis is a locally aggressive proliferative soft tissue lesion of controversial nature. The authors investigated the clonality of this process by molecular genetic analysis of dna methylation pattern at a polymorphic site at the human androgen-receptor gene (HUMARA) to examine the inactivation pattern of the x chromosome. Twenty desmoid fibromatoses including primary and recurrent lesions from 11 female patients were studied. Sixteen lesions from eight patients showed nonrandom X inactivation, consistent with a clonal origin and, therefore, a true neoplastic nature. Furthermore, multiple recurrent lesions from two patients exhibited the same inactivation pattern as the corresponding primary lesions, suggesting that they were derived from the same cell clone as the primary lesion. One patient was homozygous at the HUMARA locus, and two patients had the same skewed pattern in their normal and lesional tissues. The authors also found that digestion with HpaII, but not HhaI, failed to generate a nonrandom X inactivation pattern in some of the cases, suggesting that the methylation status at the HpaII sites was altered in some lesions, and that HhaI should be used to verify results and to avoid incorrect conclusions.- - - - - - - - - - ranking = 2keywords = nature (Clic here for more details about this article) |