1/25. Mesenteric fibromatosis: case report and literature review.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.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
2/25. Mediastinal fibromatosis presenting with superior vena cava syndrome.We encountered a fatal case of mediastinal fibromatosis in a 67-year-old female in whom there was aggressive infiltration into the large vessels, nerves and pericardium. She presented with the superior vena cava syndrome, Horner's syndrome, paralysis of bilateral vocal cords and diaphragm and heart failure. Mediastinoscopical examination revealed an extremely firm tumor adhering to the sternum, trachea and brachiocephalic artery. She died of severe heart failure due to the disturbed dilatation of the heart and ventilatory insufficiency. Although mediastinal fibromatosis is very uncommon and sometimes difficult to diagnose at an early stage, physicians should be aware of this disease for the differential diagnosis of mediastinal tumors.- - - - - - - - - - ranking = 0.75keywords = fibromatosis (Clic here for more details about this article) |
3/25. Primary fibromatosis of the breast in a patient with multiple desmoid tumors--report of a case with evaluation of estrogen and progesterone receptors.Fibromatosis (or desmoid tumor) is an infiltrative fibroblastic/myofibroblastic lesion presenting a moderate risk for local recurrence and no metastatic potential. Classically, these lesions are classified whether in abdominal or extra-abdominal sites, and may be multicentric or familial. Primary fibromatosis of the breast (PFB) is an uncommon lesion that shows histological similarities with abdominal fibromatosis (AF), and frequently poses difficulties in the differential diagnosis with other spindle cell tumors of the breast. It has been demonstrated that AF usually shows immunoreactivity for estrogen (ER) and progesterone (PR) receptors; conversely, in most of the studies, the cells from PFB are consistently negative for both receptors. We report on a case of a 41-year-old female with two desmoid tumors, affecting the abdominal wall and the breast tissue. To the best of our knowledge, there is no previous report in which hormonal receptors were evaluated in abdominal and mammary desmoid tumors in the same patient. We assessed the immunohistochemical expression of ER and PR in both lesions; while the AF showed immunoreactivity for both receptors, the cells from PFB were all negative. Although we have considered just this case, we still believe that these findings could support a distinctive etiopathogenesis of abdominal and mammary fibromatosis.- - - - - - - - - - ranking = 0.875keywords = fibromatosis (Clic here for more details about this article) |
4/25. abdominal wall and foot reconstruction after extensive desmoid tumor resection with free tissue transfer.BACKGROUND: Desmoid tumors are rare connective tissue tumors, also referred to as deep, aggressive fibromatosis. Although histologically benign, they show an invasive growth behavior and have a high local recurrence rate. methods: The treatment of choice is surgical resection with wide negative margins, while the use of radiotherapy remains controversial. Wide resection of greater desmoid tumors may result in considerable defects and functional impairment. Few papers discuss different options for defect coverage after desmoid tumor resection. Two cases of extensive desmoid tumors, one at the trunk, one at the foot, both with compromised wound margins due to multiple previous surgeries, are presented. To achieve a stable and functional soft tissue cover, the defects were treated with microvascular soft tissue transfer (one free latissimus dorsi, one free radial forearm flap). RESULTS: Both flaps healed uneventfully and patients regained full function of the abdominal wall and foot, respectively. CONCLUSIONS: The presented cases demonstrate the efficacy of free flap coverage as an ultimate therapeutic option in selected cases of critical defects after extra-abdominal desmoid tumor resection. Furthermore, free flaps provide a well vascularized ground for adjuvant radiotherapy.- - - - - - - - - - ranking = 0.125keywords = fibromatosis (Clic here for more details about this article) |
5/25. Mesenteric fibromatosis: a report of three cases and literature review.Fibromatosis is a group of relatively uncommon benign diseases showing proliferation of fibrous tissues and is liable to be confused with fibrosarcoma, thereby causing diagnostic as well as therapeutic dilemmas. Even after the correct diagnosis is made, one is not sure about the most effective treatment is in a given setting. Surgery seems to provide the best results but is not feasible at many times due to the extent of the disease, hence the need for non-surgical modalities for unresectable and incompletely resected tumors. Mesenteric fibromatosis is one of the rarer forms of fibromatosis. Three such cases are being presented here with a brief review of the literature.- - - - - - - - - - ranking = 0.75keywords = fibromatosis (Clic here for more details about this article) |
6/25. Sporadic hereditary pancreatic desmoid tumor: a new entity?Deeply seated aggressive fibromatosis also termed as desmoid tumors are rare tumors that invade surrounding structures. Although they never metastasize mortality rate may be as high as 10% due to their aggressive local behavior. Intraabdominal desmoid tumors are usually associated with familial poliposis coli and have a high recurrence rate regardless of the therapy instituted. Sporadic cases are very rare and generous surgical excision may be of benefit. We hereby report 2 siblings with sporadic pancreatic desmoid tumors who also harbor additional fibrotic masses in the pelvis. Although in previously reported cases there is usually a triggering event such as trauma, in the present cases there was no inciting event. Furthermore, the cases are without an associated FAP history, which provides the first clinical clue of a possible genetic determinant in this rare disorder.- - - - - - - - - - ranking = 0.125keywords = fibromatosis (Clic here for more details about this article) |
7/25. Response of estrogen receptor-positive intraabdominal fibromatosis to aromatase inhibitor therapy.BACKGROUND: Intraabdominal fibromatosis is a rare tumor-like lesion of uncertain etiology. CASE: A 49-year-old woman underwent abdominal hysterectomy and bilateral salpingooophorectomy in 1997 to treat uterine leiomyomata and ovarian fibromatosis. Postoperatively, she received estradiol 2 mg daily as hormone replacement therapy (HRT). In 2000, laparotomy performed for a large pelvic tumor revealed inoperable intraabdominal fibromatosis. The tumor, which was positive for estrogen and progesterone receptors, resolved during aromatase inhibitor therapy. The first follow-up computed tomographic (CT) scan revealed that the tumor masses were significantly reduced in size, and successive CT scans revealed stable disease. CONCLUSION: Intraabdominal fibromatosis that expresses estrogen and progesterone receptors may respond favorably to treatment with aromatase inhibitors.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
8/25. Mesenteric fibromatosis with involvement of the gastrointestinal tract. A GIST simulator: a study of 25 cases.Mesenteric fibromatosis (MF) and gastrointestinal stromal tumors (GISTs) are distinct lesions, but they often are confused with each other. Correct identification is essential because of their vastly different therapeutic and prognostic connotations. We reviewed 25 cases of MF with involvement of the wall of the gastrointestinal tract and found that GIST was the most common misdiagnosis (13 [52%]). MF was characterized by a spatially homogeneous proliferation of wavy spindle cells without atypia, associated with collagen deposition (often of the keloidal type), and an infiltrative border. Most cases displayed prominent muscular arteries and dilated, thin-walled veins. The mitotic count was relatively low, and no atypical mitotic figures were identified. These features are sufficiently characteristic of MF to permit distinction from GIST on the basis of routinely stained sections in the large majority of the cases, but immunohistochemical analysis provides a supporting role in the few equivocal cases.- - - - - - - - - - ranking = 0.625keywords = fibromatosis (Clic here for more details about this article) |
9/25. Diffuse intra-abdominal fibromatosis-report of a new entity with review of literature.Fibromatosis, arare non-neoplastic spindle cell proliferation of unknown aetiology, can occur anywhere in the body. Though extra-abdominal sites are commonly involved, intra-abdominal fibromatosis has also been described. Described herein is an unusual case of diffuse intra-abdominal fibromatosis in a 9-year-old boy, who could not be salvaged despite extensive medical management.- - - - - - - - - - ranking = 0.75keywords = fibromatosis (Clic here for more details about this article) |
10/25. Response of progressive fibromatosis to therapy with liposomal doxorubicin.BACKGROUND: patients with fibromatosis not amenable to surgery may suffer from high morbidity. Various chemotherapeutic regimens have been tried in these patients with limited success. Here, we report on the successful use of pegylated liposomal doxorubicin in the treatment of 4 patients with unresectable fibromatosis in unfavorable localizations. patients AND methods: 3 children and 1 adult with progressive fibromatosis were treated with 3-weekly cycles of chemotherapy with liposomal doxorubicin (dose range 20-50 mg/m2 per day every 21 days). Tumors were located at the nasal cavity, fossa infratemporalis, oral cavity, abdomen, and fossa supraclavicularis and were unresectable. 3 of the 4 patients had been heavily pretreated with various chemotherapeutic agents. Objective tumor response was monitored by magnetic resonance imaging and possible cardiotoxicity by echocardiography at regular intervals. RESULTS: A tumor response was obtained in all 4 patients. All patients showed normal cardiac function after completion of chemotherapy as evaluated by left ventricular shortening fraction. Severe neutropenia was not observed. CONCLUSION: Pegylated liposomal doxorubicin is a therapeutic option in patients with progressive unresectable fibromatosis in unfavorable localizations.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
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