1/43. 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.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/43. 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.- - - - - - - - - - ranking = 0.18614085604009keywords = soft (Clic here for more details about this article) |
3/43. 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.- - - - - - - - - - ranking = 1.0623087654714keywords = connective, soft (Clic here for more details about this article) |
4/43. Pathologic, cytologic and immunohistochemical findings of an intra-abdominal desmoplastic small round cell tumor in a 15-year-old male.The intra-abdominal desmoplastic small round cell tumor is a rare neoplasm. It usually occurs in young males and diffusely involves the peritoneum and pursues an aggressive clinical course. The present patient was a 15-year-old male who experienced abdominal pain and abdominal swelling. The patient was diagnosed with an intestinal myogenic sarcoma, and surgery for tumor resection was performed in June 1999. The tumor was a 20 x 15 x 15 cm well-defined mass in the peritoneum involving the transverse colon and stomach with peritoneal disseminations and splenic metastasis. Microscopic findings were well-defined nests composed of small round cells and separated by abundant desmoplastic stroma. Cytologically, the tumor cells consisted of small, round to oval cells with a scant amount of light blue cytoplasm. Immunohistochemically, the tumor cells were positive for anti-epithelial membrane antigen, vimentin, desmin, neuron-specific enolase and WT1 protein antibodies. Similar pathologic features with other small round cell tumors may lead to differential diagnostic difficulties that require the application of ancillary diagnostic methods, such as immunohistochemistry and cytogenetic techniques.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
5/43. Gardner-associated fibromas (GAF) in young patients: a distinct fibrous lesion that identifies unsuspected gardner syndrome and risk for fibromatosis.gardner syndrome (GS), caused by mutations in the adenomatous polyposis coli (APC) gene, is characterized by polyposis coli, osteomas, and various soft-tissue tumors. If undetected or untreated, virtually all patients develop colonic carcinoma at a young age. Early detection, while essential, can be difficult because of attenuated phenotypes or spontaneous mutations. We present the clinicopathologic features of 11 identical fibromatous lesions that we have termed Gardner-associated fibroma (GAF), which not only appear to be a part of the spectrum of lesions associated with GS but, in some cases, represent the sentinel event leading to its detection. The GAFs occurred in 11 patients (5 boys and 6 girls; age range, 3 months-14 years), were solitary (n = 7) or multiple (n = 4), and occurred in the superficial and deep soft tissues of the paraspinal region (n = 7), back (n = 3), face (n = 2), scalp (n = 2), chest wall (n = 2), thigh (n = 1), neck (n = 1), and flank (n = 1). Histologically, GAFs resemble nuchal-type fibromas (NFs), consisting of thick, haphazardly arranged collagen bundles between which are found occasional bland fibroblasts, and having margins that frequently engulf surrounding structures including adjacent fat, muscle and nerves. After surgical excision, four patients developed recurrences that were classic desmoid fibromatoses (DFs). In one patient with multiple GAFs, one lesion had the features of GAF and DF in the absence of surgical trauma. A family history of GS or polyposis (n = 6) or DF (n = 1) was known at the time of surgery in seven patients. In three patients, the diagnosis of GAF resulted in the diagnosis of unsuspected APC in older family members, with the detection of an occult colonic adenocarcinoma in one parent. In the family of the remaining patient, no stigmata of GS were present. Genetic analysis of this child was performed to investigate the presence of a spontaneous (new) mutation; however, no abnormalities were detected. The significance of GAF is that it serves as a sentinel event for identifying GS kindreds, including those with a high risk for the development of DF, and it may potentially identify children with spontaneous mutations of the APC gene. Because NFs and GAFs resemble one another, we suggest that a subset of NF occurring in multiple sites, unusual locations, or children may be GAF.- - - - - - - - - - ranking = 0.37228171208017keywords = soft (Clic here for more details about this article) |
6/43. Intrathoracic desmoid tumor mimicking primary lung neoplasm.Most reported thoracic desmoid tumors originate from the chest wall. However, intrathoracic desmoid tumors are rare. The pathogenesis of these tumors is unclear but antecedent trauma and operation have been implicated. Desmoid tumors can present either with pain or be incidentally detected on radiographic studies. We describe the case of a 60-year-old woman with an intrathoracic desmoid detected on a routine screening chest roentgenogram who underwent complete surgical resection of the tumor.- - - - - - - - - - ranking = 4keywords = neoplasm (Clic here for more details about this article) |
7/43. Aggressive fibromatosis of the head and neck (desmoid tumours).Desmoid tumours are histologically benign fibrous neoplasms arising from the musculoaponeurotic structures throughout the body. They are characterized as infiltrative, usually well-differentiated firm overgrowth of fibrous tissue and are locally aggressive. An aggressive clinical behaviour with tendency for recurrence makes the treatment of these relatively rare fibrous tumours difficult. Local recurrence rates are reported as high as 70 per cent of cases, the recurrence of desmoid fibromatosis in the head and neck is difficult to ascertain because of the different classification schemes used by different authors. A review of cases of desmoid tumours of the head and neck presented in the last 10 years is reported in this paper. Complete surgical excision of desmoid tumours is considered to be the only effective method of cure by most authorities.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
8/43. 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 = 1.0623087654714keywords = connective, soft (Clic here for more details about this article) |
9/43. Extra-abdominal fibromatosis invading the mandible: case report.Extra-abdominal fibromatosis (desmoid tumour) is a rare aggressive neoplasm with a tendency to infiltrate local structures but rarely metastasises or undergoes spontaneous malignant transformation. The treatment of choice is surgery, however, recurrences have been reported even after wide-field resection. This article presents a case of extra-abdominal fibromatosis that had extensively invaded the mandible.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
10/43. Desmoid tumour of the neck: complete regression following radiation therapy.Desmoid tumours are uncommon benign neoplasms. They arise from fascial and musculoaponeurotic tissues and are locally infiltrative, resulting in a high rate of local recurrence following surgical resection. They present difficult loco-regional control. The location and extent of the tumour, as well as the potential for significant morbidity and mortality, dictate the most appropriate therapeutic option. Complete surgical extirpation is the optimal treatment for primary and recurrent desmoid tumours. radiotherapy is indicated in incompletely excised or recurrent tumours. radiation as a primary treatment is seldom recommended. A case of desmoid tumour of the neck, primarily treated with radical radiation, is described and relevant literature reviewed.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
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