Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Desmoplastic fibroma is a rare primary tumor of bone that histologically and biologically mimics the extra-abdominal desmoid tumor of soft tissue. This study reviews 27 cases of desmoplastic fibroma, consisting of 9 from the Mayo Clinic files and 18 from our consultation files. There was a male predominance, and 74% of the patients were in the first 3 decades of life. The most frequent sites of involvement were the metaphysis of long bones and the mandible. Radiographically, the tumors were lucent, expansile lesions with well-defined margins. Histologically, they contained slender spindle cells and various amounts of collagen fibers. En bloc resection is the treatment of choice because a high incidence of recurrence was noticed after lesional curettage.
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PMID:Desmoplastic fibroma of bone. 191 45

Fifteen patients with aggressive fibromatosis are presented. The patients ranged in age from one to 64 years, an average of 26 years, and the median time of follow-up study after treatment was ten years (a range of 2.5 to 22.0). Within this period, 60 per cent (nine of 15) of the tumors recurred. In a survey of the literature, when simple excision was compared with wide extirpation, the latter was found significantly superior (p less than 0.01) in terms of limiting a recurrence of the tumor. The results of a pathoanatomic study found that the number of mast cells and the size of the primary tumor could not prognosticate the clinical course. It was concluded that aggressive fibromatosis should be evaluated and treated like a low malignant soft tissue tumor.
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PMID:Aggressive fibromatosis. 396 7

Surgery of soft tissue sarcomas in children has been modified by the introduction of multidisciplinary treatment so that major amputation and exenterations are now rare in the management of these tumors in children. Surgery must still be well planned for total removal of the tumor with preservation of limbs and, in most instances, the function of pelvic organs. The commonest soft tissue sarcomas in children are embryonal rhabdomyosarcoma (RMS), fibrosarcoma, and synovial sarcoma. Treatment of embryonal RMS can now achieve a 2-year survival of 80% at all sites. Surgery and irradiation are used to control the primary tumor and multidrug chemotherapy to control metastases or prevent dissemination of localized tumor. Fibrosarcoma in children, usually a low-grade, extra-abdominal desmoid lesion, does not respond to radiation treatment or chemotherapy, and management is by surgery alone. It shows a marked tendency to local recurrence, and multiple local resections may be necessary for cure. Ninety percent of the children with this tumor can be salvaged by surgery and careful follow-up. The management of synovial sarcoma is surgical and similar to that of RMS. Although not radiosensitive as is RMS, this tumor has responded well to multidisciplinary treatment. Adequate resection is now followed by a chemotherapy protocol similar to that used in osteogenic sarcoma. The smaller numbers of these tumors and their varied natural history make evaluation of treatment difficult. Other soft tissue sarcomas seen with extreme rarity in children are liposarcoma, angiosarcoma, and neurosarcoma. These tumors are treated with the same protocol as that of RMS.
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PMID:Surgery of soft tissue sarcomas in children. 627 15

A clinicopathologic study was made of 32 soft-tissue sarcomas of the anterior abdominal wall (ten desmoid tumors and 22 fully malignant sarcomas). Direct invasion of the costal margin or the iliac crest was observed in both types, whereas transperitoneal invasion and metastasis developed characteristically with fully malignant sarcomas. Wide monobloc resection of the abdominal wall, including, where necessary, resection of adjacent bony parts and reconstruction with a prosthetic mesh, satisfactorily controlled local disease in nine of ten patients with desmoid tumor, and in three of four patients with primary operable, fully malignant sarcoma. Adjunctive therapy with radiation and chemotherapy may improve the outcome in patients with recurrent or high-grade sarcomas, but adequate monobloc resection of the primary tumor remains the most important therapeutic objective.
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PMID:Malignant soft-tissue tumors of the anterior abdominal wall. 644 55

Desmoplastic fibroma is a very rare primary tumor of bone, closely related to aggressive fibromatosis of soft tissue. Although considered a benign lesion, it is locally destructive, can extend into the soft tissues, and has a high rate of local recurrences after incomplete surgical excision. Recognition of this entity is important to ensure proper surgical treatment. According to the published data, the tumor is most common in the long tubular bones (56%), the mandible (26%), and the pelvis (14%). Rib involvement by desmoplastic fibroma is extremely rare, and to our knowledge, only 3 cases have been reported in the literature to date. We present the case of a desmoplastic fibroma in the rib of a 19-year-old man, adding a fourth case to the previously reported cases involving this unusual location. The clinical history and the radiological and pathologic findings are presented.
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PMID:Desmoplastic fibroma of the rib. 1203 64

Desmoid tumors are uncommon benign tumors composed of fibrous tissue, which originate from aponeuroses. Little is known about their molecular pathogenesis and the reason that they recur. We report the case of a 20-year-old man with a recurrent desmoid tumor of the chest wall, focusing on our analysis of the apoptosis and its related molecular events. Immunohistochemical examination showed higher expression of antiapoptotic Bcl-2, Bcl-XL, survivin, and the transcription factor, NF-kappaB, in the recurrent tumor than in the adjoining normal tissue. Proapoptotic Bax was not detected in the tumor. Similar findings were obtained in the original primary tumor. Both tumors had a low apoptotic index according to the terminal deoxynucleotidyl transferase-mediated dUTP nick-end labeling assay. These changes occurred in the absence of cell proliferation, shown by the absence of both Ki-67 staining and increased telomerase activity. This derangement of apoptosis gives the aggressive desmoid tumor cells a proliferative advantage, and presumably, forms the basis of its high recurrence rate. Therefore, inhibitors of apoptosis proteins (IAPs) may be useful for predicting recurrence. The regulation of apoptosis by antisense therapy against these inhibitors could prove beneficial for overcoming repeated recurrence, even after surgery.
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PMID:Characterization of apoptosis-related molecular changes in a desmoid tumor of the chest wall: report of a case. 1273 31

Desmoplastic fibroma is a rare primary tumor of bone that histologically and biologically mimics the extra-abdominal desmoid tumor of soft tissue. It usually presents in patients during the first three decades of life and often involves the mandible or long bones of the skeleton. Its clinical behavior is characterized by a locally aggressive, infiltrating, and destructing course, often with invasion of surrounding tissues but without metastasis. We present herein the clinicopathological features of a desmoplastic fibroma-like tumor involving the left maxillofacial region in a 14-year-old Hispanic boy with tuberous sclerosis.
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PMID:Desmoplastic fibroma-like tumor of maxillofacial region associated with tuberous sclerosis. 1561 47

Desmoplastic fibroma is a very rare primary tumor of bone, closely related to aggressive fibromatosis of soft tissue. Although considered a benign lesion, it can be very aggressive locally and has a high rate of local recurrence after incomplete surgical excision. Radiologically, the lesion shows no distinctive features, often simulating osteosarcoma, fibrous dysplasia, or fibrosarcoma. Rib involvement by desmoplastic fibroma is extremely rare. We present two cases of desmoplastic fibroma involving this unusual location with lytic costal lesion and chest wall extension. Histological examination after surgical resection revealed that the tumor consisted of spindle cells with small, elongated nuclei in a background of numerous collagen fibers and infiltrating lamellar bone. There was no evidence of nuclear atypia, mitotic activity, or necrosis. We also discuss histological differential diagnosis as well as clinical features and the radiological and pathologic findings of this rare disease. Recognition of this entity is important to ensure proper surgical treatment.
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PMID:[Desmoplastic fibroma of the rib: two case reports]. 1649 94

Sarcomas comprise a heterogeneous group of mesenchymal neoplasms. They can be grouped into 2 general categories, soft tissue sarcoma and primary bone sarcoma, which have different staging and treatment approaches. This review includes a discussion of both soft tissue sarcomas (malignant fibrous histiocytoma, liposarcoma, leiomyosarcoma, synovial sarcoma, dermatofibrosarcoma protuberans, angiosarcoma, Kaposi sarcoma, gastrointestinal stromal tumor, aggressive fibromatosis or desmoid tumor, rhabdomyosarcoma, and primary alveolar soft-part sarcoma) and primary bone sarcomas (osteosarcoma, Ewing sarcoma, giant cell tumor, and chondrosarcoma). The 3 most important prognostic variables are grade, size, and location of the primary tumor. The approach to a patient with a sarcoma begins with a biopsy that obtains adequate tissue for diagnosis without interfering with subsequent optimal definitive surgery. Subsequent treatment depends on the specific type of sarcoma. Because sarcomas are relatively uncommon yet comprise a wide variety of different entities, evaluation by oncology teams who have expertise in the field is recommended. Treatment and follow-up guidelines have been published by the National Comprehensive Cancer Network (www.nccn.org).
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PMID:Sarcoma. 1797 62

A 25-year-old female with familial adenomatous polyposis (FAP) presented with an abdominal tumor just below the scar due to a colectomy performed 15 months previously. This tumor (tumor A) measured 7 cm in diameter, was diagnosed as a desmoid tumor of the abdominal wall, and was excised. Despite the subsequent administration of sulindac (300 mg daily for 1 year), a desmoid tumor recurred at the same site. Excision was performed again when the tumor was 8 cm in diameter, and examination revealed it to consist of a large tumor (B) and a small tumor (C) that bulged out from tumor B. Germ-line APC analysis showed a C deletion at codon 1460 resulting in a stop codon. Two somatic mutations were observed in tumor A: a TCAA deletion at codon 1068 and a deletion of a codon at bp 1192-2097. In tumor B, a somatic mutation was found at codon 1041 changing CAA to TAA. We could not detect any somatic mutations in tumor C. We conclude that somatic mutation analysis of the APC gene can be used to identify whether a recurrent desmoid tumor in a patient with FAP is a new primary tumor or a recurrence from microscopic remnants of the original tumor.
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PMID:Identification of somatic APC mutations in recurrent desmoid tumors in a patient with familial adenomatous polyposis to determine actual recurrence of the original tumor or de novo occurrence. 1870 58


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