Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Soft tissue sarcomas are uncommon malignancies, less than 10% of which arise on the distal upper extremities. Consequently, experience with treatments which preserve both the limb and its function is lacking for tumors in this region. Sixteen patients with sarcomas arising in the hand and wrist and one with an aggressive desmoid tumor were treated by combined modality therapy at the Massachusetts General Hospital. Two patients had wide resections for multiple recurrent lesions, 5 had excisional biopsies, and 9 had incomplete excisions to preserve anatomic structures of the hand. One patient refused an amputation and had no surgery. Sarcoma patients were given postoperative radiation with a dose range of 50.2 to 69 Gy (median 68 Gy). The desmoid tumor received 44 Gy. A shrinking field technique with customized castings and cerrobend blocks was used to assure precision and minimize treatment volumes. Chemotherapy was reserved for metastatic disease. Local control was achieved in 14 patients who received combined modality treatment (87%), with a follow-up 1-12 years (median 33 months). Two of the three patients with local failures subsequently obtained a local control after salvage surgery and radiation. Four patients developed metastases, one with epitrochlear lymph node metastases was salvaged by amputation, the others died with lung disease 17, 37, and 111 months after treatment. Functional integrity of the limb was primarily dependent on the extent of surgical resection required. Among 12 patients with local and distant control, one patient (who had multiple wide resections of an extensive desmoid tumor preceding irradiation) lost over 50% use of her limb, but no patients required amputation for edema or pain control. Ten of the 12 patients with local and distant control had less than a 25% decrement in limb function and had no pain or edema associated with normal use of their hand. We conclude that for selected patients with sarcomas of the distal upper extremity, combined modality therapy consisting of conservative resection and careful radiation therapy is a viable alternative to amputation.
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PMID:Extremity preservation by combined modality treatment of sarcomas of the hand and wrist. 377 12

A retrospective comparison was made between soft tissue sarcomas of the buttock treated during two consecutive periods at one institution: (1) 1966-1978, during which almost all tumors were treated by radical buttectomy with limb preservation, often in conjunction with radiation therapy, and (2) 1934-1966, as previously reported by Wanebo et al, in which treatment by hemipelvectomy was frequently offered. Desmoid tumors were excluded. The five-year survival rates after curative treatment were almost identical, 40% vs 39%, respectively. Distant metastases were the main cause of death. Very high local recurrence rates were recorded after therapy: 45% vs 39% for the respective periods. Local recurrences seemed prone to occur with large, deeply fixed sarcomas, particularly when inadequate or no radiation therapy had been utilized. These observations point to the need for innovative methods to prevent local tumor recurrence and a trial of adjuvant chemotherapy if long-term cure with limb preservation is to be achieved.
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PMID:Sarcoma of the buttock: a trend toward limb-saving resection. 617 11

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

Sixteen cases of mandibular tumors or paramandibular soft tissue tumors with mandibular involvement are reported. These include such rare mandibular tumors or tumor-like conditions as melanotic progonoma, intraosseous haematoma secondary to von Willebrand's disease, post-irradiation osteosarcoma, monostotic eosinophilic granuloma, aneurysmal bone cyst and osseous hemangiopericytoma. Three cases of cherubism, one of fibrous dysplasia or aggressive fibromatosis and one of central giant cell reparative granuloma are also reported. The soft tissue tumors comprise round cell sarcoma, parotid adeno-carcinoma with generalised metastases, embryonal rhabdo-myo-sarcoma, neuro-fibro-sarcoma and congenital cystic hygroma. In all the cases the disease was well advanced when the patient presented for X-ray examination. The specific X-ray diagnosis of mandibular and paramandibular tumors in childhood is more difficult than that of similar tumors in other parts of the body.
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PMID:Mandibular and para-mandibular tumors in children. Report of 16 cases. 627 31

Abdominal wall desmoid tumor is a rare, locally invasive proliferation of mature fibrous tissue which does not metastasize. It usually presents as an asymptomatic mass, and occurs most frequently in young women after childbirth. All three of our patients showed an echo-free mass deep in the abdominal wall with poor sonic transmission. Ultrasound cannot only accurately localize the lesion, but allows the radiologist to suggest the correct diagnosis.
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PMID:Ultrasonic diagnosis of abdominal wall desmoid tumor. 644 8

Inflammatory myofibroblastic tumor (IMT) or inflammatory pseudotumor is a spindle cell proliferation of disputed nosology, with a distinctive fibroinflammatory and even pseudosarcomatous appearance. Although the lung is the best known and most common site, inflammatory myofibroblastic tumor occurs in diverse extrapulmonary locations. We report our experience with 84 cases occurring in the soft tissues and viscera of 48 female patients and 36 male patients between the ages of 3 months and 46 years (mean, 9.7 years; median, 9 years). A mass, fever, weight loss, pain, and site-specific symptoms were the presenting complaints. Laboratory abnormalities included anemia, thrombocytosis, polyclonal hypergammaglobulinemia, and elevated erythrocyte sedimentation rate. Sites of involvement included abdomen, retroperitoneum, or pelvis (61 cases); head and neck, including upper respiratory tract (12 cases); trunk (8 cases); and extremities (3 cases). The lesions ranged in size from 1 to 17 cm (mean, 6.4; median, 6.0). Excision was performed in 69 cases. Eight had biopsy only. Five patients received chemotherapy or radiation in addition to undergoing biopsy or resection as initial treatment. Sixteen patients had multinodular masses involving one region. Clinical follow-up in 53 cases revealed that 44 patients were alive with no evidence of disease, four were alive with IMT, and five were dead. Thirteen patients had one or more recurrences at intervals of 1-24 months (mean, 6 months; median, 10 months). No distant metastases were documented. The five patients who died had complications either due to the location of the lesion (heart, peritoneum, retroperitoneum, or mesentery) or related to treatment (lymphoproliferative disorder following hepatic transplantation; sepsis following wound infection). The abdominal masses were the largest. All tumors were firm and white with infiltrative borders and focal myxoid change. Three basic histologic patterns were recognized: (a) myxoid, vascular, and inflammatory areas resembling nodular fasciitis; (b) compact spindle cells with intermingled inflammatory cells (lymphocytes, plasma cells, and eosinophils) resembling fibrous histiocytoma; and (c) dense plate-like collagen resembling a desmoid or scar. Immunohistochemistry demonstrated positivity for vimentin, muscle-specific actin, smooth muscle actin, and cytokeratin consistent with myofibroblasts. Based on this series, inflammatory myofibroblastic tumor is a benign, nonmetastasizing proliferation of myofibroblasts with a potential for recurrence and persistent local growth, similar in some respects to the fibromatoses.
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PMID:Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). A clinicopathologic and immunohistochemical study of 84 cases. 866 41

Fibromatosis can be classified in two groups: Superficial fibromatosis without capacity for infiltration and deep fibromatosis with variable capacity for infiltrative growth. One of the deep fibromatoses is aggressive fibromatosis which is a local infiltrating process never known to metastasize. Two cases of aggressive fibromatosis are presented and histological characteristics and prognosis are reviewed. Once the diagnosis aggressive fibromatosis is established, surgical removal of the lesion by large excision is the only sufficient treatment.
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PMID:[Aggressive fibromatosis]. 799 49

Twenty four cases of pathologically diagnosed aggressive fibromatosis of bone were reported. 17 patients were followed for at least 2 years. Among them 4 recurred and the histological findings of their lesional tissues obtained after recurrence remained benign; one case refused radical treatment and the second biopsy done at 3.5 years after the appearance of the tumor showed no malignant changes; but another case died of lung metastasis. The authors suggest that the criteria for final diagnosis of an aggressive fibromatosis of bone are as follows: Besides the pathological findings, the lesion does not recur or metastasize more than 2 years after operation or it does recur, but still shows benign features histologically. As for treatment, marginal or wide excision is the procedure of choice.
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PMID:[Aggressive fibromatosis of bone]. 822 13

Desmoid tumors are accounted among histologically benign soft tissue tumors. Though metastases do not occur the clinical course of the disease with their tendency to grow locally aggressive and to relapse after curative intended surgery is similar to sarcomas. After surgical excision adjuvant therapy of desmoid tumors is necessary in such cases, where a radical excision with a wide margin is not possible, or must be doubted or after the surgical excision of a locally recurrent tumor. Radiation doses of 50 to 60 Gy reduce the risk of local recurrence of desmoid tumors to 18%. In such cases, where surgery and radiotherapy have been leading to incomplete responses, systemic therapy with hormones (tamoxifen or progesterone), with alkylating agents (e. g. ifosfamide) or with prostaglandin antagonists (indomethacin) is possible. The published data on therapeutic responses to the treatment of desmoid tumors are scarce. We are reporting on seven of our own cases, treated with surgery and radiotherapy (four) or with a combination of surgery, radiotherapy, hormones and chemotherapy (three).
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PMID:[The clinical picture and therapy of aggressive fibromatosis (desmoids)]. 848 59

Aggressive fibromatoses (desmoid tumors) are rare tumors of fibroblastic origin that may arise in any musculoaponeurotic structure with a propensity of infiltrate adjacent tissues, but not to metastasize. Tumors of musculoaponeurotic origin are seldom encountered by the gynecologist. A case of pelvic fibromatosis is reported together with a discussion of theories of etiology and management options.
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PMID:Aggressive fibromatosis of the female pelvis. A case report and review of the literature. 878 Sep 20


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