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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Liposarcoma is one of the most common soft tissue sarcomas, ranking with rhabdomyosarcoma and malignant fibrous histiocytoma. We have treated 47 cases of liposarcoma, comprising 35 males and 12 females ranging in age from 22 to 86 years, with a median age of 56 years. The most common site of primary tumor was the thigh, the most common primary tumor stage by the UICC classification was T2, and the most common histological subtype by the WHO classification was myxoid type. Xerography, CT and angiography are useful forms of examination for diagnosis before surgery. Naked eye findings of the tumor often show a lobular or nodular growth pattern. Surgical therapy for the primary tumor, especially curative wide resection (by the method of Kawaguchi, et al.) is most effective for preventing local recurrence. Radiotherapy is not as effective as for rhabdomyosarcoma and chemotherapy should be applied for occult micro-metastases. Ten of the 47 cases died due to distant metastases and the 5 year survival rate was 76.2%.
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PMID:[Analysis of liposarcoma]. 329 58

A review of 1415 patients without distant metastasis from the Intergroup Rhabdomyosarcoma Study (IRS) I and II revealed an overall 10% incidence of identified lymphatic spread at diagnosis, whereas 81 of 592 children with localized rhabdomyosarcoma who had grossly complete resection (and therefore with more complete pathologic data) had histologically proven lymphatic spread (14%). The percentage of patients in this latter group with nodal metastases was highest for the prostate (41%), paratesticular sites (26%), and genitourinary sites overall (24%). Sites with a small percentage of proven lymphatic involvement were the orbit (0%), nonorbital head and neck sites (7%), and truncal sites (3%), whereas the percentage of patients with nodal metastases from extremity lesions was 12%. The primary tumor mean diameter was significantly larger in the group with nodal metastases, but there was no evidence of a relationship between lymphatic spread and age, sex, or histologic subtype. Patients with lymph node metastases who had resection had a poorer survival rate (logrank P value = 0.001), with a 3-year survival estimate of 54%, compared with 78% for patients without lymphatic metastases. Patients with extremity lesions and positive lymph nodes also did poorly when compared with patients with normal nodes (P = 0.006), and a similar observation was made for patients with paratesticular sarcoma (P = 0.06).
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PMID:Lymphatic metastases with childhood rhabdomyosarcoma. A report from the Intergroup Rhabdomyosarcoma Study. 329 2

Rhabdomyosarcoma is the most common soft tissue sarcoma in children under 15 years of age. Several decades ago it was associated with an 80% mortality rate. Recent advances with combined modality therapy have improved the 5-year survival rate to almost 70%. The focus for the improvement in treatment regimens is now shifting to concerns regarding the preservation of body function and the treatment of far advanced disease. Regardless of age, histology, stage, or site of primary tumor, there has been no group where chemotherapy has not been proven beneficial. The current recommendations used by the Intergroup Rhabdomyosarcoma Study, with regard to chemotherapy, are summarized. Radiation therapy adjunctive to surgery has a useful place in the treatment of rhabdomyosarcoma, but prophylactic nodal irradiation is not recommended. Involved nodes should, however, be treated. Since the potentially adverse side effects of chemotherapy and radiation are accentuated when combined, radiation therapy is no longer recommended in patients with group I rhabdomyosarcoma. Prognostic factors and future considerations from the operative standpoint are presented.
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PMID:Advances in the treatment of rhabdomyosarcoma. 333 Feb 73

Although combination chemotherapy and local irradiation are quite effective treatment for some children and young adults with small round cell sarcomas, high-risk patient groups, including patients with localized disease of the trunk and proximal extremity and those who present with metastases, continue to fare poorly with standard combined modality therapy. In an attempt to improve the local and systemic response of these tumors, an intensive treatment protocol was designed that integrates five cycles of chemotherapy with vincristine, doxorubicin, and cyclophosphamide (VADRIAC) plus radiation therapy to the primary tumor (55-60 Gy), bone, and soft tissue metastases (45-50 Gy). Patients achieving complete response to this induction therapy receive intensification treatment with total body irradiation (8.0 Gy), a cycle of VADRIAC, and autologous bone marrow transplantation. All treatment is completed within 6-7 months. From January 1983 to February 1986, 76 consecutive, previously untreated patients were entered in this study; 75 patients are evaluable. Twenty-five patients were diagnosed with rhabdomyosarcoma, 23 with Ewing's sarcoma, 15 with primitive neuroepithelioma, 12 with primitive sarcoma, and 1 patient with metastatic neuroblastoma. Forty-three patients (57%) had metastases at presentation. Overall, 68 of 75 patients (91%) achieved complete response. Fifty-eight of 61 patients with measurable soft tissue masses at the primary site had greater than or equal to 50% tumor reduction with two cycles of chemotherapy prior to local irradiation. Seven patients failed to have complete response at the primary site following five cycles of chemotherapy and local external beam irradiation, although 3 were subsequently rendered locally disease free by intraoperative radiotherapy (2 patients) or surgery (1 patient).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Treatment of high-risk sarcomas in children and young adults: analysis of local control using intensive combined modality therapy. 335 73

Rhabdomyosarcoma (RMS) of the hepatobiliary system is extremely rare in adults. To our knowledge only three cases have been reported in the literature, all involving the gallbladder. The present case concerns a 40-year-old woman who presented with epigastric pain and obstructive jaundice and was found to have a fusiform, submucosal neoplasm in the common bile duct. Histologically, the tumor presented a diagnostic problem due to a predominant sclerotic growth pattern suggesting an epithelial tumor. Extensive sampling revealed a focal alveolar growth pattern with rhabdomyoblasts, although cross striations were not seen. Electron microscopy failed to demonstrate the characteristic thick myofilaments and/or Z-band material. The diagnosis was supported by strongly positive immunohistochemical staining for myoglobin and desmin; the keratin stain was negative. A subsequent supraclavicular metastasis showed the typical histology of an alveolar RMS. The histologic features of the primary tumor suggest that RMS in this location may be underrecognized due to regional similarities to either primary or metastatic infiltrating carcinomas.
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PMID:Rhabdomyosarcoma of the common bile duct in an adult. 351 54

Several inflammatory processes can cause nodules or swelling in the neck. A complete physical examination and, usually, laboratory testing are required to establish the diagnosis. Common infections include cervical lymphadenitis and tuberculous lymphadenitis, cat-scratch disease, infection in the neck spaces, infectious mononucleosis, and syphilis. Primary or metastatic cancer may also be the cause. Cervical metastasis often presents as a neck mass. Although a primary tumor may not be found immediately when a neck mass is being evaluated, one is often discovered later. Other types of malignancy that may be present are histiocytic lymphoma, Hodgkin's disease, rhabdomyosarcoma, thyroid cancer, and a salivary (most often parotid) gland tumor. Symptomatic treatment is sometimes adequate for infectious disease, but administration of antituberculous drugs or antibiotics may also be necessary. Incision and drainage are required for some nodes and abscesses. For neck masses caused by neoplasms, fine-needle aspiration cytology or biopsy is performed. Depending on the diagnosis, treatment consists of dissection, radiation therapy, and/or chemotherapy.
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PMID:The neck mass. 2. Inflammatory and neoplastic causes. 355 1

The therapeutic results obtained in eight cases of childhood malignant soft-tissue sarcoma are summarized. Male to female ratio was 4:4, four were rhabdomyosarcoma and 5 were defined as Group I/II. Adjuvant combination chemotherapy was administered soon after biopsy (3 cases), or after partial or total resection (3 cases) of the primary tumor. In one case, irradiation was employed and in the remaining case, no further therapy was given after surgery. The three patients who underwent total removal of the tumor followed by adjuvant chemotherapy including adriamycin, and a patient with a partially resected intra-orbital tumor which responded well to a similar form of chemotherapy are currently alive after 26+ to 71+ months. By contrast, four patients whose tumors were too bulky to be resected responded poorly to chemotherapy and died. It must be emphasized that adjuvant chemotherapy is of limited value in the treatment of advanced childhood soft-tissue sarcoma.
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PMID:[Therapeutic effect of adjuvant combination chemotherapy in the treatment of 8 cases of childhood soft-tissue sarcoma]. 356 3

Seventy-nine patients with rhabdomyosarcoma (RMS) received treatment at the National Cancer Center Hospital between 1962 and 1985. The patients ranged in age from 4 months to 74 years with a median age of 6 years. Forty-six patients were male and 33 were female. The primary tumor site of RMS was the same as in the previous report. The head and neck region was the most frequent site (40.5%), followed by the extremities (34.1%), genitourinary region (15.2%), trunk (5.1%) and retroperitoneum (5.1%). Histologic types were embryonal RMS in 45 patients, alveolar RMS in 23 patients, pleomorphic RMS in 8 patients and unclassified RMS in 3 patients. As of October 1985, 14 of the 79 patients were still alive. Between 1962 and 1971, 38 patients were not treated by any protocol. After 1972, 41 patients received treatment using a 3 stage-related, multiple-modality program. In the first protocol, chemotherapy consisted of Vincristine, Cyclophosphamide, and Actinomycin-D, and 1 of 18 patients have survived more than 5 years. The cumulative 5-year survival rate of the first protocol was 11.1%. In the second treatment program, which involved Adriamycin in addition to the 3 drugs cited above, 4 of 23 patients have survived more than 5 years. The cumulative 5-year survival rate, 33.2%, was very improved.
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PMID:[Treatment of rhabdomyosarcoma at the National Cancer Center Hospital]. 359 4

Many Experimental and human tumor cell lines have been previously described as being dependent upon exogenous methionine for their in vitro proliferation. The rationale of the experiments described herein was to decrease the in vivo growth of malignant tumors by reducing the exogenous methionine available in diets fed to Wistar AG rats bearing the highly metastatic rhabdomyosarcoma, RMS-J1. The methionine content in the diet was reduced either by replacing casein (diet 1) with soybean protein (diet 4), or by lowering the amount of soybean protein in the diet (from 23 g/100 g to 12 g/100g) (diet 5), or by using a crystalline amino acid-defined mixture as the source of protein (diet 7). In the latter diet homocysteine replaced methionine and allowed the survival of the animals. Diet 4 significantly reduced the mean number of lung metastases without affecting the primary tumor growth. Treatment of RMS-J1 bearing rats with diet 5 led to the decrease of pulmonary invasion (78 and 21 median lung metastases, respectively, in control and treated groups). This diminished metastatic dissemination resulted from the reduced methionine consumption: the lowered casein content in diet 3 (10 g/100 g) as compared to diet 1 (23 g) did not alter primary tumor growth or the amplitude of lung invasion. Moreover, the addition of methionine to diet 5 prevented the diminution of the median number of lung metastases. Replacement of methionine with homocysteine in the crystalline amino acid-defined mixture (diet 7) fed to RMS-J1 bearing rats led to a limited retardation of primary tumor growth (less than 10%) and to a significant decrease in pulmonary invasion: the median number of pulmonary metastases was 28 and 9 for control and treated rats respectively.
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PMID:Decreased rat rhabdomyosarcoma pulmonary metastases in response to a low methionine diet. 367 75

Embryonal and alveolar rhabdomyosarcoma probably have the same histogenesis, yet alveolar rhabdomyosarcoma has a considerably worse prognosis and is generally less responsive to treatment. The metastatic behavior of rhabdomyosarcoma in 22 autopsied subjects was analyzed with respect to the histologic subtypes of the tumors to determine whether the more aggressive behavior of alveolar rhabdomyosarcoma was related to greater tumor burden and/or more widely disseminated metastases. Subjects with embryonal rhabdomyosarcoma tended to survive longer and had greater numbers of metastatic sites at autopsy compared to subjects with alveolar rhabdomyosarcoma, but these differences were not statistically significant. In addition, there were no significant differences in the overall distribution of metastases between embryonal and alveolar rhabdomyosarcoma. The most frequent sites of metastases from both types of tumor were soft tissues (73%), serosal surfaces (64%), lymph nodes (64%), lungs (55%), and bone marrow (50%). The findings suggest that the metastatic behavior of rhabdomyosarcoma does not vary as a function of length of survival, mode of therapy, or histologic subtype. We propose that factors such as stage of disease at presentation and location of the primary tumor, rather than histologic subtype, are probably the most important variables governing the therapeutic responsiveness of rhabdomyosarcoma.
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PMID:Metastatic behavior of rhabdomyosarcoma. 373 70


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