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)

Rhabdomyosarcoma is the most frequent spermatic cord tumor of infants, children, and young adults, but also occurs as a primary tumor in the testis, epididumis, and testicular tunics. In the last fifteen years, 7 patients with intrascrotal rhabdomyosarcoma were treated at our institution, and an additional 155 cases were found on review of the English literature. On the basis of these 162 cases, incidence and survival statistics were calculated with particular attention to employed forms of therapy. An over-all survival of greater than 73 per cent should be obtainable with proper utilization of surgery, radiation therapy, and combination chemotherapy.
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PMID:Intrascrotal rhabdomyosarcoma. 11 50

Twenty-two patients with rhabdomyosarcoma have presented in the years 1970--1977. Of 8 patients who had inadequate postoperative chemotherapy and radiotherapy, or none at all, only 2 patients with small completely resectable tumors are alive and well at 6 and 4 yr after surgery. Fourteen patients had intensive combination chemotherapy in addition to appropriate surgery and radiotherapy. Five have died and the remaining nine are well and free of disease at follow up ranging from 9 mo to over 5 yr. A continued place in the treatment regime is advocated for early surgery aimed at total excision of the primary tumor where this is feasible.
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PMID:Recent experience in the treatment of rhabdomyosarcoma. 42 61

The records of 27 patients with rhabdomyosarcoma involving the parameningeal area (nasopharynx, paranasal sinus, and middle ear) treated from 1961 to 1976 were reviewed. Due to the location of the primary tumor, radiation and chemotherapy were used but surgery was limited to simple biopsy. In the literature, spread of tumor from these primary sites to the meninges has been as high as 26-35%. In this series, meningeal disease developed in only 2 of the 27 patients (7%).
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PMID:Parameningeal rhabdomyosarcoma. 42 88

Rhabdomyosarcomas induced by single intramuscular injections of nickel sulphide (Ni3S2) in Fischer and Hooded rats were cultured in vivo and in vitro to study their growth characteristics and chromosomal constitution. The tumor cell suspensions cultured in vitro exhibited more myogenic differentiation on the coverslips than those cells grown in vivo in diffusion chambers. A characteristic feature of in vivo cultures was the appearance of microclusters which resembled the primary tumors. Chromosome analyses of primary tumors revealed that a majority of these had a modal number in the diploid or near diploid range. Fischer rat primary tumor cells exhibited abnormal configurations including rings, dicentrics and triradials. A comparison of the chromosome make-up of the primary tumors and their metastases was performed on four sets of tumors. Three out of four metastases examined showed the diploid chromosome make-up characteristic of the primary tumors suggesting that the tumors with the diploid or near diploid chromosome constitution are more likely to produce metastases.
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PMID:Growth and cytogenetic characteristics of nickel sulphide-induced rhabdomyosarcomas in rats. 68 91

The association of consumptive coagulopathy and malignancy is thought to be rare in pediatrics. In one patient with Ewing's sarcoma and in two with rhabdomyosarcoma consumptive coagulopathy developed in the presence of either diffuse metastatic disease or an extensive primary tumor. The coagulopathy was a major clinical problem, developing within five days of the onset of chemotherapy. Resolution coincided with the clinical response of the tumor.
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PMID:Consumptive coagulopathy. A complication of therapy of solid tumors in childhood. 68 3

Thirty-nine previously untreated children with rhabdomyosarcoma were managed by a coordinated program of surgery, radiation therapy, and chemotherapy during the years 1960 to 1973. The primary tumor was located in the head and neck (24), chest wall (1), abdomen (1), pelvis (10), and lower extremity (3). Radiation therapy consisted of tumor doses of 5000 to 6000 rads delivered in five to six weeks. Combination chemotherapy with actinomyctin-D, vincristine and cyclophosphamide was used after 1968. Seventeen of 25 cases (68%) treated after 1968 are alive two to five years following treatment. Only four of 14 cases (29%) who received less radical therapy before 1968 are alive. A relatively high incidence of local failure (23%) was noted in spite of adequate doses of radiotherapy. None of the four cases with metastatic disease at the time of diagnosis survived. Major complications were mainly noted in patients with orbital rhabdomyosarcoma. Correlation of absolute survival with dose of irradiation, primary site, extent of disease and histologic subtypes of the tumor are discussed.
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PMID:Combined treatment modalities of rhabdomyosarcoma in children. 87 41

One hundred fifty-five adult patients with "operable" soft part sarcomas including rhabdomyosarcoma, liposarcoma, leiomyosarcoma and fibrosarcoma of the trunk and extremities are reviewed. Local recurrences of 93% and 60% occurred after local and wide excisions of the primary tumor. In this series of patients, amputation was the most efficient procedure for controlling the primary site. The absolute 5 and 10-year survival rates for all groups of tumors were 50% and 26%. Development of a second primary tumor of a different cell type occurred in 9% of the patients. Local recurrence, single distant metastasis, and/or second primary tumors should be considered potentially curable and appropriate surgical and/or radiation therapy carried out.
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PMID:Soft tissue sarcomas. 113 30

A 22-year-old female patient with a swelling in the right forearm near the elbow underwent a 67Ga-citrate scan because of paraesthesia on the ulnar side of the right hand. The 67Ga total body scan showed intense focal uptake on the forearm in the same position as the swelling. At surgery a histological diagnosis of alveolar rhabdomyosarcoma was made. Five months later, a repeat 67Ga scan was normal. Eight months after the diagnosis, the patient complained of pain in the left lumbar region extending to the hypogastric area. A third 67Ga scan showed intense uptake near the spinal column which at surgery was found to be a metastasis of the primary tumor.
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PMID:67Ga-citrate imaging in rhabdomyosarcoma. 129 74

Pulmonary blastoma is a very rare primary tumor of the lung, which mostly occur at a late age in adults. Six cases in children under four years of age are reported in this series. The light-microscopic, immunohistochemical and electron-microscopic characteristics revealed that the elements of P.B. in childhood consist mainly of mesenchymal components, including undifferentiated large cells and small round cells, which tend to differentiate toward rhabdomyosarcoma. The epithelial components comprise only a small portion of the total tumor, and most appear as the well-demarcate branching tubular structures lined with columnar or cubiodal epithelium merging with the stroma. The degree of differentiation of mesenchymal elements is closely related to the prognosis.
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PMID:[Pulmonary blastoma: a light-microscopic, immunohistochemical and electron-microscopic study of six cases in childhood]. 129 30

Primary pulmonary tumors are infrequent in childhood, therefore an accurate diagnosis and treatment is often delayed. We review the English language literature and report the clinical and pathological features of eight tumors arising in the lungs of pre-adolescent children, accessioned between 1960 and 1991 in the pathology department of a children's hospital in South Africa. The ratio of pulmonary primary tumors to secondary neoplasms and to non-neoplastic lesions of the lung examined during this period was 1:5:60. Over the last 31 years we received three plasma cell granulomas, two pleuro-pulmonary blastomas, a mucoepidermoid carcinoma, an endobronchial fibrosarcoma, and a hemangioma. All patients presented with cough unresponsive to medical treatment. The incidence and spread of primary lung tumors in children was similar to that reported from other centers. Plasma cell granuloma is the most common primary tumor in the lungs of children. Aggressive behavior is most frequently encountered with pleuro-pulmonary blastoma and rhabdomyosarcoma, and because of their association with cystic lesions careful examination of lungs is required in such cases. Most other malignant neoplasms, such as muco-epidermoid carcinoma and primary fibrosarcoma, are usually of a low grade of malignancy. A decreasing incidence of bronchogenic carcinoma seems to be reported during the first two decades of life.
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PMID:Primary pulmonary tumors in childhood: a review of 31 years' experience and the literature. 133 97


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