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)

We report an unusual case of primary cutaneous embryonal rhabdomyosarcoma presenting as a solitary skin lesion on the anterior chest of a 20-month-old child. The tumor was characterized by small, round to oval, poorly differentiated cells. Immunohistochemically, the tumor was negative for NSE, S-100 protein, LCA, and keratin but positive for muscle-specific actin, myoglobin, desmin, and vimentin, thus indicating the presence of myogenous differentiation. Ultrastructural analysis demonstrated thick and thin filaments. Special studies showed no evidence of a primary rhabdomyosarcoma in the patient at a more typical location, nor was there any evidence of metastases.
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PMID:Primary cutaneous rhabdomyosarcoma. 220 84

A case is described of embryonal rhabdomyosarcoma (E.R.) of the middle ear in a 4-year-old child; survival has been over 9 years. R.E. is the most common malignant tumor of the auricular region in children and is most often fatal due to locoregional extensions or secondary metastases carried through the bloodstream and lymphatic systems. The basis for treatment is a multidisciplinary approach to the disease: surgery with as broad an exeresis as possible; radiotherapy with tumor-killing doses of 5,500/6,000 rads; and polychemotherapy (Vincristina, Endoxan, Methotrexate). Such "aggressive" treatment often results in a high rate of morbidity with complications involving the blood, bones, eyes and meninx often requiring temporary suspension of treatment and prolonged hospitalization.
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PMID:[Embryonal rhabdomyosarcoma of the middle ear: description of a case with long-term survival]. 226 Apr 43

The present paper reports a case of rhabdomyosarcoma in a 4-year-old patient, the rapidity with which it spread and the intensity of its extension bringing the case under observation. It is, in fact, a case of embryonal rhabdomyosarcoma localized in the right parotid area with an extension to the temporal fossa, to the epi-oro-hypopharynx as far as the lateral vertebral region with C5-C6 involvement and penetrating the medium cranialis fossa. Initially the child showed signs of tissue growth at the right parotid site, originating without symptoms and rapidly increasing in volume. As exploratory surgery revealed that it would be impossible to achieve radical removal of the growth, the authors opted for radio-chemotherapy treatment. This type of approach, however, gives short lived results, in this case evidenced by recurrences within six months and leading to the patient's death after just eight months. It is important to stress the rapid evolution of this neoplasm, its tendency to recur and metastasize as well as its poor prognosis in general.
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PMID:[A case of rhabdomyosarcoma of the parotid region]. 226 Apr 44

A case of prostatic rhabdomyosarcoma in a 5-year-old boy is reported. He was brought to our clinic on Apr. 1, 1982 with complaints of pollakisuria and urethral pain. X-ray examinations revealed a huge intrapelvic tumor, and it was histopathologically diagnosed as embryonal rhabdomyosarcoma with a specimen of transrectal needle biopsy. Since the tumor was too huge to resect completely, he was initially treated with combination chemotherapy regimen of vincristine, actinomycin D and cyclophosphamide (VAC therapy), and resulted in failure. Then another combination chemotherapy consisting of cis-diamminedichloroplatinum, vinblastine and bleomycin (PVB therapy) was tried, and the tumor showed reduction in size. On Oct. 15, 1982, total cystectomy with ileal conduit urinary diversion was performed. Histopathologically, degenerative change and partial necrosis of the tumor cell were recognized. After the operation, he was treated with radiation therapy and prophylactic VAC therapy. But six months later, multiple pulmonary metastases occurred and gradually increased in size and number. They did not respond to any other chemotherapy. He died on July 13, 1983. We discussed the chemotherapy for rhabdomyosarcoma, and emphasized that the PVB therapy should be tried on rhabdomyosarcoma as an initial chemotherapy.
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PMID:[Combination chemotherapy with cis-diamminedichloroplatinum, vinblastine and bleomycin for a rhabdomyosarcoma of the prostate in a child: report of a case]. 241 60

A case of prostatic rhabdomyosarcoma in a 5-year-old boy is presented. He was referred to us because of complete urinary retention. Histologically, embryonal rhabdomyosarcoma of the prostate was found. Initially, he was treated with combination chemotherapy consisting of vincristine, actinomycin-D, adriamycin and radiation therapy. This therapy reduced the size of the tumor markedly. Therefore, we performed prostatectomy. No tumor cells were found on the surgical margin of the resected prostate. About 11 months after the operation, rapidly growing recurrent lesions were found near the bladder neck. Bilateral hydronephrosis and an intravesical massive space occupying lesion were found on the excretory pyelograms and cystogram. Then three drug combination chemotherapy consisting of vinblastine, cis-diamminedichloroplatinum and bleomycin (PVB therapy) was administered and the first course of the chemotherapy reduced the size of the recurrent tumor. Drainage of contrast medium from bilateral kidneys became smooth, and no gross hematuria or severe frequency was observed. Total cystectomy with ileal conduit urinary diversion was performed. Four months after this operation, metastatic lesions appeared at the pubic bone, right ischiadic bone and rectum, and they were resected operatively. However, multiple pulmonary metastases soon occurred, and he did on June 30, 1985, approximately 2 years after the first diagnosis. We have discussed the effectiveness of combination chemotherapy, especially PVB therapy for recurrent cases.
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PMID:[Rhabdomyosarcoma of the prostate in a child: report of a case]. 244 85

A new cell line (CCA) was established from a human embryonal rhabdomyosarcoma. It showed an "early" myogenic differentiation pattern: vimentin expression was found in 100% of cells, desmin in about 40% and myosin of the embryonic isoform in about 5%. Class I HLA expression on CCA cells was undeterctable but was greatly increased by in vitro treatment with human recombinant interferon-gamma and only marginally increased by human recombinant tumour necrosis factor-alfa. CCA cell line was tumorigenic in nude mice after either subcutaneous or intramuscular injection; macroscopic spontaneous metastases were not detected. The ability to induce metastatic nodules in the lungs was found when CCA cells were injected intravenously in cyclophosphamide-pretreated nude mice and, at low frequency, in untreated nude mice.
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PMID:Metastatic ability and differentiative properties of a new cell line of human embryonal rhabdomyosarcoma (CCA). 251 17

We performed a retrospective analysis of 28 children presenting with confirmed paratesticular embryonal rhabdomyosarcoma to examine factors predictive of fatal outcome. Complete surgery, defined as radical inguinal orchiectomy with clear microscopic margins plus radical, retroperitoneal lymph node dissection with extirpation of all gross disease, was performed in 21 patients (75 per cent). All patients received chemotherapy and 20 received radiation therapy according to protocol. Of the patients 16 have survived for more than 5 years with no evidence of disease. Univariate analysis revealed that only the presence of parenchymal metastases at diagnosis (p less than or equal to 0.040) and unresectability (p less than or equal to 0.003) were significant predictors of fatal outcome. Multivariate analysis showed that unresectability was the most important predictor of mortality with an estimated relative risk of 5.8.
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PMID:Mortality in pediatric paratesticular rhabdomyosarcoma: a multivariate analysis. 274 62

Between 1972 and 1986, 37 patients with lower genital tract malignancies were treated with intracavitary or interstitial brachytherapy. Thirteen patients presented with clear cell adenocarcinoma, 14 patients with embryonal rhabdomyosarcoma, 6 patients with endodermal sinus tumor, 3 patients with sarcoma, and 1 patient with an undifferentiated tumor. FIGO classification was: Stage I, 16%; Stage II, 47%; and Stage III, 37%. Treatment policy included initial exploratory laparotomy with lymph node biopsy and ovarian transposition, chemotherapy (except in clear cell adenocarcinoma) and/or external radiotherapy prior to interstitial brachytherapy. Chemotherapy consisted of a combination of VAC-Ad (V = vincristine, A = D actinomycin, C = cyclophosphamide, Ad = adriamycin) in rhabdomyosarcoma and sarcomas, and MAC-Ad (M = methotrexate) in endodermal sinus tumor. External radiotherapy was used in seven patients: in one to reduce a bulky clear cell adenocarcinoma (20 Gy) and in six for pelvic nodal involvement (45 Gy). Brachytherapy techniques depended on tumor site and extent, and on the anatomy of the patients. Vulvar tumors were implanted with iridium-192 wires by an afterloading plastic tube technique. Cervical and vaginal tumors were treated with individually tailored moulded vaginal applicators loaded with either cesium-137 or iridium-192, with or without interstitial implants by plastic tube or guide gutter technique. Computerized dosimetry allowed calculation of treatment volumes and doses delivered on the tumor and adjacent critical organs. The prescribed dose (including external radiotherapy) was 60-75 Gy with 1-3 brachytherapy applications of a low dose rate (0.2 Gy/hr). Six patients are dead: one from chemotherapy complication, three of metastases (two sarcomas, one endodermal sinus tumor) and two of pelvic failures and metastases (two clear cell adenocarcinoma). The overall disease free 5-year survival is 72%. Actuarial 5-year local control is 84%, but including salvage is 94%: three (two rhabdomyosarcoma, one clear cell adenocarcinoma) of the five local failures were salvaged by surgery, chemotherapy and/or brachytherapy. Metastases occurred in six patients, one (sarcoma) salvaged by chemotherapy and external radiotherapy. Complications requiring surgery occurred in five patients: two hydronephroses, one urethral stricture, one ileo-cecal obstruction, and one vesicovaginal fistula. Twelve of the 17 patients (71%) over 12 years of age are normally menstruating. Two patients have produced three normal children. This multidisciplinary management of lower gynecological tract tumors including brachytherapy is both conservative and effective.
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PMID:Conservative treatment for lower gynecological tract malignancies in children and adolescents: the Institut Gustave-Roussy experience. 277 54

The patterns of disease extent, response to treatment, and survival rates were examined in 129 children registered as metastatic (Group IV) soft-tissue sarcoma patients on the Intergroup Rhabdomyosarcoma Study (IRS)-I protocol from November 1972 through October 1978. The patients' ages at diagnosis ranged from 3 months to 21 years (median, 10.5 years); 72 were male patients and 57 were female patients (male-to-female ratio, 1.3:1). The tumor arose in an extremity in 34 patients, the head and neck (exclusive of the orbit) in 27, the genitourinary tract in 25, the retroperitoneum or pelvis in 14, the trunk in 12, the intrathoracic region in nine, the hepatobiliary system in four, the perineum in two, and the orbit in two patients. The histologic types of soft-tissue sarcoma confirmed by the pathology review committee were embryonal rhabdomyosarcoma in 68 patients, alveolar rhabdomyosarcoma in 41, undifferentiated sarcoma in 14, embryonal-botryoid in two, pleomorphic in one patient, and other sarcoma in three patients. Of 105 patients who were confirmed in Group IV and had complete information concerning sites of metastatic deposits, 53 patients presented with metastases restricted to only one anatomic region or tissue; the other 52 had diffuse metastases. The most common sites of distant tumor deposits were the lungs, bone marrow, and other soft tissues. After treatment with vincristine, dactinomycin, and cyclophosphamide with or without Adriamycin (Adria Laboratories, Inc., Columbus, OH) and radiation therapy, 65 (50%) of the 129 eligible patients achieved complete disappearance of all detectable tumor. The likelihood of achieving and maintaining a complete response for 2 years was highest among patients whose tumors originated in the genitourinary tract, retroperitoneum-pelvis, or the hepatobiliary or perineum regions. Thirteen of these 45 children (29%) are alive and disease-free at a minimum of 2 years after initiation of treatment. Only 10 of the other 84 (8%) are surviving disease-free at 2 years (P = 0.02). The presence of restricted rather than diffuse metastases at diagnosis (P = 0.02) was the only other characteristic significantly related to long-term complete remissions. One hundred and two patients (79%) died at a median of 47 weeks after diagnosis, 92 from sarcoma and ten from other causes.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Disease patterns and survival rate in children with metastatic soft-tissue sarcoma. A report from the Intergroup Rhabdomyosarcoma Study (IRS)-I. 284 74

The analysis of 7358 autopsies performed in 1966-1986 showed that cardiac tumors were detected in 94 cases (1.27%) and accounted for 4.9% of cancer and 5.48% of cardiac disease cases. Detailed characteristics are provided for 9 primary tumors, of these 3 were assessed clinically and morphologically, (left ventricular elastofibroma, left ventricular pleomorphic rhabdomyosarcoma, left atrial embryonal rhabdomyosarcoma). Primary metastases of pulmonary localization were recorded in 47.9% of cases. Metastatic involvement of the heart presented in 85 cases (1.15% of autopsies, 4.43% of cancer cases, 5% of cardiac affections, 90.42% of cardiac tumors). Cardiac compartments were evaluated for the frequency of tumor emergence.
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PMID:[Clinico-morphologic analysis of primary and metastatic tumors of the heart]. 325 Mar 83


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