Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We present the result obtained by GICOP in the treatment of 12 genitourinary rhabdomyosarcoma, located in vagina-uterus (5), bladder-prostate (4) and paratesticular (3). The age of the patients ranging from 1 to 15 years. Embryonal rhabdomyosarcoma was the most common histologic type (58 for 100) being the major number of cases in stadium II. The treatment was based in the combination of chemotherapy protocolised with vincristine, d-actinomycin, cyclophosphamide (VAC), vincristine, adriamycin (VAC-VAD) and/or iphosphamide, vincristine, d-actinomycin (IVA), use of radiotherapy, marrow auto-transplantation and radical surgery avoidance. Complete remission was achieved in 11 out of 12 cases (92 for 100), relapsing in 5 cases (38 for 100) of which 4 (80 for 100) achieved a second remission. An 82 for 100 disease free survival was achieved in a 5 year period.
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PMID:[Genitourinary rhabdomyosarcoma in childhood]. 248 79

A unique case of embryonal rhabdomyosarcoma arising at the left pleura of a 7-year-old Japanese girl is reported. The present case was characterized by persistent pleural effusion, and the malignant cells incidentally found in it were the first diagnostic clue. The tumor showed a rare growth pattern involving diffuse thickening of the parietal pleura. Biopsy of the thickened parietal pleura upon thoracotomy revealed embryonal rhabdomyosarcoma largely composed of immature mesenchymal cells. Immunohistochemical demonstration of creatinine phosphokinase-MM was most helpful among several types of immunostain for the histopathological diagnosis. Ultrastructurally, thin filaments with primitive Z bands could be seen in some tumor cells. Intensive clinical examination revealed only diffuse thickening of the parietal pleura, which was reduced by chemotherapy. This is the first documented case of rhabdomyosarcoma arising at the pleura. Previous reports of rhabdomyosarcoma arising at unusual sites are reviewed and the histogenesis of this tumor is briefly discussed.
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PMID:Diffuse pleural rhabdomyosarcoma with persistent pleural effusion. 262 6

A case of rhabdomyosarcoma of the spermatic cord in a 6-year-old boy is reported. On February 26, 1986, he visited Nakamura Hospital with the chief complaint of enlargement of the left scrotal content. The contralateral scrotum was normal. The left scrotal content was a hard thumb-head-sized tumor. The left testis and epididymis were not distinguishable from the tumor. On the same day, left high inguinal orchiectomy was performed. The tumor was 3.5 by 2.5 by 2.5 cm in size and was distinguishable from the testis, epididymis and tunica vaginalis. Histopathological findings were embryonal rhabdomyosarcoma and it appeared to have originated from the spermatic cord. Two years after operation, the boy is living without metastasis. Including our experience, 101 cases of the paratesticular rhabdomyosarcoma found in Japanese literature are reviewed and briefly discussed.
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PMID:[A case of embryonal rhabdomyosarcoma of the spermatic cord]. 269 46

Of 357 patients with soft tissue sarcomas (STSs) who were registered between 1981 and 1986 in the CWS-81 study, 21 (5.8%) were younger than 1 year of age. Six cases were probably of congenital origin since they were diagnosed in the 1st month of life. There was no difference in clinical grouping (staging and primary site) between infants and older patients. The major histological subsets comprised 12 patients with embryonal rhabdomyosarcoma (57% vs. 44% in older patients) and five patients with fibrosarcoma (23% vs. 2% in older patients). Of 21 patients, 17 received chemotherapy. The response rate (tumor reduction more than 1/3) to exclusive cytostatics therapy within 7-9 weeks, of ten evaluable patients, was 100% (vs. 86% in older patients). Eight of the responders achieved complete remission after 16 weeks of therapy and are in continuous complete remission (CCR). Most patients received 20-50% of the recommended chemotherapy dose for older children, which was calculated using body surface area. No fatal or life-threatening complications were observed. The event-free survival rate for children younger than 1 year of age with rhabdomyosarcoma was 70% vs. 47% in older children (no significant difference). Now, 2-5 years after therapy all five fibrosarcoma patients are alive and free of disease. Our data indicate that children under 1 year of age with rhabdomyosarcoma have a slightly better prognosis than older children, when therapy modalities are adapted to age.
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PMID:Soft tissue sarcomas in infants younger than 1 year of age: a report of the German Soft Tissue Sarcoma Study Group (CWS-81). 270 31

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

According to immunohistochemical, ultrastructural features of neural cells, and identical 11; 22 chromosome translocation, at least some extra osseous Ewing's sarcoma, as well as the malignant small cell tumor of the thoracopulmonary region (Askin's tumor) are actually classified as peripheral neuroepitheliomas. Embryonal rhabdomyosarcoma, including the botryoid variant, is now, when treated with appropriate chemotherapy, a tumor of relatively favorable histology. Its prognosis is still primarily related to clinical stage and location of tumor. The alveolar subtype of rhabdomyosarcoma (including its solid variant) has a less favorable prognosis. Hepatoblastoma (epithelial or mixt variants) have the same long term survival, also mainly related to stage. Pancreatoblastoma is a tumor with well differentiated cytopathology and prolonged course, compared with other pancreatic tumors.
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PMID:[Solid tumors in the child. New advances and the importance of histological sub-classifications. II. Tumors of the peripheral nervous system, soft tissue, liver and pancreas]. 284 Sep 19

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

Wilms' tumor, embryonal rhabdomyosarcoma, and yolk sac tumor are the most frequently diagnosed malignant genitourinary tumors in children. The randomized therapeutic trials developed by the National Wilms' Tumor Study (NWTS) committee have produced significant improvements in the survival of Wilms' tumor patients. Those with Stage IV, favorable histology Wilms' tumor now have a 2-year survival rate of 87% to 93%. Patients with recurrent Wilms' tumor are being prospectively treated with new drugs and drug combinations with the goal of identifying active agents for inclusion in future therapeutic trials. Girls with localized embryonal rhabdomyosarcoma of the vagina have a 100% survival rate after treatment on the Intergroup Rhabdomyosarcoma Study (IRS) (IRS-1 or IRS-2). Primary treatment of patients with tumors of the prostate, bladder neck, or trigone with chemotherapy has demonstrated the need to establish local tumor control using surgery or radiation soon after diagnosis. The IRS-III currently is evaluating new drug combinations in this group of patients with locally advanced tumors. Patients with advanced yolk sac tumor have been treated using platinum-containing combination chemotherapy regimens with very encouraging results. Future research will evaluate the timing of second-look surgical procedures and the efficacy of etoposide-containing combination chemotherapy regimens.
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PMID:The treatment of advanced or recurrent malignant genitourinary tumors in children. 303 35

A correlated cytologic and histologic study of seven cases of embryonal rhabdomyosarcoma is presented. The diagnosis of rhabdomyosarcoma was established by light and electron microscopy and immunohistochemistry of the operative specimens. The cytologic appearance of the smears corresponded well with the histopathologic findings. Cytologically, two main cell types were distinguished: a predominant primitive, small round cell with scant cytoplasm and a large cell with an abundant cytoplasm, sometimes tadpole- or ribbon-shaped. The tumor cells were often enclosed in a background of mucosubstances. The lack of cytologic features proving rhabdomyoblastic differentiation, such as cross-striation, necessitates the use of additional methods in the cytologic diagnosis of embryonal rhabdomyosarcoma. The value of the embedding technique for ultrastructural analysis and immunohistochemistry in the demonstration of desmin in aspirates is emphasized in the diagnosis of embryonal rhabdomyosarcoma.
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PMID:Cytology of embryonal rhabdomyosarcoma: a cytologic, light microscopic, electron microscopic, and immunohistochemical study of seven cases. 307 10

Rhabdomyosarcoma is a diagnostic and therapeutic problem in the management of childhood tumours. A case of embryonal rhabdomyosarcoma affecting the sphenoid sinus and involving the cavernous sinus is presented. It has become evident that modern combined modality therapy, including surgery when required, radiation and adjuvant multi-drug chemotherapy offers the best chance of survival.
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PMID:Embryonal rhabdomyosarcoma of the sphenoid sinus. 317 80


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