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

The paratesticular region was the primary site in 20 of 289 children (7%) entered on the Intergroup Rhabdomyosarcoma Study. The 20 patients were 1.7- to 19-years-old at diagnosis. Fifteen underwent retroperitoneal node dissection (12) or biopsy (3), and 6/15 (40%) had nodal involvement by tumor. Nineteen of the 20 patients had no gross local or metastatic disease after surgery. All 20 received chemotherapy, and 13 also received radiotherapy. Treatment was effective: 16 of 18 evaluable patients (89%) were free of disease at a median of 23 months from diagnosis (range, 8-43 months). Since the incidence of tumor-involved retroperitoneal nodes is high, a dissection should be performed. If the nodes are free of tumor, retroperitoneal radiotherapy may not be necessary. Reduction of morbidity in patients with paratesticular rhabdomyosarcoma is desirable, because the disease has a good prognosis.
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PMID:Paratesticular rhabdomyosarcoma in childhood. 35 76

Anatomic and electrocardiographic correlations in a case of primary cardiac rhabdomyosarcoma are examined. Interatrial and atrioventricular conduction disturbances were associated with multiple lesions involving the alleged internodal pathways, together with atrial-atrioventricular nodal connections. The clinicopathologic findings seem to be consistent with the hypotheses of the functional and morphologic value of Bachmann's fascicle, and of the inherent pathways of interatrial and internodal conduction in health and disease.
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PMID:Multiple lesions of the conduction system in a case of cardiac rhabdomyosarcoma with complex arrhythmias. An anatomic and clinical study. 95 64

A case of desseminated alveolar rhabdomyosarcoma in an 18-year-old male with leuco-erythroblastic anaemia is described. Numerous bizarre malignant cells, including frequent multinucleated giant cells, were seen in bone marrow aspirates, and osteolytic lesions appeared late in the clinical course. The primary site of the neoplasm remained undertermined during life and also at necropsy, which revealed minute pulmonary metastases and extensive lymph nodal, pleural and skeletal deposits. The diagnosis was confirmed on necropsy tissue by ultrastructural examination which demonstrated numerous thin (5 nm) and thick (15 nm) intracytoplasmic filaments in tumour cells, sometimes organized in bundles; scattered dense Z-band-like bodies, and rod-shaped structures were also seen. The fine structure of the rhabdomyosarcoma in the present case is compared with previous ultrastructural studies. Elongated, thick intracytoplasmic filaments whose diameter corresponds to that of myosin myofilaments are strong evidence for rhabdomyoblastic differentiation and are considered to be the sine qua non of a positive electron microscopic diagnosis of rhabdomyosarcoma. Orgaized bundles of filaments and Z-band-like dense bodies are usually present, and rod-shaped structures are found infrequently, but none of these are necessary for the ultrastructural diagnosis.
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PMID:Bone marrow metastases in disseminated alveolar rhabdomyosarcoma: case report with ultrastructural study and review. 101 51

The pattern of malignancies among indigenous Zambian children is described. The study, based upon an analysis of histopathology, autopsy and haematology records for a 10-year period (1980-1989), reveals a total of 525 neoplasms with a peak prevalence in the 5-9 year age group. Non-Hodgkin's lymphoma (17.5%) was the most common disorder followed by Burkitt's lymphoma (13.9%), retinoblastoma (11.4%), Kaposi's sarcoma (8.8%), Hodgkin's disease (5.9%), Wilms' tumour (5.9%), acute lymphocytic leukaemia (4%), rhabdomyosarcoma (3.4%), nasopharyngeal carcinoma (2.7%) and osteogenic sarcoma (2.1%). Kaposi's sarcoma and Hodgkin's disease revealed a significant male dominance; the former presented mainly in nodal form. Compared to an earlier report from Zambia (1968-1972), a significant increase in the incidence of Kaposi's sarcoma and nasopharyngeal carcinoma was noted in the present series.
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PMID:The pattern of paediatric malignancy in Zambia (1980-1989): a hospital-based histopathological study. 156 Apr 80

We have reported a case of alveolar rhabdomyosarcoma with mediastinal involvement occurring in a 15-year-old boy. Computerized tomography revealed an anterior mediastinal mass with internal mammary nodal involvement, which had metastasized from a tumor of the abdominal wall musculature. When making the differential diagnosis of a mediastinal mass with concurrent distant soft tissue and muscle involvement in an adolescent, rhabdomyosarcoma should be considered.
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PMID:Mediastinal involvement by alveolar rhabdomyosarcoma: evaluation with computerized tomography. 206 43

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

From 1970 to 1987, 34 patients younger than 22 years of age with extremity rhabdomyosarcoma were treated at the Memorial Sloan-Kettering Cancer Center (MSKCC). All patients were treated according to protocols consisting of surgery, radiotherapy, and multiple drug chemotherapy. Sixteen of 34 patients survived, and 14 continue to be disease-free; the 5-year survival rate was 44%. A retrospective univariate analysis of data according to the TNM staging system was undertaken. Tumor invasion, regional nodal involvement, distant metastases, and alveolar histologic condition each had a significant impact on survival. However, in multivariate analysis, the stage of disease at diagnosis was the most important predictor of survival outcome.
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PMID:Prognosis in childhood rhabdomyosarcoma of the extremity. 280 12

Lymph node metastases occur in an appreciable number of soft-tissue sarcomas. The histologic subtype plays a most important role. Synovial sarcoma, malignant fibrous histiocytoma, rhabdomyosarcoma, and angiosarcoma manifest relatively frequently such metastases, whereas fibrosarcoma, liposarcoma, and malignant schwannoma do so very infrequently. With the use of the newer radiologic techniques, it is expected that this feature will be more clearly defined and taken into account in planning the treatment. For histologic subtypes known frequently to manifest metastases to the regional lymph nodes, the latter should be considered for inclusion in the surgical and/or radiation treatment plan, particularly for primary tumors situated close to a nodal basin; and careful follow-up of these nodes should be practiced.
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PMID:Incidence of metastases to lymph nodes from soft-tissue sarcomas. 283 23

Although rhabdomyosarcoma of the genitourinary tract occurs infrequently in children, it presents a challenging problem to the surgeon, radiotherapist, and oncologist. Thirty-six children with genitourinary rhabdomyosarcoma were treated at our institutions from 1957 to 1985. The primary site of tumor involved the bladder, prostate, or both in 15 patients, the paratesticular structures in 12, and female reproductive organs in nine. Although treatment modalities and philosophies of care varied over the past 27 years, the majority of patients were treated with a combination of surgery, chemotherapy, and radiation therapy. The event-free survival for all patients is 74%. Patients with paratesticular tumors had the best prognosis (88% event-free survival). Regional nodal radiation therapy is not necessary in this favorable group if the retroperitoneal lymph node dissection is negative. Of 15 children with bladder/prostate tumors nine had initial conservative surgery. Of these nine six are long-term survivors, but only three patients have intact bladders. The girls with tumors of the reproductive organs had an event-free survival of 63%; all patients in this group had hysterectomies. Chemotherapy and radiotherapy have not substituted for radical surgical procedures in most children with bladder or prostate rhabdomyosarcomas. Innovative therapeutic approaches are necessary for this group of patients.
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PMID:Genitourinary rhabdomyosarcoma in children. 292 67

Modern multidisciplinary treatment of childhood cancer has made extent of disease evaluation important for proper treatment planning. Accurate staging is essential to cooperative group studies and for comparing treatment modalities at different centers. Operative staging plays an important role where clinical or imaging methods are limited, as in abdominal Hodgkin's disease or regional nodal metastasis. Operative staging is carried out either as a special diagnostic procedure, as in lymphoma, or as part of a planned surgical resection of a solid tumor. For lymphomas: Operative staging of abdominal Hodgkin's disease is required where protocols include involved field irradiation and sparing of normal growing tissue in the child. In non-Hodgkin's lymphoma, bulky abdominal tumor may be surgically evaluated after intensive chemotherapy either in delayed primary surgery or in second look procedures. Residual tumor may be excised or tagged with clips for localized irradiation to the tumor sparing normal abdominal organs. For solid tumors: During surgical resection of neuroblastoma, Wilms' tumor and rhabdomyosarcoma, the correct procedure involves regional staging either by formal node dissections or by multiple biopsies to determine extent of spread. Regional node dissections are often part of a correct cancer operation for cure, but also give staging information unobtainable by other methods. The surgeon must plan every procedure carefully with the aim of curing the patient and also deriving maximum information from the operation to enable correct planning of further treatment.
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PMID:The objectives and importance of operative staging of children with cancer. 301 92


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