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)

A new retravirus (SMRV) isolated from a squirrel monkey, Saimiri sciureus, has an Mg2+-dependen reverse transcriptase and a buoyant density of 1.17 g/cm3 in sucrose and 1.21 g/cm3 in cesium chloride, similar to the mouse mammary tumor virus and the Mason-Pfizer monkey virus. The polypeptide patter of SMRV as determined by sodium dodecyl sulfate-polyacrylamide gel electrophoresis was distinct from the reported polypeptide patterns of known retraviruses. Four major polypeptides of molecular weights 40,000, 20,000, 14,000 and 8,000 were resolved in virus propagated in human, mink, and canine cells. In A204 human rhabdomyosarcoma cells, a protein of 73,000 daltons (gp73) represented the major viral glycoprotein as determined by [3H]glucosamine labeling. Additional proteins were also observed, but their presence depended on the cell type in which the virus was propagated. In both species-and interspecies-specific assays, no antigenic relatedness was observed between SMRV and Mason-Pfizer monkey virus, mouse mammary tumor virus, baboon endogenous virus (BaLV), woolly monkey virus (SSV-1), murine leukemia virus, endogenous feline type C virus (RD-114), bovine leukemia virus, and equine infectious anemia virus. These findings indicate that SMRV represents a new retravirus and the first isolate from a New World monkey.
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PMID:Characterization of a retravirus isolated from squirrel monkeys. 6 28

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

Twenty-one children were admitted to a single paediatric institution between 1964-1990 with histologically proven primary liver tumours. The diagnosis was hepatoblastoma (HBL) in 15 patients, hepatocellular carcinoma (HCA) in 2, rhabdomyosarcoma (RMS) in 2, non-Hodgkin's lymphoma (NHL) in 1, and haemangioendothelioma (HE) in 1. The common presenting clinical features were anaemia, abdominal mass, and abdominal pain. Serum alpha-foetoprotein was useful in establishing a diagnosis in HBL and in monitoring disease activity. Computed tomographic (CT) scan, ultrasound, and angiography were useful preoperative investigations for assessing site and resectability of tumour. There were no survivors in patients with malignant hepatic tumours (n = 10) who had surgery alone prior to 1981. Of 7 patients with HBL diagnosed after 1981 who had adequate surgical resection and chemotherapy, 5(72%) are currently alive and disease free between 15 months and 8 years from diagnosis. We conclude that adequate surgical resection and adjuvant chemotherapy can improve disease free survival for children with HBL. Optimal treatment has yet to be devised for other malignant hepatic tumours.
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PMID:Primary hepatic tumours in children: a 26-year review. 131 8

Carboplatin was administered at 1,000 mg/m2/course in combination with etoposide at 300 mg/m2/course to 23 patients aged 5 months to 16 years. Five patients were affected by neuroblastoma, four by CNS tumors, three by Ewing's sarcoma, two by rhabdomyosarcoma, two by malignant teratoma, two by Wilms' tumor, two by head and neck carcinoma, one by hepatoblastoma, one by synovial sarcoma, and one by Langerhans-cell histiocytosis. Eleven patients were pretreated, seven of them with high-dose cisplatin. The overall response rate was 7/11 (64%) for pretreated and 10/12 (83%) for previously untreated patients. Myelosuppression was the main side effect, with anemia and thrombocytopenia more pronounced than leukopenia. Gastrointestinal toxicity and ototoxicity were very mild; nephrotoxicity and neurotoxicity other than hearing loss were not observed. In children with malignant tumors, the therapeutic activity of carboplatin at high doses, even in combination chemotherapy, deserves further studies.
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PMID:A pilot study of high-dose carboplatin and pulsed etoposide in the treatment of childhood solid tumors. 220 54

Pericardial effusion (P.E.) has been described during the course of malignant neoplasms and leukemia, but in children its frequency is not well-known. It is possible by echocardiography to identify very small P.E. M-mode echocardiograms were performed for periodic evaluation of the patients treated by antraciclinic drugs and when P.E. was suspected by the physical examination, chest roentgenogram and electrocardiographic data. A total of 265 echocardiograms were performed in 210 children with malignant neoplasms and leukemia from 1981 to 1985 and 12 cases (5.7%) of P.E. were identified: 7 affected by acute lymphatic leukemia (ALL), 3 Rhabdomyosarcoma, 1 by Wilm's tumor and 1 by Ewing's sarcoma. In all patients was carried out and Ecg, and a chest roentgenogram was performed to evaluate cardiac shadow. Hemoglobin, serum protein and VES values were evaluated to control, respectively, anemia, dysprotidemia and flogistic condition. Pericardiocentesis was played when clinical findings indeed. P.E. was identified in 6 out all cases, 4 affected by ALL and 2 by Rhabdomyosarcoma, during the first clinical approach. Two cases, both affected by ALL, showed large P.E., x-ray enlarged cardiac shadow and typical electrocardiographic findings. The disappearance of the P.E., resulted few days after the beginnings of antiblastic therapy. Four cases were in maintenance therapy: 2 affected by ALL, 1 Ewing's sarcoma and 1 by Rhabdomyosarcoma. The last two showed very large P.E. and pericardiocentesis was necessary: only in patient with rhabdomyosarcoma fluid containing malignant cells was yielded, and reproducing itself one day after.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Pericardial effusion in neoplasms and malignant hemopathies in children. Study of 12 cases and pathogenetic considerations]. 282 17

A 20-year-old white male was initially suspected clinically and pathologically of having an acute lymphoblastic leukemic process because of fatigue, severe anemia, thrombocytopenia, a leuko-erythroblastic peripheral blood picture, and a diffusely infiltrated bone marrow. Subsequent review of the bone marrow material indicated cytologic features consistent with either an embryonal, undifferentiated small cell mesenchymal malignancy or reticulo-endothelial malignancy. Ultimately, the electron microscopic (EM) study of the tumor proved to be diagnostic of rhabdomyosarcoma. An extensive search for a primary site of rhabdomyosarcoma did not show any lesion, although the genitourinary region was clinically suspected. The clinical course was a rapidly downhill one with extensive bone and CNS involvement. The patient died 5 months later. An autopsy permit was not obtained. This case emphasizes the occasional tendency of rhabdomyosarcoma to masquerade as a hematopoietic malignancy at the time of presentation and the usefulness of EM study in confirming a diagnosis.
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PMID:Systemic rhabdomyosarcoma presenting as leukemia: case report with ultrastructural study and reviews. 330 41

Rhabdomyosarcoma and other small round-cell neoplasms of infancy and childhood frequently involve the bone marrow. An unusual clinical presentation of diffuse bone marrow involvement as the sole manifestation of the disease represents a difficult diagnostic challenge. We report the case of an 18-year-old boy presenting with lower back pain, sternum tenderness, anemia, thrombocytopenia, leukoerythroblastic blood film, hypercalcemia, and renal failure. No solid tumor was found. A diagnosis of rhabdomyoblastic bone marrow metastatic involvement was initially suggested by the morphological picture, and later confirmed by the ultrastructural findings. Hypercalcemia and renal failure were controlled, initially, with furosemide and calcitonin. The patient was treated with intensive chemotherapy, including vincristine, actinomycin D, cyclophosphamide, and doxorubicin. A remission was achieved, lasting 5 months until relapse.
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PMID:Rhabdomyosarcoma presenting with diffuse bone marrow involvement, hypercalcemia and renal failure. 335 35

Fifteen patients with soft-part sarcoma were treated with combination chemotherapy consisting of vincristin, actinomycin D, cyclophosphamide and adriamycin (VACA therapy). The cumulative five-year survival rate by the Kaplan-Meier method was about 73%. This VACA therapy was effective for malignant fibrous histiocytoma and synovial sarcoma as well as rhabdomyosarcoma. Side effects such as anemia, leucocytopenia, nausea and alopecia were observed, but could be managed. VACA therapy is considered to be useful as a combination chemotherapy for patients with soft-part sarcoma.
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PMID:[Combination chemotherapy with vincristin, actinomycin D, cyclophosphamide and adriamycin in soft-part sarcoma]. 374 64

Two children with rhabdomyosarcoma developed severe anemia following chemotherapy; anemia was more severe compared to that observed following earlier chemotherapy cycles. While one patient had a brisk reticulocytosis, the other had no demonstrable reticulocytes. Both patients had evidence of acute B19 parovirus infection and subsequently developed appropriate antibody response. A diagnosis of B19 parvovirus infection should be considered in any patient who develops persistent or severe anemia while on chemotherapy.
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PMID:B19 parvovirus infection in children with malignant solid tumors receiving chemotherapy. 810 56

Thirty patients were examined initially because of neurologic problems and later were diagnosed as having systemic malignant disorders. Acute leukemia was the most common malignancy (36.6%), followed by neuroblastoma (33.3%), non-Hodgkin lymphoma (13.3%), rhabdomyosarcoma (10%), Ewing tumor (3.3%), and Hodgkin lymphoma (3.3%). Four of the 11 acute leukemia patients had nervous system involvement due to meningeal, orbital, or cerebellar infiltration. The complaints of the remaining patients included back pain, weakness, and difficulty in walking, all of which were caused by anemia or bone pain. Neurologic involvement in systemic malignancies, other than acute leukemia, mainly appeared as spinal cord compression (7 with neuroblastoma, 3 non-Hodgkin lymphoma, 1 rhabdomyosarcoma, 1 Ewing tumor), orbital or cavernous sinus infiltration (3 with acute leukemia, 1 rhabdomyosarcoma), and VIIth cranial nerve involvement (2 with rhabdomyosarcoma). One patient had skull infiltration without any neurologic deficit. Cerebellar signs were caused by the remote effects of cancer. It is concluded that acute leukemia is the first and neuroblastoma is the second most common malignancy among childhood systemic malignancies presenting with neurologic involvement; however, neuroblastoma is the most common cause of spinal cord compression.
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PMID:Neurologic features as initial presentations of childhood malignancies. 819 71


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