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At least 1,387 records · Page 77Linked to original sources
PubMed1977

Prognosis in children with metastatic rhabdomyosarcoma.

Distant metastases were documented in 83 of 161 children with rhabdomyosarcoma seen between 1953 and 1974. Of these, 31 had metastases at diagnosis (Group IV) and 7 were noted to have metastases only at autopsy. Only 6 are living with no evidence of disease activity at 6, 20, 77, 85, 105, and 112 months from time of metastasis. One child is living with disease at 98 months. Late deaths occurred in 2 at 31 and 41 months after metastasis. Sixty-four of the 66 deaths occurred within 24 months. The median durations of life after metastasis were less than 9 months in Groups I and II patients, less than 6 months in Group III patients, and about 12 months in 31 Group IV patients. The median time to metastasis in Groups I, II, and III patients was 9 months.

Adolescent↗
PubMed1978

Meningeal seeding from rhabdomyosarcoma of the head and neck with base of the skull invasion: recognition of the clinical evolution and suggestions for management.

Rhabdomyosarcomas of the head and neck may spread by diffuse local invasion, resulting in erosion of the base of the skull with possible extension to the leptomeninges, brain, and cranial nerves, as well as invasion of the ventricular system with possible intradural, extramedullary metastases anywhere along the neuraxis. Meningeal spread of tumor is difficult to eradicate, and nearly all patients reviewed died soon after meningeal disease was evident. Patients with erosion of the base of the skull or abnormal cranial nerve findings seem to have a risk of seeding the meninges with tumor. The clinical evolution of this pattern of metastatic spread is reviewed, diagnostic studies are recommended, and therapeutic considerations are discussed. Tentative treatment guidelines are also offered.

Ataxia↗
PubMed1979

Orbital rhabdomyosarcoma in a neonate.

A newborn with a pleomorphic rhabdomyosarcoma of the eyelid was treated by chemotherapy, surgery, and radiation therapy and experienced a good response. The nine previous cases in the literature are reviewed and this patient's therapy discussed.

Antineoplastic Agents↗
PubMed1985

Graft-versus-host disease in rhabdomyosarcoma following transfusion with nonirradiated blood products.

Graft-versus-host disease is a complication of transfusion of nonirradiated blood products into an immunocompromised patient, and it has been reported in hematologic and lymphoid malignancies as well as in neuroblastoma in children. We report the first case of acute graft-versus-host disease in a child undergoing chemotherapy for a rhabdomyosarcoma, who received transfusions during an intercurrent illness. The graft-versus-host disease was accompanied by an allogeneic lymphoid graft and was diagnosed on the basis of HLA studies on the patient's and her family's circulating lymphocytes and confirmed by HLA typing of the patient's skin fibroblasts.

Child↗
PubMed1988

Prognostic factors in 281 children with nonmetastatic rhabdomyosarcoma (RMS) at diagnosis.

Pretreatment characteristics of 281 children with nonmetastatic rhabdomyosarcoma, included in the registry of the International Society of Pediatric Oncology (SIOP) between January 1975 and December 1983, were examined to study the children's prognosis. The multivariate statistical method (Cox regression model) was used for each of two endpoints: survival time and disease-free time. The three most important predictors for survival time were primary site (p less than .001), clinical stage (p = .009), and sex (p = .020). The best results involved paratesticular and orbital primary sites, regardless of the clinical stage; males fared slightly better than females. These same three factors were also significant predictors for disease-free time.

Adolescent↗
PubMed1988

Rhabdomyosarcoma presenting with diffuse bone marrow involvement, hypercalcemia and renal failure.

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.

Adult↗
PubMed1989

Rhabdomyosarcoma in infants under one year of age: experience of the Institut Gustave-Roussy.

Forty-three infants under 1 year of age (median 4 months) with rhabdomyosarcoma (RMS), representing 11% of all childhood RMS seen over the same period, were treated and followed between 1955 and 1984 at our institute. Compared to the 340 older children (1-16 years of age), there were no significant differences in male/female ratio, primary site, or clinical stage, but there was a higher frequency of alveolar and poorly differentiated histologic subtypes in infants aged under 1 year (P = 0.05 and P less than 0.001, respectively) and increased frequency of botryoid RMS in older children (P less than 0.02). Although children older than 1 year of age had a significantly higher complete remission rate (83% vs. 51%, P less than 0.001), their 5 year overall survival (43%) was no different from that of infants under 1 year of age (35%). In the older group there was no survival difference between alveolar (38%) and nonalveolar (44%) histologic subtypes, but in infants younger than 1 year, alveolar subtype had a significantly poorer prognosis (13% vs. 47%, P = 0.01). Management of patients at this age group is difficult and major advances are needed to increase survival rates with a minimum of morbidity.

Adolescent↗
PubMed1990

Prognostic significance of regional lymph node involvement in childhood extremity rhabdomyosarcoma.

Thirty-four patients with extremity rhabdomyosarcoma were treated at Memorial Sloan-Kettering Cancer Center during the years 1970 to 1987. The regional lymph node status at diagnosis was histologically confirmed in 27 patients. Of these 27 patients, there were 20 males and 7 females, ranging in age from 6 months to 22 years (median 10 years). The primary tumor was located in the upper and lower extremity in 11 and 16 patients, respectively. The histologic subtypes included embryonal in 13 patients and alveolar in 14 patients. Fourteen patients had no evidence of regional lymph node involvement (N0) and 13 patients had lymphatic metastases (N1). All patients were further staged according to the extent of surgical resection, the local invasiveness and size of the primary tumor, and the presence (M1) or absence (M0) of distant metastases. With a median follow-up time from diagnosis of 9.2 years (minimum 5.3 years), the overall survival was 48% (13/27): N0, 12/14 and N1, 1/13 (P = .0002). Excluding those patients with M1 disease, the survival rate was: N0, 11/12; N1, 1/10 (P = .0001). The first site of relapse in the M0 patients was distant metastases in 8/10 cases. Our results suggest that regional lymph node involvement at diagnosis is an important prognostic factor in extremity RMS. Its presence appears to be associated with a high incidence of relapse in metastatic sites and poor survival outcome.

Adolescent↗
PubMed1993

Local failure in childhood rhabdomyosarcoma and undifferentiated sarcoma: prognostic factors and implications for curative therapy.

In this retrospective review, the risk factors for local failure in childhood rhabdomyosarcoma and undifferentiated sarcoma were assessed in 49 patients managed by a multi-disciplinary team at the Prince of Wales Children's Hospital, Sydney, between 1970 and 1988. Average follow-up time was 6.1 years. Sixteen of 49 patients experienced local failure defined as local recurrence after complete excision (5/20) or progressive local disease following incomplete resection (11/29). Fourteen of 16 patients who experienced local failure have died. Using logrank analysis of time to local failure, we found significantly increased risk with "non-embryonal" histology (P = 0.032), residual tumour (P = 0.052, higher IRS group (P = 0.088), "inadequate radiotherapy" for residual tumour (P = 0.001), delay in definitive local treatment (P = 0.038) and Adriamycin-containing chemotherapy (P = 0.017). When these factors were examined by multivariate analysis (Cox regression), only the presence of residual tumour after resection, "inadequate radiotherapy" for residual tumour (P < 0.001), and delay in definitive local therapy (P < 0.037) were shown to have independent significant association with local failure. We conclude that local failure may be avoided by prompt local treatment by either complete surgical resection or adequate radiotherapy.

Adolescent↗
PubMed1993

Intracerebral recurrence of primary intrathoracic rhabdomyosarcoma.

We present two cases of intracerebral recurrence of primary intrathoracic rhabdomyosarcoma, occurring within 2 months of each other. Both had embryonal histological features. One (case 1) almost certainly arose within the lung adjacent to a developmental cyst; the intrathoracic site of origin of the other (case 2) was difficult to ascertain. Both were treated on SIOP MMT protocols. The intracerebral deposits became clinically apparent soon after completing chemotherapy. They were removed surgically and second-line chemotherapy was given both systemically and intrathecally. Neither received radiotherapy. The disease recurred in both children whilst on therapy and they died as a consequence of raised intracranial pressure.

Brain Neoplasms↗
PubMed1994

Bone and gallium scintigraphy in children with rhabdomyosarcoma: a 10-year review.

Technetium-99m bone and Gallium-67 scintigraphy has been widely used in the management of children with solid tumors. A retrospective review of all patients with rhabdomyosarcoma (RMS) from January 1980 to December 1989 was undertaken in order to determine the sensitivity and specificity of scintigraphy in detection of metastatic disease and to determine optimum frequency of serial scans in follow up. Over the 10-year period, 40 patients were diagnosed and treated for RMS: 22 were newly diagnosed from 1980-1984 and of these 16 had a minimum 5-year disease-free period whilst 6 died of the disease, giving a cure rate of 73%. A total of 271 bone and 236 Gallium-67 scans were performed. With respect to detection of metastatic disease in all tissues, Gallium-67 scans had a sensitivity of 84%, specificity 95% and bone scans had a sensitivity of 70% and specificity 95%. Bone scan sensitivity and specificity for skeletal metastases were 100% and 95%, respectively. Considering only patients with Gallium avid primary tumors, the Gallium-67 scan sensitivity was 94%. Ten patients developed new metastatic disease or primary recurrence after starting therapy, 8/10 within 12 months of diagnosis. Frequent surveillance scanning in the treatment phase, 2 years post-diagnosis, is of value in the early detection of metastases and monitoring disease response to therapy. Surveillance scanning after completion of treatment is more difficult to justify.

Adolescent↗
PubMed1995

Veno-occlusive disease of the liver after chemotherapy for rhabdomyosarcoma: case report with a review of the literature.

We describe the case of a 3-year-old girl who developed veno-occlusive disease of the liver while receiving chemotherapy for parameningeal rhabdomyosarcoma. After suffering lethargy and oral mucosal bleeding for one day, the patient exhibited a sudden weight gain and refractoriness to platelet transfusions. Symptoms rapidly worsened with elevation of liver enzymes, bleeding diatheses, and respiratory failure. An ultrasound scan of the liver demonstrated reversed flow in the portal vein. Maximal supportive care, including tracheal intubation and mechanical ventilation, was required. The patient gradually recovered with no respiratory and minimal neurological sequelae. Veno-occlusive disease of the liver should be considered in children receiving chemotherapy who develop weight gain, a sudden drop in platelet count and derangement of liver enzymes. Aggressive supportive measures should be instituted if necessary, as patients surviving the acute phase can expect to make a full recovery.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2003

Molecular characterization of Patched-associated rhabdomyosarcoma.

Mutations in the human homologue of Drosophila Patched1 (PTCH1) have been found in several common tumours including basal cell carcinoma, medulloblastoma, and rhabdomyosarcoma (RMS). Medulloblastoma and RMS are also present in the murine model for Ptch1 deficiency. Tumours in heterozygous Ptch1(neo67/+) mice consistently exhibit elevated transcript levels of the proto-oncogene Gli1, of Ptch1 itself, and of the insulin-like growth factor 2 (Igf2). The present study has investigated additional molecular changes in RMSs of Ptch1 mutant mice by means of microarray analysis and protein expression analysis. The data show activation of the cell survival-promoting Akt/protein kinase B (Pkb). Furthermore, RMSs express increased levels of the anti-apoptotic protein Bcl-2 and of genes and proteins known to inhibit cell proliferation, including Gadd45a and p27kip1. Taken together, the data suggest that the formation of RMSs in Ptch1 mutants is associated with the ability of tumour cells to resist apoptosis.

Animals↗
PubMed1980

Primary rhabdomyosarcoma of the cerebellum.

A case of primary rhabdomyosarcoma of the cerebellum is reported. This is a very rare form of primary tumour in the brain and probably arises from the ectomesenchyme of the neural crest.

Cerebellar Neoplasms↗
PubMed1988

Immunohistochemical study of rhabdomyosarcoma. Unexpected staining with S100 protein and cytokeratin.

The immunohistochemical study of 60 cases of rhabdomyosarcomas made it possible to test eight different antibodies currently used in tumour pathology: i.e., antisera to vimentin, desmin, myoglobin, cytokeratin, epithelial membrane antigen, S100 protein, neurofilaments, and leukocyte common antigen. Vimentin was found in 58 cases (97 per cent), desmin in 49 cases (82 per cent), myoglobin in 23 cases (38 per cent), S100 protein in 7 cases (12 per cent), and cytokeratin in 3 cases (5 per cent). Other markers were negative. S100 protein was present in large round tumour cells with abundant eosinophilic cytoplasm (round rhabdomyoblasts), whereas cytokeratin was present in small tumour cells similar to those observed in rhabdoid sarcoma. This unexpected staining should become common knowledge for the correct interpretation of the immunohistochemical study of small cell tumours in the young.

Adolescent↗
PubMed1994

The expression pattern of contractile and intermediate filament proteins in developing skeletal muscle and rhabdomyosarcoma of childhood: diagnostic and prognostic utility.

In order to investigate whether rhabdomyosarcoma (RMS) can be related to equivalent stages of skeletal muscle development, muscle tissue of 21 human foetuses and 112 primary RMSs were characterized immunohistochemically using antibodies directed against vimentin, desmin, muscle-specific actin (HHF35), sarcomeric actin (sr-actin), smooth muscle actin (sm-actin), and troponin-T. During fetal skeletal muscle development, all myotubes/fibres of the first and second generations expressed desmin, HHF35, and sr-actin. Vimentin was almost exclusively present in immature primary and secondary myotubes/fibres. Troponin-T was expressed in immature myotubes/fibres of the first and second generations as well as mature fibres of the second generation. Sm-actin was never expressed. Vimentin was expressed in 96 per cent of primary and 98 per cent of relapsed RMS; HHF35 in 96 and 98 per cent, respectively; desmin in 95 and 100 per cent; troponin-T in 82 and 75 per cent; sr-actin in 71 and 86 per cent; and sm-actin in 13 and 17 per cent. The proportion of RMS cells reacting with vimentin, HHF35, and desmin was consistently higher than those expressing sr-actin and troponin-T. Neither the shape nor size of neoplastic RMS cells nor the histopathological types were related to the expression pattern of the investigated markers. RMS with aberrant expression of two or more markers predicted a worse prognosis than RMS in which at most one marker was aberrantly expressed (25 per cent and 54 per cent 10-year survival, P = 0.01). These results demonstrate that HHF35, desmin, sr-actin, and troponin-T have the potential to confirm the commitment of the tumours to the myogenic pathway which supports the diagnosis of RMS. However, it was impossible to relate RMS to equivalent stages of skeletal muscle development. Aberrant marker expression by RMS cells correlated significantly with patients' survival.

Actins↗