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PubMedDate not supplied

Imaging of rhabdomyosarcomas of the head and neck.

Rhabdomyosarcoma (RMS) is the most common childhood malignancy of the head and neck. The Intergroup Rhabdomyosarcoma Study now divides head and neck RMS into three categories by site of origin: orbital, parameningeal (middle ear, paranasal sinuses, and nasopharynx), and all other head and neck sites. CT is clinically applicable in the diagnosis of RMS of the head and neck, in treatment planning, and in the follow-up of patients with these tumors. Specific areas of applicability include determination of the presence/absence of intracranial and meningeal involvement, definition of tumor extent to guide radiation therapy planning, and demonstration of tumor regression or recurrence during and after treatment. CT has played an important role in the dramatically improved prognosis seen in RMS over the last 10 years. The role of MR in evaluating these patients is not yet defined, but it has promise because of the ease of obtaining multiple projections and the avoidance of ionizing radiation.

Adolescent↗
PubMed1988

Primary cardiac rhabdomyosarcoma: definition of a rare entity.

Primary cardiac rhabdomyosarcoma (PCR) is a highly malignant tumor that is rarely recognized prior to surgery or necropsy. We present the pathologic findings of a primary atrial rhabdomyosarcoma in an 82-year-old woman and an update on PCR as a clinicopathologic entity. In addition to pleomorphic tumor cells with cross-striations and a positive immunoperoxidase reaction for myoglobin, some areas of our patient's tumor had features of sarcoma botryoides. Electron optic studies showed primitive cells with fibroblastic and/or myoblastic differentiation. A comprehensive study of 77 cases in the international literature yielded new information and perspectives on PCR. The incidence of PCR is bimodal among males, being highest in infancy and early childhood with a secondary peak in the sixth and seventh decades. Females, however, have a single peak incidence in the fifth decade. Septal origin is 35.2% among infants and children but only 5.2% among adults. The predilection for the pediatric age group in conjunction with common septal origin among these patients suggests a congenital form of PCR that may arise from embryonic cell tests.

Aged↗
PubMed1988

[Subcutaneous rhabdomyosarcoma in children. Clinical, immunologic and ultrastructural aspects].

Embryonal rhabdomyosarcoma is the most frequent of tissue sarcomas in children. Its location in the subcutaneous tissue makes it a dermatological diagnosis. In childhood, the exact histological type of the tumour, sometimes difficult to determine, is absolutely necessary since prognosis and treatment differ according to the histogenetic form. Rhabdomyosarcoma is rare in adults. One must rule out malignant pleomorphic histiocytoma which has a more favourable prognosis. Among the antisera recently made available, those directed against desmin, foetal skeletal myosin and/or specific skeletal muscle myofilament seem to be most useful when associated with the anti-myoglobin antibody.

Child↗
PubMedDate not supplied

Fine needle aspiration biopsy diagnosis of rhabdomyosarcoma. An immunocytochemical study.

Specific antibodies against desmin, skeletal muscle actin and myosin were assessed for their usefulness in the cytodiagnosis of five rhabdomyosarcomas: one well-differentiated, two moderately differentiated and two poorly differentiated lesions. Acetone-fixed smears from fine needle aspiration biopsies and the avidin-biotinyl-peroxidase complex technique were used. All aspirates were positively immunostained with antibodies against desmin and actin. Myosin could only be detected in the moderately and well-differentiated tumors. The percentage of tumor cells positive for any of the three proteins was positively correlated with the overall degree of differentiation. However, the number of positive tumor cells decreased in the sequence desmin-actin-myosin. The results indicate the value of antibodies, especially those against skeletal muscle actin, in aiding in the cytodiagnosis of rhabdomyosarcoma, particularly with respect to its differential diagnosis from small round cell tumors in children and pleomorphic sarcomas in adults.

Actins↗
PubMed1988

[Multiple primary malignant tumors of rhabdomyosarcoma in an upper arm and breast cancer].

Multiple primary malignant tumors of rhabdomyosarcoma in a upper arm and cancer of breast are extremely rare. In September 1970, a female, aged 86 years, underwent the first resection operation performed on a pleomorphic rhabdomyosarcoma of the right upper arm. She underwent radiation therapy (8000 rads at the operative field) postoperatively. In August 1980, a simple mastectomy was performed for a cancer of the right breast. In December 1984, fourteen years after her first operation, a recurrence was detected. The tumor recurred five times during the next two years and was resected each time. The patient died of cachexia sixteen years after the first operation.

Aged↗
PubMed1988

[Capacity of rhabdomyosarcoma cells to produce substances with somatomedin and mitogenic activity].

The rats aged 1, 3, 12 and 15 months were intravenously injected with 10,000 of the rhabdomyosarcoma cells. 3 weeks following the injection multiple tumour clones were observed in the lungs of experimental animals, the rats aged 1 and 15 months showing a significantly greater tumour yield than those aged 3 and 12 months. The level of the mitogenic and somatomedin activity increased both in the blood serum and in the lung tissue, the activity varying with the number of tumour clones. Rhabdomyosarcoma cells, probably, synthesize and release growth factors into the blood.

Animals↗
PubMedDate not supplied

Diagnosis of a case of pulmonary carcinosarcoma by detection of rhabdomyosarcoma cells in sputum.

Cytologic examination of sputum samples from an elderly patient revealed the presence of two cell populations: squamous cell carcinoma cells and rhabdomyosarcoma cells. The abnormal squamous cells showed both keratinizing and nonkeratinizing forms while some of the rhabdomyosarcoma cells showed cross striations. Sputum cytology was thus able to suggest a diagnosis of pulmonary carcinosarcoma. Histologically, the tumor was composed mainly of sarcomatous tissue showing various kinds of cells: fusiform or fibrous cells, round anaplastic cells, spindled cells with typical cross striations and myoblastic cells. A partially myxomatous degeneration was present. In addition, squamous cell carcinoma proliferated along the bronchi and formed small invasive cell nests in the sarcomatous tissue. No transition between the two components was noted. Both cellular constituents had metastasized to an interlobar lymph node.

Aged↗
PubMed1986

Immunohistochemical studies of rhabdomyosarcoma.

We performed immunoperoxidase studies in 29 cases of rhabdomyosarcoma from the Intergroup Rhabdomyosarcoma Study I using antisera against actin, myosin, myoglobin, alpha-actinin, and tropomyosin. Although each of these antisera reacted with some of the tumors, none reacted with all of the tumors, and some tumors showed no reactivity. Antimyosin reacted with more tumors than any of the others, while antiactin and antimyoglobin were about equally sensitive. Antitropomyosin and anti-alpha-actinin reacted with few of the tumors. The better-differentiated tumors were more likely to react compared with the poorly differentiated tumors.

Actins↗
PubMed1986

[An immunohistochemical study of myoglobin and S-100 protein in rhabdomyosarcoma].

Twenty seven cases of rhabdomyosarcoma from pathologic files of the Hokkaido University Hospital from 1975 to 1985 were reviewed and the presence of myoglobin and S-100 protein was investigated immunohistochemically. Their average age at diagnosis was 30.7 years (ranging from two months to 77 years) and there was a predilection for males in a ratio of 1.7:1. The common locations were the head and neck. Nine cases were positively stained for myoglobin, mainly in the cytoplasm of rhabdomyoblasts and 19 cases were positive for S-100 protein and alpha-subunit of S-100 protein in the cytoplasm of immature tumor cells as well as of rhabdomyoblasts. In seven cases, both of myoglobin and S-100 protein were demonstrated. Although S-100 protein is not strictly specific, however, these results suggest that the immunohistochemical demonstration of S-100 protein can be a useful diagnostic tool of rhabdomyosarcoma.

Adolescent↗
PubMed1987

Childhood rhabdomyosarcoma in Hungary.

56 children with rhabdomyosarcoma were treated in Hungary between 1975 and 1984. Tumor localization, age and sex distribution was similar to reported figures. Survival analysis demonstrated a better prognosis for orbital and urogenital rhabdomyosarcoma. Except for Stage I patients the more advanced cases had an inferior survival to other reported series. Intensification of therapy did not seem to clarify this point. Improving survival necessitates a uniform therapeutic approach that takes prognostic factors into consideration.

Child↗
PubMed1987

Orbital rhabdomyosarcoma in childhood.

Rhabdomyosarcoma is a diagnostic and therapeutic problem in the management of pediatric tumors, representing the most common malignant orbital tumor of childhood. In this discussion of the diagnosis of rhabdomyosarcoma, the role of CT is detailed through the case reports and the radiographs presented. CT can accurately define the anatomic location of the orbital mass, the involvement of various intraorbital structures, and the extension of the pathologic process into the periorbital regions such as sinuses, nasal cavity, and intracranial compartment. Thin-needle aspiration biopsies can be done under CT guidance without complications as well as follow-up monitoring of tumor regression following therapy.

Adult↗
PubMed1987

Rhabdomyosarcoma in children. Correlation of form and prognosis in one institution's experience.

All cases of rhabdomyosarcoma and undifferentiated sarcoma accessioned at Texas Children's Hospital since 1954 were reviewed. Electron microscopy and immunohistochemistry were used as aids to diagnosis. Rhabdomyosarcoma was diagnosed by study of biopsy specimens obtained prior to therapy in 63 of these 105 cases. Clinical information extending for at least 1 year was available in 47 cases; these cases were used for correlative study. Cellular anaplasia--defined as nuclear enlargement, hyperchromasia, and abnormal mitoses--was noted in 14 cases; 12 of these 14 died an average of 1.53 years after diagnosis. Only 10 of the 33 remaining patients died, including only one of eight with well-differentiated tumors. The mean duration of follow-up for survivors was 4.8 years. By chi-square analysis, the difference in survival between the groups with and without anaplasia was highly significant (p = 0.0009). The relative risk of death within 5 years was 2.48 for the group with anaplasia. Patients with well-differentiated tumors were significantly more likely to survive (p = 0.03). In this study, cellular anaplasia correlated strongly with unfavorable prognosis.

Adolescent↗
PubMed1987

[A case of intrascrotal rhabdomyosarcoma].

We report a case of intrascrotal rhabdomyosarcoma. A 15-year-old boy visited our clinic with the chief complaint of swelling of the right hemiscrotum. He had a history of fever and right hemiscrotal pain. With the initial diagnosis of acute epididymitis, the patient underwent medical therapy with antibiotics. One month later, because the mass had not responded to medical therapy, a right inguinal orchiectomy was done. Histologic examination revealed rhabdomyosarcoma. The patient received retroperitoneal lymph node dissection and combined chemotherapy with cyclophosphamide, bleomycin, actinomycin-D, adriamycin and vinblastine. He was well 5 years and 10 months postoperatively with no evidence of tumor recurrence or metastasis.

Combined Modality Therapy↗
PubMed1986

Paratesticular rhabdomyosarcoma. Case reports.

During the 30-year period 1955-1984, 9 cases of paratesticular rhabdomyosarcoma were seen in the pathology departments of the University of Cape Town, including the Red Cross War Memorial Children's Hospital. Three of these patients were lost to follow-up; 2 were alive at 6 months and 6 years after presentation. Two patients absconded with clinical disease and are presumed dead and 2 died of disease 6 and 18 months after presentation. Although rhabdomyosarcoma is known to be a disease of the young, none of the patients in this series was in the first decade of life and only 3 were below 20 years of age.

Adolescent↗
PubMed1986

Methotrexate as relapse therapy for rhabdomyosarcoma.

Four patients had a local relapse after standard therapy for rhabdomyosarcoma and were treated with high-dose 42-hour MTX infusions. All patients responded to this therapy, one patient had a complete, and two patients a partial remission. Long duration MTX infusion should be part of a combination chemotherapy for relapsed rhabdomyosarcomas.

Child↗
PubMed1986

[Rhabdomyosarcoma of the prostate].

We report a case of rhabdomyosarcoma of the prostate. The patient was a 56-year-old man who complained of anal pain and dysuria. Tumor of the prostate was suspected after rectal examination. Multiple metastatic lesions were found in the lungs and liver. A needle biopsy of the prostate revealed rhabdomyosarcoma. He received chemotherapy, using Etoposide and responded slightly. Subsequently VAC-therapy was also performed. Although the patient improved temporarily, he died 4 months after admission.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1985

Desmin is a specific marker for rhabdomyosarcomas of human and rat origin.

Putative human rhabdomyosarcoma (RMS) has been divided into two groups according to desmin content. Twenty-five tumors with histologic features consistent with but not necessarily sufficient to prove a diagnosis of RMS were desmin-positive. More than 95% of the tumor cells were desmin-positive, suggesting a muscle origin and supporting the diagnosis of RMS. Nine tumors for which the preferred first histologic diagnosis was also RMS were desmin-negative. Reexamination of the original histologic slides together with results from intermediate filament typing resulted in a diagnosis other than RMS for all tumors in this second group, and in several instances other tests were used to prove the correctness of the final diagnosis. The results on human material were extended to a rat model system in which RMS was induced by nickel sulfide. Again, all 24 tumors tested were desmin-positive. Vimentin was coexpressed in a varying percentage of tumor cells in RMS of human and rat origin. The results show that desmin is an excellent marker for rhabdomyosarcoma, yielding few if any false-positive or false-negative results in frozen or alcohol-fixed material.

Adolescent↗
PubMed1985

[An autopsy case of alveolar rhabdomyosarcoma of the prostate].

A 48-year-old man who was diagnosed as undifferentiated malignant tumor by needle biopsy was treated with irradiation and anticancer drugs. Autopsy revealed alveolar rhabdomyosarcoma of the prostate with marked infiltration into periprostatic tissue and distant metastasis. Histological diagnosis was performed by special staining as well as the immunoperoxidase technique with myoglobin. This was the first case of alveolar rhabdomyosarcoma of the prostate in Japan.

Humans↗