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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Survival with embryonal rhabdomyosarcoma of all sites has improved dramatically in recent years with the increased use of long-term, cyclic, multidrug chemotherapy. Protocols have been established and are currently being evaluated by the Intergroup Rhabdomyosarcoma Study. The management of embryonal rhabdomyosarcoma of the pelvic viscera, though, remains troublesome. Limited surgical excision is rarely possible and high-dose radiotherapy to the bony pelvis may cause severe and disabling growth disorders. Yet, survival with these lesions is increasing as with rhabdomyosarcoma from all sites and is directly related to a well-planned and aggressive multidisciplinary program. We have seen 12 cases of pelvic rhabdomyosarcoma within the last seventeen years at this institution. These cases will be reviewed in regard to varying modes of therapy and survival. Our current therapeutic approach, based on national and local experience, will be presented.
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PMID:Changing concepts in management of pelvic rhabdomyosarcoma in children. 45 95

Malignant lesions of the bladder in infancy and early childhood arise from mesenchymal tissue. Among the mesenchymal tumors embryonal rhabdomyosarcoma is more common in young children than in adults. The gross appearance of rhabdomyosarcoma of the bladder is typically that of polypoid grapelike clusters of tissue, termed "sarcoma botryoides". Histologically, the lesion is consistent with embryonal rhabdomyosarcoma. The growths originate in the submucosal layer of the trigone and the internal urethral orifice. Rhabdomyosarcoma (RMS) of the bladder in children tends to remain within the disease. Deep extension and metastasis to regional lymph nodes and to distant sites is a relatively late occurrence. The symptoms are characteristically those of obstruction and strangury. Hematuris is uncommon. The intravenous urogram shows characteristic lobulated filling defects. Treatment of choice is almost always total cystectomy. Suprapubic cystotomy must be prohibited both as a method of drainage and as an approach for biopsy since tumors ulcerating through the abdominal wall after cystotomy and local excision have been observed. With concomitant administration of radiation therapy in selected cases and the combination of surgery with multiple drug chemotherapy, promising results have been obtained in the therapy of embryonal RMS of the bladder in children.
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PMID:[Embryonal rhabdomyosarcoma of the urinary bladder in childhood. Report of five own cases]. 97 79

Of 30 children treated for embryonal rhabdomyosarcoma the primary site of tumor was the bladder in 14 cases (9 boys and 5 girls) and the prostate in 16 cases. The mean age at diagnosis was 3 years in patients with bladder sarcoma and 6.5 years in patients with sarcoma of the prostate. Over-all survival rate has been 23 per cent--5 children with rhabdomyosarcoma of the bladder and 2 with rhadbomyosarcoma of the porstate are alive from 1 1/2 to 23 years postoperatively. Aggressive coordinated treatment with surgery, radiotherapy and cyclic combination chemotherapy is recommended.
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PMID:Embryonal rhabdomyosarcoma of the bladder and prostate in childhood. 112 15

A full-term black boy had a 2- to 3-cm, round, bluish mass on his right lower eye-lid at birth, later diagnosed as rhabdomyosarcoma. It was cystic in nature and extended into the nasal cavity. The tumor was initially classified as neuroblastoma. The child died eitht months later and necropsy report confirmed an original ophthalmologic pathology diagnosis of embryonal rhabdomyosarcoma.
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PMID:Rhabdomyosarcoma of the orbit in the newborn. 120 95

Comparison of the clinical and pathologic features of two orbital leiomyomas, two leiomysarcomas, and one embryonal rhabdomyosarcoma showed the leiomyomas occurred in young individuals and the leiomyosarcomas in older patients. The histopathologic diagnosis rested on the intense cytoplasmic eosinophilia and nostriated longitudinal cytoplasmic filaments demonstrated by means of the trichrome stain. The leiomyosarcomas disseminated 15 months and seven years after their orbital presentations. The treatment of both leiomyoma and leiomyosarcoma is surgical. Leiomyomas are encapsulated growths that may have small satellite nodules projecting from the main tumor mass; thus, a margin of normal tissue should also be excised, lest a small lobulation be left behind to serve as the seed for a late recurrence. Once the diagnosis of leiomyosarcoma has been made, and no evidence of metastasis has been found after a thorough systemic evaluation, the orbit should be exenterated, because the tumor is unencapsulated and liable to widespread dissemination. Rhabdomyosarcoma has a much more fulminant course than leiomyosarcoma, and especially more so than that of leiomyoma of childhood. The histopathologic diagnosis of a malignant smooth muscle tumor in a child should always be questioned, since embryonal rhabdomyosarcoma is a much more likely diagnosis.
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PMID:Leiomyoma and leiomyosarcoma of the orbit. 120 96

Pure rhabdomyosarcomas originate in the female genital tract. They are uncommon and most often occur in infancy or childhood as sarcoma botryoides (embryonal rhabdomyosarcoma) which involve the vagina and cervix. Such tumors rarely occur in adults. A pure rhabdomyosarcoma of the uterus that arose in a postpartum patient is described. The pertinent literature is discussed.
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PMID:Pure rhabdomyosarcoma of the corpus uteri in a postpartum patient: report of a case and review of the literature. 132 92

The identification of acquired homozygosity in human cancers implies locations of tumor suppressor genes without providing functional evidence. The localization of a defect in embryonal rhabdomyosarcomas to chromosomal region 11p15 provides one such example. In this report, we show that transfer of a normal human chromosome 11 into an embryonal rhabdomyosarcoma cell line elicited a dramatic loss of the proliferative capacity of the transferrants. Indeed, the majority of the viable microcell hybrids had either eliminated genetic information on the short arm of the transferred chromosome 11 or increased the copy number of the rhabdomyosarcoma-derived chromosomes 11. Cells that possessed only the long arm of chromosome 11 also demonstrated a decreased growth rate. In contrast, all microcell hybrids retained the ability to form tumors upon inoculation into animals. These functional data support molecular studies indicating loss of genetic information on chromosome 11p15 during the development of embryonal rhabdomyosarcoma. In addition, our studies demonstrate the existence of a second gene on the long arm, previously unrecognized by molecular analyses, which negatively regulates the growth of embryonal rhabdomyosarcoma cell lines.
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PMID:Human chromosome 11 contains two different growth suppressor genes for embryonal rhabdomyosarcoma. 134 25

A case of a mammary leiomyosarcoma in a 23-year-old woman is presented. The tumor appeared 16 years after successful treatment of an embryonal rhabdomyosarcoma of the orbit. Rhabdomyosarcomas are the most frequent soft tissue tumors of childhood, the orbit and the paratesticular region being the most common primary site for this tumor. In contrast, leiomyosarcomas other than those evolving from the viscera or the urogenital organs are rare neoplasms at any age. With the improvement of cancer treatment and survival rates, the risk of late effects after successful treatment for malignant tumors during childhood is increasing. Growth, development and fertility may be impaired and cosmetically disturbing facial and dental complications are common. Development of novel primary tumors is a known further consequence of successful treatment of brain tumors, retinoblastoma and acute leukemias. This is the case when high dose local radiation therapy and/or chemotherapy, especially alkylating agents, were used. Development of novel primary tumors is also known after treatment of childhood rhabdomyosarcomas. This report is intended to show that a second primary tumor may occur many years after a first successfully treated malignant neoplasm, and that young people are at risk for development of tumors at sites that are uncommon to this age group.
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PMID:[Leiomyosarcoma of the breast 16 years following successful treatment of a rhabdomyosarcoma of the orbit in childhood]. 141 91

Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood, but it is rare in young adults. It usually occurs in the pelvis or genito-urinary tract. We report a case of abdominal embryonal rhabdomyosarcoma who received a combined treatment modality with radical pelvic surgery and combination chemotherapy of vincristine, actinomycin-D and cyclophosphamide (VAC). She is well with no evidence of tumour recurrence for 3 years after primary surgery.
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PMID:Abdominal embryonal rhabdomyosarcoma. A case report. 151 77

Twenty-one cases of embryonal rhabdomyosarcoma, composed mainly of elongated spindle cells arranged in a fasciculated or storiform pattern, were retrieved from the files of the German-Italian Cooperative Soft Tissue Sarcoma Study. The term spindle cell rhabdomyosarcoma is proposed to designate this histotype. Spindle cell rhabdomyosarcoma predilected male patients (18 males, three females) and involved mostly the paratesticular area (12 cases) and the head and neck region (six cases). Histologically, all cases were characterized by a uniform proliferation of elongated spindle cells with eosinophilic and fibrillar cytoplasm mimicking smooth muscle fibers; immunocytochemical studies disclosed high expression of the muscle markers titin, desmin, and myoglobin. Clinical information was available in 17 cases; according to the Intergroup Rhabdomyosarcoma Study (IRS) grouping system, 13 were classified in group I, two in group II, and two in group III. Sixteen patients were well and alive 24 to 100 months after diagnosis; one patient died from disease progression 24 months after diagnosis. Analysis of our results determined that spindle cell rhabdomyosarcoma constitutes a rare variant of the embryonal form, showing a high degree of skeletal muscle differentiation and a low malignant potential; it should therefore be distinguished from classical forms of embryonal rhabdomyosarcoma.
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PMID:Spindle cell rhabdomyosarcoma. A prognostically favorable variant of rhabdomyosarcoma. 159 14


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