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)

Rhabdomyosarcoma is a rare malignant neoplasm of the biliary tract. Including all the historical recent probable, and established cases that are carefully classified by Hayes and Synder, only 24 cases have been reported to date. Because of the extreme rarity of this tumor in this location, correct diagnosis was not made until second laparotomy or autopsy in several reported cases. The present report describes a child who recently underwent excision of such a tumor at our institution.
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PMID:Rhabdomyosarcoma of the bile ducts. 59 61

Rhabdomyosarcoma usually presents as a soft tissue mass, which may invade adjacent bone. However, the patient presents occasionally with bony metastases. Recognition of these is important for staging and management. Fifty-eight cases have been reviewed; 14 of these had local bone invasion by the soft tissue tumour. All bones involvel were flat bones; 12 showed permeated bone destruction and two showed geographic destruction. Bone expansion was seen in half the involved bones. Twelve of the 58 cases showed secondary bone deposits, which were the presenting feature in five. Although 10 cases had permeated bone destruction, two were very well defined with a wide range of radiological appearances. The radiological differential diagnosis includes neuroblastoma, leukaemic infiltration, lymphoma, histiocytosis X, solitary and multifocal osteosarcoma and other deposits.
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PMID:The radiology of bone changes in rhabdomyosarcoma. 62 1

Rhabdomyosarcoma is a rare tumour of mesenchyme-like tissue in which differentiation of rhabdomyoblasts has occurred. It is found mainly in infants and children. The prognosis, until recent years very grave, has been improved by a combination of chemotherapy and radiotherapy occasionally with surgery. Survival depends on the extent of the disease at the time of diagnosis. Diagnostic radiology has an important role to play in the demonstration of this, illustrations of which are given from a series of seven cases. The primary tumour has no pathognomonic appearances and is demonstrated essentially as a soft tissue mass lesion which displaces adjacent soft tissue structures such as arteries, veins, bladder and colon, or erodes adjacent bones whether in the extremities or the skull. The main role of diagnostic radiology is the detection of metastases. Chest radiography and skeletal survey are mandatory. Lymphangiography may reveal filling defects in regional lymph nodes but these are indistinguishable from any other metastases. Arteriography as well as revealing displacement of larger vessels, may show typical tumour neovascularity in the lesion. Serial skeletal radiographs are of help in assessing response of metastases to therapy.
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PMID:The role of diagnostic radiology in the diagnosis and management of rhabdomyosarcoma in young persons. 62 2

Twenty-six of three hundred and fourteen (26/314) patients whose tumors were reviewed by the Pathology Evaluation Committee of the Intergroup Rhabdomyosarcoma Study have histologic subtypes I and II which have morphologic characteristics similar to Ewing's sarcoma of bone. They are somewhat more common in an extremity lesion but have no relation to patient age. Disease-free survival is good; seventeen of twenty-six (17/26) or sixty-five (65%) percent thus far.
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PMID:Extraskeletal Ewing's sarcoma: a preliminary review of 26 cases encountered in the Intergroup Rhabdomyosarcoma Study. 66 97

Rhabdomyosarcoma, the most common primary malignant childhood orbital tumor, is composed of neoplastic striated muscle cells (rhabdomyoblasts) in various stages of differentiation and in patterns suggestive of neoplastic analogs of normal muscle embryogenesis. Orbital rhabdomyosarcoma is most commonly seen in children and adolescents, the average age of onset of symptoms being 7.8 years. The tumor usually presents as a rapidly evolving exophthalmos, often associated with drooping of the upper eyelid. A mass is palapable in only 25% of cases, loss of central vision at the time of presentation is uncommon, and laboratory studies are often of little help in diagnosis. The best diagnostic aid is a high index of suspicion whenever one sees a rapidly progressive exophthalamos in a child. Orbital rhabdomyosarcoma is almost always of the embryonal type, believed to originate in the orbital soft tissues from undifferentiated pluripotential embryonic mesenchyme. In the past, orbital exenteration has been the primary therapy. Review of 162 literature cases of orbital rhabdomyosarcoma, generally treated by unassisted surgery, revealed that only 25% of the patients survived 3 or more years. Recently, it has been shown that radiation therapy, alone or combined with chemotherapy, can be successful. A multidisciplinary approach, utilizing surgery, radiation therapy and chemotherapy has also been advocated. Both approaches appear to offer greater survival than unassisted orbital exenteration. The possibility of primary radiation therapy is extremely promising; if it becomes increasingly effective, a mutilating surgical procedure may become obsolete.
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PMID:Ophthalmic striated muscle neoplasms. 79 29

Clinico-pathologic reviews of series of children with rhabdomyosarcoma have yielded conflicting information regarding frequency of lymphatic spread of this disease. The 264 eligible entries in the Intergroup Rhabdomyosarcoma Study (from November 1972 to September 1975) have been categorized by a prospective staging system and pre-treatment characteristics and pathologic findings reviewed. Data accumulated thus far have revealed a higher than expected incidence of lymphatic metastases from extremity (17%) and genito-urinary sites (19%) with a somewhat lower incidence from the orbit (0%), the head and neck region (3%), and trunk (10%). These differences in regard to lymphatic metastases were found to have no relationship to age, sex, tumor size, or histologic type when these factors were simultaneously examined.
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PMID:Lymphatic metastasis with childhood rhabdomyosarcoma. 83 39

Rhabdomyosarcoma of the urinary bladder in adults is exceedingly rare. Reported cases have been of the embryonal botryoid type, as seen in children. We will describe a case of pleomorphic rhabdomyosarcoma occurring in the urinary bladder of an adult. In this case, we noted striking intraepidermal migration of tumor cells, resembling the epidermotropic growth characteristic of tumor cells in Paget disease of the breast. The pathological features of this apparently unique case form the subject of this report.
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PMID:Rhabdomyosarcoma of the urinary bladder with intraepithelial spread in an adult. 94 59

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

Rhabdomyosarcoma of the spermatic cord is a rare, highly malignant lesion, for which the optimum treatment has yet to be ascertained. Results of treatment of 3 patients are presented, making the total reported experiences 53 cases. Two of our 3 patients are alive. One survivor underwent retroperitoneal lymphadenectomy and the other received radical orchiectomy only. The third patient had metastasis at the time of diagnosis and subsequently died. Because of a paucity of knowledge concerning the disease, its propensity for youthful patients and its highly malignant characteristics, all treatment modalities must be considered and tailored to the needs of the individual patient.
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PMID:Rhabdomyosarcoma of the spermatic cord: report of 3 cases with review of the literature. 116 11

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


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