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

Embryonal and alveolar rhabdomyosarcoma probably have the same histogenesis, yet alveolar rhabdomyosarcoma has a considerably worse prognosis and is generally less responsive to treatment. The metastatic behavior of rhabdomyosarcoma in 22 autopsied subjects was analyzed with respect to the histologic subtypes of the tumors to determine whether the more aggressive behavior of alveolar rhabdomyosarcoma was related to greater tumor burden and/or more widely disseminated metastases. Subjects with embryonal rhabdomyosarcoma tended to survive longer and had greater numbers of metastatic sites at autopsy compared to subjects with alveolar rhabdomyosarcoma, but these differences were not statistically significant. In addition, there were no significant differences in the overall distribution of metastases between embryonal and alveolar rhabdomyosarcoma. The most frequent sites of metastases from both types of tumor were soft tissues (73%), serosal surfaces (64%), lymph nodes (64%), lungs (55%), and bone marrow (50%). The findings suggest that the metastatic behavior of rhabdomyosarcoma does not vary as a function of length of survival, mode of therapy, or histologic subtype. We propose that factors such as stage of disease at presentation and location of the primary tumor, rather than histologic subtype, are probably the most important variables governing the therapeutic responsiveness of rhabdomyosarcoma.
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PMID:Metastatic behavior of rhabdomyosarcoma. 373 70

We report on 17 patients with rhabdomyosarcoma of the genitourinary organs, retroperitoneal, pelvic, or perineal regions. Treatment was refused by three patients with either bladder or prostatic lesions. The remaining patients were treated with combined modality therapy. Five of 6 patients with bladder lesions are free of tumor 24 to 108 months (mean 51) after diagnosis, while one patient died 4 months postoperatively; two patients with paratesticular lesions are free of disease 3 years and 10 months following diagnosis, respectively. Of two patients with perineal lesions, one patient died, probably due to metastases 21 months after diagnosis, while another patient with local tumor recurrence was treated by reexcision with follow-up for 12 months without evidence of tumor recurrence. The worst results were obtained with the four patients with retroperitoneal pelvic lesions; one patient died 3 months after gross resection of tumor, and the other three patients deteriorated in the 4 months after diagnosis. Overall, 7 of 8 operated patients with bladder or paratesticular lesions are free of disease with follow-up for 10 to 108 months. The biological behavior of embryonal rhabdomyosarcoma, with the sex and age distribution and the management are discussed.
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PMID:Rhabdomyosarcoma involving the genitourinary organs, retroperitoneum, and pelvis. 395 Aug 46

In the cytogenetic analysis of an embryonal rhabdomyosarcoma after short-term culture, individual cells were found to contain multiple copies of chromosomes #2, #6, #8, #12, #13, #18, #20 and #21, and del(1)(:p21----qter). The tumor was hypotriploid (mode, 56 chromosomes). The relationship between these findings and published reports of karyotypes from rhabdomyosarcoma is discussed.
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PMID:A cytogenetic study of embryonal rhabdomyosarcoma. 396 22

A review of eight cases of rhabdomyosarcoma in childhood seen at the Ahmadu Bello University Hospitals in Kaduna and Zaria (Nigeria) over five years is presented. The cases were equally divided between the head region and the urogenital system. Embryonal rhabdomyosarcoma and sarcoma botryoides were the only histologic types seen. Although the clinical presentations were not different from those reported in other series, the correct diagnosis was not suspected clinically in most of the patients, so the institution of rational management was delayed. Certain aspects of the disease and its therapy in this part of the world are discussed.
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PMID:Rhabdomyosarcoma in Nigerian children. 401 51

Histological structure of rhabdomyosarcoma was studied in a group of 37 children. The classification was: embryonal rhabdomyosarcoma in 28 cases, alveolar rhabdomyosarcoma in 9 cases. 5 cases showed an ambivalent structure. The diagnosis of rhabdomyosarcoma is based on cytology: various rhabdomyoblasts are to be identified as well as distinguished from similar cells. The structural point of view is helpful but diagnostic only in typical cases of sarcoma botryoides and alveolar rhabdomyosarcoma. Undifferentiated cells maturating into rhabdomyoblasts combined with numerous polymorphic cells were found consecutive investigations of individual cases.
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PMID:[Rhabdomyosarcoma in childhood. I. Histologic structure]. 401 32

Although numerous investigations of soft-tissue sarcomas of childhood have been done, there are still many unsolved problems in the diagnosis, histogenesis, and biological behaviour of such tumours. In the present study, the soft-tissue sarcomas collected at the Paediatric Tumour Registry in Kiel were investigated. There was a total of 262 cases, including 169 (64.5%) in which a definite diagnosis of rhabdomyosarcoma had been made. An analysis of the age distribution showed that rhabdomyosarcoma is more than three times as frequent as non-rhabdomyosarcomatous soft-tissue tumours in the first quinquennium. Rhabdomyosarcoma exhibited male predominance (male-to-female ratio 1.45:1), whereas non-rhabdomyosarcomatous tumours showed an approximately equal sex distribution. Among the cases of rhabdomyosarcoma, the embryonal type clearly predominated (approx. 70%); the alveolar type was less common (approx. 20%). Correlation of histology with prognosis revealed that even tumours showing tiny foci with an alveolar pattern in otherwise solid, undifferentiated rhabdomyosarcoma, must be classified as alveolar rhabdomyosarcoma. Cases of alveolar rhabdomyosarcoma showed a higher incidence of local recurrence, lymph node metastasis, distant metastatic spread, and death, than did cases of embryonal rhabdomyosarcoma. It is concluded that "cytological differentiation" does not improve the prognosis of rhabdomyosarcoma.
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PMID:Soft-tissue sarcomas in childhood. A study of 262 cases including 169 cases of rhabdomyosarcoma. 403 65

A case of embryonal rhabdomyosarcoma of the left kidney is reported. A 16-year-old boy was admitted with the complaint of left abdominal pain and fever on January 6, 1983. Radiological examination showed a tumor of the left kidney; and, nephrectomy was performed. Histopathologically the entire tumor was composed of undifferentiated round cells. Diagnosis of embryonal rhabdomyosarcoma was made on the basis of special stains including immunohistochemical study with nervous tissue specific enolase. Although radiation and chemotherapy were performed postoperatively, the tumor recurred and the patient died on October 22, 1983. The problems of differential diagnosis of embryonal rhabdomyosarcoma from sarcomatous types of nephroblastoma, particularly rhabdoid tumor and other undifferentiated renal tumors were discussed. Fifteen rhabdomyosarcoma of the kidney including our case have been reported in the Japanese literature.
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PMID:[A case of embryonal rhabdomyosarcoma of the kidney]. 406 Dec 1

The diagnosis of rhabdomyosarcoma was established or proved by electron microscopy in two thirds of 21 cases. Histological classification was not adequate or formulated as "undifferentiated sarcoma" in half the cases. Electron microscopy did not succeed in one third of cases and rhabdomyosarcoma was classified according to simultaneous or successive study. Undifferentiated tumour cells resembled myoblastema cells of the 5th-6th gestation weeks. Differentiation of myoblasts and myotubes corresponded to the muscle development of the 7th-16th weeks. Immature cells showed features of fibroblasts and myofibroblasts in most cases of embryonal rhabdomyosarcoma. Alveolar rhabdomyosarcomas were characterized by exclusive myoblastic differentiation. Different cellular sources were discussed in rhabdomyosarcomas with differentiation into one cell type or into various mesenchymal cells.
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PMID:[Rhabdomyosarcoma in childhood. II. Ultrastructure]. 406 35

Of 32 patients seen in Memorial Center since 1920 with myosarcomas of the bladder or prostate 26 were male and six were female. The tumors arose in the bladder in 20, in the prostate in 11 and in one patient both organs were involved. Sarcomas of the bladder account for two or three of every thousand bladder cancers and for one of every thousand prostate cancers. Seventy-six patients, or approximately 10% of reported cases, have survived 3 years or more from diagnosis. Forty-five of those 76 sarcomas were reported as specific myosarcomas, i.e., embryonal rhabdomyosarcoma, adult rhabdomyosarcoma, leiomyosarcoma or combinations of those three. Thirty-six arose in the bladder, nine arose in the prostate. The most successful methods of treatment have been cystectomy for embryonal rhabdomyosarcoma of the bladder and segmental resection for leiomyosarcoma of the bladder. Rhabdomyosarcoma of the bladder and prostate has seldom been managed successfully, so that no particular treatment can be unconditionally recommended. Embryonal rhabdomyosarcoma of the prostate is as yet an incurable condition.
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PMID:Myosarcomas of the bladder and prostate. 521 7

Eleven male patients had germ cell tumors of the testis (7), mediastinum (3), or retroperitoneum (1) in which non-germ cell malignancies developed. Such malignant non-germ cell elements were present in the primary excisions of five patients and were subsequently found in additional resected tissue in 10 of 11 patients. In the patients who had multiple pathology specimens examined, a progression from atypia to predominant non-germ cell malignancy was often found. The authors believe these malignant elements arose within teratomatous foci, since eight of nine cases had teratoma in the primary tumor, and teratoma was found in subsequently resected tissue in one additional case. Cisplatin therapy frequently "unmasked" the non-germ cell malignant elements by destroying the more chemosensitive germ cell cancers. The prognosis was worst for five patients who developed progressive embryonal rhabdomyosarcoma: two of these patients died of local spread of tumor, whereas a third died of metastatic sarcoma. Only one patient, who had total surgical excision of rhabdomyosarcoma, survived. Other forms of sarcoma that developed within germ cell tumors did not appear to adversely affect the prognosis beyond that of teratoma. It is currently recommended, when feasible, that patients with teratoma and sarcoma undergo total surgical excision. Further treatment with cisplatin regimens, after eradication of the germ cell component, has not been helpful. The role of other forms of chemotherapy remains speculative.
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PMID:The development of non-germ cell malignancies within germ cell tumors. A clinicopathologic study of 11 cases. 609 1


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