Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Eighteen patients with parameningeal rhabdomyosarcoma (RMS) involving the orbit were treated at Memorial Sloan-Kettering Cancer Center (MSKCC) between July 1971 and October 1983. Fifteen patients were children with a mean age of 6 years and three patients were adults with a mean age of 21 years. In four patients, the primary tumor originated in the orbit, while the remaining 14 had other parameningeal primary sites. The tumors were in a very progressive local stage, with destruction of facial bones in most patients. Six patients were treated with the T2 chemotherapy protocol and 12 received the T6 protocol. The radiation treatment plan for all patients was designed to deliver between 4500 and 7200 rad delivered to the primary tumor over 4 to 16 weeks. Eleven of the 18 patients (61%) are alive and well with a median follow-up time of six years. Two patients died of therapeutic complications and five died of tumor spread with central nervous system involvement. Ocular complications included acute and chronic conjunctival, corneal, lens, and retinal changes, which were less severe than those reported in previous series.
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PMID:Radiation and chemotherapy of parameningeal rhabdomyosarcoma involving the orbit. 376 44

Of a total of 1561 patients registered in the Intergroup Rhabdomyosarcoma Study (IRS) as of May 1983, 78 patients (5%) were younger than 1 year of age. These infants did not differ from the older children (1-20 years of age) in male/female ratio, clinical grouping, or survival rates. Infants younger than 1 year of age had a significantly greater frequency of undifferentiated sarcoma, 18% versus 7% in older children (P less than 0.005). Infants also had a significantly greater proportion of cancers with botryoid pathology, 10% versus 4% in older children (P less than 0.005). When reviewed by the newly proposed IRS cytopathologic classification, there was no difference in pathologic types between the two age groups. Infants younger than 1 year of age had a higher rate of bladder-prostate-vagina primary tumor sites than older children, 24% versus 10% (P less than 0.05). In addition, infants tended to receive less of the prescribed doses of chemotherapy and radiation therapy than older children, and to develop more toxicity to treatment than older children. Despite these differences, the overall survival curve for the two age groups appears to be similar. In contrast to Wilms' tumor and neuroblastoma, in which age (less than 1 year) is a favorable prognostic factor, age does not appear to be an important prognostic factor in rhabdomyosarcoma.
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PMID:Infants younger than 1 year of age with rhabdomyosarcoma. 377 10

Ewing's sarcoma is a rare, but highly malignant bone tumor. During the years 1966-1983 we diagnosed and treated this tumor in 84 patients. Diagnosis of the tumor in an early stage is a prerequisite for successful therapy. Clinical and radiological findings contribute to the diagnosis, but histologic confirmation is indispensible. In addition to histomorphology we regard electron microscopy and the demonstration of glycogen as essential for the final diagnosis. The differential diagnosis includes haematogeneous osteomyelitis, malignant lymphoma, metastases from neuroblastomas, rhabdomyosarcoma and some benign bone tumors. New observations concerning the histogenesis of Ewing's sarcoma are discussed. The introduction of chemotherapy as part of a multimodality approach to treatment has substantially improved the prognosis in comparison to the previous results with surgery and radiation therapy. The role of surgery and radiation independently and in combination is discussed. We propose an approach to treatment based on our experience which involves surgical removal of the primary tumor after preoperative chemotherapy. The overall survival rate among our 84 patients was 40%. Among those who were diagnosed early and treated according to the recommended protocol 54% are alive.
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PMID:[Ewing's sarcoma. New clinico-pathological aspects]. 378 6

In the first Intergroup Rhabdomyosarcoma Study (IRS-I), it was found that, of 57 patients with nonorbital cranial parameningeal sarcoma treated from 1972 to 1976, 20 patients (35%) developed meningeal sarcoma and 90% (18/20) died of this complication. Since about half of these 20 patients had received inadequate radiation therapy and all had radiation therapy delayed until week 6, the treatment program was modified in December 1977 to begin radiation therapy on day 0 to the entire neuraxis and the primary tumor for all patients presenting with one or more risk factors indicating a high risk of meningeal involvement, and to administer periodic intrathecal medications to the same patients. The risk factors were intracranial extension of tumor, bony erosion at the cranial base, and cranial nerve palsy. In this report, the prognoses of two groups of patients with nonorbital cranial parameningeal sarcoma are compared: 95 patients treated before modification of the protocol in December 1977 with chemotherapy and nonintensive meningeal radiotherapy (preintensive group) and 68 patients treated subsequently with intensive meningeal radiotherapy and chemotherapy (intensive group). All patients received vincristine and actinomycin D (dactinomycin) with or without cyclophosphamide and Adriamycin (doxorubicin), according to the protocol. Radiation therapy consisted of 4000 to 5500 rad to the primary tumor in 5 to 6 weeks for most patients. The intensively treated patients also received intrathecal drugs via lumbar puncture and whole cranial or craniospinal radiation when one or more meningeal risk factors were present. In the preintensive group, the complete remission rate was 68% (65/95); the percentages of patients tumor free or alive at 3 years were 33% and 41%, respectively. In intensively treated patients, the complete remission rate was 76% (52/68); the percentages of patients tumor free or alive at 3 years were 57% and 68%, respectively. The improvements in the percentages of intensively treated patients tumor free or surviving were highly statistically significant (P less than 0.01 for both comparisons). Within the intensive treatment group, meningeal involvement at diagnosis was an unfavorable prognostic sign, since patients with no meningeal involvement had much better tumor-free survival at 3 years (81% vs. 51%) and survival (90% vs. 57%) than patients with evidence of meningeal involvement; the differences were highly statistically significant (P = 0.01).
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PMID:Improved prognosis with intensive treatment of children with cranial soft tissue sarcomas arising in nonorbital parameningeal sites. A report from the Intergroup Rhabdomyosarcoma Study. 379 Nov 41

Rhabdomyosarcoma of the heart is a rare tumor, especially in childhood and particularly in the left ventricle. A primary tumor of this type was successfully resected after echocardiographic assessment alone in a young girl exposed prenatally to diphenylhydantoin. The patient is well and free of recurrence 2 1/2 years later.
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PMID:Primary left ventricular rhabdomyosarcoma in a child: noninvasive assessment and successful resection of a rare tumor. 382 Nov 53

Rhabdomyosarcoma represents the most common soft tissue sarcoma in children. The CT findings in five patients with pelvic rhabdomyosarcoma are reported. In the three male patients the primary tumor originated in the prostate gland whereas in the two female patients the bladder was the primary site. The CT findings in the prostatic tumors included an enlarged, inhomogeneous prostatic mass with invasion of the perirectal fat. In two of these patients there was thickening of the levator ani muscle and one showed tumor invasion through the ischiorectal fossa and sciatic and obturator foramina into the pelvic musculature and bone. In the two patients with bladder tumors, one showed a large mass adjacent to the lateral aspect of the bladder and ascites, suggesting an ovarian primary tumor. The other patient with sarcoma botryoides (a subtype of rhabdomyosarcoma) had a diffusely thickened bladder. Two patients have had a follow-up CT demonstrating tumor regression posttherapy. The characteristically large size of these masses and propensity for local invasion in a young patient are typical features of pelvic rhabdomyosarcoma.
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PMID:Computed tomography of prostatic and bladder rhabdomyosarcomas. 401 36

Twenty-three patients with parameningeal (including orbital rhabdomyosarcoma (RMS)) were treated at Memorial Sloan-Kettering Cancer Center (MSKCC) between July 1971 and January 1983. Twenty were children with a mean age of 6 and 3 were adults. In 6 patients, the primary tumor was from the orbit, whereas the remaining 17 had other parameningeal primary sites. The tumors were in a very progressive local stage, with extensive destruction of the facial bones in 19 patients. Eight patients were treated with T2 chemotherapy protocol and 15 received T6. Seven patients received 5,000 to 7,200 rad delivered to the primary tumor in 11-16 weeks, 15 patients received between 4,500 to 5,000 rad in 4-7 weeks, and 1 patient received 3,000 rad in 3 weeks for residual microscopic disease following surgery. Two patients were treated with radiation to the whole brain; no patients received radiation of the whole central nervous axis (CNA). Fifteen of the 23 patients (65%) are alive and well with a medical follow-up time of 5 years. Two patients died of therapeutic complications and six died of tumor spread. In five patients, involvement of the central nervous system (CNS) was the cause of death. The prognosis of orbital RMS with parameningeal involvement is no better than in other tumors of parameningeal sites. In those patients who had impaired vision because of optic nerve damage prior to treatment, the vision did not improve following treatment. There was no impaired vision seen due to radiation damage of eye structures except in the lens.
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PMID:Parameningeal rhabdomyosarcoma (including the orbit): results of orbital irradiation. 406 37

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

The results of the treatment of the commonest soft tissue sarcoma in childhood have improved dramatically during the past decade. The combination of reasonable surgery, radiotherapy and multiagent chemotherapy has been demonstrated to provide effective control of both the primary tumor and distant metastases in patients with rhabdomyosarcoma. Similar progress with the less common soft tissue malignancies will be more difficult to attain. Current therapeutic trends appear to favor a similar multimodal approach with a conservative but complete surgical excision combined with varying regimens of radiation and chemotherapy. Because of the infrequent occurrence of these unusual tumors (450 cases per year in the United States) no single institution can possibly attract a significant number of new cases to enter on treatment protocols. Substantial improvement in our current therapeutic techniques will be possible in the future only if our combined multicenter studies are continuously refined, re-examined, and extended. A plea is made for early referral of these difficult patients to regional study centers.
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PMID:The pediatric patient with sarcoma. 626 51

Three enolase isozymes (alpha alpha, alpha gamma, and gamma gamma) and S-100 protein in the extract of neuroendocrine tumors (neuroblastoma, ganglioneuroblastoma, ganglioneuroma, and pheochromocytoma) and nonneuroendocrine tumors (Wilms' tumor, rhabdomyosarcoma, and hepatoblastoma) were determined by means of enzyme immunoassay systems. All of the tumors examined showed a high level of alpha alpha-enolase (1.71 to 19.0 micrograms/mg protein). Levels of nervous system-specific enolases (NSE; alpha gamma and gamma gamma) in the neuroendocrine tumors were also rather high (alpha gamma, 1.64 to 7.45 micrograms/mg protein; gamma gamma, 0.052 to 5.56 micrograms/mg protein). However, the NSE concentration in the extract of nonneuroendocrine tumors was low (alpha gamma, less than 0.88 micrograms/mg protein; gamma gamma, 0 microgram/mg protein). The level of S-100 protein was relatively high in ganglioneuroma (greater than 500 ng/mg protein) and ganglioneuroblastoma (greater than 100 ng/mg protein), but low in neuroblastoma (less differentiated neuroendocrine tumor) and nonneuroendocrine tumors. Serum levels of enolase isozymes were also determined in neuroblastoma patients before and after resection of primary tumor or effective chemotherapy. The elevated level of serum NSE (alpha gamma and gamma gamma) was markedly decreased with little change in the alpha alpha level by the treatment.
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PMID:Determination of three enolase isozymes and S-100 protein in various tumors in children. 631 26


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