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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

Twenty-four children aged 3 months to 18 years (median, 12 years) were treated for neurogenic sarcoma at the Children's Hospital of Philadelphia Cancer Research Center from 1958 through 1984. Sixteen patients had neurofibromatosis (NF). The tumors arose in an extremity or in the trunk (15 patients), the retroperitoneum-pelvis (6), or other sites (3). Twelve children underwent grossly complete excision of localized sarcoma; of them, five had no known residual tumor and seven had proven microscopic residual disease. Ten of the remaining 12 patients had grossly visible, residual localized disease, and two had lung metastases. After operation, nine were treated on a protocol with local radiation therapy (4000-6000 rad) plus vincristine, actinomycin D, and cyclophosphamide with or without Adriamycin (doxorubicin). The other 15 were treated variably. At 3 years, the proportion of tumor-free survivors was 9 of 24 (37.5%). The significant favorable factors were the initial surgical removal of all gross tumor (9 of 12 with tumor excision were tumor-free survivors at 3 years compared to none of 12 with gross residual sarcoma; P less than 0.01), and a tumor mitotic rate under one per high-power field. No significant correlation was found between tumor-free survival expectancy and age, race, sex, the presence of NF, the site and size of the primary tumor, or use of the chemoradiotherapy regimen. More effective treatment programs are needed for children with neurogenic sarcoma, especially for those with unresectable tumors.
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PMID:Treatment of children with neurogenic sarcoma. Experience at the Children's Hospital of Philadelphia, 1958-1984. 379 Nov 40

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

Operations for pulmonary metastases were carried out on 33 patients from January 1st 1976 to December 31st 1985 at the Surgical Division, University of Cologne. In six cases each, the primary tumors were hypernephromas, colorectal carcinomas and osteocarcinoma. There were five cases of melanoma, three cases of testicular carcinoma, two cases of corpus carcinoma and two cases of soft-tissue sarcoma as well as one case each of parotid, tonsillar and laryngeal carcinoma. The large number of metastases could be determined in 21 cases (64%) by means of preoperative radiodiagnostics with X-rays of the chest in two planes, conventional tomography and computer tomography of the thorax. Computer tomography was the most efficient single investigation. 17 patients received atypical resection and lobectomy was carried out in 16 cases. For all patients, the cumulative survivals revealed a one-year actuarial survival of around 70% and a three-year actuarial survival of about 20%. Patients with several metastases (two to six) had a very much poorer prognosis than patients with solitary pulmonary metastases. There was no connection between the surgical technique selected and the expected survival time in any case. The tumor-free interval between operation on the primary tumor and resection of the pulmonary metastases only had a slight prognostic significance.
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PMID:[Diagnosis and surgical therapy of lung metastases]. 380 39

The clinical characteristics and response to therapy of a patient with meningeal sarcoma, one of four patients over a twenty-five year period at Memorial Sloan-Kettering Cancer Center, are described. The light and electron microscopic characteristics of the primary tumor and as a heterotransplant in nude mice showed minimal differences. The tumor was resistant to conventional chemotherapeutic agents, both in the patient, in vitro as a cell culture and ex vivo as a heterotransplant. Only a combination of L-phenylalanine mustard and dianhydrogalactitol produced a limited response in both the patient and the mice. This combination may have some utility in future cases.
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PMID:Human meningeal sarcoma heterotransplants in Nu/Nu mice treated with L-phenylalanine mustard (L-PAM) and dianhydrogalactitol (DAG). 384 Jun 66

We present a series of eight cases of pleomorphic carcinoma of the pancreas, an uncommon lesion that contains bizarre giant cells and resembles sarcoma histologically. To our knowledge, this entity has not been described in the radiological literature. Clinical symptoms are similar to those of the usual pancreatic ductal cell carcinoma, but at presentation the primary tumor mass is usually large, and widespread metastatic disease is present. The most striking finding is massive lymphadenopathy, which may mimic lymphoma. A combination of clinical history, imaging findings, and results of percutaneous biopsy should lead to the proper diagnosis and may help to differentiate this entity from others that may affect lymph nodes.
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PMID:Pleomorphic carcinoma of the pancreas: computed-tomographic, sonographic, and pathologic findings. 388 Sep 2

Glucan, a particulate beta-1,3-polyglucose immunomodulator, was evaluated for its ability to modify hepatic metastases and survival in mice with reticulum cell sarcoma. Sarcoma M5076 cells were injected subcutaneously (1 X 10(5) cells) into syngeneic C57BL/6J male mice. On Day 20, histopathological studies indicated the presence of hepatic micrometastases. At this time, glucan (0.45 mg per mouse) or dextrose was administered intravenously. Therapy was continued at 3-day intervals up to Day 50. By Day 36 postchallenge, the glucan-treated group, when compared to the control group, showed a marked decrease in hepatic metastases, both grossly and histopathologically. A significant inhibition in the growth of the primary tumor also occurred. Plasma clearance of bromosulfophthalein measured on Day 36, denoted that glucan therapy maintained hepatic parenchymal cell functional integrity, while a 4-fold impairment in bromosulfopthalein removal was observed in control mice. Glucan-treated mice showed a 28% (p less than 0.05) long-term survival. In contrast, control mice showed a 100% mortality by Day 42 postchallenge. Studies to evaluate the mechanism of the anti-metastatic action of glucan indicated that 8 days after glucan administration, isolated hepatic macrophages were significantly more cytotoxic to sarcoma cells in vitro than were normal Kupffer cells. At this time, the cytotoxic activity of peritoneal and splenic macrophages from glucan-treated mice were unaltered. Additionally, co-incubation of particulate glucan with diverse populations of normal or tumor cells in vitro indicated that glucan exerted a direct cytostatic effect on sarcoma and melanoma cells and, in contrast, had a proliferative effect on normal spleen and bone marrow cells.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Therapeutic efficacy of glucan in a murine model of hepatic metastatic disease. 388 76

A 14-year-old boy was presented with a mass in the epigastrium. The tumor developed 13 years after removal of a neuroblastoma of the left adrenal gland. Therapy of the primary tumor had included irradiation and chemotherapy. Diagnosis of the gastric tumor was made by ultrasound and endoscopy. A total gastrectomy was performed showing a malignant undifferentiated sarcoma. Primary malignant tumors of the stomach such as gastric teratoma of the newborn [4], leiomyosarcoma [6], malignant lymphoma and carcinoma [1] are very rare in childhood. As cancer is treated more aggressively with radiation and cytostatics, more therapy-induced-tumors, including gastric sarcoma, must be expected in children.
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PMID:Malignant gastric sarcoma--diagnosis by ultrasound and endoscopy. 394 5

The results of treatment of brain metastases in a series of 125 patients who underwent surgery with or without postoperative radiation from 1978 through 1982 were analyzed. The major sites of primary tumor included the lung (40%), melanoma-skin (11%), kidney (11%), colon (8%), soft tissue sarcoma (8%), breast (6%), and a variety of others (15%). At the time of craniotomy, disease was considered limited to the central nervous system in 63 patients (50%). After surgery, 83 patients (66%) were neurologically improved, and 26 (21%) had their deficits stabilized. The overall median survival was 12 months, and 25% lived 2 years. Eight patients (12%) are alive 5 years or more following surgery. Survival varied with site of primary tumor, location of brain metastasis, extent of systemic disease, and neurologic deficit at time of craniotomy. Over a follow-up period ranging from 18 months to 6 years, 42 patients (34%) developed either local recurrences or other sites of brain metastases. These data suggest that although craniotomy followed by radiation is highly effective in the initial treatment of selected patients with brain metastases, alternate therapies require investigation in view of the high central nervous system relapse rate in long-term survivors.
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PMID:Surgical treatment of brain metastases. Clinical and computerized tomography evaluation of the results of treatment. 397 8

A retrospective clinicopathologic review of 32 patients with mammary sarcoma exclusive of angiosarcoma or lymphoma was performed. For 25 patients with previously untreated lesions, the median tumor diameter was 4 cm and 14 patients had high or intermediate grade lesions. One of 22 patients treated by mastectomy and one of three patients treated by local excision died of sarcoma yielding an actuarial 5-year survival of 91%. None of the 25 patients had received adjuvant chemotherapy and only one treated by mastectomy had post-operative radiation therapy. Seven other patients were referred for treatment of recurrent mammary sarcoma. In this group, median size of the primary tumor was 6 cm and four had high or intermediate grade histology. Tumor control was achieved for only one of five patients with local recurrence and neither of the two with distant metastases. Median survival was 6 months following initiation of treatment for recurrence. Whenever possible breast sarcomas should be classified according to histologic cell type and grade. For lesions not readily classified, the terms unclassified or anaplastic sarcoma should be used. The diagnosis of stromal sarcoma is best reserved for those infrequent sarcomas that can be traced to the specialized periductal and perilobular stroma of the breast. Total mastectomy is recommended for most patients with postoperative radiation therapy indicated when the adequacy of the margin is in doubt. The role of adjuvant chemotherapy in the primary management of mammary sarcoma is yet to be determined.
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PMID:Sarcoma of the breast. A study of 32 patients with reappraisal of classification and therapy. 397 55


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