Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029463 (osteosarcoma)
 16,637 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Because of the great risk for development of pulmonary metastases following amputation for osteosarcoma, 24 consecutive patients with "clinically localized" osteosarcoma of an extremity were given adjuvant combination chemotherapy with adriamycin-cyclophosphamide-high-dose methotrexate-citrovorum factor. Thirteen of these patients remain free of tumor from 11 to 48 months following amputation. The median disease-free survival is estimated to be 18 months and the median survival 27 months. No relapses have been observed in any patients surviving free of disease beyond 23 months. Combination chemotherapy was also given to 16 patients whose disease was not localized; eight with pulmonary metastases at or following diagnosis, one with nodal metastases at diagnosis, two with osteosarcoma following radiation therapy for other malignant tumors, three with osteosarcoma of flat bones, one with parosteal osteosarcoma, and one with multifocal osteosarcoma. Three of this latter group of patients are surviving free of tumor at 8, 17, and 19 months from diagnosis. Two young patients died from complications of methotrexate and adriamycin toxicity.
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PMID:Combination chemotherapy for osteosarcoma. 34 14

The pattern of malignancies among indigenous Zambian children is described. The study, based upon an analysis of histopathology, autopsy and haematology records for a 10-year period (1980-1989), reveals a total of 525 neoplasms with a peak prevalence in the 5-9 year age group. Non-Hodgkin's lymphoma (17.5%) was the most common disorder followed by Burkitt's lymphoma (13.9%), retinoblastoma (11.4%), Kaposi's sarcoma (8.8%), Hodgkin's disease (5.9%), Wilms' tumour (5.9%), acute lymphocytic leukaemia (4%), rhabdomyosarcoma (3.4%), nasopharyngeal carcinoma (2.7%) and osteogenic sarcoma (2.1%). Kaposi's sarcoma and Hodgkin's disease revealed a significant male dominance; the former presented mainly in nodal form. Compared to an earlier report from Zambia (1968-1972), a significant increase in the incidence of Kaposi's sarcoma and nasopharyngeal carcinoma was noted in the present series.
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PMID:The pattern of paediatric malignancy in Zambia (1980-1989): a hospital-based histopathological study. 156 Apr 80

The presence of lymph node metastases from osteosarcoma was rare at diagnosis, occurring in only four of 176 patients (2.3%) at St. Jude Children's Research Hospital. An additional patient presented with calcified lymph nodes, which were demonstrated to contain no evidence of metastatic osteosarcoma. These patients did not differ in race, sex or age at diagnosis from other osteosarcoma patients. Each of the four patients with lymph node metastases of osteosarcoma had tumors composed predominantly of the osteoblastic type. Regional nodal metastases, whether alone or in conjunction with other metastatic disease were a poor prognostic factor. Despite aggressive therapy, median survival was 8 1/2 months from diagnosis, which did not differ significantly from the survival achieved by other osteosarcoma patients who had other hematogenous metastases.
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PMID:The significance of calcified regional lymph nodes at the time of diagnosis of osteosarcoma. 386 62

Sarcomas of childhood rank fifth in incidence of malignant tumors in children younger than 15 years. Among the soft tissue sarcomas, approximately 50% are rhabdomyosarcomas. The remainder represent a heterogeneous group of diverse sarcomas which are not unique to children and include fibrosarcoma, synoviosarcoma, malignant fibrous histiocytoma, malignant schwannoma, angiosarcoma, leiomyosarcoma, and others. The most common bone cancers in childhood are osteosarcoma and Ewing's sarcoma. Although a multidisciplinary approach utilizing surgery, irradiation, and combination chemotherapy is routinely used in management of virtually all children with solid tumors, the value of adjuvant chemotherapy in select bone and rare soft tissue sarcomas is currently being tested. Multiagent chemotherapy including vincristine, dactinomycin, cyclophosphamide, and Adriamycin (doxorubicin) contribute to cure rates in 65% to 75% of children with localized rhabdomyosarcoma, Stages I to III, when combined with surgery and/or irradiation. Other drugs which hold promise include platinum, DTIC, methotrexate, and VP-16. The efficacy of similar drugs in the rarer pediatric soft tissue sarcomas other than rhabdomyosarcoma and its variants requires prospective randomized trials evaluating histologic grade, tumor size, and nodal status. It has been suggested that the high-grade sarcomas presenting with minimal tumor bulk are most sensitive to combined radiotherapy-chemotherapy, whereas the low-grade sarcomas are more resistant to such therapy. Tumor cell heterogeneity contributes to biologic diversity and response to treatment. Chemotherapy as adjuvant therapy to irradiation is currently recommended and utilized for Ewing's sarcoma with survival rates approaching 80%, and disease-free survival of approximately 75% for those with localized disease. Children with widespread and metastatic disease at presentation fare less well. Although multiple single agents exhibit response rates ranging from 40% to 60%, including cyclophosphamide, Adriamycin, dactinomycin, BCNU, mithramycin, and 5-fluorouracil, new and more effective agents are needed. Controversy regarding the value of multiagent chemotherapy in osteosarcoma has stimulated prospective randomized trials. Evaluation of local control rates as well as sites and occurrence of metastases are essential in assessing the contribution of aggressive combined modality therapy in the pediatric sarcomas. Emphasis on refinement of therapy in determining the risk/benefit ratio from adjuvant chemotherapy in pediatric sarcomas is mandatory. Enhancement of early local reactions is apparent when adjuvant chemotherapy is used with local radiotherapy.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:The value of adjuvant chemotherapy in the management of sarcomas in children. 388 37

A retrospective review of 112 cases treated by one author (J.C.) over the 21 year period 1953-1973 is performed. In 62% of the cases squamous cell carcinoma was the histologic diagnosis, with adenoid cystic carcinoma accounting for a further 16%. The remaining histologic types were few in number and included fibrosarcoma, muco-epidermoid carcinoma, melanoma, chondrosarcoma, adenocarcinoma, malignant Schwannoma, lymphosarcoma, malignant mixed tumor, rhabdomyosarcoma, angiosarcoma, and osteogenic sarcoma. Three years following diagnosis more than 1/3 of the squamous cell carcinomas were alive but by 10 years the survival rate had fallen to 16%. Orbital involvement at the time of presentation or noted at surgery had a poor prognosis with a 17% five year cure rate and only 2% alive at 10 years. Erosion of the pterygoids or pterygopalatine fossa invasion as diagnosed radiologically or intra-operatively revealed all patients dead of disease within five years despite radical surgery and irradiation. Cervical nodal metastases at primary presentation was a particularly ominous finding with all patients dead of disease within three years. Metastases developing later had a better prognosis with 39% surviving five years when treated aggressively.
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PMID:Carcinoma of the maxillary sinus. A correlation of the clinical course with orbital involvement, pterygoid erosion or pterygopalatine invasion and cervical metastases. 630 78

Osteogenic melanoma is a rare variant of malignant melanoma; only eight cases have been reported. To characterize this unusual neoplasm further, we present four new cases. Two patients were men and two were women (average age, 56 years; range, 47-78 years). All tumors arose from acral lentiginous melanomas. Three were subungual finger lesions and one was on the sole of the foot. All four had been previously diagnosed as or were suspected to have been primary osseous lesions. The vertical growth components were high-grade, amelanotic sarcomatoid malignancies with abundant osteoid matrix. Two tumors also had chondroblastic differentiation. Cells with epithelioid features, including prominent eosinophilic nucleoli, were discernible in every tumor. Regional lymph node metastases in two cases retained osteocartilaginous differentiation, whereas metastatic cells in another case were purely epithelioid. Tumor cells in every case were immunoreactive for S-100 protein and vimentin, and non-reactive for cytokeratin. Two tumors also expressed HMB-45. Melanosomes were identified ultrastructurally in every tumor. Follow-up information was available on every patient. Three developed regional lymph node metastases and are currently alive and well after 14, 39, and 101 months. The fourth patient died of metastatic uterine carcinoma 20 months postoperatively. The differential diagnosis of osteogenic melanoma includes osteosarcoma as well as atypical fibro-osseous proliferations. Clinico-pathologic features that support a diagnosis of osteogenic melanoma include junctional activity, absence of primary bony involvement, regional nodal metastases, immunoreactivity for S-100 protein and/or HMB-45, lack of cytokeratin reactivity, and ultrastructural identification of melanosomes.
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PMID:Osteogenic melanoma. A rare variant of malignant melanoma. 803 2

To date, intraoperative radiotherapy has been performed at Kyoto University Hospital in a total of 480 patients with malignancies various sites. Among these, patients with disease of 4 major sites were selected for prognostic analysis. For gastric cancer patients survival was improved by the adjuvant intraoperative radiotherapy compared with gastrectomy alone in those with advanced nodal or serosal involvement [P < 0.05 for n2 + n3' 0.1 > P > 0.05 for s(+)]. Also, the survival for the adjuvant radiotherapy group was significantly improved in patients with stage II-III pancreatic cancer who underwent non-curative resection (P < 0.001). For patients with soft tissue sarcomas of the extremities, intraoperative radiotherapy was performed after the tumours were surgically removed to the greatest e* possible. Operation curability had a great impact on the local tumour control rate (82% for curative resection versus 18% for non-curati resection) and on the long-term survival rates (65% versus 8%, respectively). In patients with osteosarcoma, a large single do* 50-55 Gy was applied to the affected limb in an attempt to achieve local control and limb salvage. By this method, good local control attained, and a 10-year survival rate showed improvement following the introduction of cisplatinum into intensive chemotherapy.
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PMID:Kyoto University experience with intraoperative radiation therapy. 887

A middle-aged man was being investigated for constipation. Abdominal radiographs incidentally revealed a large, densely calcified, rounded mass within the pelvic cavity. A CT scan was performed followed by surgical excision with a differential diagnosis of calcified hematoma and an enlarged calcified lymph nodal mass. Histopathological investigation revealed a primary mesenteric extraskeletal osteosarcoma. To the best of our knowledge, a primary extraskeletal osteosarcoma arising from the mesentery has not been described previously in the English literature. The radiological features and differential diagnosis are discussed.
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PMID:Primary mesenteric extraskeletal osteosarcoma in the pelvic cavity. 1583 64

Spindle cell (sarcomatoid) carcinoma of the breast is a rare variant of breast cancer that has been classified under the broad rubric of metaplastic carcinoma. Because the term "metaplastic carcinoma" comprises a heterogeneous group of tumors, it has been difficult to reliably predict biologic potential or to determine optimal therapy. To better characterize the spindle cell subset of metaplastic breast carcinomas, we reviewed 29 cases. All patients were adult females ranging from 40 to 96 years of age (median, 68 years). Tumor size ranged from 1.5 to 15 cm (median, 4 cm). Treatment was by excision and/or mastectomy with axillary node evaluation in most cases, often combined with postoperative radiation and/or chemotherapy. All cases were clinically of breast origin, showed >or=80% spindled/sarcomatoid morphology, and demonstrated keratin positivity and/or close association with ductal carcinoma in situ. Immunohistochemical studies showed evidence suggesting myoepithelial differentiation as exhibited by immunoreactivity for smooth muscle actin, cytokeratin 14, and p63 in a subset of cases (39%). Twenty-seven cases exhibited pure spindled or sarcomatoid morphology of variable appearance and nuclear grade, whereas 2 contained high-grade invasive ductal carcinoma comprising <or=20% of the tumor mass. Two cases exhibited heterologous elements (1 rhabdomyosarcoma and 1 with both chondrosarcoma and osteosarcoma) and 4 were associated with ductal carcinoma in situ. Follow-up data were available on 24 of 29 patients (range, 1-120 months; median, 20 months). Of 20 cases in which axillary nodes were biopsied, definitive nodal metastases were identified in only 1 (5%), and this was in a case with a significant component of invasive ductal carcinoma. Three patients developed local recurrences. Extranodal metastases occurred in 11 of 24 patients (46%), most commonly to the lungs. Ten of 24 patients (42%) died of disease at a median interval of 11.5 months (range, 1-46 months) and 3 patients were alive with metastatic disease. Eight patients were alive with no evidence of recurrent or metastatic disease (median, 29.5 months). Based on this series, spindle cell/sarcomatoid carcinoma of the breast is a highly aggressive neoplasm with a high rate of extranodal metastases. Purely spindled/sarcomatoid tumors have a significantly lower rate of nodal metastases than conventional ductal and lobular breast carcinomas.
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PMID:Spindle cell (sarcomatoid) carcinoma of the breast: a clinicopathologic and immunohistochemical analysis of 29 cases. 1725 81

Bone scans are widely utilized for detection of metastases from bone sarcomas. Technetium methylene diphosphonate scan ([(99m)Tc]MDP) is one of the most popular radiotracers used for that purpose. Lymphatic spread of bone sarcomas is unusual and often difficult to diagnose. Unfortunately, bone scans are not as sensitive in demonstrating lymphatic spread of sarcomas as they are at demonstrating hematogenous spread. A bone scan will often fail to demonstrate lymph nodes metastases until there is mineralization at the affected node. In this report, we highlight an interesting case of a patient with secondary osteogenic sarcoma (OS) from Paget's disease in the distal femur with non-ossified inguinal nodal metastasis diagnosed with [(99m)Tc]MDP. Lymph node involvement was not appreciated on plain radiographs or computed tomography (CT).
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PMID:Lymphatic spread of pagetic osteogenic sarcoma detected by bone scan. 1858 31


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