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Extraosseous osteogenic sarcoma. Clinicopathological study of eight cases and review of literature
Author(s) -
Rao Uma,
Cheng Anthony,
Didolkar Mukund S.
Publication year - 1978
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(197804)41:4<1488::aid-cncr2820410437>3.0.co;2-a
Subject(s) - medicine , calcification , amputation , sarcoma , soft tissue , mesenchymal stem cell , lung , surgery , pathology
Since 1963, eight patients with extraosseous osteogenic sarcoma were treated at Roswell Park Memorial Institute; these constituted 4.6% of all the osteogenic sarcoma patients during the period. The mean age of the patients was 58.7 years and a ratio of male to female was equal. Local swelling of insidious onset was the commonest symptom. All the tumors originated in extremities; the lower extremity was the more frequent site. At the time of diagnosis, seven patients had localized tumor and one had pulmonary metastases. Radiologically, a soft tissue mass with spotty calcification without any adjacent bone involvement, was the classical sign. Elevated alkaline phosphatase without liver metastases was observed in five of six patients when disease progressed. Ultrastructurally, the prominent cell type was a fibroblast‐like cell intermingled with varying numbers of osteoblasts and undifferentiated mesenchymal cells. Extreme morphologic variability probably accounts for the difference in composition of this tumor. Radical soft part excision or amputation should be the treatment of choice. Local recurrence was not seen in four patients after radical surgery, but it was observed in three of the four patients who had simple excision. The lung was the commonest site of metastases. No objective responses were observed after chemo‐ or immunotherapy. The overall median survival was 20 months and 5‐year survival rate was 25%.