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Alveolar rhabdomyosarcoma. An analysis of 110 cases
Author(s) -
Enzinger F. M.,
Shiraki M.
Publication year - 1969
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(196907)24:1<18::aid-cncr2820240103>3.0.co;2-5
Subject(s) - medicine , alveolar rhabdomyosarcoma , rhabdomyosarcoma , metastasis , giant cell , pathology , medullary cavity , lymph node , lung , sarcoma , cancer
A series of 110 cases of alveolar rhabdomyosarcoma was reviewed. The neoplasms chiefly occurred in young patients between 10 and 20 years of age and were most common in the muscles of the forearms, the hands, and the perirectal and perineal regions. The microscopic picture varied, but in most cases recognition was readily accomplished on the basis of the characteristic pseudoalveolar pattern and the presence of differentiated rhabdomyoblasts and multinucleated giant cells having peripherally placed nuclei. Solid or medullary portions of undifferentiated tumor cells closely mimicking a malignant lymphoma occurred in about half the cases. Follow‐up information was obtained in 102 patients. Ninety‐four (92%) had died from widespread metastasis within the first 4 years after the diagnosis had been made. Seventy‐four of the 94 patients had died during the first year, most with evidence of metastasis to the regional lymph nodes and the lung. (Median actuarial survival, 83 3/4 months.) In several instances, lymph node metastasis led to the detection of the primary tumor. Prognosis and the management of alveolar rhabdomyosarcoma are discussed and analyzed on the basis of the actuarial survival rates.