Rhabdomyosarcoma presenting as acute hematologic malignancy: case report and review of the literature
Ambrosiani, L.; Bellone, S.; Betto, F.S.; Cecchetti, G.; Ceretti, E.; Dagani, R.; Morelli, A.; Pavia, G.; Schiaffino, E.; Tavani, E.; Vismara, A.
Tumori 82(4): 408-412
1996
ISSN/ISBN: 0300-8916 PMID: 8890982 Document Number: 467300
The authors describe a case of undifferentiated rhabdomyosarcoma from unknown primary site, presenting as an acute hematologic malignancy with generalized lymphadenopathy, extensive bone marrow involvement and clinical and laboratory features of disseminated intravascular coagulation. Such a peculiar behaviour is known for rhabdomyosarcoma but is rare and can be a serious diagnostic problem for the clinician and the pathologist. The importance of a large spectrum immunohistochemistry as first diagnostic approach to any undifferentiated small-cell malignant tumor is stressed, together with the knowledge of the different immunoreactivity patterns. Desmin, MS-actin and myoglobin are the most reliable markers of this type of myogenic sarcoma.