Two cases of large, multiple splenic hamartomas in children with pancytopenia, bone marrow hyperplasia, lymphadenopathy, hepatosplenomegaly, frequent infections, growth retardation, and fever are reported. These symptoms were relieved by splenectomy, and have not recurred during follow-up periods of one year and nine years. The sharply circumscribed lesions comprised large portions of the resected spleens and were composed of dilated vascular channels filled with mononuclear cells and immunoblasts. The lesions lacked splenic cords or trabeculae, lymphoid follicles, Reed-Sternberg cells, and granulomas or other evidence of infection. Splenic hamartomas are usually single small lesions found incidentally at necropsy or laparotomy. Splenic hamartomas associated with symptoms and hypersplenism are large, and often confluent multiple tumors. Recognition of their benign nature is important in light of the current practice of laparotomy for staging and diagnosis of malignant conditions.
- Received August 6, 1979.
- Accepted October 15, 1979.
- Copyright © 1980 by the American Academy of Pediatrics