PEDIATRICS Vol. 66 No. 2 August 1980, pp. 261-265
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Symptomatic Splenic Hamartoma: A Report of Two Cases and Review of the Literature

Renato V. Iozzo MD1, Joel E. Haas MD1, and Ronald L. Chard MD1

1 Departments of Pathology and Pediatrics, the Children's Orthopedic Hospital and Medical Center and the University of Washington School of Medicine, Seattle

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.

Submitted on August 6, 1979
Accepted on October 15, 1979




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