PEDIATRICS Vol. 101 No. 5 May 1998, p. e10

ELECTRONIC ARTICLE:
Symptomatic Splenic Hamartoma: Case Report and Literature Review

Received Jul 22, 1997; accepted Jan 14, 1998.

Howard A. Britton^{, ¶}

E. Bruce Mewborne^§

Dean A. Troyer^#

Victor A. Saldivar^*

Irving A. Ratner

Departments ^* Pathology,  Pediatrics, ^§ Radiology, and  Surgery Santa Rosa Children's Hospital San Antonio, TX 78207 ^¶ Division of Pediatric Hematology and Oncology and ^# Department of Pathology University of Texas Health Science Center San Antonio, TX 78207

An 11-year-old girl with low-grade fever, night sweats, thrombocytopenia, and an 8-year history of progressive splenomegaly underwent an elective splenectomy. Pathologic diagnosis was multiple splenic hamartoma. The patient's symptoms resolved after the splenectomy. Since first described by Rokitansky in 1861, ~140 cases of splenic hamartoma have been described in the literature. Most of the splenic hamartomas were discovered incidentally. A minority of these lesions were associated with hematologic symptoms such as pancytopenia, anemia, and thrombocytopenia. Only 20 of the reported cases of splenic hamartoma occurred in pediatric patients. However, compared with the adult patients, nearly half of these cases in pediatric patients was associated with symptoms. Splenectomy and partial splenectomy have relieved these symptoms. With advances in imaging, splenic hamartomas are being discovered with increasing frequency. A multimodal radiologic work-up has enabled some cases of splenic hamartoma to be diagnosed preoperatively. Inclusion of this benign entity in the differential diagnoses of symptomatic splenomegaly in a pediatric patient is important in the preoperative management and counseling of the patient and family. In patients who have discrete lesions, consideration of this entity preoperatively may avoid total splenectomy.

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