Symptomatic Splenic Hamartoma: Case Report and Literature Review

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. Pediatrics 1998;101(5). URL: http://www.pediatrics.org/ cgi/content/full/101/5/e10; splenic hamartoma, pancytopenia, hypersplenism, splenomegaly, hemangiomas. ABBREVIATIONS. ANA, antinuclear antibody; CT, computed tomography; EBV, Epstein–Barr virus; MRI, magnetic resonance imaging. Rokitansky, according to Friedreich, first described splenic hamartomas1 .100 years ago in 1861. Since then, ;140 cases of true hamartomas have been reported or mentioned in the literature. Synonyms have included splenomas, spleen within a spleen,2 hemangiomas, posttraumatic scars,3 fibrotic nodules,4 and hyperplastic nodules. They are usually asymptomatic, particularly in the adult population, and are discovered either incidentally during work-up of other problems or during postmortem examination. It appears that Von Falkowski reported the first case of splenic hamartoma in a pediatric patient in 1914.5 Approximately 20 of the reported cases of splenic hamartoma have been in pediatric patients (,16 years of age). An association with hematologic disorders or other symptoms is rarely reported. Therefore, splenic hamartomas are seldomly included in the differential diagnoses of a left upper quadrant mass in pediatric patients. In 1953, Videbaek6 first reported the association of splenic hamartoma with hematologic disorders in a 30-year-old woman who had onset of symptoms in childhood. Thirty such symptomatic cases have been reported since then. Symptoms reported most frequently include pancytopenia, anemia, and thrombocytopenia. Less commonly, fever, malaise, and weight loss have been reported. Nine cases of symptomatic splenic hamartoma have been described in pediatric patients. The symptoms observed in the pediatric patients are similar to those in the adult patient population. In addition, growth retardation, night sweats, and recurrent infections have been reported in the pediatric patient population.7 Here, we report an example of symptomatic splenic hamartoma in a pediatric patient who had an 8-year span of clinical course from the time of initial presentation to the time of diagnosis.