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Symptomatic Splenic Hamartoma: Case Report and Literature Review

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Symptomatic Splenic Hamartoma: Case Report and Literature Review Symptomatic Splenic Hamartoma: Case Report and Literature Review ABSTRACT. An 11-year-old girl with low-grade fever, In 1953, Videbaek6 first reported the association of night sweats, thrombocytopenia, and an 8-year history of splenic hamartoma with hematologic disorders in a progressive splenomegaly underwent an elective sple- 30-year-old woman who had onset of symptoms in nectomy. Pathologic diagnosis was multiple splenic childhood. Thirty such symptomatic cases have been hamartoma. The patient’s symptoms resolved after the reported since then. Symptoms reported most fre- splenectomy. Since first described by Rokitansky in quently include pancytopenia, anemia, and throm- 1861, ;140 cases of splenic hamartoma have been de- scribed in the literature. Most of the splenic hamartomas bocytopenia. Less commonly, fever, malaise, and were discovered incidentally. A minority of these lesions weight loss have been reported. Nine cases of symp- were associated with hematologic symptoms such as pan- tomatic splenic hamartoma have been described in cytopenia, anemia, and thrombocytopenia. Only 20 of the pediatric patients. The symptoms observed in the reported cases of splenic hamartoma occurred in pediat- pediatric patients are similar to those in the adult ric patients. However, compared with the adult patients, patient population. In addition, growth retardation, nearly half of these cases in pediatric patients was asso- night sweats, and recurrent infections have been re- ciated with symptoms. Splenectomy and partial splenec- ported in the pediatric patient population.7 Here, we tomy have relieved these symptoms. With advances in report an example of symptomatic splenic hamar- imaging, splenic hamartomas are being discovered with toma in a pediatric patient who had an 8-year span of increasing frequency. A multimodal radiologic work-up has enabled some cases of splenic hamartoma to be di- clinical course from the time of initial presentation to agnosed preoperatively. Inclusion of this benign entity the time of diagnosis. in the differential diagnoses of symptomatic splenomeg- aly in a pediatric patient is important in the preoperative CASE REPORT management and counseling of the patient and family. In patients who have discrete lesions, consideration of this The patient was an 11-year-old girl with an 8-year history of progressive splenomegaly who was admitted for elective splenec- entity preoperatively may avoid total splenectomy. tomy. She was first seen in 1988 at 3 years of age for splenomegaly. Pediatrics 1998;101(5). URL: http://www.pediatrics.org/ Complete blood count findings were normal. She then developed cgi/content/full/101/5/e10; splenic hamartoma, pancytope- severe thrombocytopenia. Work-up revealed both direct and in- nia, hypersplenism, splenomegaly, hemangiomas. direct platelet antibodies. Antinuclear antibody (ANA) was ,1:20. A bone marrow aspirate showed abundant marrow megakaryo- cytes. A liver spleen scan demonstrated splenomegaly, without a ABBREVIATIONS. ANA, antinuclear antibody; CT, computed to- shift in radionuclide uptake. Computed tomography (CT) images mography; EBV, Epstein–Barr virus; MRI, magnetic resonance showed splenomegaly. Immune thrombocytopenia subsequently imaging. was diagnosed. The patient responded to intravenous gamma- globulin therapy with a rise in platelet count from 11 000/mm3 to 152 000/mm3 in 3 days. Biopsies of a subcutaneous buttock mass okitansky, according to Friedreich, first de- and a left axillary lymph node revealed capillary hemangioma and scribed splenic hamartomas1 .100 years ago reactive hyperplasia, respectively. During the next several years, in 1861. Since then, ;140 cases of true hamar- the spleen continued to enlarge. There were intervening episodes R of urinary tract infection at 4 years of age, Epstein–Barr virus tomas have been reported or mentioned in the liter- (EBV) infection (documented by EBV IgG titer of 640, EBV-EA titer ature. of 320, and EBNA antibody titer of 40), and unclassified glomer- Synonyms have included splenomas, spleen ulonephritis at 7 years of age. Sonograms and repeat CT images within a spleen,2 hemangiomas, posttraumatic scars,3 showed splenomegaly and no focal lesions. fibrotic nodules,4 and hyperplastic nodules. They are When seen in March 1996 at 11 years of age, the patient had symptoms of periodic low-grade fever and night sweats. Physical usually asymptomatic, particularly in the adult pop- examination showed both weight and height to be less than the ulation, and are discovered either incidentally dur- fifth percentile for age. The spleen was then 11 cm below the left ing work-up of other problems or during postmor- costal margin (Fig 1). White blood count was 3800/mm3, with a tem examination. It appears that Von Falkowski normal differential. The hemoglobin was 7.8 g/dL, and the he- matocrit was 22.8%. The red cell indices were as follows: mean reported the first case of splenic hamartoma in a corpuscular volume 70.2 fL, mean corpuscular hemoglobin 24.1 5 pediatric patient in 1914. Approximately 20 of the pg, and mean corpuscular hemoglobin concentration 34.3 dL. The reported cases of splenic hamartoma have been in platelets count was 90 000/mm3. The reticulocyte count was 6%. pediatric patients (,16 years of age). An association The erythrocyte sedimentation rate was elevated at 54 mm/h. with hematologic disorders or other symptoms is Prothrombin time was 44.6 and partial thromboplastin time 39.9. Lactate dehydrogenase was normal. Total bilirubin was slightly rarely reported. Therefore, splenic hamartomas are elevated at 1.4 mg/dL, with direct fraction of 0.1 mg/dL. Bone seldomly included in the differential diagnoses of a marrow findings were consistent with the diagnosis of hyper- left upper quadrant mass in pediatric patients. splenism. Chest x-ray was normal. Tuberculin skin test, coccidio- mycoses, and ANA serology were noncontributory. A splenic arteriogram showed irregular distribution of contrast material and Received for publication Jul 22, 1997; accepted Jan 14, 1998. marked splenomegaly (Fig 2). The patient underwent elective Reprint requests to (T.C.H.) Department of Pathology, Methodist Women’s splenectomy in March 1996. During the operation, the spleen was and Children’s Hospital, 8109 Fredericksburg Rd, San Antonio, TX 78229. noted to be enlarged. An accessory spleen was identified at the PEDIATRICS (ISSN 0031 4005). Copyright © 1998 by the American Acad- hilum, along with large perisplenic lymph nodes. The postopera- emy of Pediatrics. tive course was uneventful, and the platelet count increased to http://www.pediatrics.org/cgi/content/full/101/5/Downloaded from www.aappublications.org/newse10 by guestPEDIATRICS on September Vol.28, 2021 101 No. 5 May 1998 1of6 Fig 1. The enlarged spleen was out- lined, and the tip was measured to be 11 cm below the left costal margin. Fig 2. Splenic arteriogram with nonionic iodinated contrast. within normal range. Examination of the patient 9 months later Histologically, the nodules were less defined and showed an increase in both height and weight and resolution of merged imperceptibly with the surrounding splenic fever and night sweats. The complete blood count and erythrocyte sedimentation rate returned to within normal range. parenchyma. The nodules were composed of irregu- larly arranged sinusoids admixed with intervening PATHOLOGIC FINDINGS pulp cord-like elements (Fig 4). Endothelial cells sim- The resected spleen weighed 921 g (expected ilar to sinusoidal lining cells lined these channels. weight for height is 128 g) and measured 20 3 15 3 These lining cells, by immunoperoxidase stains, 8 cm. The hilum showed several lymph nodes at #8 showed reactivity for CD8 and factor VIII-related mm each. An accessory spleen, measuring 1.5 cm, antigen, consistent with splenic type endothelium. was present. Cut surface of the spleen showed sev- There was no reactivity to CD34, which highlights eral large bulging circumscribed solid nodules that the cord capillaries. The pulp-like elements consisted measured up to 4.5 cm (Fig 3). The largest nodule of lymphocytes and macrophages along with few contained a central tan–white stellate scar. Numer- immunoblasts (Fig 5). There was focal stromal scle- ous similar but smaller nodules ranging from a few rosis, hyalinization, and hemosiderosis. Foreign millimeters to 1.2 cm were noted throughout the body giant cells were present with engulfed pig- spleen. ments. Foci of extramedullary hematopoiesis were 2of6 SYMPTOMATICDownloaded SPLENIC HAMARTOMAfrom www.aappublications.org/news by guest on September 28, 2021 Fig 3. A cut surface of the spleen showing circumscribed nodules of var- ious sizes. Fig 4. The nodule is composed of ir- regularly arranged sinusoids admixed with intervening pulp cord-like ele- ments (hematoxylin–eosin, 100 3). observed. No lymphoid follicles of the white pulp bound vesicles, and mitochondria. Occasional Wei- were identified within these nodules. The rest of the bel–Palade bodies are seen in the lining cells of the spleen and the accessory spleen showed normal vascular channels (Fig 6). components of red and white pulp. The hilar lymph nodes showed mild sinus histiocytosis. DISCUSSION EBV EBER1 transcripts by in situ hybridization test Primary nonlymphomatous tumors of the spleen demonstrated a latent EBV transcript in rare lym- are uncommon. Most are either cysts or hemangio- phoid cells of the spleen and hilar lymph node. This mas. Hamartomas of the spleen are rarely reported result suggested that the patient was a virus carrier entities. The incidence of splenic hamartomas has from previous viral exposure. However, the spleno- been reported to be 3 in 200 000 splenectomies.3 With megaly was not a result of proliferation of EBV- the use of modern radiologic imaging techniques infected lymphocytes. such as sonography, CT, radionuclide scintigraphy, The vascular spaces are lined by cells with ultra- and MRI, splenic hamartoma may not be as uncom- structural features consistent with endothelial cells. mon as thought previously. In fact, one third of the These rounded cells rest on basement membrane cases reported in the past 135 years occurred just in material that is present only intermittently.
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