Renal Splenic Heterotopia With Extramedullary Hematopoiesis in a Thalassemic Patient, Simulating
Renal Neoplasm: A Case Report
Mehran Hiradfar, MD,* Nona Zabolinejadm, MD,* Abdolla Banihashem, MD,*
and Abdol-Mohammad Kajbafzadeh, MDw

Summary: Intrarenal splenic tissue (splenorenal heterotopia) is a rare condition. Simultaneous existence of splenorenal heterotopia and extramedullary hematopoiesis in a same patient can present as a mass of kidney. We present an interesting case of splenorenal heterotopia with extramedullary hematopoiesis in a thalassemic child simulating as a renal neoplasm. This is the first reported case in the right kidney.

Key Words: spleen, renal, thalassemia, heterotopia, neoplasm

(J Pediatr Hematol Oncol 2007;29:195–197) Heterotopia (or choriostoma) is applied to microscopically normal cells or tissues, which are present in an abnormal location. Aside from normal location of spleen, this tissue could be found as an accessory spleen, splenovisceral fusion, and splenosis. Heterotopic splenic tissue in the kidney can occur in patients with intact spleens (splenorenal fusion) or those who had had splenic trauma or had been splenectomized (splenosis).
Despite from many reports of splenogonadal (splenic-mesonephric) fusion,1 splenorenal (splenometanephric)
fusion is a very rare condition, which was first reported by Gonzalez-Crussi et al in 1977.2 Here, we describe a case of heterotopic spleen with extramedullary hematopoiesis within the right kidney of a boy with major thalassemia simulating primary renal neoplasm.

CASE REPORT
An 11-year-old thalassemic boy was referred for splenectomy owing to need for transfusion, which exceeded 200mL of packed red cells/kg per year. Complete blood count (WBC) was
2100/mL (normal=5000 to 10,000/mL) with 36% segmented neutrophils, 62% lymphocytes and 2% eosinophiles, hematocrit (Hct) 19.9%, hemoglobin 6.8 g/dL, mean corpuscular volume=78.3 fL, mean corpuscular hemoglobin=26.8 pg,