Presentation
Known haemoglobin H-constant spring (Hb H-CS) thalassaemia with right abdominal discomfort.
Patient Data
There is a well circumscribed heterogeneous large right adrenal mass measuring 6.7 x 6.7 x 9.1 cm (AP x ML x CC). It has central necrosis. The soft tissue component demonstrates enhancement on the portovenous phase. No calcification or fatty component within. No surrounding fat streakiness.
On coronal images, it causing mass effect onto the right kidney.
Hepatosplenomegaly and cholelithiasis.
No ascites.
Impression: Right adrenal mass with underlying haematological disorder suggestive of extramedullary haematopoietic tumour.
Ultrasound guided biopsy of the right adrenal mass was performed.
Histopathological examination revealed proliferation of erythroblasts, myeloblasts and megakaryocytes, along with red blood cells and haemosiderin-laden macrophages. Findings consistent with adrenal extramedullary haematopoiesis.
Case Discussion
Extramedullary haematopoiesis (EMH) is a compensatory mechanism in response to an imbalance between bone marrow erythropoiesis and circulatory blood demand. It occurs most often due to haemolytic anaemias e.g. thalassaemia, hereditary spherocytosis, and sickle cell disease.
Adrenal EMH tumour should be considered in thalassaemic patients with an adrenal mass, thus avoiding unnecessary surgical procedures.
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