Adrenal extramedullary haematopoietic tumour
Teenage girl, underlying HbH Constant Spring Thalasemia with right abdominal discomfort.
Contrast enhanced CT abdomen pelvis
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There is a well circumscribed heterogeneous large right adrenal mass measuring 6.7 x 6.7 x 9.1cm (APxWxCC). 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.
Impression: Right adrenal mass with underlying haematological disorder suggestive of extramedullary hematopoietic tumour.
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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.
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 hemolytic anaemia such as thalassemia, hereditary spherocytosis, and sickle cell anaemia.
Adrenal EMH tumour should be considered in thalassemic patients with an adrenal mass, thus avoiding unnecessary surgical procedures.