Adrenal extramedullary haematopoietic tumour

Case contributed by Dr Noriza Zainol Abidin

Presentation

Teenage girl, underlying HbH Constant Spring Thalasemia with right abdominal discomfort.

Patient Data

Age: 16 years old
Gender: Female

Contrast enhanced CT abdomen pelvis

Modality: CT

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.

No ascites.

Impression:  Right adrenal mass with underlying haematological disorder suggestive of extramedullary hematopoietic 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 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.

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Case Information

rID: 51579
Case created: 27th Feb 2017
Last edited: 25th Mar 2017
Inclusion in quiz mode: Included

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