Endocrine Abstracts

Introduction: Extramedullary hematopoiesis (EMH) is most commonly seen in reticuloendothelial system tissues such as liver, spleen and lymph nodes. Rarely, it can be detected in non-hepatosplenic atypical localizations such as the adrenal glands. Although the underlying mechanism is not clearly elucidated, it is suggested that the adrenal glands have hematopoietic activity in the fetal period and EMH may develop from the remains here due to chronic anemia. We aimed to present a case of myelodysplastic syndrome (MDS) presenting with an adrenal mass that secretes autonomic cortisol and contains EMH foci.

Case Report: A 67-year-old female patient was diagnosed with MDS 5 years ago and erythropoietin treatment was started due to anemia. In the abdominal magnetic resonance imaging (MRI) performed 4 years ago, a 33*25 mm adenoma with signal loss in the external phase sequence was detected in the right adrenal gland. Cortisol level was not suppressed in the 1 mg dexamethasone suppression test (DST) performed for hypercortisolemia. As a result of further investigations (Table 1), autonomic cortisol hypersecretion originating from adrenal adenoma was considered. Pheochromocytoma and hyperaldosteronism tests were normal. It was decided to follow-up because there was no indication for treatment. No significant change was observed in adenoma size and clinic in 3-year follow-up. However, in the control abdominal MRI performed at the end of the 4th year, it was observed that there was a minimal increase in the size of the lesion and heterogeneous signal loss that was not present in the previous imaging. PET CT was performed to exclude malignancy since collision tumor was prediagnosed in MRI findings, no metastasis was detected, then laparoscopic right adrenalectomy was performed. Microscopic and immunohistochemical examination revealed an cortisol-secreting adrenocortical adenoma containing EMH foci. She was referred to the hematology department due to the development of adrenal EMH due to MDS and chronic anemia.

Conclusion: The development of EMH foci due to MDS in a stable autonomic cortisol-secreting adrenocortical adenoma is not a common condition, therefore our patient is a rare case in the literature. It should be kept in mind that EMH may develop in atypical localizations in hematological diseases.