Diagnostic challenges: suspected adrenal myeloid sarcoma

Kouloud Hachani; Sabrine Mekni; Sawsen Essayeh; Rihab Laamouri; Meriem Werfelli; Nadia Mchirgui; Karima Khiari; Imen Rojbi; Nacef Ibtissem Ben

doi:10.1530/endoabs.99.EP1265

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Endocrine Abstracts (2024) 99 EP1265 | DOI: 10.1530/endoabs.99.EP1265

ECE2024 Eposter Presentations Late Breaking (127 abstracts)

Diagnostic challenges: suspected adrenal myeloid sarcoma

Kouloud Hachani ¹ , Sabrine Mekni ^1,2 , Sawsen Essayeh ^1,2 , Rihab Laamouri ¹ , Meriem Werfelli ¹ , Nadia Mchirgui ¹ , Karima Khiari ¹ , Imen Rojbi ^1,2 & Ibtissem Ben Nacef ^1,2

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¹Charles Nicolle Hospital, Tunis, Tunisia; ²Laboratory of Renal Pathology LR00SP01, Tunis, Tunisia

Introduction: Myeloid sarcoma is a rare etiology of adrenal tumors, with both diagnostic and therapeutic difficulties. This clinical case highlights the diagnostic challenges inherent in the coexistence of these two pathologies, underscoring the complexity of their management.

Case report: We present the case of a 40-year-old woman with a history of polycystic ovary syndrome since her youth. She was referred to our department to explore adrenal incidentaloma as a CT scan of the abdomen showed a 55 × 49 mm heterogeneous adrenal mass with a density of 35 HU. Investigations revealed a suspicious, non-secreting mass, with morning cortisol levels after a 1-mg overnight dexamethasone suppression test at 22 nmol/l and normal Urinary metanephrins and normetanepherins, testosterone and SDHEA levels. Clinicobiological assessments and CT chest-abdomen-pelvis excluded metastasis from another cancer. The patient was referred to the urology department for total adrenalectomy, but she developed (2month later) bicytopenia, presenting with anemia at 5 g/dl and thrombocytopenia at 50, 000 / microliter. Faced with the hematological emergency, surgery and biopsy could not be performed, and the patient was referred to the hematology department. The diagnosis of acute myeloid leukemia was established, and the patient underwent chemotherapy (protocol combining intensive induction chemotherapy, and consolidation treatment ). The course was marked by a significant decrease in adrenal tumor size by 47% in the adrenal CT scan follow up. Given this evidence, the most probable diagnosis appears to be myeloid sarcoma of the adrenal gland.

Conclusion: Myeloid sarcoma can develop before the onset of acute myeloid leukemia (Aml), concurrently with AMl, or during Aml relapse. Its localization in various organs is possible, however, adrenal localization remains rare. Diagnosis can be concluded by clinical and evolutive evidence, but definitive confirmation always relies on histopathology.