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Borderline Myxoid Adrenocortical Neoplasm: A Case Report
Maria-Anthi Chantzi1, Theodoros Sidiropoulos2, Spyridon Christodoulou2
1School of Medicine, National and Kapodistrian University of Athens, Athens, GRC.
Abstract:
Myxoid adrenocortical neoplasm (MAN) is an exceptionally rare variant of adrenal tumor arising from the cortex, with limited cases reported globally. A minor proportion of these are classified as having borderline malignant potential (BMAN). Typically, these neoplasms exhibit a biphasic histological pattern, with both myxoid and conventional non-myxoid regions. Their identification remains challenging, particularly when the myxoid component predominates. The presence of extensive myxoid stroma can obscure traditional histological criteria crucial for malignancy assessment, such as those outlined in the Weiss criteria, thus complicating risk stratification. We report the case of a 34-year-old female patient who presented to the outpatient clinic with a three-month history of persistent headaches, episodic hypertension, and fatigue. Her blood pressure fluctuated between 140-200 and 90-110 mmHg. Imaging revealed an incidental adrenal mass. No significant past medical history or family history of hypertension or cardiovascular disease was reported. The patient subsequently underwent a posterior retroperitoneoscopic adrenalectomy of the left adrenal gland. Histopathological analysis revealed a tumor with prominent myxoid features and a low Weiss score (2), without evidence of capsular or periadrenal invasion, confirming the diagnosis of BMAN. The rarity and distinct histomorphological features of BMANs complicate their diagnosis. This case contributes to the limited literature and underscores the diagnostic challenges involved. The inclusion of these tumors in the differential diagnosis of adrenal masses, particularly when related hormonal abnormalities are present, as well as thorough clinical and histopathological evaluation, is crucial. The favorable outcome of the patient highlights the significance of early diagnosis and complete surgical excision. However, further research is essential for understanding the biological behavior of these neoplasms and for refining the diagnostic criteria and management strategies.