A Case Report: A rare primary adrenal malignant rhabdoid tumor in a 42-year-old man

Background Primary adrenal malignant rhabdoid tumor (MRT) is a rare, highly aggressive neoplasm lacking specific clinical manifestations and imaging characteristics. Methods This case report delineates the clinical course of a patient with primary adrenal MRT managed at our institution. We provide a comprehensive characterization of the patient’s clinical presentation, histopathological and molecular genetic profiles, and the therapeutic strategy employed. Furthermore, we critically examine contemporary evidence regarding its pathogenesis, diagnostic challenges, and therapeutic strategies. Results A 42-year-old man was initially admitted to the Department of Endocrinology presenting with pyrexia and an adrenal mass. The patient underwent surgical resection of an adrenal mass; definitive histopathological examination and molecular genetic profiling confirmed a diagnosis of primary adrenal MRT. Despite undergoing surgical resection and adjuvant chemotherapy, the patient ultimately succumbed to sudden cardiac death. Conclusion Primary adrenal MRT represent an exceptionally rare clinical entity. Consequently, clinicians must maintain high suspicion for this diagnosis when evaluating non-functioning adrenal masses, especially those with atypical radiological features or aggressive behavior.