A Unique Combined Ganglioneuroma Schwannoma Tumor Mimicking Adrenal Malignancy

A 28-year-old woman with a past medical history significant for cervical cancer was diagnosed with a 2.5 cm adrenal tumor but was lost to follow-up. Two years later, she presented to the emergency room with worsening right upper abdominal and flank pain. The computed tomography (CT) and magnetic resonance imaging (MRI) of the abdomen and pelvis revealed that the right adrenal mass had nearly doubled in size (4.3 cm), was heterogeneous with calcifications, central necrosis and actively uptaking the intravenous (IV) contrast with a delayed washout. The biochemical workup was negative for hyperaldosteronism, hypercortisolism, and pheochromocytoma. She reported an unintentional body weight loss of 40 pounds. Adrenocortical carcinoma or a metastatic malignancy was high on the differential diagnoses list. She underwent a successful laparoscopic adrenalectomy, and final pathology revealed a benign extra-adrenal combined ganglioneurofibroma and schwannoma. These rare benign malignancies alone or in combination may closely mimic the clinical and imaging characteristics of adrenal malignancy and pose a diagnostic and therapeutic dilemma to surgeons as well as cause a significant distress to patients and their families. Thus, it is important to thoroughly document and report these cases in order to increase awareness and improve our understanding of the biology, natural history and management of these extremely rare tumors.