A 53‐year‐old African American man presented with 1 month of diffuse abdominal pain associated with nausea, emesis, and anorexia. He also reported 15 pounds of unintentional weight loss and fatigue. He denied any medical history beyond an episode of Bell's palsy of the left side in 1997. He is a cigarette smoker with a history of 35 pack‐years. Vital signs were stable on admission. Physical examination revealed normoactive bowel sounds, abdominal tenderness to palpation with guarding. No masses were appreciated due to pain on minimal palpation. Computed tomography (CT) scanning confirmed a 9.1‐cm heterogeneous and necrotic left adrenal lesion, multiple necrotic mesenteric lymph nodes, a 4.3‐cm retroperitoneal necrotic mass inferior to the cecum, two cavitated masses in the right upper lobes, and scattered nodules in the left upper lobes. Initial laboratory studies revealed the following: normal complete blood count, renal and liver function tests, amylase, lipase, lactate and coagulation panel. LDH was 1222 U/L, and uric acid was 3.6 mg/dL. Urine and serum metanephrines were sent and pending. Ultrasound guided core lymph node biopsy was performed. The patient developed progressive sensorineural hearing loss while pathology results were pending. A slight left ear hearing difficulty he endorsed on presentation worsened to complete bilateral hearing loss over one week. MRI of the brain showed leptomeningeal disease with bilateral metastases to the internal auditory canals (Fig. 1). Biopsy results revealed malignant histology, and immunocytochemical stains positive for chromogranin and synaptophysin. These findings were diagnostic of metastatic pheochromocytoma, and consistent with initial laboratory studies showing urine metanephrines 541 μg/24h and chromogranin A 261 ng/mL.
The classically described pheochromocytoma is a neuroendocrine tumor of the adrenal glands that over secrete neurogenic amines. A flood of catecholamines drives the syndrome of episodic headaches, sweating, elevated blood pressure and other signs and symptoms of sympathetic hyperactivity. The presentation in our patient was remarkably dissimilar. The abdominal pain, nausea, emesis, and progressive hearing loss could be partly explained by his advanced disease state; however, infiltration of the auditory canals with resultant hearing loss has not been described in malignant pheochromocytomas. Distant metastases most often occur in the lymph nodes, liver, lung, and skeleton. Once metastasized, the clinical presentation is highly variable and often marked by nonspecific signs and symptoms of local disease, and lack of neuroendocrine symptoms. Metastasis with auditory tropism has not been described.
Pheochromocytoma is a disorder with a highly variable clinical presentation that when advanced can present with symptoms of local infiltration in many systems, including the auditory canals.
To cite this abstract:Cho J, Belletti L. Abdominal Pain and Hearing Loss: An Unusual Presentation of Malignant Pheochromocytoma. Abstract published at Hospital Medicine 2013, May 16-19, National Harbor, Md. Abstract 363. Journal of Hospital Medicine. 2013; 8 (suppl 2). https://www.shmabstracts.com/abstract/abdominal-pain-and-hearing-loss-an-unusual-presentation-of-malignant-pheochromocytoma/. Accessed April 5, 2020.