Diaphoresis as the Prominent Manifestation of Pheochromocytoma

Pheochromocytoma is a rare neuroendocrine tumor, affecting 0.6 to 0.8 of 100,000 people per year. The "classic triad" of headache, diaphoresis, and tachycardia is well documented in the literature, although its clinical utility has come into question. Diaphoresis is part of the "classic triad" and occurs in <50% of patients with pheochromocytoma. There are few reports of diaphoresis as the sole symptom of pheochromocytoma. Our objective is to report a patient with diaphoresis as the only prominent manifestation of pheochromocytoma.

A 20-year-old man presented with 5 years of worsening diaphoresis; diffuse, but predominantly in the upper half of his body. No other symptoms were present. His blood pressure was 138/82 mm Hg and had a heart rate of 60 bpm. The physical examination was unremarkable. Thyrotoxicosis, infection (including tuberculosis), and lymphoma/leukemia were ruled out. The 24-hour urine norepinephrine level was 1002 ug/24hours (0-135 ug/24 hours), plasma normetanephrine was 2873 pg/mL (0-145 pg/mL), and plasma norepinephrine was 2869 pg/mL (0-874 pg/mL). Computed tomography of the abdomen revealed a 4.0 × 3.1 × 4.3 cm left adrenal mass. After pre-operative preparation with doxazosin, the patient underwent laparoscopic left adrenalectomy. The diaphoresis resolved. Pathology confirmed a completely resected pheochromocytoma. Genetic testing for germline mutations was negative.

This patient was a young adult and did not exhibit features of pheochromocytoma common to the pediatric/adolescent or adult populations. Monosymptomatic presentations are sparse in the literature.

This case reflects an atypical presentation of pheochromocytoma, a disease with high cardiovascular morbidity and mortality, and helps to establish the need to better quantify individual symptoms of patients to better understand the entire spectrum of this disease.