39-Year-Old Man With a Large Adrenal Mass

Neuroblastomas are heterogeneous neuroblastic tumors that occur almost exclusively in childhood. Most neuroblastomas occur in the abdomen, usually in the adrenal gland. Catecholamine secretion may occur in approximately 70% of cases. Patient age at diagnosis, stage of disease, and cytogenetics have prognostic implications

Case Report

The patient was a 39-year-old man who initially presented with abdominal discomfort and symptoms of radiculopathy. In addition, he reported occasional spells with anxiety and episodic elevated blood pressure. Initially, he was treated with physical therapy to improve his symptoms of radiculopathy. However, because of incomplete improvement, spine imaging was recommended. Unexpectedly, magnetic resonance imaging (MRI) of the spine demonstrated metastatic bone disease, and further imaging revealed a large adrenal mass ( Fig. 78.1 ).

INVESTIGATIONS

Workup for catecholamine excess was pursued and demonstrated elevated urinary normetanephrine and dopamine ( Table 78.1 ). A preliminary diagnosis of pheochromocytoma with bone metastases was considered, and open left adrenalectomy was recommended. Appropriate preparation with α- and β-adrenergic blockade was initiated before adrenalectomy. During surgery, in addition to the left adrenal mass, multiple lymph nodes were removed. Surprisingly, pathologic diagnosis was neuroblastoma with lymph node metastases.

TABLE 78.1

Laboratory Tests

Biochemical Test	Result	Reference Range
Cortisol after overnight 1-mg DST, mcg/dL	Not performed	<1.8
Cortisol, mcg/dL	12	7–21
ACTH, pg/mL	Not performed	7.2–63
DHEA-S, mcg/dL	172	65–344
Aldosterone, ng/dL	13	<21
Plasma renin activity, ng/mL per hour	Not performed	2.9–10.8
24-Hour urine metanephrine, mcg/24 h	126	<400
24-Hour urine normetanephrine, mcg/24 h	1582	<900
24-Hour urine epinephrine, mcg/24 h	3	<21
24-Hour urine norepinephrine, mcg/24 h	72	15–80
24-Hour urine dopamine, mcg/24 h	1016	65–400