Dopamine secreting adrenal tumor—ganglioneuroma rather than pheochromocytoma: case report

Abstract

Ganglioneuromas are rare, benign, well-differentiated neural crest tumors arising in the paravertebral sympathetic chain, and are classically non-secretory and clinically asymptomatic. As the diagnosis of ganglioneuroma is based on histopathology, the clinical presentation prior to surgical excision often mirrors that of pheochromocytoma or adrenal cortical adenoma. We describe a case of an incidentally found right sided calcified adrenal mass with evidence of marked dopamine excess, suspicious for pheochromocytoma in a 70-year-old female. The patient endorsed a 6-month history of intermittent right flank pain and a 2-year history of weight loss and fatigue. She reported mild symptoms of hypomania but denied other symptoms of dopamine excess including agitation, anxiety, nausea, and vomiting. Exam revealed isolated mild hypertension. The imaging features of this mass were concerning for malignancy including the presence of macrocalcification and irregular borders. After preoperative alpha blockade, the patient underwent open right adrenalectomy and the final pathology was consistent with ganglioneuroma rather than pheochromocytoma. Following resection, the dopamine level normalized, confirming the resected right adrenal ganglioneuroma as the source of dopamine excess. This case represents a rare presentation of dopamine-secreting adrenal ganglioneuroma. This illustrates that although rare, ganglioneuroma should be included on the differential diagnosis for functional adrenal tumors.

Adrenal ganglioneuroma: Prognostic factors (Review)

“…It has a negative endocrine profile, but it has the potential to mimic other adrenal cancers when it comes to imaging findings; thus, the importance of its removal in order to obtain post-operative pathological identification (10). Non-specific abdominal distention and complaints which are not directly regarded as a marker of poor prognosis have been described; oversecretion of epinephrine/norepinephrine/dopamine is exceptional; also high blood pressure is extremely rarely associated (11,12). Exceptionally, immune-mediated paraneoplasic neurologic syndromes have been reported including: NMDAR (anti-N-methyl-D-aspartate receptor) encephalitis and opsoclonus-myoclonus-ataxia syndrome (13).…”