Rare Case of Large Catecholamine Secreting Ganglioneuroma in an Asymptomatic Elderly Male

Abstract

Ganglioneuromas (GNs) are benign tumors that originate from neural crest cells, composed mainly of mature ganglion cells. These tumors, which are usually hormonally silent, tend to be discovered incidentally on imaging tests and occur along the paravertebral sympathetic chain, from the neck to the pelvis and occasionally in the adrenal medulla. Rarely, GNs secrete catecholamines.¹ Adrenal GNs occur most frequently in the fourth and fifth decades of life, whereas GNs of the retroperitoneum and posterior mediastinum are usually encountered in younger adults.² Adrenal GNs are commonly hormonally silent and asymptomatic; even when the lesion is of substantial size.³ We report an incidentally detected asymptomatic case of an adrenal ganglioneuroma with mildly elevated urinary catecholamine levels in an elderly male. After preoperative alpha blockade, the patient underwent open right adrenalectomy. Upon microscopic examination, the right adrenal mass proved to be a ganglioneuroma, maturing type and the immunohistochemistry examination showed immunoreactivity to synaptophysin, chromogranin, and CD 56, while S100 was strongly positive at the Schwannian stroma. Following resection, catecholamine levels normalized, confirming the resected right adrenal ganglioneuroma as the source of the catecholamine excess. This case represents a rare presentation of catecholamine-secreting adrenal ganglioneuroma in the elderly.

Keywords: adrenal glands; catecholamine; ganglioneuroma.

Figure 1

Coronal Section Contrast-Enhanced CT Adrenal images in portovenous phase showing a large heterogeneous enhancing right adrenal mass with no calcification or fat component within (arrow). The density of the mass on the plain study is 41 HU, in portovenous phase 53 HU, and delayed study 71 HU.

Figure 2

Macroscopic findings. (A) Right adrenalectomy specimen: Large, well-circumscribed lobular mass weighing 1167 g and measuring 155 × 140 × 85 mm. The mass is smooth and shiny on the outer surface with some attached fatty tissues. Fine vessels are seen on its brownish-grey surface; (B) Right adrenalectomy specimen. Cut surface showing soft dark brownish multicystic areas with extensive hemorrhage. The cystic spaces range from 5-20 mm in diameter.

Figure 3

Histopathology of the mass. (A) Ganglioneuroma. The neoplastic cells are arranged in clusters set in loose fibrocollagenous stroma (H&E, 4x); (B) Ganglioneuroma. Neoplastic cells exhibiting mature ganglion cells with eccentric nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm with surrounding Schwannian stroma and spindle cells (H&E, 20x). Ganglioneuroma. Immunohistochemical stains show that the neoplastic cells are positive for (C) Synaptophysin, (D) Chromogranin, (E) CD56, and (F) negative for GFAP.