Adrenal ganglioneuroma

Abstract

SUMÁRIOIncidentalomas adrenais (IA) são massas descobertas ao acaso na investigação de distúrbios não relacionados a patologias das glândulas adrenais e tem se tornado cada vez mais frequentes. A maioria dos casos é constituída por adenomas não secretores, embora possa também representar uma neoplasia maligna primária ou metastática. Porém, no diagnóstico diferencial, doenças menos prevalentes não podem ser esquecidas. Relatamos um caso de massa adrenal em mulher jovem, sem sinais de hiperfunção clínica ou laboratorial. O diagnóstico foi feito após episódio de pielonefrite aguda, em que o exame de imagem foi realizado para excluir complicações locais. Durante a abordagem cirúrgica, o aspecto em carne de peixe da lesão fez suscitar a hipótese de uma lesão de origem neural, a qual foi prontamente definida pelo exame histopatológico. A paciente se apresentava com a manifestação mais comum do ganglioneuroma de adrenal, um incidentaloma. Arq Bras Endocrinol Metab. 2012;56(4):270-4 SUMMARY Adrenal incidentalomas (AI) are unsuspected adrenal masses discovered during investigation of unrelated diseases, and are increasing in frequency. The majority of the AI is non-secretory adenomas, although it can also represent primary or metastatic malignant neoplasia. However, less frequent diseases should not be forgotten in the differential diagnosis. We describe a case of a young woman with an adrenal mass without clinical or laboratorial signs of hormonal hyperfunction. Diagnosis was performed after an episode of acute pyelonephritis in which the imaging study was carried out in order to exclude local complications. During the surgical procedure, the fish flesh aspect of the mass raised the suspicion of a neurogenic tumor, which was diagnosed in the histophatological analysis. The patient presented the most common manifestation of adrenal ganglioneuroma, an incidentaloma. Arq Bras Endocrinol Metab. 2012;56(4):270-4

Occurrence of paratesticular ganglioneuroma 18 years after concurrent adrenal ganglioneuroma and papillary thyroid carcinoma – a case report

“…The majority of adrenal GNs manifest as adrenal incidentalomas, and a correct diagnosis is difficult to obtain preoperatively. It has been reported that more than 85% of adrenal incidentalomas are benign nonfunctioning adenomas [15], whereas some have hormone manifestations such as Cushing’s syndrome or pheochromocytoma. Thus, hormonal screening tests should be assessed to exclude functional tumours by means of the evaluation of urinary free cortisol and late-night salivary cortisol, the 1-mg overnight dexamethasone suppression test for Cushing syndrome, and the assessment of urinary catecholamines/metanephrines for pheochromocytoma [15].…”

Report on 5 Cases of Adrenal Ganglioneuromas

“…Generally, ganglioneuroma has been considered to occur mainly in children and young adults, and to be most commonly diagnosed between the mean ages of 5.5 to 10 years (4,12,13). However, recent reports suggest that the mean age at diagnosis is 40 to 50 years (2,5,(14)(15)(16)(17). GN is slightly more common in women than in men (1.13:1 to 1.5:1) (1, 4).…”

“…In order to distinguish between these tumors some authors advocate performing MIGB and PET scans (14,15). The 123I-MIBG scan is considered to be highly specific for pheochromocytoma, although Geoerger et al report that approximately 57% of GNs also take up MIBG, which is often associated with increased catecholamine secretion by the tumor (1).…”