A mild acute pancreatitis and adrenal incidentaloma: only two synchronous findings?

Background Ganglioneuroma (GN) is a rare tumor arising from the neural crest tissue and is most commonly located in the posterior mediastinum and retroperitoneum, and rarely in the adrenal gland. The prevalent use of imaging procedures such as ultrasonography (US) and computed tomography (CT) is increasing the number of incidental diagnosis of adrenal GNs. Adrenal GN is usually hormonally silent, and can therefore asymptomatic even when the size of tumor is large 1. Case history A 43-year-old healthy female went to the ED for epigastric pain and vomiting. Her medical history included cholecystectomy for cholelithiasis. The laboratory examinations at the entrance showed an increase in pancreatic enzymes, the abdominal US was inconclusive as it was hindered by abundant meteorism. In 48 hours we observed a regression of the symptoms and a normalization of the laboratory tests. The patient underwent instrumental examinations such as abdominal CT and MRI, with findings of a polylobed expansive formation (67 x 65 mm) in the left adrenal lodge, dislocating pancreatic tail, splenic vein, left kidney and spleen, and with a thin cleavage plane (Fig.1). Hormonal tests and tumor markers were negative. The patient was referred to the surgical ward for exeresis of the neoformation. Histological examination was conclusive for a ganglioneuroma-type adrenal neuroblastic tumor. Discussion GN is a rare, differentiated, benign, and slow-growing tumor arising from the neural crest tissue of the sympathetic nervous system, and is histologically composed of mature Schwann cells and ganglion cells with fibrous stroma. Recent studies have shown that the mean age at diagnosis of GN is around 40 to 50 years old. It is estimated that an adrenal tumor is incidentally found in 1-10% of cases on abdominal CT, and 1-6% of these are GNs. Adrenal GN is usually hormonally silent, however some adrenal GNs occasionally secrete catecholamine’s and their metabolites, because they can occur as a composite tumor with pheochromocytoma (PC), and they rarely secrete cortisol and androgen. GN often has radiological phenotype characteristics such as those seen in our case Careful evaluation by endocrine examinations and multiple imaging procedures is necessary to provide a differential diagnosis from other adrenal Tumors such as PC and Adrenocortical carcinoma. The first choice of treatment for adrenal GN, especially for large Tumors, is surgical resection. We assumed that the episode of mild acute pancreatitis in the patient may have been favoured by the extrinsic compression of GN.

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