gms | German Medical Science

Einleitung: Gangliocytic paraganglioma is an extremely rare duodenal tumor located predominantly in the periampullary region. The majority of reported duodenal gangliocytic paraganglioma were benign, but the disease can spread in regional lymphatics.

Material und Methoden: A 56-year old man presenting with episodes of melena. Upper gastrointestinal endoscopy revealed a globular ulcerated mass in the ampullary region. At endosonography, the gangliocytic paraganglioma appeared as a submucosal tumor, measuring 3.4 x 3 cm, in the second portion of duodenum. Magnetic resonance imaging (MRI) showed a solid inhomogenous tumor shifting the distal bile duct and the duct. of Wirsungianus. Positron emission tomography (PET) revealed a solitary circumscribed area of pathologically elevated glucose uptake in the proximal duodenum. The patient underwent pylorus-preserving pancreaticoduodenectomy (Longmire-Traverso) and lymph node dissection of the compartiment II.

Ergebnisse: Pathology evaluation confirmed typical features of a duodenal gangliocytic paraganglioma.Histologically, the tumor consisted of epithelioid cells, neuroid spindle cells, and gangliocytic elements. Immunhistochemical the tumor stained positively for S-100, synaptophysin, NCAM (CD56), NSE and cytokeratin AE1 and AE3.

Schlussfolgerung: We report a case of duodenal gangliocytic paraganglioma included the papilla of Vateri, with the most common clinical presentation and the typical immunohistochemical features. It is a rare tumor with mostly benign character and low malignant potential.In our knowledge it is the first reported case of duodenal gangliocytic paraganglioma demonstrating this lesion with PET. A review of literature confirmed our radical surgical approach by pancreatico-duodenectomy and lymph node dissection of the compartiment II.