[Gangliocytic paraganglioma--a rare cause of an upper gastrointestinal bleeding]

Introduction: Gangliocytic paraganglioma is a rare tumour, occurring nearly exclusively in the descending part of the duodenum. It is regarded as a mostly benign tumour but of unknown malignant potential, which rarely metastasises to local lymph nodes or distantly.

Case report: Here we report on a 62-year-old female patient with a marked microcytic anaemia with a haemoglobin concentration of 3.4 mg/dL. Oesophagogastroduodenoscopy showed an ulcerous periampullary tumour in the duodenum with a diameter of approximately 5 cm. Endoscopic ultrasonography showed no evidence of tumour infiltration of the tunica muscularis and of locoregional lymph node metastasis. Therefore, complete endoscopic resection of the tumour was achieved after ligating the tumour base by an endoloop using a dual channel endoscope. In a second step, the tumour base was resected by endoscopic submucosal dissection (ESD) and revealed no residual tumour. The histological evaluation showed a gangliocytic paranganglioma consisting of three specific cell types: epithelioid cells arranged in typical carcinoid-like patterns, spindle cells wrapped around nests of epithelioid cells and ganglion cells. All cell types expressed neuron-specific enolase (NSE) as a neuroendocrine marker. Synaptophysine was expressed mainly by the epithelioid and ganglion cells while the protein S 100 was expressed mainly by the spindle cells, which surround the epithelioid cell nests as a sustentacular network. The proliferation rate determined by Ki67 staining was only < 5 %.

Conclusion: Gangliocytic gangliocytomas of the duodenum can be safely removed by endoscopic submucosal dissection as long as there is no evidence of infiltration of the tunica muscularis or of local lymph node metastasis. Because of the unknown malignant potential, these patients have to be controlled by regular ultrasonographic and endosonographic procedures.