Gastric paraganglioma: A case report and a review of the literature. Published online (EP) 29 December 2014 - Ann. Ital. Chir 1 Pervenuto in Redazione Novembre 2014. Accettato per la pubblicazione Dicembre 2014 Correspondence to: Maurizio Rossitto, MD, University of Messina, Department of Human Pathology, General Surgery Unit, Via Consolare Vaeria, 98125 Messina, Italy (e-mail: rossitt@unime.it) Vincenzo Pruiti*, Francesca Mazzeo**, Maurizio Rossitto*, Agata Foti*, Antonio Macrì*, Eugenio Cucinotta* University of Messina, Messina, Italy *General Surgery Unit, Department of Human Pathology **Pathological Anatomy, Department of Human Pathology Gastric paraganglioma: A case report and review of the literature. AIM: Paragangliomas are neural crest-derived neuroendocrine tumors, originating from paraganglia, which are dispersed neuroendocrine organs characterized by catecholamine and peptide-producing cells. With an annual incidence estimated at 1/100,000, paragangliomas represent 10% of catecholamine secreting tumors. MATERIAL OF STUDY: We report a case of a 76-year-old man who was submitted to a subtotal gastrectomy with omen- tectomy and gastrojejunal anastomosis. The Hystologic exam has revealed an ulcerative polypoid gastric carcinoma with cell poorly cohesive and infiltration of the muscular gastric wall and an incidental parietal gastric lesion which was a paraganglioma with immunocytochemical investigations positive for NSE and negative for CD117, S100, CD34 e SMA. DISCUSSION: Pheochromocytoma indicates exclusively tumors arising from the adrenal medulla, while the extra-adrenal paraganglioma suggests tumors of the chromaffin cells with other locations. Gastric or paragastric localization, as in our case, is very rare for these neoplasms, and in literature there are only isolated case reports. Genetical predisposition is observed in 30% of these tumors and can be responsible of hereditary disease characterized for differences in tumor dis- tribution, catecholamine production, risk of metastasis, and association with others types of tumors. CONCLUSION: In asymptomatic patients and when biochemical and clinical suspicion of neuroendocrine tumor is strong, you have to perform anatomical and functional investigations to detect these neoplasms. The first line treatment for resectable tumors is complete surgical resection, that can be performed with open surgery or laparoscopic technique. Surgical therapy is also indicated to palliative intent when a complete eradication of disease is not achievable for metastatic sta- tus of malignancies. KEY WORDS: Autonomic nervous system, Gastrectomy, Gastric cancer, Gastric paraganglioma associated with genetic syndromes. The clinical presentation depends on secretion of catecholamines, malignancy or mass effect; paragangliomas may also be discovered incidentally and they are called “incidentalomas” [1,2] . Most paragangliomas are benign and are characterized by a strong vascularization; about 20% are malignant with poor survival. We report a rare case of parietal gastric paraganglioma fortuitously detected during intraoperative exploration on subtotal gastrectomy performed for gastric cancer endoscopically diagnosed. If this cancer is uncommon, gastric localization even more. Therefore, we briefly discuss the clinical and therapeutic approach for these tumors and the cytomorphological features which are important also for diagnosis. Ann. Ital. Chir. Published online (EP) 29 December 2014 pii: S2239253X14023469 www.annitalchir .com Introduction Paragangliomas are rare neuroendocrine tumors arising from the embryonic neural crest. They may be located from the upper cervical region to the pelvis, related to the autonomic nervous system. The majority of paragangliomas appear to be sporadic but there are forms READ-ONLY COPY PRINTING PROHIBITED