Pruiti Vincenzo, Mazzeo Francesca, Rossitto Maurizio, Foti Agata, Macrì Antonio, Cucinotta Eugenio
Ann Ital Chir. 2014 Dec 29;85(ePub):S2239253X14023469.
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.
We report a case of a 76-year-old man who was submitted to a subtotal gastrectomy with omentectomy 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.
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 distribution, catecholamine production, risk of metastasis, and association with others types of tumors.
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 status of malignancies.
副神经节瘤是神经嵴衍生的神经内分泌肿瘤，起源于副神经节，副神经节是由分泌儿茶酚胺和肽的细胞构成的散在神经内分泌器官。副神经节瘤的年发病率估计为1/100,000，占分泌儿茶酚胺肿瘤的10%。
我们报告一例76岁男性患者，该患者接受了胃次全切除术、网膜切除术和胃空肠吻合术。组织学检查显示为溃疡性息肉样胃癌，细胞黏附性差，侵犯胃肌层，同时偶然发现胃壁病变，为副神经节瘤，免疫细胞化学检查显示神经元特异性烯醇化酶（NSE）阳性，CD117、S100、CD34和平滑肌肌动蛋白（SMA）阴性。
嗜铬细胞瘤仅指起源于肾上腺髓质的肿瘤，而肾上腺外副神经节瘤是指肾上腺髓质外嗜铬细胞的肿瘤。胃或胃旁部位的副神经节瘤，如我们病例中的情况，非常罕见，文献中仅有个别病例报道。30%的此类肿瘤存在遗传易感性，可导致遗传性疾病，其特征为肿瘤分布、儿茶酚胺产生、转移风险以及与其他类型肿瘤的关联存在差异。
对于无症状患者，以及神经内分泌肿瘤的生化和临床怀疑强烈时，必须进行解剖学和功能检查以检测这些肿瘤。可切除肿瘤的一线治疗是完整手术切除，可通过开放手术或腹腔镜技术进行。当恶性肿瘤因转移状态无法完全根除疾病时，手术治疗也可用于姑息治疗目的。
