Servicio de Endocrinología y Nutrición, Complejo Asistencial Universitario de Salamanca, Salamanca, Spain.
Servicio de Endocrinología y Nutrición, Complejo Asistencial Universitario de Salamanca, Salamanca, Spain.
Endocrinol Diabetes Nutr (Engl Ed). 2024 Aug-Sep;71(7):317-320. doi: 10.1016/j.endien.2024.03.023. Epub 2024 Aug 1.
Paragangliomas (PGLs) are rare and encapsulated neuroendocrine tumors (NET), located in the adrenal gland or the extra-adrenal paraganglia. Extra-adrenal PGLs may develop a gangliocytic component with ganglion cells which are called gangliocytic paragangliomas (GPs). The most common location is the duodenum, and they appear with digestive symptoms or as an incidental finding. We described a 43 years old patient, with epigastric pain, nausea and vomiting. The CT-scan reveals a nodular image in the duodenum. An ultrasound-guided FNA was performed and the pathological report revealed neuroendocrine cell groups and neural tissue. Surgery was the chosen treatment. As the patient did not present lymphatic or pancreatic parenchyma invasion, radiotherapy (RT) was not administered. The management of GPs is not well established and multidisciplinary team approach is recommended to lead to therapeutic options. Surgical resection is still key in the treatment, and adjuvant RT may be considered in cases of lymph node invasion.
嗜铬细胞瘤（PGL）是罕见的包裹性神经内分泌肿瘤（NET），位于肾上腺或肾上腺外副神经节。肾上腺外 PGL 可能会发展出具有神经节细胞的神经节细胞成分，称为神经节细胞瘤（GP）。最常见的部位是十二指肠，它们可能表现为消化症状或偶然发现。我们描述了一位 43 岁的患者，表现为上腹痛、恶心和呕吐。CT 扫描显示十二指肠有结节状影像。进行了超声引导下细针穿刺活检，病理报告显示神经内分泌细胞群和神经组织。选择手术治疗。由于患者没有淋巴结或胰腺实质侵犯，未进行放疗（RT）。GP 的治疗方法尚未确定，建议采用多学科团队方法以确定治疗选择。手术切除仍然是治疗的关键，对于淋巴结侵犯的病例可考虑辅助放疗。
