Essafi Mohammed Amine, Habibi Sanae, Aynaou Hayat, Salhi Houda, El Ouahabi Hanan
Department of Endocrinology, Diabetology, Metabolic Diseases and Nutrition, Hassan II University Hospital Center, Fez, MAR.
Cureus. 2022 Jul 30;14(7):e27492. doi: 10.7759/cureus.27492. eCollection 2022 Jul.
Pheochromocytomas and paragangliomas are rare neuroendocrine tumors developed from chromaffin cells. They are exceptional in children with an atypical symptomatology. We report here a case of a 17-year-old boy who presented with a left retroperitoneal mass discovered on thoracic-abdominal-pelvic computed tomography (CT) scan in the presence of diffuse abdominal pain, more pronounced in the left hypochondrium. The exploration had objectified an exclusive secretion of urinary normetanephrine over 24 hours. Metanephrine and 3 ortho methyldopamine were within normal limits. He had adrenalectomy after controlling his blood pressure with an alpha blocker. Histology had confirmed a pheochromocytoma of non-aggressive potential of the Adrenal gland Scaled (PASS) 2. The evolution was favorable with normalization of blood pressure and urinary catecholamines at one week, three months, six months, and one year. He tested negative for hereditary syndromic including Von Hippel-Lindau (VHL), RET genes, subject to the succinate dehydrogenase complex B and D subunit genes (SDHB-D), which have been requested.
嗜铬细胞瘤和副神经节瘤是起源于嗜铬细胞的罕见神经内分泌肿瘤。它们在儿童中较为特殊，症状不典型。我们在此报告一例17岁男孩，因弥漫性腹痛（左上腹更明显）行胸腹盆腔计算机断层扫描（CT）时发现左腹膜后肿块。检查发现24小时尿去甲肾上腺素分泌异常。间甲肾上腺素和3-邻甲基多巴胺在正常范围内。在用α受体阻滞剂控制血压后，他接受了肾上腺切除术。组织学证实为肾上腺低危嗜铬细胞瘤（PASS）2级。术后一周、三个月、六个月和一年时，血压及尿儿茶酚胺恢复正常，病情进展良好。对其进行了包括冯·希佩尔-林道（VHL）、RET基因、琥珀酸脱氢酶复合物B和D亚基基因（SDHB-D）等遗传性综合征检测，结果均为阴性。
