Loukili Mohamed Aymane, Assarrar Imane, El Yamani Nada, Haloui Anass, Rouf Siham, Latrech Hanane
Department of Endocrinology-Diabetology and Nutrition, Mohammed VI University Hospital Center, Faculty of Medicine and Pharmacy, University of Mohammed 1st, Oujda, Morocco.
Department of Anatomical Pathology, Mohammed VI University Hospital Center, Faculty of Medicine and Pharmacy, University of Mohammed 1st, Oujda, Morocco.
Int J Surg Case Rep. 2023 Aug;109:108547. doi: 10.1016/j.ijscr.2023.108547. Epub 2023 Jul 21.
Phaeochromocytomas and paragangliomas are rare neuroendocrine neoplasms that grow outside the adrenal gland and arise from the primitive neural crest cells. The retroperitoneal location is extremely rare with an incidence of 2-8 per million.
Here we report a case of an 80 years old man presenting with abdominal pain and vomiting associated with hypertensive peaks and weight loss. CT scan showed a retroperitoneal para-aortic tumor invading the inferior vena cava, with significantly elevated urinary catecholamine levels. Histopathological and immunohistochemistry examinations confirmed the diagnosis of paraganglioma. A medical preparation by alpha-blockers was performed. Complete resection of the tumor with the reconstruction of the vena cava was achieved without postoperative complications. After surgery, blood pressure and HbA1c were on the targets and the urinary catecholamine levels were normal.
The diagnosis of paragangliomas is suspected by clinical symptoms in the case of functional paragangliomas and the confirmation is biological by the plasmatic or urinary catecholamines. Non-functional paragangliomas often represent a diagnostic challenge. In our case, the large size, the location of the tumor, and the invasion of adjacent structures represented a surgical challenge to perform a complete resection.
In the elderly, this pathology is quite uncommon. Retroperitoneal paraganglioma is a rare location of this type of tumor. Endocrinologists, surgeons, and anesthesiologists should work together to ensure an appropriate diagnosis and treatment of paraganglioma. The gold standard treatment is the complete resection after a medical preparation.
嗜铬细胞瘤和副神经节瘤是罕见的神经内分泌肿瘤，生长于肾上腺外，起源于原始神经嵴细胞。腹膜后位置极为罕见，发病率为百万分之二至八。
我们在此报告一例80岁男性患者，出现腹痛、呕吐，伴有高血压峰值和体重减轻。CT扫描显示腹膜后主动脉旁肿瘤侵犯下腔静脉，尿儿茶酚胺水平显著升高。组织病理学和免疫组化检查确诊为副神经节瘤。进行了α受体阻滞剂药物准备。成功完整切除肿瘤并重建下腔静脉，术后无并发症。术后血压和糖化血红蛋白达到目标值，尿儿茶酚胺水平正常。
功能性副神经节瘤通过临床症状怀疑诊断，通过血浆或尿儿茶酚胺进行生物学确诊。非功能性副神经节瘤常带来诊断挑战。在我们的病例中，肿瘤体积大、位置特殊以及侵犯相邻结构对完整切除构成了手术挑战。
在老年人中，这种疾病相当罕见。腹膜后副神经节瘤是这类肿瘤的罕见发病部位。内分泌科医生、外科医生和麻醉科医生应共同努力，确保对副神经节瘤进行恰当的诊断和治疗。金标准治疗方法是在药物准备后进行完整切除。
