Rodríguez Lina, Girón Felipe, Chaves Carlos Eduardo Rey, Venegas David, Núñez-Rocha Ricardo E, Nassar Ricardo
School of Medicine, Universidad de los Andes, 111711 Bogotá D.C., Colombia.
School of Medicine, Universidad del Rosario, 111711 Bogotá D.C., Colombia.
Int J Surg Case Rep. 2022 Jun;95:107119. doi: 10.1016/j.ijscr.2022.107119. Epub 2022 Apr 22.
Paraganglioma and pheochromocytoma are uncommon conditions that affect around 1.5-9 patients per million. The most frequent symptoms are headache, hypertension and diaphoresis; however, palpitations or tachycardia could be present. Malignancy is not frequent, and when is suspected, positron emission tomography (PET) should be performed. Surgery it's the gold standard treatment, with acceptable rates of morbidity and mortality.
A 33-year-old woman presented to private practice with long-standing symptoms consisting of asthenia, adynamia, and sensation of palpable masses in the neck. Due to her medical history and imaging findings, urine metanephrines were obtained, showing high values of adrenaline 6.69 (μg/24 h), noradrenaline 130.09 (μg/24 h), dopamine 262.59 (μg/24 h). PET was performed to identify hyperfunctioning masses in other locations, finding bilateral carotid hypermetabolic masses and a nodular lesion anterior to the aortoiliac bifurcation, probably malignant. Laparoscopic retroperitoneal tumor resection was performed by a laparoscopic and metabolic surgeon, with intraoperative findings of a vascularized mass (30 × 25 mm) closely related to the left aortoiliac bifurcation and peritoneal fluid.
Paragangliomas are rare tumors that frequently produce catecholamines with varied symptoms. Diagnosis requires patient history, laboratory studies including 24-hour urine-metanephrines and plasma metanephrine levels. Imaging such as CT, MRI and PET scan are necessary. Perioperative management needs to be performed and surgery is the basis of the treatment in patients with localized disease. Metastatic disease has a 50% mortality at 5 years and requires a different approach.
Paraganglioma is a rare and complex entity that requires a multidisciplinary approach.
副神经节瘤和嗜铬细胞瘤是罕见疾病，每百万人口中约有1.5 - 9人受其影响。最常见的症状是头痛、高血压和多汗；然而，也可能出现心悸或心动过速。恶性情况并不常见，当怀疑有恶性时，应进行正电子发射断层扫描（PET）。手术是金标准治疗方法，发病率和死亡率可接受。
一名33岁女性因长期存在乏力、动力不足和颈部可触及肿块的感觉而就诊于私人诊所。根据她的病史和影像学检查结果，检测了尿间甲肾上腺素，结果显示肾上腺素值为6.69（μg/24小时）、去甲肾上腺素值为130.09（μg/24小时）、多巴胺值为262.59（μg/24小时）均偏高。进行PET检查以确定其他部位的功能亢进肿块，发现双侧颈动脉高代谢肿块以及主动脉髂动脉分叉前方的一个结节状病变，可能为恶性。由一名腹腔镜和代谢外科医生进行了腹腔镜腹膜后肿瘤切除术，术中发现一个血管丰富的肿块（30×25毫米），与左主动脉髂动脉分叉和腹腔积液密切相关。
副神经节瘤是罕见肿瘤，常分泌儿茶酚胺，症状多样。诊断需要患者病史、包括24小时尿间甲肾上腺素和血浆间甲肾上腺素水平的实验室检查。CT、MRI和PET扫描等影像学检查是必要的。围手术期管理需要进行，手术是局限性疾病患者治疗的基础。转移性疾病5年死亡率为50%，需要不同的治疗方法。
副神经节瘤是一种罕见且复杂的疾病，需要多学科方法进行治疗。
