Paz-Ibarra José Luis, Suarez-Rojas Jacsel, García-Ruiz Víctor Raúl, Álvarez-Gamero Julio César, Somocurcio-Peralta José
Hospital Nacional Edgardo Rebagliati Martins, Servicio de Endocrinología. Lima, Perú.
Rev Med Inst Mex Seguro Soc. 2021 Aug 2;59(4):352-357.
Adrenal incidentalomas are tumors that are usually detected in imaging studies indicated without suspicion of adrenal disease. Pheochromocytoma is a neuroendocrine tumor that can occur sporadically or associated with genetic syndromes, having been described in 0.1-5% of patients with type 1 neurofibromatosis, which is a progressive multisystemic disease of autosomal dominant inheritance that affects 1 / 2600-3000 individuals .
We present the case of a 50-year-old patient who was admitted to the Emergency Department for acute appendicitis and whose CT scan showed the presence of bilateral adrenal masses is reported. After the surgical intervention, he was hospitalized for study. The patient denied headache, sweating, hypertension, palpitations, and weight loss; Physical examination revealed the presence of multiple lentigines, café-au-lait spots > 15 mm, fixed and pedunculated nodular lesions, compatible with focal neurofibromas, without signs suggestive of hypo or hypercortisolism. In the analysis, total metanephrines and vanillyl-mandelic acid were identified in urine, they were found in very high values, being subjected to laparoscopic adrenalectomy in 2 stages, after adequate preparation that included alpha and beta adrenergic blockade.
In patients with adrenal masses, it is necessary to recognize the importance of a comprehensive clinical evaluation to guide an adequate diagnostic study, as well as the performance of an optimal preoperative study that includes hormonal tests to rule out functionality.
肾上腺偶发瘤是在影像学检查中偶然发现的肿瘤,之前并无肾上腺疾病的怀疑。嗜铬细胞瘤是一种神经内分泌肿瘤,可散发或与遗传综合征相关,在1型神经纤维瘤病患者中占0.1%-5%,1型神经纤维瘤病是一种常染色体显性遗传的进行性多系统疾病,影响1/2600-3000的个体。
我们报告一例50岁患者,因急性阑尾炎入住急诊科,其CT扫描显示双侧肾上腺肿块。手术干预后,他住院进行检查。患者否认头痛、出汗、高血压、心悸和体重减轻;体格检查发现多处雀斑样痣、直径>15mm的咖啡牛奶斑、固定和带蒂的结节性病变,与局灶性神经纤维瘤相符,无提示皮质醇增多或减少的体征。分析发现尿中总甲氧基肾上腺素和香草扁桃酸含量极高,在充分准备(包括α和β肾上腺素能阻滞剂)后,分两期进行腹腔镜肾上腺切除术。
对于肾上腺肿块患者,必须认识到全面临床评估对指导充分诊断研究的重要性,以及进行包括激素检测以排除功能性的最佳术前研究的重要性。