Department of Pediatrics, University Hospital Farhat Hached, Sousse, Tunisia.
Faculty of Medicine, Ibn El Jazzar, Sousse, Tunisia.
Pediatr Endocrinol Diabetes Metab. 2021;27(2):141-145. doi: 10.5114/pedm.2020.101804.
Pheochromocytoma is a rare tumor during childhood, originating from the chromafine tissue. The clinical presentation can be variable and assembling many other diseases. This tumor submits specific care problems. We report the case of a 7-year-old boy who presented with headache, fever, abdominal pain and vomiting evolving for 3 days. The physical examination revealed a painful abdomen, a high blood pressure and profuse sweating. As an acute appendicitis was suspected, abdominal ultrasound then abdominal CT were performed, revealing multiple bilateral adrenal masses. Measurement of 24-hour urinary catecholamines showed a marked increase in normetanephrines: 7,18 mg/24 h (normal range: 0.07-0.46 mg/24 h). The MIBG scintigraphy revealed a bilateral fixation in the two adrenals. During the next weeks, the patient developed close peaks of threatening hypertension, controlled with difficulty through the concomitant use of three anti hypertensive treatments. He underwent surgery two months after the first consultation, and had a total adrenalectomy on the right side and subtotal on the left side. The pathological examination concluded with multiple and bilateral pheochromocytomas with a PASS score between 2 and 6. The patient was treated with hormone replacement therapy immediately after the surgery. The subsequent course with a 10-month follow-up was favorable with disappearance of functional signs, correct blood pressure, good general condition, normal growth and a normal biological balance.
嗜铬细胞瘤在儿童期较为罕见,来源于嗜铬组织。临床表现多样,可能与多种其他疾病相混淆。这种肿瘤存在特定的护理问题。我们报告了一例 7 岁男孩的病例,他因头痛、发热、腹痛和呕吐持续 3 天就诊。体格检查显示腹痛、高血压和大量出汗。由于怀疑急性阑尾炎,进行了腹部超声和腹部 CT 检查,显示双侧肾上腺多个肿块。24 小时尿儿茶酚胺测量显示去甲变肾上腺素明显增加:7.18mg/24h(正常范围:0.07-0.46mg/24h)。MIBG 闪烁显像显示双侧肾上腺均有固定摄取。在接下来的几周里,患者出现了几次高血压危象高峰,尽管同时使用了三种降压药物治疗,但仍难以控制。他在首次就诊后两个月接受了手术,右侧行全肾上腺切除术,左侧行次全切除术。病理检查结果为双侧多发嗜铬细胞瘤,PASS 评分为 2-6 分。术后患者立即接受激素替代治疗。随后的 10 个月随访过程良好,功能体征消失,血压正常,一般情况良好,生长正常,生物学平衡正常。