儿科罕见的肾上腺髓质细胞瘤（PPC）和副神经节瘤（PGL）（合称PPGL）是起源于嗜铬细胞的肿瘤。肾上腺髓质细胞瘤位于肾上腺，而副神经节瘤则可以发生在头部到骨盆的其他部位。临床表现为持续性高血压、头痛、多汗和心悸。这些症状是由于儿科罕见的肾上腺髓质细胞瘤分泌儿茶酚胺或肿瘤对其他器官施加压力所致。尿液或血浆中的儿茶酚胺及其代谢物水平对进一步诊断至关重要。儿科患者中的这些肿瘤与多发性内分泌综合征2型、神经纤维瘤病1型、Von Hippel-Lindau综合征、家族性副神经节瘤综合征等多个家族综合征有关，与特定突变（SDHx、RET、VHL、NF1）导致的特征表型相关。放射学和核医学成像是检查的重要组成部分。尽管CT和MR被报道具有良好的肿瘤检测敏感性，但还是建议进行核医学成像的深入分析以进行具体的诊断。目前68GA-DOTATATE被认为是首选示踪剂，可用于对不同突变和转移疾病的患者进行复杂评估。治疗选择是切除肿瘤。此外，最近还在研究基因靶向治疗等新的治疗方法，用于处理具有潜在遗传原因或转移疾病的肿瘤。术后需进行长期随访，以避免复发或及早发现。

Pheochromocytoma (PPC) and paraganglioma (PGL) are the tumors that rarely occur in the pediatric population (PPGL). Both originate from chromaffin cells, pheochromocytoma is localized in the adrenal gland, whereas paragangliomas are regarded as the tumors present in other localizations, from head to the pelvis. The clinical image is characterized by the presence of the sustained hypertension, headaches, sweating, palpitations. The symptoms are caused by the catecholamine secretion or are related to tumor mass pressure on different organs. The catecholamines and their metabolites levels in urine collection or plasma are necessary for further evaluation of the diagnosis. In pediatric population the tumors occur in multiple familial syndromes such as Multiple Endocrine type 2, Neurofibromatosis type 1, Von Hippel-Lindau syndrome, Familial Paraganglioma syndrome are related to specific mutations (SDHx, RET, VHL, NF1) leading to the characteristic phenotype. The radiological and nuclear imaging are an important part of the examination. Although CT and MR are reported to have overall good sensitivity for the tumor detection, further analysis with nuclear imaging is recommended for the specified diagnosis. Right now 68GA-DOTATATE is regarded as the tracer of choice, leading to the complex evaluation of patients with different mutations and metastatic disease. The treatment of choice is the tumor excision. Also, lately new therapeutic approaches including genetically targeted therapies are under investigation for more complex treatment of tumors with underlying genetic cause or metastatic disease. Long term follow-up after treatment to avoid recurrence or to detect it in early stadium must be performed.