Tripathy Prasanta Kumar, Pattnaik Kaumudee, Nayak Manjushree, Mohanty Hiranya Kishor
Department of Pediatric Surgery, SCB Medical College, Cuttack, Odisha, India.
Department of Pathology, SCB Medical College, Cuttack, Odisha, India.
Indian J Med Paediatr Oncol. 2017 Jan-Mar;38(1):59-61. doi: 10.4103/0971-5851.203503.
Pheochromocytoma (PCC) is a neuroendocrine tumor originating from chromaffin tissue in adrenal medulla. Its diagnosis and treatment are well defined in adults, but experience in children is limited. Children constitute only 10% of reported cases, the average age at presentation being 11 years. The most common presentation is sustained hypertension, which is absent in only 10% of children. We managed a 14-month-old female child with PCC, but she was not hypertensive. We report two unusual features, in this case, an extremely young age at presentation and a childhood case of nonhypertensive PCC contributing for "rule of 10s."
嗜铬细胞瘤（PCC）是一种起源于肾上腺髓质嗜铬组织的神经内分泌肿瘤。其诊断和治疗在成人中已有明确界定，但儿童方面的经验有限。儿童仅占报告病例的10%，发病时的平均年龄为11岁。最常见的表现是持续性高血压，仅10%的儿童无此症状。我们诊治了一名14个月大的患有嗜铬细胞瘤的女童，但她并无高血压症状。我们报告了该病例的两个不寻常特征，一是发病时年龄极小，二是儿童非高血压性嗜铬细胞瘤病例，这为“10%规则”提供了实例。
