Pheochromocytoma in neurofibromatosis type 1 during pregnancy

Abstract

Pregnant women with neurofibromatosis type 1 (NF-1) have increased complications during gestation, including hypertensive disorders that are sometimes caused by pheochromocytoma. Pheochromocytoma is an extremely rare condition during pregnancy, and the main clinical manifestation is hypertension. If not properly treated, pheochromocytoma has high maternal and fetal mortality rates. Early recognition and adequate clinical management before delivery have led to better outcomes in the last few decades. Despite the association of NF-1 and pheochromocytoma, there are few clinical reports of these two conditions in pregnant patients. We present a rare case of pheochromocytoma diagnosed during pregnancy in a patient with NF-1, and we describe the treatment and the obstetric and fetal outcomes. We also review other medical conditions related to NF-1 that complicated this patient’s pregnancy.

Chinese abstract

患有I型神经纤维瘤(NF-1)的妊娠妇女更可能发生包括高血压在内的妊娠期并发症, 有时高血压是由嗜铬细胞瘤引起。嗜铬细胞瘤是一种极为罕见的妊娠期并发症, 主要临床表现为高血压。如果不能得到妥善处理, 嗜铬细胞瘤可导致高母胎死亡率。在近数十年, 早期诊断和分娩前适当处理改善了妊娠期嗜铬细胞瘤的妊娠结局。尽管NF-1和嗜铬细胞瘤存在联系, 妊娠妇女同时发生这两种疾病的报告也非常少见。 我们报告了一例NF-1患者在妊娠期被诊断为嗜铬细胞瘤的病例, 并描述了治疗, 分娩以及胎儿结局。同时我们回顾了这名患者与NF-1有关的使妊娠情况更加复杂的其它医疗状况。

Keywords:

• Hypertension
• neurofibromatosis type 1
• obstetric labor complications
• pheochromocytoma
• pregnancy

Declaration of interest

The authors report no conflict of interest.