From the Department of Obstetrics and Gynecology, University of Texas Southwestern Medical Center, and Parkland Health and Hospital System, Dallas, Texas.
Obstet Gynecol. 2010 Aug;116 Suppl 2:507-509. doi: 10.1097/AOG.0b013e3181da0113.
Neurofibromatosis is a rare pregnancy complication that can present with rapidly enlarging masses that have malignant potential.
A 19-year-old primagravid woman with a known history of neurofibromatosis presented in her first trimester with pulmonary complaints. A malignant mediastinal mass was diagnosed and resected, with additional treatment options declined. In less than 3 months, the patient presented with a recurrent mass that resulted in fatal airway compromise during the same gestation.
Neurofibromatosis type 1 with malignant peripheral nerve sheath tumors complicating pregnancy requires an experienced, multidisciplinary team of care offering an aggressive evaluation to rule out malignant transformation or recurrence when there is any change in clinical status of a patient, as this may signal a potentially fatal change in the lesion.
神经纤维瘤病是一种罕见的妊娠并发症，可表现为迅速增大的具有恶性潜能的肿块。
一位 19 岁的初产妇，已知患有神经纤维瘤病，在孕早期出现肺部不适。诊断为恶性纵隔肿块并进行了切除，但拒绝了其他治疗选择。不到 3 个月，患者再次出现肿块，导致同一孕期气道严重受阻而致命。
神经纤维瘤病 1 型伴恶性外周神经鞘肿瘤合并妊娠需要一个经验丰富的多学科护理团队，当患者的临床状况有任何变化时，提供积极的评估以排除恶性转化或复发的可能性，因为这可能预示着病变有潜在的致命变化。
