Neurofibromatosis and pregnancy: A report of maternal cardiopulmonary compromise

Background: Neurofibromatosis is a rare pregnancy complication that can present with rapidly enlarging masses that have malignant potential. Case: 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. Conclusion: 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.