Background/aim: Pheochromocytomas (PHEOs) are a rare entity in the common population but have higher prevalence in neurofibromatosis type I (NFI) patients. In combination with pregnancy there are several reports on perioperative complications due to endocrine tumor activity; however, case reports on the malignant course of the disease could not be identified.
Case report: We report the case of a pregnant female patient with diagnosed NF1, who was referred to our hospital with metastatic PHEO. Because of worsening state, emergency cesarian section was performed at 33 weeks gestation and the patient required CPR and avECMO. Diagnostic workup showed a tumor of the right adrenal gland infiltrating the liver continuously, as well as the right kidney and vena cava inferior, and multiple disseminated bone metastases.
Conclusion: As NF1 patients seem to have a significantly higher risk for PHEO, routine testing before or during pregnancy needs to be discussed, as perioperative adverse events due to endocrine activity are common. In case of malignant PHEO, guidelines for surgical management of metastasis are still missing. As our case shows however, interdisciplinary management and constant readaptation of therapy regimen according to disease progress are important.
Keywords: Pheochromocytoma; Van Recklinghausen’s disease; neurofibromatosis type I; pregnancy.
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