Recurrent Pheochromocytoma With Bone Metastasis Eight Years After Bilateral Adrenalectomies in a Patient With Neurofibromatosis Type 1

Background/ObjectivePheochromocytoma can recur years after curative surgical resection. Rarely, it may reoccur as metastasis. Here, we present a case of metastatic pheochromocytoma to the bones in a patient with neurofibromatosis type 1, eight years after initial resection of primary bilateral adrenal pheochromocytomas without metastases.Case ReportA 44-year-old woman presented with diffuse body pain and palpitations. Her past medical history included neurofibromatosis type 1 and hypertension. Eight years prior to her current presentation, she had undergone a bilateral adrenalectomy for the management of bilateral adrenal pheochromocytomas. Her plasma metanephrines normalized after surgery and remained normal at her one-year post-operative visit. She was subsequently lost to follow-up until her current presentation. Our evaluation revealed significantly elevated urine and plasma metanephrines as well as innumerable DOTATATE avid lesions along the axial and perpendicular spine compatible with a metastatic neuroendocrine tumor. She was started on doxazosin and metoprolol and discharged home with a plan to be seen by Oncology to discuss systemic therapy.DiscussionPredicting malignant disease in patients with primary tumors without metastases is challenging. There is no single factor that can reliably predict tumor behavior. It is unknown if individuals with neurofibromatosis type 1, who have a genetic predisposition for developing pheochromocytomas, are at an increased risk of malignant disease.ConclusionDue to a lack of accurate predictors, annual biochemical testing is recommended after primary tumor resection and in patients with a genetic predisposition, strict lifelong follow-up should be strongly considered due to a possible higher risk of malignant disease.