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Malignant nonfunctioning pheochromocytoma occurring in a mixed multiple endocrine neoplasia syndrome
R P Mozersky1, R Girdhar, S Palushock
1Division of Endocrinology, Shadyside Hospital, and the University of Pittsburgh, Department of Medicine, Pittsburgh, Pennsylvania, USA.
Objective:
To present the first documented case of malignant nonfunctioning pheochromocytoma occurring in a mixed multiple endocrine neoplasia (MEN) syndrome.
Methods:
We describe the personal and family history of a 34-year-old man with headaches, increased serum prolactin levels, and hyperparathyroidism and review the details of his clinical course and treatment.
Results:
After diagnosis of a prolactinoma, bromocriptine therapy was implemented but eventually discontinued by the patient when severe headaches recurred. A transsphenoidal resection of a prolactinoma was performed; the patient continued to have abnormally high serum prolactin levels. Subsequent left lower flank pain prompted an exploratory laparotomy, and a left nephroad-renalectomy was performed. Pathologic examination disclosed a 1-cm tumor, consistent with malignant pheochromocytoma. Electron microscopy demonstrated neurosecretory granules. A hypercoagulable state complicated the course postoperatively. Despite chemotherapy, extensive metastatic disease ensued, and the patient died.
Conclusion:
Although pheochromocytomas are usually associated with MEN type IIA and type IIB, this patient had a malignant pheochromocytoma, without the usual clinical and biochemical manifestations, in the setting of MEN type I.
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