Malignant paraganglioma of the uterus

We report a malignant uterine paraganglioma in a 41-year-old woman who unde rwent a hysterectomy for meno-metrorrhagia. It was initially thought to be a leiomyoma in necrobiosis, The clinical outcome was characterized by an ea rly regional recurrence tin the left Fallopian tube). Later, vertebral and lung metastasis occured, leading to death 22 months after the initial diagn osis. Paragangliomas are uncommon neuroendocrine tumors, related to pheochromocyt omas. They are mainly found in the para-aortic and retroperitoneal region, and less commonly in the pelvic area. Location in the uterus is extremely r are: 5 cases were previously reported and only one malignant.