Paragangliome malin de l’uterus [Malignant paraganglioma of the uterus]

Service de Médecine Interne et Oncologie, Hôpital d'Instruction des Armées Bégin, Saint-Mandé.
Annales de medecine interne 07/2000; 151(4):311-3.
Source: PubMed


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

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    ABSTRACT: Paragangliomas are uncommon neoplasms of the female genital tract, with fewer than 15 reported cases arising from the uterus, ovary, vagina, and cervix. Three of these tumors presented in the vagina. This report describes the authors' experience with a paraganglioma of the vagina in a 24-year-old woman that presented as an asymptomatic mass. The histologic features in the initial biopsy proved again that the paraganglioma may be confused with other vascular neoplasms. The clinical course after the biopsy implied that this neoplasm had been awakened from its functionless dormancy.
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