Article

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

ABSTRACT

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.

0 Followers
 · 
15 Reads
  • [Show abstract] [Hide abstract]
    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.
    No preview · Article · Nov 2003 · International Journal of Gynecological Pathology
  • [Show abstract] [Hide abstract]
    ABSTRACT: Paraganglioma is rare tumor arising from chromaffine cells. We report a 39-year-old man who was diagnosed with anterior mediastinal paraganglioma. Computed tomography (CT) revealed a solid mass in the anterior mediastinum. Surgical biopsies obtained by sternotomy confirmed histologic and immunohistochemical features of paraganglioma. Surgical removal of this vascularized and invasive tumor was not possible to. External radiotherapy was performed. Three years later, the patient developed chest pain. CT findings showed right nodular pleural masses. Transparietal biopsy confirmed the same pathological aspect of paraganglioma. Chemotherapy was proposed without significant response. This observation confirmed the rare course of paraganglioma to pleural metastases. Treatment of paraganglioma is mainly surgery if the mass is resectable.
    No preview · Article · Nov 2005 · Revue de Pneumologie Clinique
  • [Show abstract] [Hide abstract]
    ABSTRACT: Paraganglioma is one of the rarest neoplasms to involve the ovary, whether primary or metastatic, with only two previous reports. We describe three examples that occurred in patients 22, 58, and 68 years of age. Two patients had hypertension. Two tumors involved the left ovary and one the right ovary; they ranged from 8 to 22 cm, were solid, and were tan, brown, or yellow. One tumor was confined to the ovary; in the second case, there were tumor deposits on the posterior surface of the uterus and the contralateral ovary; in the other case, there was peri-aortic lymph node involvement and peritoneal deposits. In all cases, however, radiologic investigations did not reveal an alternative primary site. On microscopic examination, all three tumors showed a predominantly nested "zellballen" pattern with groups of cells surrounded by a vascular stroma. Tumor cells largely had abundant granular eosinophilic cytoplasm with, in 2 cases, focal clear cell areas. In 1 case, bizarre tumor giant cells were present. Immunohistochemically, all neoplasms were cytokeratin negative and diffusely positive with neuroendocrine markers. In 1 case, there was an S-100-positive population of sustentacular cells. Two cases were positive for inhibin, one focal and one diffuse, and the other was focally positive for calretinin. Electron microscopy performed in 2 cases revealed dense core neuroendocrine granules. One patient has been followed up for 15 years and is alive and well. Although metastatic spread from an undetected primary outside the ovary cannot be totally excluded for the 2 cases with extraovarian disease, we think that the neoplasms most likely represent primary ovarian paragangliomas. Because various neoplasms in the sex cord-stromal and steroid categories are likely to enter into the differential diagnosis, inhibin and calretinin positivity represents a significant potential diagnostic pitfall. The differential is broad and may include many other ovarian tumors, particularly those with an oxyphilic cell type. Possible theories of histogenesis of primary ovarian paraganglioma include an origin from extra-adrenal paraganglia in the region of the ovary or unidirectional differentiation within a teratoma.
    No preview · Article · Jun 2006 · American Journal of Surgical Pathology
Show more