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: 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.
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    ABSTRACT: Paragangliomas are rare tumors arising from the chromaffin cells in the autonomic nervous system. While they both occur most frequently along the paraaortic chain, paraganglioma and ovarian carcinoma very rarely occur together. A 61-year-old, post-menopausal woman visited our hospital, with complaints of abdominal pain and genital bleeding. Image analysis showed a 21 x 18 x 10 cm ovarian mass, and a 38 mm tumor at the paraaortic lesion. First, she underwent bilateral salpingo-oophorectomy. Serous papillary cystadenocarcinoma of the left ovary was found, and so a second surgery was performed. The paraaortic tumor was completely eliminated in spite of fluctuating blood pressure intraoperatively. Microscopic examination revealed that the paraaortic tumor was paraganglioma. She was ultimately diagnosed as having ovarian carcinoma stage Ia (FIGO) with coincident paraganglioma. If blood pressure fluctuation is observed during dissection of the paraaortic lymph node, paraganglioma should be suspected and blood pressure must be carefully controlled.
    Journal of Obstetrics and Gynaecology Research 02/2010; 36(1):204-8. DOI:10.1111/j.1447-0756.2009.01094.x · 0.93 Impact Factor
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    ABSTRACT: Vaginal paraganglioma is a rare and unusual tumor occurring in the vaginal wall. It is a solitary primary paraganglioma, especially in atypical sites. Herein, we report an unusual case of a 17-year-old woman who had not experienced vomiting, or hypertension. She was found to have an immobile solid mass in the right side of her vaginal wall. Positron emission tomography/computed tomography scans revealed a well-defined solid ovoid mass adjacent to the bladder and pelvic floor. Tumor markers were within the normal range. A transient blood pressure increase occurred during the biopsy. After oral administration of antihypertensive drugs, surgery was performed to completely remove the mass. Histopathological examination indicated that it was a paraganglioma of the vagina. Repeat computed tomography examination did not reveal any local recurrence or distant metastasis during the 12-month follow-up period.
    OncoTargets and Therapy 06/2014; 7:965-8. DOI:10.2147/OTT.S62174 · 2.31 Impact Factor