Primary ependymoma of the ovary: a case report and literature review

Abstract

Ependymoma is a glioma with differentiation toward ependymal cells that usually arises in the central nervous system. Ovarian ependymoma is extremely rare, and the treatment strategies for this disease have not been established. This is the first report of a patient with advanced ovarian ependymoma who received fertility-sparing surgery and is now alive without disease. A 23-year-old Japanese woman, gravida 0, presented with a chief complaint of lower abdominal distension and weight loss. She underwent left salpingo-oophorectomy for suspected ovarian cancer. Microscopic examination revealed a highly cellular tumor composed of small cells with hyperchromatic, round-to-oval nuclei and scanty cytoplasm. Perivascular pseudorosettes, ependymal rosettes, and extensive necrosis were observed. After thorough pathologic examination, she was diagnosed as having stage IIIC ovarian ependymoma. Postoperatively, she received adjuvant chemotherapy and underwent secondary cytoreductive surgery that preserved the uterus and right ovary. Her menstrual cycle has resumed, and she is alive without evidence of disease 16 months after the start of treatment. Although rare, primary ovarian ependymoma must be kept in mind in the differential diagnosis of ovarian tumors, especially in young women. Administration of etoposide-based chemotherapy along with cytoreductive surgery is a potential standard treatment for advanced ovarian ependymoma.

Ovarian ependymomas of extra-axial type or central immunophenotypes

“…No cases of ependymomas of the female genital tract arising from the tissues of mature teratoma have previously been reported. Two cases were associated only with contralateral teratomas [5,10], and although areas of cartilage were present in 2 further tumors [16,18], no complex admixture of tissues that characterize teratoma was found. The presence of cartilage alone is not proof of an origin from a teratoma because it is known that central nervous system ependymomas can also show foci of heterologous cartilage and bone [20].…”

“…They are found rarely only in the female genital tract; to date, only 16 cases have been reported in the ovary [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17] and 3 [18,19] in the broad ligament, and none of these cases originated unequivocally from a preexistent mature teratoma.…”

Retinal ependymoma: an immunohistologic and ultrastructural study

“…On the other hand, of all the neuroectodermal tumors of the CNS, ependymomas have the greatest propensity to occur at ectopic sites, presumably from ependymal rests [2]. Further removed are examples occurring in broad ligament [21,22], liver [23], small bowel [24], and ovaries [25], as well as in lung and mediastinum [26,27]. A morphological variant, the myxopapillary ependymoma, occurs primarily in the presacral and postsacral soft tissue, as well as in lumbosacral dermal/ subcutaneous tissue.…”

Paraneoplastic Internal Jugular Vein Thrombosis Leading to Diagnosis of Bilateral Ovarian Ependymoma

“…As such there are no standard guidelines for the management of ovarian ependymomas. Administration of etoposide-based chemotherapy along with cytoreductive surgery is a potential standard treatment for advanced ovarian ependymoma [10]. A combination of cisplatin and etoposide (EP) or bleomycin, etoposide, and cisplatin (BEP) is preferred because of a lower relapse rate and shorter treatment time.…”