Ewing sarcoma-primitive neuroectodermal tumor of the uterus: a clinicopathologic, immunohistochemical and ultrastructural study of one case

Abstract

In spite of the rarity of ES/PNET, we should consider it in the differential diagnosis of small cell neoplasms of the uterus.

Primitive Neuroectodermal Tumors of the Female Genital Tract

“…Ewing sarcoma/peripheral PNETs may also be encountered at these 2 sites (3, 7); however, findings from our study and few others (4, 10, 13) suggest that central PNETs predominate in the ovary and uterine corpus either alone or association with another tumor type, particularly an ovarian teratoma or a uterine carcinoma, carcinosarcoma, or sarcoma (2, 4–6, 9–12, 18). Interestingly, regardless of whether the tumor is central or peripheral type, patients with ovarian PNETs tend to be of reproductive age (median, 23 years) (1, 3–7, 45) compared to those with uterine PNETs who are typically postmenopausal (median, 57 years) (1, 9–18, 40, 41). Among the 14 PNETs with known EWSR1 rearrangement status reported in the cervix, vagina, and vulva (26, 30, 31, 34, 36–38), approximately 87%, including our single vulvar tumor, are Ewing sarcoma/peripheral PNETs confirmed by FISH or RT-PCR.…”

“…The identification of teratoma in a significant minority of ovarian PNETs based on our study and others (2, 4–6) suggests germ cell derivation in at least a subset of tumors arising at this site. There have been rare reports of teratomas (45, 56–60) arising in the uterus, and while it is conceivable that teratoma may be a source of uterine PNETs as it is in the ovary, teratoma has not been found in association with PNET in the uterus (1, 9–25, 40, 41). The identification of benign glial tissue (61–63) in the uterus which may result from implantation of aborted fetal tissue (63), represents another possible source of both uterine gliomas (64, 65) and PNETs.…”

“…Gynecologic PNETs have been reported in the ovary (1–7), broad ligament (1, 8), uterine corpus (1, 9–18), uterine cervix (1, 19–25), vagina (26–32), and vulva (1, 26, 31, 33–39); to date, none have been reported to have arisen in the fallopian tube. PNETs of the ovary and uterus are frequently associated with another tumor type (2, 4–6, 10, 11, 18), although many, including those arising elsewhere, occur in pure form (1, 3, 4, 7, 9, 10, 13–17, 19–37, 39–41). Together, they represent a peculiar group of rare neoplasms that show varying degrees of neuroectodermal differentiation and remain poorly understood when compared to their bone and soft tissue counterparts (42) and tumors that until recently were classified as PNETs of the CNS (43).…”

Adjuvant chemotherapy for a primitive neuroectodermal tumor of the uterine corpus: A case report and literature review

“…Reports with incomplete information about chemotherapy were also excluded. Of a total of 178 papers identified from the search terms in the MEDLINE database, 49 cases fulfilled the mentioned criteria (Table ). We analyzed the choice of chemotherapy regimen in relation to the patients’ ages and divided patients into the following groups: adolescence (<20 years), reproductive (20–40 years), premenopausal (40–60 years) and elderly (>60 years).…”

Extraosseous Ewing's sarcoma arising in the perineal region (Rectovaginal Septum)

“…1 The occurrence of primary ES in the female genital tract is exceptional, and only rare cases arising from the ovary, uterus, vagina, and vulva have been reported. [2][3][4][5][6][7] Moreover, only one case of primary ES arising in the rectovaginal septum has been documented. 8 Herein, we describe the second documented case of ES arising in the perineal region (rectovaginal septum) and discuss the usefulness of cytological examination in the diagnosis of this lesion.…”