Primary primitive neuroectodermal tumor of the ovary

Chu, Ling-Hui; Chang, Wen‐Chun; Kuo, Kuan-Ting; Sheu, Bor‐Ching

doi:10.1016/j.tjog.2013.08.005

Cited by 9 publications

(13 citation statements)

References 11 publications

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“…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).…”

Section: Discussionsupporting

confidence: 72%

“…Based on our study and several others with well-annotated microscopic descriptions of tumor morphology and documentation of EWSR1 rearrangement status, central PNETs appear to arise only in the ovary and uterine corpus (3, 4, 7, 10, 13, 18). 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).…”

Section: Discussioncontrasting

confidence: 66%

“…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).…”

Section: Introductionmentioning

confidence: 99%

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Primitive Neuroectodermal Tumors of the Female Genital Tract

Chiang

Snuderl

Kojiro-Sanada

et al. 2017

American Journal of Surgical Pathology

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Primary primitive neuroectodermal tumor (PNET) of the female genital tract is rare, and its proper classification remains unclear. The clinical, histologic, and immunophenotypic features as well as EWSR1 rearrangement status of 19 gynecologic PNETs, including 10 ovarian, 8 uterine, and 1 vulvar tumors, are herein reported. Patient age ranged from 12 to 68 years, with a median age of 20 and 51 years among those with ovarian and uterine PNETs, respectively. Morphologic features of central nervous system (CNS) tumors were seen in 15 PNETs, including 9 medulloblastomas, 3 ependymomas, 2 medulloepitheliomas, and 1 glioblastoma, consistent with central PNET. The remaining 4 PNETs were composed entirely of undifferentiated small round blue cells and were classified as Ewing sarcoma/peripheral PNET. Eight PNETs were associated with another tumor type, including 5 ovarian mature cystic teratomas, 2 endometrial low-grade endometrioid carcinomas and a uterine carcinosarcoma. By immunohistochemistry, 17 PNETs expressed at least 1 marker of neuronal differentiation, including synaptophysin, NSE, CD56, S100, and chromogranin in 10, 8, 14, 8, and 1 tumors, respectively. GFAP was positive in 4 PNETs, all of which were of central type. Membranous CD99 and nuclear Fli-1 staining was seen in 10 and 16 tumors, respectively, and concurrent expression of both markers was seen in both central and Ewing sarcoma/peripheral PNETs. All tumors expressed vimentin; while keratin cocktail (CAM5.2, AE1/AE3) staining was only focally present in 4 PNETs. Fluorescence in situ hybridization was successful in all cases and confirmed EWSR1 rearrangement in 2 of 4 tumors demonstrating morphologic features of Ewing sarcoma/peripheral PNET and concurrent CD99 and Fli-1 expression. In conclusion, central and Ewing sarcoma/peripheral PNETs may be encountered in the female genital tract with central PNETs being more common. Central PNETs show a spectrum of morphologic features that overlaps with CNS tumors but lack EWSR1 rearrangements. GFAP expression supports a morphologic impression of central PNET and is absent in Ewing sarcoma/peripheral PNET. Ewing sarcoma/peripheral PNETs lack morphologic features of CNS tumors.

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Section: Discussionsupporting

confidence: 72%

Section: Discussioncontrasting

confidence: 66%

Section: Introductionmentioning

confidence: 99%

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Primitive Neuroectodermal Tumors of the Female Genital Tract

Chiang

Snuderl

Kojiro-Sanada

et al. 2017

American Journal of Surgical Pathology

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“…PNETs are uncommon entities especially for the female genital tract and the ovaries are the most common location ( 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 ) . It seems that the exact age for non-skeletal ES is not clear, but cases in the literature were seen between the second and third decades of the life.…”

Section: Discussionmentioning

confidence: 99%

“…The distinction between ES of the ovary and other tumors is made through immunohistochemistry studies. As seen in Table 2 , ( 12 , 13 , 14 , 15 , 24 ) on immunohistochemistry, diffuse membranous positivity for MIC2 (CD99), CD56 (neural cell adhesion molecule), HMW CK and FL1 led to the consideration of PNETs. Negativity for epithelial markers such as CK, EMA, desmin, and WT-1 led to the consideration of desmoplastic small round cell tumors (SRCTs).…”

Section: Discussionmentioning

confidence: 99%

Primitive neuroectodermal tumor of genital tract in hysterectomized patient: A case report

Yakıştıran

Taşkın

Ersöz

et al. 2018

tjod

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Primitive neuroectodermal tumors are high-grade malignant neoplasms. These are uncommon entities for the female genital tract. The treatment, management and follow-up period of Ewing’s tumors are not well-defined because of their rarity in the genital tract. Surgical debulking is the mainstay treatment in all cases. After debulking surgery, patients receive chemotherapy and/or radiotherapy and there is a relation between disease stage and survival. Herein, we present a case of ovarian primitive neuroectodermal tumor with a review of previously reported cases.

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Mesenchymal Tumors of the Ovary

Nucci,

Baniak

2023

Essentials of Diagnostic Gynecological Pathology

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Primary primitive neuroectodermal tumor of the ovary

Cited by 9 publications

References 11 publications

Primitive Neuroectodermal Tumors of the Female Genital Tract

Primitive Neuroectodermal Tumors of the Female Genital Tract

Primitive neuroectodermal tumor of genital tract in hysterectomized patient: A case report

Mesenchymal Tumors of the Ovary

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