Primary uterine primitive neuroectodermal tumor
Primitive neuroectodermal tumors (PNETs) are rare malignant tumors and extremely rare in uterine corpus. We report a case of primary uterine PNET in Taiwan. A 68-year-old woman presented with vaginal bleeding and abdominal fullness for 2 weeks. Computed tomography revealed one 8.0 cm uterine corpus...
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2018-01-01
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| Series: | Journal of Medical Sciences |
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| Online Access: | http://www.jmedscindmc.com/article.asp?issn=1011-4564;year=2018;volume=38;issue=2;spage=81;epage=84;aulast=Chen |
| _version_ | 1818671429299732480 |
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| author | Yen-Chang Chen Yung-Hsiang Hsu Yu-Chi Wei Tang-Yuan Chu Dah-Ching Ding |
| author_facet | Yen-Chang Chen Yung-Hsiang Hsu Yu-Chi Wei Tang-Yuan Chu Dah-Ching Ding |
| author_sort | Yen-Chang Chen |
| collection | DOAJ |
| description | Primitive neuroectodermal tumors (PNETs) are rare malignant tumors and extremely rare in uterine corpus. We report a case of primary uterine PNET in Taiwan. A 68-year-old woman presented with vaginal bleeding and abdominal fullness for 2 weeks. Computed tomography revealed one 8.0 cm uterine corpus tumor with carcinomatosis peritonei and ascites. The cancer antigen 125 level was high (280.7 IU/ml). Pathology of endometrial biopsy diagnosed PNET according to the characteristic of small blue round cells with Homer-Wright rosettes and Friend leukemia integration 1 transcription factor (FLI-1) positive. She received dose-dense chemotherapy with paclitaxel and carboplatin, but the response was poor and the PENT metastasized to liver. PNETs belong to small blue round cell tumor group that is difficult to be diagnosed on histopathology. The immunohistochemistry for CD99 and FLI-1 provides high sensitivity and specificity for diagnosis, respectively. No optimal treatment is established due to rarity. The prognosis is poor, usually <3 years survival. |
| first_indexed | 2024-12-17T07:23:52Z |
| format | Article |
| id | doaj.art-9ef40cfc32dd4fd99ce5362459f2fa82 |
| institution | Directory Open Access Journal |
| issn | 1011-4564 |
| language | English |
| last_indexed | 2024-12-17T07:23:52Z |
| publishDate | 2018-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Journal of Medical Sciences |
| spelling | doaj.art-9ef40cfc32dd4fd99ce5362459f2fa822022-12-21T21:58:41ZengWolters Kluwer Medknow PublicationsJournal of Medical Sciences1011-45642018-01-01382818410.4103/jmedsci.jmedsci_88_17Primary uterine primitive neuroectodermal tumorYen-Chang ChenYung-Hsiang HsuYu-Chi WeiTang-Yuan ChuDah-Ching DingPrimitive neuroectodermal tumors (PNETs) are rare malignant tumors and extremely rare in uterine corpus. We report a case of primary uterine PNET in Taiwan. A 68-year-old woman presented with vaginal bleeding and abdominal fullness for 2 weeks. Computed tomography revealed one 8.0 cm uterine corpus tumor with carcinomatosis peritonei and ascites. The cancer antigen 125 level was high (280.7 IU/ml). Pathology of endometrial biopsy diagnosed PNET according to the characteristic of small blue round cells with Homer-Wright rosettes and Friend leukemia integration 1 transcription factor (FLI-1) positive. She received dose-dense chemotherapy with paclitaxel and carboplatin, but the response was poor and the PENT metastasized to liver. PNETs belong to small blue round cell tumor group that is difficult to be diagnosed on histopathology. The immunohistochemistry for CD99 and FLI-1 provides high sensitivity and specificity for diagnosis, respectively. No optimal treatment is established due to rarity. The prognosis is poor, usually <3 years survival.http://www.jmedscindmc.com/article.asp?issn=1011-4564;year=2018;volume=38;issue=2;spage=81;epage=84;aulast=ChenPrimitive neuroectodermal tumorsmall blue round cell tumorHomer-Wright rosettesCD99Friend leukemia integration 1 transcription factor protein |
| spellingShingle | Yen-Chang Chen Yung-Hsiang Hsu Yu-Chi Wei Tang-Yuan Chu Dah-Ching Ding Primary uterine primitive neuroectodermal tumor Journal of Medical Sciences Primitive neuroectodermal tumor small blue round cell tumor Homer-Wright rosettes CD99 Friend leukemia integration 1 transcription factor protein |
| title | Primary uterine primitive neuroectodermal tumor |
| title_full | Primary uterine primitive neuroectodermal tumor |
| title_fullStr | Primary uterine primitive neuroectodermal tumor |
| title_full_unstemmed | Primary uterine primitive neuroectodermal tumor |
| title_short | Primary uterine primitive neuroectodermal tumor |
| title_sort | primary uterine primitive neuroectodermal tumor |
| topic | Primitive neuroectodermal tumor small blue round cell tumor Homer-Wright rosettes CD99 Friend leukemia integration 1 transcription factor protein |
| url | http://www.jmedscindmc.com/article.asp?issn=1011-4564;year=2018;volume=38;issue=2;spage=81;epage=84;aulast=Chen |
| work_keys_str_mv | AT yenchangchen primaryuterineprimitiveneuroectodermaltumor AT yunghsianghsu primaryuterineprimitiveneuroectodermaltumor AT yuchiwei primaryuterineprimitiveneuroectodermaltumor AT tangyuanchu primaryuterineprimitiveneuroectodermaltumor AT dahchingding primaryuterineprimitiveneuroectodermaltumor |