| CASE REPORT |
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| Year : 2018 | Volume
: 38
| Issue : 2 | Page : 81-84 |
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Primary uterine primitive neuroectodermal tumor
Yen-Chang Chen1, Yung-Hsiang Hsu1, Yu-Chi Wei2, Tang-Yuan Chu2, Dah-Ching Ding2
1 Department of Pathology, Hualien Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Tzu Chi University, Hualien, Taiwan, Republic of China
2 Department of Obstetrics and Gynecology, Hualien Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Tzu Chi University, Hualien, Taiwan, Republic of China
Correspondence Address:
Dr. Dah-Ching Ding
Department of Obstetrics and Gynecology, Hualien Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, 707, Section 3, Chung-Yang Road, Hualien 970
Republic of China
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/jmedsci.jmedsci_88_17
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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. |
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