Desmoplastic Small Round Cell Tumors in a Young Man

被引:2
|
作者
Hirano, Genryu [1 ]
Irie, Makoto [1 ]
Nakashima, Yuta [2 ]
Shakado, Satoshi [1 ]
Sohda, Tetsuro [1 ]
Tanaka, Toshihiro [2 ]
Takamatsu, Yasushi [2 ]
Aoki, Mikiko [3 ]
Tamura, Kazuo [2 ]
Nabeshima, Kazuki [3 ]
Sakisaka, Shotaro [1 ]
机构
[1] Fukuoka Univ, Fac Med, Dept Gastroenterol & Med, Fukuoka, Japan
[2] Fukuoka Univ, Fac Med, Dept Med Oncol Hematol & Infect Dis, Fukuoka, Japan
[3] Fukuoka Univ, Fac Med, Dept Pathol, Fukuoka, Japan
关键词
chemotherapy; desmoplastic small round cell tumor; EWS-WT1 gene fusion transcript; reverse transcriptase polymerase chain reaction; TRANSLOCATION; 22)(P13; T(11;
D O I
10.2169/internalmedicine.52.0431
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
A previously healthy 18-year-old man was admitted to our hospital with abdominal pain in September 2010. We performed a percutaneous biopsy of multiple intrahepatic masses. A diagnosis of desmoplastic small round cell tumors was confirmed based on the presence of a polyphenotypic immunoprofile (positivity for EMA, vimentin, cytokeratin, desmin and WT1) and characteristic EWS-WT1 gene fusion. Because the mass had invaded the mesentery and the disease had disseminated to liver, the patient received palliative chemotherapy with carboplatin, paclitaxel, vincristine, doxorubicin, cyclophosphamide, ifosfamide, etoposide and irinotecan. The maximal response to the chemotherapy was a partial remission. The patient died 20 months after diagnosis.
引用
收藏
页码:1909 / 1914
页数:6
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