Desmoplastic Small Round Cell Tumors in a Young Man

Genryu Hirano; Makoto Irie; Yuta Nakashima; Satoshi Shakado; Tetsuro Sohda; Toshihiro Tanaka; Yasushi Takamatsu; Mikiko Aoki; Kazuo Tamura; Kazuki Nabeshima; Shotaro Sakisaka

doi:10.2169/internalmedicine.52.0431

CASE REPORTS

Desmoplastic Small Round Cell Tumors in a Young Man

Genryu Hirano, Makoto Irie, Yuta Nakashima, Satoshi Shakado, Tetsuro Sohda, Toshihiro Tanaka, Yasushi Takamatsu, Mikiko Aoki, Kazuo Tamura, Kazuki Nabeshima, Shotaro Sakisaka

Author information

Genryu Hirano
Department of Gastroenterology and Medicine, Fukuoka University Faculty of Medicine, Japan
Makoto Irie
Department of Gastroenterology and Medicine, Fukuoka University Faculty of Medicine, Japan
Yuta Nakashima
Department of Medical Oncology, Hematology and Infectious Disease, Fukuoka University Faculty of Medicine, Japan
Satoshi Shakado
Department of Gastroenterology and Medicine, Fukuoka University Faculty of Medicine, Japan
Tetsuro Sohda
Department of Gastroenterology and Medicine, Fukuoka University Faculty of Medicine, Japan
Toshihiro Tanaka
Department of Medical Oncology, Hematology and Infectious Disease, Fukuoka University Faculty of Medicine, Japan
Yasushi Takamatsu
Department of Medical Oncology, Hematology and Infectious Disease, Fukuoka University Faculty of Medicine, Japan
Mikiko Aoki
Department of Pathology, Fukuoka University Faculty of Medicine, Japan
Kazuo Tamura
Department of Medical Oncology, Hematology and Infectious Disease, Fukuoka University Faculty of Medicine, Japan
Kazuki Nabeshima
Department of Pathology, Fukuoka University Faculty of Medicine, Japan
Shotaro Sakisaka
Department of Gastroenterology and Medicine, Fukuoka University Faculty of Medicine, Japan

Keywords: chemotherapy, desmoplastic small round cell tumor, EWS-WT1 gene fusion transcript, reverse transcriptase polymerase chain reaction

JOURNAL OPEN ACCESS

2013 Volume 52 Issue 17 Pages 1909-1914

DOI https://doi.org/10.2169/internalmedicine.52.0431

Details

Abstract

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.

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