Intra-Abdominal Desmoplastic Small Round Cell Tumor with Elevated Serum CA 125: A Case Report
Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive tumor usually involving the peritoneum. It occurs more commonly in young males and is characterized by distinctive clinical, histologic, and immunophenotypic features. The histogenesis of DSRCT remains unknown. Coexpression...
| Main Authors: | , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2003-10-01
|
| Series: | Kaohsiung Journal of Medical Sciences |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S1607551X09705030 |
| _version_ | 1819105283896508416 |
|---|---|
| author | Sheau-Fang Yang Sheng-Lan Wang Chee-Yin Chai |
| author_facet | Sheau-Fang Yang Sheng-Lan Wang Chee-Yin Chai |
| author_sort | Sheau-Fang Yang |
| collection | DOAJ |
| description | Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive tumor usually involving the peritoneum. It occurs more commonly in young males and is characterized by distinctive clinical, histologic, and immunophenotypic features. The histogenesis of DSRCT remains unknown. Coexpression of epithelial, mesenchymal, and neural antigens in the same cell provides evidence that DSRCT may arise from a primitive pluripotent stem cell with divergent differentiation. Recently, according to cytogenetic studies, some authors have proposed that the divergent differentiation of DSRCT may be the result of the fusion of Ewing's sarcoma gene and Wilms' tumor suppressor gene. Clinically, an elevated serum CA 125 concentration is found in some patients with DSRCT. We present the case of a 29-year-old man with diffuse intra-abdominal DSRCT and elevated serum CA 125 concentration and briefly review the relevant literature. |
| first_indexed | 2024-12-22T02:19:48Z |
| format | Article |
| id | doaj.art-70f007b039ef41d2b0947d0f9938930b |
| institution | Directory Open Access Journal |
| issn | 1607-551X |
| language | English |
| last_indexed | 2024-12-22T02:19:48Z |
| publishDate | 2003-10-01 |
| publisher | Wiley |
| record_format | Article |
| series | Kaohsiung Journal of Medical Sciences |
| spelling | doaj.art-70f007b039ef41d2b0947d0f9938930b2022-12-21T18:42:09ZengWileyKaohsiung Journal of Medical Sciences1607-551X2003-10-01191053153510.1016/S1607-551X(09)70503-0Intra-Abdominal Desmoplastic Small Round Cell Tumor with Elevated Serum CA 125: A Case ReportSheau-Fang YangSheng-Lan WangChee-Yin ChaiDesmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive tumor usually involving the peritoneum. It occurs more commonly in young males and is characterized by distinctive clinical, histologic, and immunophenotypic features. The histogenesis of DSRCT remains unknown. Coexpression of epithelial, mesenchymal, and neural antigens in the same cell provides evidence that DSRCT may arise from a primitive pluripotent stem cell with divergent differentiation. Recently, according to cytogenetic studies, some authors have proposed that the divergent differentiation of DSRCT may be the result of the fusion of Ewing's sarcoma gene and Wilms' tumor suppressor gene. Clinically, an elevated serum CA 125 concentration is found in some patients with DSRCT. We present the case of a 29-year-old man with diffuse intra-abdominal DSRCT and elevated serum CA 125 concentration and briefly review the relevant literature.http://www.sciencedirect.com/science/article/pii/S1607551X09705030desmoplastic small round cell tumorintra-abdominalCA 125 |
| spellingShingle | Sheau-Fang Yang Sheng-Lan Wang Chee-Yin Chai Intra-Abdominal Desmoplastic Small Round Cell Tumor with Elevated Serum CA 125: A Case Report Kaohsiung Journal of Medical Sciences desmoplastic small round cell tumor intra-abdominal CA 125 |
| title | Intra-Abdominal Desmoplastic Small Round Cell Tumor with Elevated Serum CA 125: A Case Report |
| title_full | Intra-Abdominal Desmoplastic Small Round Cell Tumor with Elevated Serum CA 125: A Case Report |
| title_fullStr | Intra-Abdominal Desmoplastic Small Round Cell Tumor with Elevated Serum CA 125: A Case Report |
| title_full_unstemmed | Intra-Abdominal Desmoplastic Small Round Cell Tumor with Elevated Serum CA 125: A Case Report |
| title_short | Intra-Abdominal Desmoplastic Small Round Cell Tumor with Elevated Serum CA 125: A Case Report |
| title_sort | intra abdominal desmoplastic small round cell tumor with elevated serum ca 125 a case report |
| topic | desmoplastic small round cell tumor intra-abdominal CA 125 |
| url | http://www.sciencedirect.com/science/article/pii/S1607551X09705030 |
| work_keys_str_mv | AT sheaufangyang intraabdominaldesmoplasticsmallroundcelltumorwithelevatedserumca125acasereport AT shenglanwang intraabdominaldesmoplasticsmallroundcelltumorwithelevatedserumca125acasereport AT cheeyinchai intraabdominaldesmoplasticsmallroundcelltumorwithelevatedserumca125acasereport |