Desmoplastic Small Round Cell Tumor of Stomach
Desmoplastic small round cell tumor (DSRCT) is an extremely uncommon, highly aggressive, and malignant mesenchymal neoplasm of undetermined histogenesis. Less than 200 case reports have been documented in literature so far. Herein, we report a 26-year-old otherwise healthy female patient who present...
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| Format: | Article |
| Language: | English |
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Wiley
2013-01-01
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| Series: | Case Reports in Gastrointestinal Medicine |
| Online Access: | http://dx.doi.org/10.1155/2013/907136 |
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| author | Ahmed Abu-Zaid Ayman Azzam Asma AlNajjar Hussa Al-Hussaini Tarek Amin |
| author_facet | Ahmed Abu-Zaid Ayman Azzam Asma AlNajjar Hussa Al-Hussaini Tarek Amin |
| author_sort | Ahmed Abu-Zaid |
| collection | DOAJ |
| description | Desmoplastic small round cell tumor (DSRCT) is an extremely uncommon, highly aggressive, and malignant mesenchymal neoplasm of undetermined histogenesis. Less than 200 case reports have been documented in literature so far. Herein, we report a 26-year-old otherwise healthy female patient who presented with a 1-month history of epigastric pain. On physical examination, a palpable, slightly mobile, and tender epigastric mass was detected. All laboratory tests were normal. A chest, abdominal, and pelvic contrast-enhanced computed tomography (CT) scans showed a 3.8 × 7.2 × 8.7 cm ill-defined mass, involving gastric fundus and extending into gastric cardia and lower gastroesophageal junction. It was associated with multiple enlarged gastrohepatic lymph nodes; the largest measured 1.2 cm. There was no evidence of ascites or retroperitoneal or mesenteric lymphatic metastases. Patient underwent total gastrectomy with D2 lymphadenectomy, splenectomy, and antecolic Roux-en-Y esophagojejunal anastomosis. Histopathological examination revealed coexpression of mesenchymal, epithelial, and neural markers. The characteristic chromosomal translocation (t(11; 22)(p13; q12)) was demonstrated on fluorescence in situ hybridization (FISH) technique. Diagnosis of DSRCT of stomach was confirmed. Patient received no postoperative radiotherapy or chemotherapy. A postoperative 3-month followup failed to show any recurrence. In addition, a literature review on DSRCT is included. |
| format | Article |
| id | doaj-art-26e859513ef04be48b9f3442d6572a3b |
| institution | Kabale University |
| issn | 2090-6528 2090-6536 |
| language | English |
| publishDate | 2013-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Gastrointestinal Medicine |
| spelling | doaj-art-26e859513ef04be48b9f3442d6572a3b2025-02-03T05:58:16ZengWileyCase Reports in Gastrointestinal Medicine2090-65282090-65362013-01-01201310.1155/2013/907136907136Desmoplastic Small Round Cell Tumor of StomachAhmed Abu-Zaid0Ayman Azzam1Asma AlNajjar2Hussa Al-Hussaini3Tarek Amin4College of Medicine, Alfaisal University, P.O. Box 50927, Riyadh 11533, Saudi ArabiaDepartment of General Surgery, Faculty of Medicine, Alexandria University, Alexandria 21526, EgyptCollege of Medicine, Alfaisal University, P.O. Box 50927, Riyadh 11533, Saudi ArabiaDepartment of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Center (KFSH&RC), P.O. Box 3354, Riyadh 11211, Saudi ArabiaDepartment of Surgical Oncology, King Faisal Specialist Hospital and Research Center (KFSH&RC), P.O. Box 3354, Riyadh 11211, Saudi ArabiaDesmoplastic small round cell tumor (DSRCT) is an extremely uncommon, highly aggressive, and malignant mesenchymal neoplasm of undetermined histogenesis. Less than 200 case reports have been documented in literature so far. Herein, we report a 26-year-old otherwise healthy female patient who presented with a 1-month history of epigastric pain. On physical examination, a palpable, slightly mobile, and tender epigastric mass was detected. All laboratory tests were normal. A chest, abdominal, and pelvic contrast-enhanced computed tomography (CT) scans showed a 3.8 × 7.2 × 8.7 cm ill-defined mass, involving gastric fundus and extending into gastric cardia and lower gastroesophageal junction. It was associated with multiple enlarged gastrohepatic lymph nodes; the largest measured 1.2 cm. There was no evidence of ascites or retroperitoneal or mesenteric lymphatic metastases. Patient underwent total gastrectomy with D2 lymphadenectomy, splenectomy, and antecolic Roux-en-Y esophagojejunal anastomosis. Histopathological examination revealed coexpression of mesenchymal, epithelial, and neural markers. The characteristic chromosomal translocation (t(11; 22)(p13; q12)) was demonstrated on fluorescence in situ hybridization (FISH) technique. Diagnosis of DSRCT of stomach was confirmed. Patient received no postoperative radiotherapy or chemotherapy. A postoperative 3-month followup failed to show any recurrence. In addition, a literature review on DSRCT is included.http://dx.doi.org/10.1155/2013/907136 |
| spellingShingle | Ahmed Abu-Zaid Ayman Azzam Asma AlNajjar Hussa Al-Hussaini Tarek Amin Desmoplastic Small Round Cell Tumor of Stomach Case Reports in Gastrointestinal Medicine |
| title | Desmoplastic Small Round Cell Tumor of Stomach |
| title_full | Desmoplastic Small Round Cell Tumor of Stomach |
| title_fullStr | Desmoplastic Small Round Cell Tumor of Stomach |
| title_full_unstemmed | Desmoplastic Small Round Cell Tumor of Stomach |
| title_short | Desmoplastic Small Round Cell Tumor of Stomach |
| title_sort | desmoplastic small round cell tumor of stomach |
| url | http://dx.doi.org/10.1155/2013/907136 |
| work_keys_str_mv | AT ahmedabuzaid desmoplasticsmallroundcelltumorofstomach AT aymanazzam desmoplasticsmallroundcelltumorofstomach AT asmaalnajjar desmoplasticsmallroundcelltumorofstomach AT hussaalhussaini desmoplasticsmallroundcelltumorofstomach AT tarekamin desmoplasticsmallroundcelltumorofstomach |