Desmoplastic small round cell tumor of the stomach mimicking a gastric cancer in a child.
10.4174/jkss.2011.80.Suppl1.S80
- Author:
Suk Bae MOON
;
Jung Min HUR
;
Hong Hoe KOO
;
Yeon Lim SUH
;
Hyun Baek SHIN
;
Jeong Meen SEO
;
Suk Koo LEE
- Publication Type:Case Report
- Keywords:
Stomach;
Desmoplastic small round cell tumor;
Children
- MeSH:
Abdominal Pain;
Biopsy;
Child;
Desmoplastic Small Round Cell Tumor;
Fluorescence;
Gastrectomy;
Gastroscopy;
Humans;
In Situ Hybridization;
Laparotomy;
Neoplasm Metastasis;
Neoplasm, Residual;
Positron-Emission Tomography;
Stomach;
Stomach Neoplasms
- From:Journal of the Korean Surgical Society
2011;80(Suppl 1):S80-S84
- CountryRepublic of Korea
- Language:English
-
Abstract:
Intra-abdominal desmoplastic small round cell tumor (DSRCT) is a highly malignant tumor of uncertain histogenesis. Here we report a case of DSRCT involving the stomach, initially misdiagnosed as gastric cancer. A 12-year-old boy presented with upper abdominal pain developed 1 month prior. On gastroscopy, a 7-cm mass was noted involving the esophago-gastric junction to the fundus, and positron emission tomography showed multiple hot uptakes suggesting distant metastasis. Gastroscopic biopsy showed poorly differentiated malignant cells. We diagnosed as stage IV gastric cancer and treated with 6 cycles of chemotherapy. Laparotomy revealed a huge gastric mass along with peritoneal disseminations. Palliative proximal gastrectomy was performed. Pathological examination revealed transmural involvement of DSRCT, and t(11;22)(p12;q12) was demonstrated on fluorescence in situ hybridization test. The chemotherapeutic regimen was changed and the patient underwent 8 additional cycles of post-operative chemotherapy. The patient is now alive and the residual tumor shows no significant changes after chemotherapy.
