J Korean Surg Soc.  2011 Jun;80(Suppl 1):S80-S84. 10.4174/jkss.2011.80.Suppl1.S80.

Desmoplastic small round cell tumor of the stomach mimicking a gastric cancer in a child

• Moon SB
• Hur JM
• Koo HH
• Suh YL
• Shin HB
• Seo JM
• Lee SK

• KMID: 2096658
• DOI: http://doi.org/10.4174/jkss.2011.80.Suppl1.S80

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.

Keyword

Stomach; Desmoplastic small round cell tumor; Children

MeSH Terms

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

Figure

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  Fig. 1 Pre-operative gastroscopy shows huge mass located at the esophago-gastric junction.

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  Fig. 2 Multiple, enlarged (A) retropancreatic and (B) aortocaval, retrocaval lymph nodes are seen on abdominal computed tomography scan (line-indicated).

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  Fig. 3 Positron emission tomography scan. Numerous hot uptakes are seen at left supraclavicular area, retroperitoneum, pelvis, and pleura, thus suggesting metastases.

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  Fig. 4 (A) Sharply demarcated aggregates of small round cells are seen within the dense fibrous stroma (H&E, ×200), and (B) tumor cells are positive for desmin (dot-like expression, ×200) and (C) cytokeratin (×200) in immunohistochemical staining.

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