Two Cases of Succinate Dehydrogenase-Deficient Juvenile Gastric Gastrointestinal Stromal Tumor

SDH-deficient GIST is a part of WT GIST that constitutes approximately 10% of gastric GISTs and has no mutation of proto-oncogene receptor tyrosine kinase or PDGFR-α. In this paper, we present 2 cases of juvenile WT gastric GIST with SDH deficiency: a woman who underwent initial surgical treatment in junior high school and subsequently underwent 2 surgical treatments, and a man with lymph node metastasis who underwent distal gastrectomy with lymphadenectomy.

The 1st case was a woman who was diagnosed with gastric GIST and underwent distal gastrectomy at another institution when she was in junior high school. And she was diagnosed with gastric GIST again at our institution after a close examination for anemia and underwent laparoscopic partial gastrectomy. Two years ago, a GIS revealed another multiple gastric GIST in the remnant stomach, and a total remnant gastrectomy with lymphadenectomy was performed. The 2nd case was a man who was diagnosed with gastric GIST after a thorough examination of the cause of anemia. A 30-mm gastric GIST was found in the antrum, and a distal gastrectomy with lymphadenectomy was performed in this case as well. Pathological findings showed a metastatic lymph node in the subpyloric region.

Lymphadenectomy may be needed to improve the prognosis of juvenile GIST patients without distant metastasis because SDH-deficient GIST is more frequent in the younger generation, and SDH-deficient GIST has a higher frequency of lymph node metastasis.