Moreover, GI LMS is an extremely rare cancer representing 3%–6% of all GI mesenchymal tumors.5) In the past, before the diagnosis of KIT mutations by IHC, the prognosis of CLMS was generally considered to be a benign tumor that displayed optimism with a low propensity for recurrence and distant metastasis.6) Later on, the number of reports on true CLMS has rapidly decreased, and those reports have shown that frequent recurrences and distant metastasis have been observed in the CLMS.7) The gene discussed is KIT; the disease is mesenchymal cell neoplasm.