von Recklinghausen病に胃癌をみた1例

Advanced Gastric Cancer with Massive Lymphoid Stroma in von Recklinghausen Neurofibromatosis, Report of a Case

要旨　von Recklinghausen病に進行胃癌を合併した症例を報告した．患者は53歳，男性．主訴は心窩部不快感．上部消化管造影，内視鏡検査で胃角前壁に径60mmのBorrmann 2型の腫瘍がみられ手術を施行した．手術材料では胃角前壁に60×50mmのBorrmann 2型の腫瘍が認められ，組織所見は中分化型管状腺癌であり，粘膜下層から固有筋層を貫いて胚中心を伴ったリンパ組織の著明な増生を伴っていた．腫瘍細胞に対するリンパ組織-間葉系のhyperreactivityを表しているものと思われる．

　A 53-year-old man with von Recklinghausen neurofibromatosis was admitted to Hakodate Chuo Hospital because of epigastrial discomfort. Laboratory findings revealed hypochromic and microcytic anemia, but negative for fecal occult blood. X-ray and endoscopic examinations revealed Borrmann 2 type advanced gastric carcinoma at the anterior wall of the gastric angle. Histopathological findings showed moderately differentiated tubular adenocarcinoma (pm, INFγ, V_o, Ly₃) with massive lymphoid stroma which revealed transluminar proliferation. Massive lymphoid stroma may be due to hyperreactivity of lymphoid tissue against cancer cell in von Recklinghausen neurofibromatosis. This case of von Recklinghausen disease may be caused by mutation, because no relatives had neurofibromatosis. Genetic basis of neurofibromatosis which seems to be related to mutation may have some role in the occurrence of gastric cancer. Neurofibromatosis must be considered as precancerous state in the stomach.