食道に発症したcalcifying fibrous pseudotumorの1例

Calcifying Fibrous Pseudotumor of the Esophagus, Report of a Case

要旨　CFP（calcifying fibrous pseudotumor，石灰化線維性偽腫瘍）は四肢，体幹，腋窩，胸膜，腹膜などの軟部組織に発生するまれで病理学的に特異的な偽腫瘍である．患者は60歳代，女性．食後のつまり感をもち，胃集団検診にて異所指摘を契機に紹介医を受診した．X線所見や内視鏡所見では下部食道に正常粘膜で覆われた粘膜下腫瘍様病変が存在していた．比較的立ち上がりが急峻で粘膜筋板から粘膜下層に発生した腫瘤と考えられた．CT検査所見では石灰化を伴う境界明瞭な腫瘤で造影効果は認めなかった．変性した平滑筋腫の術前診断にて胸部下部食道切除術を施行した．切除標本では5.8cm大の硬い腫瘤が粘膜下層に存在し，割面は境界明瞭な白色充実性腫瘤であった．組織学的には，硝子化した膠原線維成分が主体で，異型のない紡錘形細胞が散在していた．砂粒体を含む石灰化を認め，軽度のリンパ球，形質細胞浸潤を伴っていた．免疫組織化学的にはvimentinのみが陽性であり，食道CFPと診断した．消化管に発生するCFPはまれであり，食道での発生は本例が第1例目である．

　We reported our experience with one patient of CFT（calcifying fibrous pseudotumor）which occurred in the esophagus. The case was a 61-year-old women. We consulted a specialist about her chief complaint which was a feeling of some sort of blockage in the esophagus after editing a submucosal tumor lesion in the lower esophagus, was found. X-ray examination and endoscopy showed no abnormality in the mucosal surface. However, a mass with clear boundaries with calcification was shown by CT. We undertook lower esophagus resection because of the diagnosis of a denatured leiomyoma. The comparatively firm submucosal mass,5.8cm in size, was present in the esophageal lumen, and a white enhancement with clear boundaries in the sectioned surface-related mass showed a lot of calcification. The cell density was poor, and the fusiform cells were not of the aberrant type and, histologically, were shown to be scattered a resected specimen showed hyalinization and calcification including psammoma body, and there was mild lymphocytic and plasma cell infiltration into the mass. Immunohistologically tumor was CD34 negative,α-smooth muscle actin negative,S-100 negative, c-kit negative, vimentin positive.

　We presented a case of CFT of the esophagus. To our knowledge, this is the first case to be described.