色素性乾皮症に伴った結膜扁平上皮癌の1例

Conjunctival squamous cell carcinoma in a patient with xeroderma pigmentosum

　41歳女性の色素性乾皮症に伴った結膜扁平上皮癌を経験した。症例は6歳頃から顔面に色素斑が出現し，22歳で皮膚生検により色素性乾皮症と診断された。その後7回の顔面腫瘍切除術を受け，いずれも基底細胞癌であった。1987年5月，左眼の充血を主訴に当院眼科にて受診したところ結膜腫瘍が認められ，切除標本は扁平上皮癌と診断された。そののち，更に顔面皮膚の5か所の腫瘍切除術を受けたが，組織学的にはそれぞれ基底細胞型扁平上皮癌，基底細胞癌，毛嚢上皮腫及び腺扁平上皮癌と診断は多岐にわたっていた。このような多段階にわたる悪性像を呈する腫瘍を合併することは稀と考えるので報告する。

A 41-year-old female presented with hyperemia in her left eye since 2 weeks before. She had manifested signs of xeroderma pigmentosum since 6 years of age and was diagnosed as such at the age of 22 years. The family history was negative. No consanguinity was present. She had undergone multiple excisions of dermal tumors which proved to he basal cell carcinoma. She manifested atumor-like mass nasal to the left conjunctive in May 1987. Surgical removal of the conjunctival tumor led to the diagnosis of squamous cell car-cinoma. After that operation, she underwent exci-sion of five dermal tumors on her face which proved to be a basalsquamous cell carcinoma, a basal cell epithelioma, two trichoepitheliomas and an adenosquamous cell carcinoma. No recurrence of the tumors has been observed during 2 years after surgery.