BOWENOID PAPULOSIS, BOWENS-DISEASE AND SQUAMOUS-CELL CARCINOMA OF THE ANUS AND VULVA IN CONGENITAL INTESTINAL LYMPHANGIECTASIA

A 32-year-old woman with congenital intestinal lymphangiectasia (CIL) and warts (condylomata acuminata) was found to have bowenoid papulosis, Bowen's disease and squamous-cell carcinoma, at first in the anus, later also in the vulva. Limited surgical measures and laser vaporization with systemic and topical administration of interferon controlled the tumour development for some time. But after 7 years the squamous-cell carcinoma recurred with infiltration of the outer anogenital region. The patient then had an episode of thrombotic cerebral ischaemia, which prevented a planned abdominoperineal resection with radical vulvectomy. Instead she received chemotherapy with bleomycin, mitomycin and cisplatin. But she died 8 weeks later, from tumour cachexia. Occurrence of a, squamous-cell carcinoma of the anus and vulva in this young patient suggests a high oncogenic potential of the papilloma virus (HIV) infection. The latent period was probably shortened by a cellular immune deficiency as part of CIL. Treatment of the various carcinomatous manifestations should as long as possible be by local measures and interferon administration.