Abstract
A case of Cole-Engman syndrome with multiple oral cancers is reported. The patient had reticulated telangiectatic pigmented skin, lack or dystrophy of nails, epiphora, hyperkeratinization of the palms and soles, hyperhidrosis, extensive teeth loss, interstitial pneumonia, mild gastritis, and stenosis of the external urethral ostium, but no anemia or splenomegaly.
Intraorally, there were a squamous cell carcinoma of the left mandibular gingiva and leukoplakias of the tongue and the right buccal mucosa. After radiotherapy with Co60 in a total dose of 34 Gy and chemotherapy with 6.9g of UFT, the patient underwent resection of the carcinoma by segmental mandibulectomy and bilateral upper neck dissection with removal of the leukoplakias. An additional resection was carried out 4 months later, because of the development of a tumor in the right buccal mucosa. There has been no sign of recurrence 2 and a half years after the second operation.
A review of the Japanese literature from 1960 through 1998 revealed 37 cases of the Cole-Engman syndrome.
