A Case of Dyskeratosis Congenita Complicated by Tongue Cancer
| Autor: | Eiji Yumoto, Masamitsu Hyodo, Yasuyuki Hinohira, Akiko Sadamoto |
|---|---|
| Rok vydání: | 1999 |
| Předmět: | |
| Zdroj: | Practica Oto-Rhino-Laryngologica. 92:253-257 |
| ISSN: | 1884-4545 0032-6313 |
| DOI: | 10.5631/jibirin.92.253 |
| Popis: | Dyskeratosis congenita (DC) is a rare form of ectodermal dysplasia characterized by reticulated hyperpigmentation of the skin, leukoplakia of the mucosa, and dystrophic nails. Furthermore, malignant neoplasmas arising from the leukoplakia and bone marrow aplasia are critical complications. We reported a female case of DC with tongue cancer. She complained of leukoplakia of the tongue since the age of 6 years. She was diagnosed with DC when she was 20 years old based on typical findings of the skin and nails. Four years later, she returned to our clinic because of prolonged tongue pain, and tongue cancer (T3N1M0) was detected. Radiation was administered as an initial treatment, however, it was suspended due to development of severe radiation mucositis. Severe pancytopenia prohibited the choice of chemotherapy. We performed unilateral radical neck dissection and subtotal glossectomy followed by reconstruction of the tongue. Postoperative phonatory and swallowing functions were acceptable and she returned to work. However she subsequently died of recurrence due to contralateral neck lymph node and lung metastasis 8 months postoperatively. Surgical therapy seems to be beneficial in some patients suffering from DC complicated by malignancy, although careful management of the general condition is mandatory. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|