TY - JOUR
T1 - A case of laryngeal carcinoma in a young adult with dyskeratosis congenita
AU - Komune, Noritaka
AU - Hara, Takashi
AU - Tamae, Akihiro
AU - Izu, Kunio
AU - Tokura, Yoshiki
AU - Joe, Andrew K.
AU - Masuda, Muneyuki
N1 - Funding Information:
This study was supported in part by funds from Grants-in-Aid for Scientific Research (C): 21592195 to Muneyuki Masuda.
PY - 2010/8
Y1 - 2010/8
N2 - Dyskeratosis congenita (DC) is an inherited disorder that is characterized by the triad of skin pigmentation, nail dystrophy, and mucosal leukoplakia. Individuals with DC suffer from premature mortality because of bone marrow failure, pulmonary disease, or malignant transformation within the areas of mucosal leukoplakia, caused by telomerase dysfunction. We present a case of a 31-year-old Japanese man with DC who developed laryngeal cancer (supraglottic T4aN0M0). To avoid the serious risks of accelerating the DC-associated complications by DNA-damaging therapies, he was treated with a total laryngectomy plus right modified neck dissection (levels IB, IIA, III, and IV). A contralateral nodal metastasis appeared 4 months after initial surgery and was salvaged by a left radical neck dissection. Our strategy to spare DNA-damaging therapies has proven effective so far. This is the first reported case of laryngeal cancer in a patient with DC in the English-language medical literature.
AB - Dyskeratosis congenita (DC) is an inherited disorder that is characterized by the triad of skin pigmentation, nail dystrophy, and mucosal leukoplakia. Individuals with DC suffer from premature mortality because of bone marrow failure, pulmonary disease, or malignant transformation within the areas of mucosal leukoplakia, caused by telomerase dysfunction. We present a case of a 31-year-old Japanese man with DC who developed laryngeal cancer (supraglottic T4aN0M0). To avoid the serious risks of accelerating the DC-associated complications by DNA-damaging therapies, he was treated with a total laryngectomy plus right modified neck dissection (levels IB, IIA, III, and IV). A contralateral nodal metastasis appeared 4 months after initial surgery and was salvaged by a left radical neck dissection. Our strategy to spare DNA-damaging therapies has proven effective so far. This is the first reported case of laryngeal cancer in a patient with DC in the English-language medical literature.
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U2 - 10.1007/s10147-010-0053-x
DO - 10.1007/s10147-010-0053-x
M3 - Article
C2 - 20198396
AN - SCOPUS:77955710945
SN - 1341-9625
VL - 15
SP - 428
EP - 432
JO - International Journal of Clinical Oncology
JF - International Journal of Clinical Oncology
IS - 4
ER -
