A case of laryngeal carcinoma in a young adult with dyskeratosis congenita

Noritaka Komune, Takashi Hara, Akihiro Tamae, Kunio Izu, Yoshiki Tokura, Andrew K. Joe, Muneyuki Masuda

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)428-432
Number of pages5
JournalInternational Journal of Clinical Oncology
Volume15
Issue number4
DOIs
Publication statusPublished - Aug 1 2010
Externally publishedYes

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Dyskeratosis Congenita
Young Adult
Carcinoma
Leukoplakia
Neck Dissection
Laryngeal Neoplasms
Genetic Therapy
Skin Pigmentation
Laryngectomy
Premature Mortality
Telomerase
Nails
Lung Diseases
Language
Bone Marrow
Neoplasm Metastasis

All Science Journal Classification (ASJC) codes

  • Surgery
  • Hematology
  • Oncology

Cite this

A case of laryngeal carcinoma in a young adult with dyskeratosis congenita. / Komune, Noritaka; Hara, Takashi; Tamae, Akihiro; Izu, Kunio; Tokura, Yoshiki; Joe, Andrew K.; Masuda, Muneyuki.

In: International Journal of Clinical Oncology, Vol. 15, No. 4, 01.08.2010, p. 428-432.

Research output: Contribution to journalArticle

Komune, Noritaka ; Hara, Takashi ; Tamae, Akihiro ; Izu, Kunio ; Tokura, Yoshiki ; Joe, Andrew K. ; Masuda, Muneyuki. / A case of laryngeal carcinoma in a young adult with dyskeratosis congenita. In: International Journal of Clinical Oncology. 2010 ; Vol. 15, No. 4. pp. 428-432.
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