Natural history and management of Fanconi anemia patients with head and neck cancer: A 10-year follow-up.
OBJECTIVE
To describe the management and outcomes of Fanconi anemia (FA) patients with head and neck squamous cell carcinoma.


METHODS
Cohort study.


METHODS
Demographic information, prognostic factors, therapeutic management, and survival outcomes for FA patients enrolled in the International Fanconi Anemia Registry who developed head and neck squamous cell carcinoma (HNSCC) were analyzed.


RESULTS
Thirty-five FA patients were diagnosed with HNSCC at a mean age of 32 years. The most common site of primary cancer was the oral cavity (26 of 35, 74%). Thirty patients underwent surgical resection of the cancer. Sixteen patients received radiation therapy with an average radiation dose of 5,050 cGy. The most common toxicities were high-grade mucositis (9 of 16, 56%), hematologic abnormalities (8 of 16, 50%), and dysphagia (8 of 16, 50%). Three patients received conventional chemotherapy and had significant complications, whereas three patients who received targeted chemotherapy with cetuximab had fewer toxicities. The 5-year overall survival rate was 39%, with a cause-specific survival rate of 47%.


CONCLUSIONS
Fanconi anemia patients have a high risk of developing aggressive HNSCC at an early age. Fanconi anemia patients can tolerate complex ablative and reconstructive surgeries, but careful postoperative care is required to reduce morbidity. The treatment of FA-associated HNSCC is difficult secondary to the poor tolerance of radiation and chemotherapy. However, radiation should be used for high-risk cancers due to the poor survival in these patients.


METHODS
4.
Department of Otolaryngology-Head and Neck Surgery, New York Presbyterian Hospital/Weill Cornell Medical College, New York, New York, U.S.A.
David I
DI
Kutler
Weill Cornell Medical College, The Rockefeller University, New York, New York, U.S.A.
Krupa R
KR
Patel
Program in Human Genetics and Hematology, The Rockefeller University, New York, New York, U.S.A.
Arleen D
AD
Auerbach
Laboratory of Genome Maintenance, The Rockefeller University, New York, New York, U.S.A.
Jennifer
J
Kennedy
Laboratory of Genome Maintenance, The Rockefeller University, New York, New York, U.S.A.
Francis P
FP
Lach
Laboratory of Genome Maintenance, The Rockefeller University, New York, New York, U.S.A.
Erica
E
Sanborn
Department of Otolaryngology-Head and Neck Surgery, New York Presbyterian Hospital/Weill Cornell Medical College, New York, New York, U.S.A.
Marc A
MA
Cohen
Department of Otolaryngology-Head and Neck Surgery, New York Presbyterian Hospital/Weill Cornell Medical College, New York, New York, U.S.A.
William I
WI
Kuhel
Laboratory of Genome Maintenance, The Rockefeller University, New York, New York, U.S.A.
Agata
A
Smogorzewska
D000293:Adolescent; D000328:Adult; D002294:Carcinoma, Squamous Cell / Q000150:complications* / Q000628:therapy; D005199:Fanconi Anemia / Q000209:etiology* / Q000628:therapy*; D005260:Female; D005500:Follow-Up Studies; D006258:Head and Neck Neoplasms / Q000150:complications* / Q000628:therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D012307:Risk Factors; D015996:Survival Rate; D016896:Treatment Outcome
Fanconi anemia; head and neck; squamous cell carcinoma