TY - JOUR
T1 - Characterization of Fanconi Anemia Patients with Head and Neck Squamous Cell Carcinoma
T2 - Israel Fanconi Registry
AU - Tsur, Nir
AU - Frig, Omri
AU - Steinberg-Shemer, Orna
AU - Tamary, Hannah
AU - Kurman, Noga
AU - Mizrachi, Aviram
AU - Popovtzer, Aran
N1 - Publisher Copyright:
© 2022 Israel Medical Association. All rights reserved.
PY - 2022/8/1
Y1 - 2022/8/1
N2 - Background: Recent studies show a high risk of developing malignancy in patients with Fanconi anemia. The most common solid tumor in this condition is head and neck squamous cell carcinoma (HNSCC) and there is often uncertainty and about disease behavior as well as chemotherapy and radiation response. Objectives: To describe and characterize HNSCC among Fanconi anemia patients on the Israeli Fanconi Registry Methods: Our study population included patients in Israel's inherited bone marrow failure registry who were diagnosed with Fanconi anemia between 1980 and 2016. Demographic, clinical, and laboratory data were collected from patient charts. Results: From the collected data, HNSCC was confirmed in 6/111 (5.4%) Fanconi anemia patients; 1 (17%) had classic HNSCC risk factors of tobacco abuse and 4 (56%) had undergone primary surgery. The 3 (50%) receiving concurrent chemora-diotherapy had mild side effects, while half developed metachronous primary malignancy, and all developed > 2 primary malignancies. The overall median survival of the patients in our study was 14 (0.5-57) months. Conclusions: Fanconi anemia patients have a very high risk of developing HNSCC. Proactive screening for malignancies is needed for the head and neck regions. We also found that chemoradiotherapy can be used safely in high-stage cancers.
AB - Background: Recent studies show a high risk of developing malignancy in patients with Fanconi anemia. The most common solid tumor in this condition is head and neck squamous cell carcinoma (HNSCC) and there is often uncertainty and about disease behavior as well as chemotherapy and radiation response. Objectives: To describe and characterize HNSCC among Fanconi anemia patients on the Israeli Fanconi Registry Methods: Our study population included patients in Israel's inherited bone marrow failure registry who were diagnosed with Fanconi anemia between 1980 and 2016. Demographic, clinical, and laboratory data were collected from patient charts. Results: From the collected data, HNSCC was confirmed in 6/111 (5.4%) Fanconi anemia patients; 1 (17%) had classic HNSCC risk factors of tobacco abuse and 4 (56%) had undergone primary surgery. The 3 (50%) receiving concurrent chemora-diotherapy had mild side effects, while half developed metachronous primary malignancy, and all developed > 2 primary malignancies. The overall median survival of the patients in our study was 14 (0.5-57) months. Conclusions: Fanconi anemia patients have a very high risk of developing HNSCC. Proactive screening for malignancies is needed for the head and neck regions. We also found that chemoradiotherapy can be used safely in high-stage cancers.
KW - Fanconi anemia
KW - chemoradiation therapy
KW - head and neck squamous cell carcinoma (HNSCC)
KW - survival
UR - http://www.scopus.com/inward/record.url?scp=85136077893&partnerID=8YFLogxK
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C2 - 35971997
AN - SCOPUS:85136077893
SN - 1565-1088
VL - 24
SP - 491
EP - 496
JO - Israel Medical Association Journal
JF - Israel Medical Association Journal
IS - 8
ER -