2021
DOI: 10.1158/1078-0432.ccr-21-1259
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Abstract: Fanconi anemia, the most frequent genetic cause of bone marrow failure, is characterized by an extreme predilection toward multiple malignancies, including a greater than 500-fold incidence of head and neck squamous cell carcinoma (HNSCC) relative to the general population. Fanconi anemia–associated HNSCC and esophageal SCC (FA-HNSCC) often present at advanced stages with poor survival. Surgical resection remains the primary treatment for FA-HNSCC, and there is often great reluctance to administer systemic age… Show more

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Cited by 9 publications
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“…Targeted chemo-therapy (such as cetuximab or gefitinib) or immuno-therapy (e.g., nivolumab, pembrolizumab) are definitely intriguing, but their actual efficacy and complication rates have still to be assessed overall [ 37 ]. It follows that the first-line therapeutic strategy is surgery.…”
Section: Discussionmentioning
confidence: 99%
See 1 more Smart Citation
“…Targeted chemo-therapy (such as cetuximab or gefitinib) or immuno-therapy (e.g., nivolumab, pembrolizumab) are definitely intriguing, but their actual efficacy and complication rates have still to be assessed overall [ 37 ]. It follows that the first-line therapeutic strategy is surgery.…”
Section: Discussionmentioning
confidence: 99%
“…It follows that the first-line therapeutic strategy is surgery. As demonstrated by Lee, patients with FA can also undergo major surgery without developing particular complications [ 37 ]. Conspicuous resective interventions are also possible, associated with reconstructive surgery which may include free flaps as well.…”
Section: Discussionmentioning
confidence: 99%
“…However, some reports suggest that HPV may be a major contributor to HNSCC development in patients with FA 3 , whereas other studies dispute these results 4 . HNSCC in FA is difficult to treat, as patients cannot tolerate current therapies, including ionizing radiation or DNA cross-linking, platinum-based chemotherapies 5 . In addition, most tumors are detected at advanced stages.…”
Section: Introductionmentioning
confidence: 99%
“…Multiple oral cavity carcinomas are distressingly common (3), and an elevated risk of cutaneous squamous carcinomas has been documented together with a continuing, elevated risk of leukemia (3,4). However, SCC may be the first clinical presentation of FA at any age (1,5). Many other cancer types have been reported in individuals with FA, but only a small subset - liver and brain tumors, lymphomas, and embryonal tumors such as Wilms tumor and neuroblastoma - are observed at higher-than-expected frequencies versus population controls (3), and none with risk estimates approaching those of squamous mucosal carcinomas.…”
Section: Introductionmentioning
confidence: 99%
“…Effective therapies for FA-associated SCC have also proven elusive: many cancers are detected too late for surgical cure, and FA patients cannot tolerate effective doses of standard-of-care therapies including DNA cross-linking drugs and ionizing radiation (5). These features of cancer in FA - the often-late clinical recognition, synchronous or metasynchronous tumors and therapeutic intractability - have collectively made SCC the leading cause of premature morbidity and mortality in adults with FA.…”
Section: Introductionmentioning
confidence: 99%