What are the management and outcomes for Fanconi anemia (FA) patients with head and neck squamous cell carcinoma (HNSCC)?
Background: FA is a rare recessive disorder characterized clinically by bone marrow failure that can affect any of the hematopoietic lineages, along with congenital malformations, sensitivity to DNA cross-linking agents, and increased malignancy risk. In particular, HNSCCs are significantly more common in FA patients relative to the general population. Because hematopoietic stem cell transplantation (HSCT), preleukemia detection, leukemia therapies, and other advances have improved FA patient survival, an increasing number of these patients are developing HNSCCs. Management is often difficult because of sensitivity to both radiation therapy and chemotherapy agents.
Explore This IssueApril 2016
Study design: Cohort study of 35 FA patients with HNSCC over a 10-year period.
Setting: International Fanconi Anemia Registry (IFAR).
Synopsis: Twenty-seven of 35 patients had tumors with a known clinical stage (eight stage I; three stage II, one stage III, 15 stage IV). Cancer sites included the oral cavity, larynx, oropharynx, and esophagus. Information concerning tumor differentiation was available for 26 patients. (Nine well differentiated, 13 moderately differentiated, four poorly differentiated.) Margin status was available for 24 patients (18 negative margins, six microscopically positive margins). Thirty patients underwent surgical resection of their primary HNSCC. (Nine underwent flap reconstruction; the remaining patients had primary closure of their defects). Seven patients had a total of nine postoperative complications. Sixteen patients received radiation therapy. The most prevalent complications during radiation treatment were high-grade mucositis, hematologic abnormalities, and dysphagia. Eleven patients were able to complete the planned radiation dose; five patients had to prematurely halt or interrupt radiation therapy. Three patients received conventional chemotherapy; another three patients received targeted chemotherapy with cetuximab. Seventeen patients had recurrence of their tumor, with a median disease-free interval of 22 months. Thirty patients died during the study period; 16 died as a consequence of HNSCC. Of the 16 patients who underwent radiation therapy, 15 died, with four dying from complications while receiving radiation therapy.
Bottom line: FA 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.
Citation: Kutler DI, Patel KR, Auerbach AD. Natural history and management of Fanconi anemia patients with head and neck cancer: a 10-year follow-up. Laryngoscope. 2016;126:870-879.