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      Call for Papers: Digital Diagnostic Techniques

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      Extensive Lower Lip Squamous Cell Carcinoma in a Fanconi Anaemia Patient and Treatment Delays during COVID-19 Pandemic

      case-report

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          Abstract

          Fanconi anaemia (FA) is an autosomal recessive inherited disease that renders patients susceptible to congenital anomalies, bone marrow failures, leukaemia, and solid malignancies. FA is caused by the loss of function of at least one gene in the FA/BRCA biological pathway, which is involved in DNA repair. Patients with FA have an increased risk of developing head and neck cancer, particularly oral squamous cell carcinoma (SCC). Due to susceptibility of head and neck cancer at a very young age, relatively poor survival rate, low tolerance to oncologic interventions, and complexity of treatments, strict follow-up is mandatory to detect any changes or recurrence of SCC in the head and neck region in FA patients. Surgery is the mainstay of treatment, but adjuvant therapy should be instituted when needed. This short report describes a rare case of lower lip SCC in FA and its management. It also highlights the impact of the COVID-19 pandemic on healthcare practices.

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          Radiotherapy plus cetuximab for squamous-cell carcinoma of the head and neck.

          James A. Bonner, Paul Harari, Jordi Giralt … (2006)
          We conducted a multinational, randomized study to compare radiotherapy alone with radiotherapy plus cetuximab, a monoclonal antibody against the epidermal growth factor receptor, in the treatment of locoregionally advanced squamous-cell carcinoma of the head and neck. Patients with locoregionally advanced head and neck cancer were randomly assigned to treatment with high-dose radiotherapy alone (213 patients) or high-dose radiotherapy plus weekly cetuximab (211 patients) at an initial dose of 400 mg per square meter of body-surface area, followed by 250 mg per square meter weekly for the duration of radiotherapy. The primary end point was the duration of control of locoregional disease; secondary end points were overall survival, progression-free survival, the response rate, and safety. The median duration of locoregional control was 24.4 months among patients treated with cetuximab plus radiotherapy and 14.9 months among those given radiotherapy alone (hazard ratio for locoregional progression or death, 0.68; P=0.005). With a median follow-up of 54.0 months, the median duration of overall survival was 49.0 months among patients treated with combined therapy and 29.3 months among those treated with radiotherapy alone (hazard ratio for death, 0.74; P=0.03). Radiotherapy plus cetuximab significantly prolonged progression-free survival (hazard ratio for disease progression or death, 0.70; P=0.006). With the exception of acneiform rash and infusion reactions, the incidence of grade 3 or greater toxic effects, including mucositis, did not differ significantly between the two groups. Treatment of locoregionally advanced head and neck cancer with concomitant high-dose radiotherapy plus cetuximab improves locoregional control and reduces mortality without increasing the common toxic effects associated with radiotherapy to the head and neck. (ClinicalTrials.gov number, NCT00004227.) Copyright 2006 Massachusetts Medical Society.
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            Solid cancers after bone marrow transplantation.

            J Fraumeni, J A Boice, G. Socié … (1997)
            The late effects of bone marrow transplantation, including cancer, need to be determined in a large population at risk. We studied 19,229 patients who received allogeneic transplants (97.2 percent) or syngeneic transplants (2.8 percent) between 1964 and 1992 at 235 centers to evaluate the risk of the development of a new solid cancer. Risk factors relating to the patient, the transplant, and the course after transplantation were evaluated. The transplant recipients were at significantly higher risk of new solid cancers than the general population (observed cases, 80; ratio of observed to expected cases, 2.7; P<0.001). The risk was 8.3 times higher than expected among those who survived 10 or more years after transplantation. The cumulative incidence rate was 2.2 percent (95 percent confidence interval, 1.5 to 3.0 percent) at 10 years and 6.7 percent (95 percent confidence interval, 3.7 to 9.6 percent) at 15 years. The risk was significantly elevated (P<0.05) for malignant melanoma (ratio of observed to expected cases, 5.0) and cancers of the buccal cavity (11.1), liver (7.5), brain or other parts of the central nervous system (7.6), thyroid (6.6), bone (13.4), and connective tissue (8.0). The risk was higher for recipients who were younger at the time of transplantation than for those who were older (P for trend <0.001). In multivariate analyses, higher doses of total-body irradiation were associated with a higher risk of solid cancers. Chronic graft-versus-host disease and male sex were strongly linked with an excess risk of squamous-cell cancers of the buccal cavity and skin. Patients undergoing bone marrow transplantation have an increased risk of new solid cancers later in life. The trend toward an increased risk over time after transplantation and the greater risk among younger patients indicate the need for life-long surveillance.
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              Impact of chronic GVHD therapy on the development of squamous-cell cancers after hematopoietic stem-cell transplantation: an international case-control study.

              Catherine Metayer, Mary Flowers, M Horowitz … (2005)
              Previous studies of recipients of hematopoietic stem-cell transplants suggest that graft-versus-host disease (GVHD) and its therapy may increase the risk for solid cancers, particularly squamous-cell carcinomas (SCCs) of the buccal cavity and skin. However, the importance and magnitude of these associations are not well characterized. We conducted a case-control study of 183 patients with posttransplantation solid cancers (58 SCCs, 125 non-SCCs) and 501 matched control patients within a cohort of 24,011 patients who underwent hematopoietic stem-cell transplantation (HSCT) at 215 centers worldwide. Our results showed that chronic GVHD and its therapy were strongly related to the risk for SCC, whereas no increase in risk was found for non-SCCs. Major risk factors for the development of SCC were long duration of chronic GVHD therapy (P < .001); use of azathioprine, particularly when combined with cyclosporine and steroids (P < .001); and severe chronic GVHD (P = .004). Given that most patients who received prolonged immunosuppressive therapy and those with severe chronic GVHD were also treated with azathioprine, the independent effects of these factors could not be evaluated. Additional analyses determined that prolonged immunosuppressive therapy and azathioprine use were also significant risk factors for SCC of the skin and of the oral mucosa. These data provide further encouragement for strategies to prevent chronic GVHD and for the development of more effective and less carcinogenic treatment regimens for patients with moderate or severe chronic GVHD. Our results also suggest that clinical screening for SCC is appropriate among patients exposed to persistent chronic GVHD, prolonged immunosuppressive therapy, or both.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Case Rep Oncol
                Journal ID (iso-abbrev): Case Rep Oncol
                Journal ID (publisher-id): CRO
                Title: Case Reports in Oncology
                Publisher: S. Karger AG (Allschwilerstrasse 10, P.O. Box · Postfach · Case postale, CH–4009, Basel, Switzerland · Schweiz · Suisse, Phone: +41 61 306 11 11, Fax: +41 61 306 12 34, karger@karger.com )
                ISSN (Electronic): 1662-6575
                Publication date Collection: Sep-Dec 2022
                Publication date (Electronic): 27 September 2022
                Publication date PMC-release: 27 September 2022
                Volume: 15
                Issue: 3
                Pages: 848-853
                Affiliations
                [1] aOral and Maxillofacial Surgery Unit, School of Dental Sciences, Health Campus, Universiti Sains Malaysia, Kota Bharu, Malaysia
                [2] bOral and Maxillofacial Surgery Unit, Hospital Universiti Sains Malaysia, Kota Bharu, Malaysia
                Author notes
                *Mohd Faizal Abdullah, mohdfaizalkk@ 123456usm.my
                Article
                Publisher ID: cro-0015-0848
                DOI: 10.1159/000526698
                PMC ID: 9941789
                PubMed ID: 36825104
                SO-VID: 46310ac8-0a00-49de-ba8a-37db63dd718c
                Copyright © Copyright © 2022 by The Author(s). Published by S. Karger AG, Basel
                License:

                This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission.

                History
                Date received : 11 July 2022
                Date accepted : 5 August 2022
                Date: 2022
                Page count
                Figures: 3, References: 15, Pages: 6
                Funding
                No funding was secured for the publication of this manuscript.
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Oncology & Radiotherapy
                Keywords: fanconi anaemia,head and neck cancer,oral squamous cell carcinoma,covid-19
                Data availability:
                ScienceOpen disciplines: Oncology & Radiotherapy
                Keywords: fanconi anaemia, head and neck cancer, oral squamous cell carcinoma, covid-19

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