Ezgi DOĞAN, Hüseyin Gencay KEÇELİ, Feriha ÇAĞLAYAN

Fanconi Anemili Hastada Gingival Skuamo z Hu creli Karsinom: Olgu Sunumu ve Literatu r Derlemesi

Fanconi anemisi; kromozomal instabilite, aplastik anemi ve kansere yatkınlık ile karakterize nadir görülen, otozomal resesif geçişli genetik bir bozukluktur. Bu hastalarda skuamöz hücreli karsinom yaygın olarak baş boyun bölgesinde görülmüştür. Kliniğimize sağ alt birinci büyükazı dişindeki sallanma şikayeti ile başvuran 29 yaşındaki kadın hastada Fanconi anemisi hikayesine rastlandı. Klinik ve radyografik muayene sonucunda generalize periodontitis teşhisi konulduktan sonra dişin çekimine karar verildi. Hematoloji bölümüne yapılan konsültasyon doğrultusunda diş çekimi yapıldı ve aynı seansta lingualdeki dişetinden alınan örnek histopatolojik incelemeye gönderildi. İnceleme sonucunda iyi diferansiye skuamöz hücreli karsinom teşhisi konuldu. Tümör rezeksiyonu amacıyla yapılan commando cerrahisi ve sonrasında uygulanan kemoterapi, radyoterapi sonucunda dört sene boyunca rekürrens görülmedi. Bu vaka raporu; Fanconi anemisi gibi kansere yatkınlığı olan hastalarda periodontal hastalık gibi eş zamanlı malignensi riski yaratabileceği düşünülen faktörlerin farkında olmanın, Fanconi anemisi teşhisi alan hastaların düzenli diş hekimi kontrolünün ve dolayısıyla erken teşhisin önemini vurgulamaktadır.

Gingival Squamous Cell Carcinoma in a Fanconi Anemia Patient: Case Report and Review of Literature

Fanconi anemia (FA) is a rare autosomal recessive genetic disorder characterized by chromosomal instability, aplastic anemia, and predisposition for malignancies. Squamous cell carcinomas(SCC) are commonly seen in head and neck region. A 29- year-old female, diagnosed with FA, was referred to our clinic with chief complaint of loose tooth in right mandibula. Clinical and radiological examination revealed diagnosis of generalized periodontitis and extraction of tooth was decided. An incisional biopsy was performed from lingual gingiva at time of extraction. After histopathological examination, lesion was diagnosed as welldifferentiated SCC. The patient underwent commando surgery and postoperative concurrent chemoradiotherapy was performed. No evidence of recurrence was found after four-year follow-up. This case highlights malignancy possibility of patients, exhibiting periodontal disease, who also have concurrent risk factors for SCC development such as FA and emphasizes importance of regular dental review of all patients receiving a diagnosis of FA.

PDF

___

1. Giampietro PF, Davis JG, Adler-Brecher B, et al. The need for more accurate and timely diagnosis in Fanconi anemia: a report from the International Fanconi Anemia Registry. Pediatrics. 1993;91:1116-20.
2. Velleuer E, Dietrich R. Fanconi anemia: young patients at high risk for squamous cell carcinoma. Mol Cell Pediatr. 2014;1:1-6.
3. Balta G. (13.03.2005) Fankoni Anemisinin Moleküler Biyolojisi ve Genetiği. Erişim tarihi: 11.01.2020, http://www.turkhematolojidernegi.org.tr/thdData/u serfiles/file/gunaybalta.pdf.
4. Bagby GC, Alter BP. Fanconi anemia. Semin Hematol. 2006;43:147-156.
5. Kutler DI, Singh B, Satagopan J, et al. A 20-year perspective on the International Fanconi Anemia Registry (IFAR). Blood. 2003;101:1249-56.
6. Rosenberg PS, Greene MH, Alter BP. Cancer incidence in persons with Fanconi anemia. Blood. 2003;101:822-6.
7. Kutler DI, Patel KR, Auerbach AD, et al. Natural history and management of Fanconi anemia patients with head and neck cancer: A 10‐year follow‐up. Laryngoscope. 2016;126:870-9.
8. Furquim CP, Pivovar A, Amenábar JM, et al. Oral cancer in Fanconi anemia: review of 121 cases. Crit Rev Oncol Hematol. 2018;125:35-40.
9. Alter BP. Fanconi's anemia and malignancies. Am J Hematol. 1996;53:99-110.
10. Sarna G, Tomasulo P, Lotz MJ, et al. Multiple neoplasms in two siblings with a variant form of Fanconi's anemia. Cancer. 1975;36:1029-33.
11. Ward G, Edgerton M, Chambers R, et al. Cancer of the oral cavity and pharynx and results of treatment by means of the composite operation (in continuity with radical neck dissection). Ann Surg. 1959;150:202-220.
12. Snow D, Campbell J, Smallman L. Fanconi's anaemia and post-cricoid carcinoma. J Laryngol Otol. 1991;105:125-7.
13. Lustig J, Lugassy G, Neder A, et al. Head and neck carcinoma in Fanconi's anaemia—report of a case and review of the literature. Eur J Cancer B Oral Oncol. 1995;31:68-72.
14. Kennedy AW, Hart WR. Multiple squamous‐cell carcinomas in fanconi's anemia. Cancer. 1982;50:811-4.
15. Mayne ST, Cartmel B, Kirsh V, et al. Alcohol and tobacco use prediagnosis and postdiagnosis, and survival in a cohort of patients with early stage cancers of the oral cavity, pharynx, and larynx. Cancer Epidemiol Biomarkers Prev. 2009;18:3368-74.
16. Rodriguez T, Altieri A, Chatenoud L, et al. Risk factors for oral and pharyngeal cancer in young adults. Oral Oncol. 2004;40:207-13.
17. Scheckenbach K, Wagenmann M, Freund M, et al. Squamous cell carcinomas of the head and neck in Fanconi anemia: risk, prevention, therapy, and the need for guidelines. Klin Padiatr. 2012;224:132-8.
18. Kaplan MJ, Cantrell RW, Sabio H, et al. Squamous cell carcinoma in the immunosuppressed patient: Fanconi's anemia. Laryngoscope. 1985;95:771-5.
19. Millen F, Rainey M, Hows J, et al. Oral squamous cell carcinoma after allogeneic bone marrow transplantation for Fanconi anaemia. Br J Haematol. 1997;99:410-4.
20. Maier H, Zöller J, Herrmann A, et al. Dental status and oral hygiene in patients with head and neck cancer. Otolaryngol Head Neck Surg. 1993;108:655-61.
21. Fitzpatrick SG, Katz J. The association between periodontal disease and cancer: a review of the literature. J Dent. 2010;38:83-95.
22. Opinya GN, Kaimenyi JT, Meme JS. Oral findings in Fanconi's anemia: a case report. J Periodontol. 1988;59:461-3.
23. Nowzari H, Jorgensen MG, Ta TT, et al. Aggressive periodontitis associated with Fanconi's anemia. A case report. J Periodontol. 2001;72:1601-6.