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

Öz

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 well-differentiated 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.

Anahtar Kelimeler

• Fanconi anemia; squamous cell carcinoma; periodontal disease

Fanconi Anemili Hastada Gingival Skuamöz Hücreli Karsinom: Olgu Sunumu ve Literatür Derlemesi

Öz

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.

Anahtar Kelimeler

• Fanconi anemisi
• Skuamöz hücreli karsinom
• Periodontal hastalık

Kaynakça

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