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Fanconi Anemisi ile Birliktelik Gösteren Adolesan Dönemde Vulvar Kanser: Olgu Sunumu

Vulvar Cancer Associated with Fanconi’s Anemia in Adolescent Patient: A Case Report

Journal Name:

  • İnönü Üniversitesi Tıp Fakültesi Dergisi

Publication Year:

  • 2012

Key Words:

Keywords (Original Language):

Author NameUniversity of AuthorFaculty of Author
Abstract (2. Language): 
Introduction: Fanconi’s anemia (FA) is a rare autosomal recessive disorder characterized by aplastic anemia, congenital anomalies, and cancer susceptibility. Vulvar cancer, mostly seen in postmenopausal, is fourth common gynecologic cancer. In this report, we present a 17- years- old virgin patient, who had diagnosis of Fanconi’s anemia 9 years before, with vulvar squamous cell carcinoma. Case Report: A 17- years-old female presented to our department with complait of growing fragile mass of her vulva which have appeared 2-3 months ago. The vulvar biopsy showed a vulvar squamous cell carcinoma. Despite prompt modified radical vulvectomy-gluteal fold flep closure and radiotherapy lung methastasis was observed two months after the surgery. The prognosis is poor. Conclusion: Vulvar cancer can be seen with FA in adolescent. These patiens could have poor prognosis despite prompt and agressive treatment. Further studies to evaluate the importance of HPV vaccia ne in patients with FA are needed.
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Abstract (Original Language): 
Giriş: Fanconi anemisi; aplastik anemi, konjenital anomali ve maligniteye yatkınlık ile karakterize otozomal resesif geçiş gösteren nadir bir hematolojik hastalıktır. Sıklıkla postmenapozal dönemde, ortalama 65 yaşta, tanı konulan vulva kanserleri 4. sıklıkla görülen jinekolojik kanserlerdir. Bu olguda 17 yaşında, virjin, dokuz yıl önce Fanconi anemisi tanısı almış adolesanda vulvar squamoz hücreli carsinom (SCC) sunulmuştur. Olgu Sunumu: Son 2-3 aydır vulvar bölgede büyüyen kitle nedeniyle kliniğimize başvuran hastanın yapılan vulvar biopsi sonucu differansiye SCC olarak gelmiştir. Geniş tabanlı vulvektomi ve gluteal fold fleb uygulanan hastaya postoperatif radyoterapi verilmiştir. Hastada operasyondan 2 ay sonra akciğer metastazları izlenmiştir. Prognoz kötüdür. Sonuç: Fanconi anemili hastalarda, adolesan dönemde vulva kanseri görülebileceği, hızlı ve agresif bir tedaviye rağmen prognozun kötü olabileceği göz önünde bulundurulmalıdır. Fanconi anemisi tanısı konulan hastalarda HPV aşısının önemini ortaya koyan yeni çalışmalara ihtiyaç vardır
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  • 1
46-49
  • Turkish

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