Çocukluk çağı medulloblastom olgularında P53, ERBB2, c-Kit ve BCL2 Ekspresyonunun prognostik ve klinik önemi

Amaç: Bu çalışmanın amacı pediatrik medullbolastom olgularında p53, ERBB2, c-Kit ve Bcl-2 ekspresyonunun klinik ve prognostik öneminin araştırılmasıdır.Gereç ve Yöntem: Toplam 29 medulloblastom olgusu demografik, klinik, histopatolojik özellikler ve immünhistokimyasal olarak p53, ERBB2, c-Kit ve Bcl-2 ekspresyonlarının prognostik anlamı açısından değerlendirmeye alındı. Bulgular: Ortanca tanı yaşı 69 aydı (21-122 ay). Ortalama takip süresi 54 ay (2-209 ay) olarak belirlendi. Ondört olgu klasik (%48), 13 olgu nodüler/dezmoplastik (%45) ve 2 olgu anaplastik (%7) olarak değerlendirildi. İmmünhistokimyasal olarak 28 olguda c-Kit, 10 olguda Bcl-2, 9 olguda p53 ve 2 olguda ERBB2 pozitifliği gösterildi. Genel sağkalım oranı %62 ve hastalıksız sağkalım oranı %52 olarak bulundu. Bcl-2 ekspresyonunun nodüler/dezmoplastik alt tipte arttığı gösterildi. Klinik, histopatolojik ve immünhistokimyasal özelliklerin herhangi birisinin prognoz üzerinde etkisi gösterilemedi.Sonuç:  Bu çalışma medulloblastom tedavisinde kombine tedavi protokollerinin kullanılmaya başladığı ilk yılları yansıtmaktadır. Sağkalım oranları tanı yıllarına göre literatür ile benzer saptanmıştır. Değerlendirilen klinik, histopatoloijk ve immünhistokimyasal özelliklerin prognozla ilişkisi gösterilemese de, gelecekte moleküler ve immünhistokimyasal yöntemlerin birlikte kullanıldığı çalışmalarla medulloblastom olgularında prognostik faktörlerin saptanmasının mümkün olacağı düşüncesindeyiz.

Prognostic and clinical significance of expressions of P53, Erbb2, c-Kit and Bcl2 in childhood medulloblastoma

Purpose: The objective of this study is to evaluate prognostic implications of clinical, histopathological features and immuhistochemical expressions of p53, ERBB2, c-Kit and Bcl-2 in pediatric medulloblastoma.Materials and Methods: A total of 29 pediatric medulloblastoma cases were evaluated for prognostic association of demographic, clinical, histopathological features and immunohistochemical expressions of p53, ERBB2, c-Kit and Bcl-2. Results: Median age at diagnosis was 69 months (21-122 months). Median duration of follow-up was 54 months (2-209 months). Fourteen of samples were diagnosed as classical (48%), thirteen cases as nodular/desmoplastic (45%) and two cases as anaplastic (7%) subtype. Staining for c-Kit, Bcl-2, p53 and ERBB2 was positive in 28, 10, 9 and 2 samples, respectively.  Overall  (OS) and event-free survival (EFS) were 62 % and 52%, respectively. Bcl-2 expression was found to be significantly increased in nodular/desmoplastic subtype. None of the clinical, histopathological and immunohistochemical features were related to survival. Conclusion: This study reflects the earliest periods of current multimodal treatment protocols of medulloblastoma with similar survival rates in literature. Although none of the proposed factors have been associated with survival, future studies combining molecular and immunohistochemical methods would be more convenient for detecting new prognostic criteria in pediatric medulloblastoma.

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