Göğüs duvarı yerleşimli, nadir bir yumuşak doku sarkomu: Berrak hücreli sarkom
Yükleniyor...
Tarih
2013
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Dicle Üniversitesi Tıp Fakültesi
Erişim Hakkı
Attribution-NonCommercial 3.0 United States
info:eu-repo/semantics/openAccess
info:eu-repo/semantics/openAccess
Özet
Yumuşak dokunun berrak hücreli sarkomları, nöral krest hücrelerinden köken alan, kötü prognozlu ender tümörlerdir. Histolojik özelliklerinin malign melanoma benzemesi sebebiyle (S-100 ve HMB- 45 pozitifliği, ultrastrüktürel melanozomların varlığı gibi) yumuşak dokuların malign melanomu olarak da dlandırılmıştır. Ancak berrak hücreli sarkom kutanöz melanomdan farklı olarak hemen her zaman derin yerleşimlidir ve davranışı farklıdır. Desmoplastik veya iğsi hücreli malign melanomun, dermis yerleşimli oluşu nedeniyle ayırıcı tanıları daha zor olabilmektedir. Vakamızda, geniş nekroz alanları yanısıra, gevşek stroma içinde, uniform görünümlü, veziküler nükleuslu, belirgin nükleollü, dar ve soluk-eozinofilik, kısmen şeffaf
sitoplazmalı hücrelerden oluşan infiltratif tümör izlendi. İmmünohistokimyasal çalışmada tümörün vimentin, S-100, HMB45 ve SMA eksprese ettiği, CD34, PanCK, EMA, LCA, CD99 ve desmin ile immünreaksiyon göstermediği Ki-67 proliferasyon indeksinin ise %50 civarında olduğu tespit edildi. Derin yerleşim özelliği ve morfolojik-immünohistokimyasal bulguları bir araya getirildiğinde vaka ‘’berrak hücreli sarkom’’ olarak değerlendirildi. Hastanın 1 ay sonra gönderilen vertebra biyopsisinde benzer morfolojide tümör izlendi ve tümörün vertebraya metastazı şeklinde yorumlandı.
The clear cell sarcomas of soft tissue are rare tumors originating from neural crest cells and presenting with poor prognosis. By the reason of the resemblance of histological properties to malign melanoma (eg. the immunoreactivity to S100 and HMB45, the presence of melanosomes ultrastructurally), these tumors are also defined as malign melanomas of soft tissue. But distinctively from cutaneous melanoma, clear cell sarcoma is almost always deeply localized and the biological behaviour of the last one is also different. The differential diagnosis between clear cell sarcoma and desmoplastic or spindle cell malign melanoma may be more difficult because of the dermal localization of the last ones. In our case, it was observed an infiltrative tumor composed of uniform seeming cells with vesicular nuclei, distinct nucleoli, pale eosinophilic and sometimes clear, scant cytoplasms, in addition to necrotic areas. On immunohistochemical examination, the tumoral cells showed a positive immunoreactivity to vimentin, S100, HMB45, and SMA, while showing negative immunoreactivity with CD34, PanCK, EMA, LCA, CD99 and desmin. Ki-67 proliferation index was determined as approximately 50%. Because of deep localization and different morphological-immunohistochemical findings of the tumor, the case was diagnosed as “clear cell sarcoma”. It was observed a tumor with similar morphology in the biopsy sample taken from vertebra of the patient one month later than the first material and this was commented as the metastasis of the tumor to vertebra.
The clear cell sarcomas of soft tissue are rare tumors originating from neural crest cells and presenting with poor prognosis. By the reason of the resemblance of histological properties to malign melanoma (eg. the immunoreactivity to S100 and HMB45, the presence of melanosomes ultrastructurally), these tumors are also defined as malign melanomas of soft tissue. But distinctively from cutaneous melanoma, clear cell sarcoma is almost always deeply localized and the biological behaviour of the last one is also different. The differential diagnosis between clear cell sarcoma and desmoplastic or spindle cell malign melanoma may be more difficult because of the dermal localization of the last ones. In our case, it was observed an infiltrative tumor composed of uniform seeming cells with vesicular nuclei, distinct nucleoli, pale eosinophilic and sometimes clear, scant cytoplasms, in addition to necrotic areas. On immunohistochemical examination, the tumoral cells showed a positive immunoreactivity to vimentin, S100, HMB45, and SMA, while showing negative immunoreactivity with CD34, PanCK, EMA, LCA, CD99 and desmin. Ki-67 proliferation index was determined as approximately 50%. Because of deep localization and different morphological-immunohistochemical findings of the tumor, the case was diagnosed as “clear cell sarcoma”. It was observed a tumor with similar morphology in the biopsy sample taken from vertebra of the patient one month later than the first material and this was commented as the metastasis of the tumor to vertebra.
Açıklama
Anahtar Kelimeler
Berrak hücreli sarkom, Göğüs duvarı, Metastaz, Vertebra, Clear cell sarcoma, Chest wall, Metastasis, Vertebral, HMB-45, S-100
Kaynak
Dicle Tıp Dergisi
WoS Q Değeri
Scopus Q Değeri
Cilt
40
Sayı
1
Künye
Alabalık, U., Keleş, A.N., Büyükbayram, H., Fırat, U., Küçüköner, M. ve Hamidi, A. (2013). Göğüs duvarı yerleşimli, nadir bir yumuşak doku sarkomu: Berrak hücreli sarkom. Dicle Tıp Dergisi, 40(1), 124-127.
