A rare chest wall localized soft tissue sarcoma: Clear cell sarcoma

Öz

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.

Anahtar Kelimeler

• Clear cell sarcoma, chest wall, metastasis, vertebral, HMB-45, S-100

Göğüs duvarı yerleşimli, nadir bir yumuşak doku sarkomu: Berrak hücreli sarkom

Öz

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 adlandırılmıştır. 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.

Anahtar Kelimeler

• Berrak hücreli sarkom, göğüs duvarı, metastaz, vertebra, HMB-45, S-100

Kaynakça

1. Sara AS, Evans HL, Benjamin RS. Malignant melanoma of soft parts (clear cell sarcoma); a study of 17 cases, with emphasis on prognos- tic factors. Cancer 1990;65:367-74.
2. Weiss SW, Goldblum JR. Enzinger and Weiss’s soft tissue tumors; ma- lignant tumors of the peripheral nerves, 4th edition USA, Mosby, 2001:1241-50.
3. Ferrari A, Casanova M, Bisogno, et al. Clear cell sarcoma of tendons and aponeuroses in pediatric patients; a report from the Italian and German Soft Tissue Sarcoma Coop Group. Cancer 2002;94:3269- 76.
4. Chung EB and Enzinger FM. Malignant melanoma of soft parts. A reassessment of clear cell sarcoma. Am J Surg Pathol 1983;7:405-13.
5. Bridge JA, Borek DA, Neff JR, Huntrakoon M. Chromossomal abnor- malities in clear cell sarcoma. Implications for histogenesis. Am J Clin Pathol 1990;93: 26-31.
6. Yalnız E, Alıcıoğlu B, Yılmaz B, Özyılmaz F, Yalçın Ö. Nadir görülen bir yumuşak doku sarkomu: berrak hücreli sarkom. Pamukkale Tıp Dergisi 2009;2:41-4.
7. Bonetti F, Pea M, Martignoni G, et al. Clear cell (‘sugar’) tumor of the lung is striktly related to angiomyolipoma the concept of a family of lesions characterized by the presence of the perivascular epithelioid cell (PEC). Pathology 1994;26:230-6.