Çocukluk Çağında İnmemiş Testis Dokusundan Gelişen Primer Histiyositik Sarkom

Abstract

Histiyositik sarkom morfolojik ve immünhistokimyasal olarak histiyositik diferansiasyon gösteren, genellikle ekstranodal tutulum yapan, oldukça nadir görülen, agresif seyirli bir malignitedir. Burada 12 yaşında adrenolökodistrofi tanılı takipte olan erkek hastada, inmemiş testis dokusundan gelişen primer histiyositik sarkomu ayırıcı tanıları eşliğinde tartıştık. Genellikle erişkinlerde 45-55 yaş arasında görülen bu malignitenin bizim olgumuzda çocukluk çağında izlenmesi ve inmemiş testis dokusundan kaynaklanması olguya ait dikkat çekici özelliklerdir. Günümüze kadar İngilizce literatürde böyle bir birliktelik bildirilmemiştir

Keywords

• Histiyositik sarkom, Çocukluk çağı, İnmemiş testis, Adrenolökodistrofi , CD68, Extranodal tutulum

Primary Histiocytic Sarcoma Arising from Undescended Testis in Childhood

Abstract

Histiocytic sarcoma is a rare, aggressive malignancy that shows histiocytic differentiation morphologically and immunohistochemically, often with extranodal involvement. Here we discuss the differential diagnosis of primary histiocytic sarcoma of undescended testicular tissue in a 12-yearold male patient with adrenoleukodystrophy. The presence of malignancy in childhood arising from undescended testicular tissue was noteworthy in our case. This concurrence has not previously been reported in the English literature

Keywords

• Histiocytic sarcoma, Childhood, Undescended testis, Adrenoleukodystrophy, CD68, Extranodal involvement

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