Afroz Nishat, Shamim Nida, Jain Anshu, Soni Mayank
Department of Pathology, Jawaharlal Nehru Medical College, Aligarh, Uttar Pradesh, India.
BMJ Case Rep. 2014 Apr 11;2014:bcr2013203420. doi: 10.1136/bcr-2013-203420.
Giant cell fibroblastoma (GCF) is a rare soft tissue tumour that occurs almost exclusively in children younger than 10 years of age and is mostly located in the superficial soft tissues of the back and thighs. We present a rare case of GCF with encephalocele in a 1.5-year-old boy who presented with a swelling in the occipital area of the scalp since birth. CT scan suggested encephalocele without any suspicion of a mass lesion. On histopathology, an ill-defined proliferation of fibroblasts in a heavily collagenised and focally myxoid stroma was seen containing numerous multinucleated cells having a floret-like appearance along with mature glial tissue bordering a cystic space. Immunohistochemically, the stromal cells were positive for both, vimentin (diffuse) and CD34 (focal) thereby confirming the histological diagnosis of GCF. This case highlights the unusual coexistence of GCF with congenital defects and its histogenetic resemblance to dermatofibrosarcoma protuberans.
巨细胞成纤维细胞瘤（GCF）是一种罕见的软组织肿瘤，几乎仅发生于10岁以下儿童，且大多位于背部和大腿的浅表软组织。我们报告1例1.5岁男孩罕见的伴有脑膨出的GCF病例，该患儿自出生以来枕部头皮出现肿胀。CT扫描提示脑膨出，未怀疑有任何肿块病变。组织病理学检查显示，在高度胶原化且局部呈黏液样的基质中，成纤维细胞呈边界不清的增生，可见大量具有小花样外观的多核细胞，以及与囊性间隙相邻的成熟神经胶质组织。免疫组织化学检查显示，基质细胞波形蛋白（弥漫性）和CD34（局灶性）均呈阳性，从而证实了GCF的组织学诊断。该病例突出了GCF与先天性缺陷的异常共存及其与隆突性皮肤纤维肉瘤的组织发生学相似性。
