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Coexistence of giant cell fibroblastoma and encephalocele
Nishat Afroz1, Nida Shamim, Anshu Jain
1Department of Pathology, Jawaharlal Nehru Medical College, Aligarh, Uttar Pradesh, India.
Insights
Giant cell fibroblastoma (GCF), a rare pediatric soft tissue tumor, was found coexisting with an encephalocele in a young boy. This unusual case highlights GCF
Area of Science:
- Pediatric Oncology
- Dermatopathology
- Soft Tissue Tumors
Background:
- Giant cell fibroblastoma (GCF) is a rare soft tissue neoplasm typically affecting children under 10.
- GCF commonly presents in the superficial soft tissues of the back and thighs.
Observation:
- A rare case of GCF coexisting with an occipital encephalocele in a 1.5-year-old boy is presented.
- Initial CT scans suggested encephalocele, with no initial suspicion of a mass lesion.
- Histopathology revealed a fibroblast proliferation with multinucleated giant cells and mature glial tissue.
Findings:
- Immunohistochemistry confirmed GCF diagnosis, with stromal cells positive for vimentin and CD34.
- The presence of mature glial tissue within the tumor was a notable finding.
- The tumor exhibited an ill-defined proliferation in a collagenized and myxoid stroma.
Implications:
- This case underscores the unusual association of GCF with congenital defects.
- The findings suggest a potential histogenetic link between GCF and dermatofibrosarcoma protuberans.
- Highlights the importance of thorough histopathological examination in diagnosing rare pediatric soft tissue tumors.
Abstract:
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.
