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Congenital infantile fibrosarcoma: a clinical mimicker of hemangioma
1Department of Pathology, Maulana Azad Medical College, New Delhi, India. deepalijain76@gmail.com
Insights
Congenital infantile fibrosarcoma (CIFS) is a rare pediatric soft tissue tumor. This case highlights CIFS presenting as an ulcerated mass, initially misdiagnosed as a vascular lesion.
Area of Science:
- Pediatric Oncology
- Surgical Pathology
- Soft Tissue Tumors
Background:
- Congenital infantile fibrosarcoma (CIFS) is a rare, often congenital, soft tissue neoplasm in infants.
- CIFS typically presents as a rapidly growing mass, frequently in the extremities.
- While generally having a better prognosis than other pediatric sarcomas, early and accurate diagnosis is crucial.
Observation:
- A case of CIFS in an infant presenting as a nonspecific, ulcerated mass over the right shoulder.
- The lesion was initially suspected to be a benign vascular tumor, such as a hemangioma, due to its highly vascular appearance.
- The clinical presentation mimicked other common pediatric skin lesions, delaying definitive diagnosis.
Findings:
- Histopathological examination revealed a highly cellular tumor composed of densely packed spindle cells arranged in fascicles.
- Immunohistochemical analysis demonstrated that the tumor cells were positive for vimentin, a common marker for mesenchymal tumors.
- These findings confirmed the diagnosis of congenital infantile fibrosarcoma.
Implications:
- This case underscores the importance of considering CIFS in the differential diagnosis of pediatric soft tissue masses, even with atypical presentations.
- Accurate histopathological and immunohistochemical evaluation is essential for differentiating CIFS from benign vascular lesions and other sarcomas.
- Timely diagnosis and appropriate management can lead to favorable outcomes for patients with CIFS.
Abstract:
Congenital infantile fibrosarcoma (CIFS) is a rare pediatric soft tissue tumor; clinically, it presents as a highly vascular mass and may simulate a hemangioma. It usually occurs in the first year of life and has a relatively better prognosis compared to other aggressive spindle cell sarcomas of childhood. We report a patient with CIFS who presented with a nonspecific ulcerated mass lesion over the right shoulder region that was clinically diagnosed as a benign vascular lesion. Histology revealed a highly cellular tumor with closely packed fascicles of spindle cells. Immunohistochemically, the tumor cells expressed vimentin.
