Related Experiment Video
Updated: May 10, 2026

Tumorsphere Derivation and Treatment from Primary Tumor Cells Isolated from Mouse Rhabdomyosarcomas
Published on: September 13, 2019
Infantile rhabdomyofibrosarcoma: A potentially underdiagnosed aggressive tumor
Narendra Chaudhary1, Tanuja Shet, Anupama Borker
1Department of Child Health, Christian Medical College, Vellore, Tamil Nadu, India.
Insights
Infantile Rhabdomyofibrosarcoma (IRMFS) is a rare soft-tissue tumor. Differentiating IRMFS from infantile fibrosarcoma (IFS) is crucial for appropriate treatment and prognosis.
Area of Science:
- Pediatric Oncology
- Surgical Pathology
- Soft Tissue Tumors
Background:
- Infantile Rhabdomyofibrosarcoma (IRMFS) is a rare tumor mimicking infantile fibrosarcoma (IFS).
- Distinguishing IRMFS from IFS is critical due to differing prognoses and treatment strategies.
Observation:
- A case report of a 2-year, 6-month-old boy with a left axillary soft-tissue mass.
- Initial histopathology suggested IFS, but subsequent immunohistochemistry confirmed IRMFS (desmin-positive).
Findings:
- The patient received aggressive adjuvant chemotherapy following diagnosis.
- Complete remission was achieved 6 months post-chemotherapy.
Implications:
- Accurate differentiation of IRMFS from IFS is essential for effective treatment planning.
- Early and correct diagnosis can prevent under-treatment of this aggressive tumor.
Abstract:
Infantile Rhabdomyofibrosarcoma (IRMFS) is a rare clinicopathological entity that resembles infantile fibrosarcoma (IFS) but has ultrastructural and immunohistochemical evidence of rhabdomyoblastic differentiation. We report a 2 years and 6 months old boy who presented with a slowly progressive large soft-tissue mass in left axillary region. After complete excision, histopathology report revealed diagnosis of IFS. Review of the histopathology with immunohistochemistry (positive for desmin) revealed diagnosis of IRMFS. He was treated with aggressive adjuvant chemotherapy. He was in complete remission 6 months after completion of chemotherapy. In view of poor prognosis and aggressive treatment approaches for IRMFS, it must be differentiated from IFS to avoid under treatment.
Related Concept Videos
Rous Sarcoma Virus (RSV) and Cancer
RSV is a retrovirus that contains two copies of a plus-strand RNA genome. Its genome consists of four main open...
Rous Sarcoma Virus (RSV) and Cancer
RSV is a retrovirus that contains two copies of a plus-strand RNA genome. Its genome consists of four main open...
The Retinoblastoma Gene
The first-ever tumor suppressor gene called Rb was identified in retinoblastoma - a rare eye tumor in children. In inherited forms of the disease, a child inherits one defective copy of the Rb gene, which predisposes them to retinoblastoma. However,...
The Retinoblastoma Gene
The first-ever tumor suppressor gene called Rb was identified in retinoblastoma - a rare eye tumor in children. In inherited forms of the disease, a child inherits one defective copy of the Rb gene, which predisposes them to retinoblastoma. However,...
Abnormal Proliferation
Abnormal Proliferation