Year: 2017 | Month: Aug | Volume: 7 | Issue: 8 | Pages: 499-502
Aggressive Fibromatosis of the Chest Wall Mimicking Low Grade Fibrosarcoma – An Unusual Clinical Presentation
Dr. Nimisha Sharma1*, Dr. Sumanashree Mallappa2**, Dr. Sujata Raychaudhuri3*, Dr Amit Yadav3**, Prof. Dr. A. K. Mandal4**
1Assistant Professor, 2Senior Resident, 3Associate Professor, 4Director Professor,
*Dept. of Pathology, ESIC Medical College and Hospital, Faridabad
**Dept. of Pathology, VMMC and Safdarjung Hospital, New Delhi
Corresponding Author: Dr. Nimisha Sharma
Background: Aggressive fibromatosis (AF), also known as desmoid tumour or musculoaponeurotic fibromatosis, is a monoclonal fibroblastic proliferative disease. It can present as abdominal and extra-abdominal fibromatosis. Extra abdominal deep fibromatosis represent 3.5% of fibrous tissue tumor and 0.03% of all neoplasms. Anterior chest wall forms 10% of Extra abdominal deep fibromatosis cases.
Case history: Patient presented with a progressively increasing large swelling on the chest wall since 2 years. On examination, firm to hard ill defined growth was found. Microscopic examination showed a cellular spindle cell tumour. Strong positivity for VIMENTIN and focal positivity for SMA and DESMIN was seen on Immunohistochemistry. Final Diagnosis of aggressive fibromatosis was made.
Conclusion: This case is being presented for its rarity, unusual clinical presentation and overlapping clinical and histopathological features with fibrosarcoma, which is a malignant entity.
Key words: Aggressive fibromatosis (AF), desmoid tumour, fibrosarcoma.