Infantile (Desmoid Type) Anterior Mediastinal Fibromatosis-A Case Report
S. K. Andola, Vatsala Kishore, S. J. Rajshekhar, Shivanand Melkundi and S. R. Harwal
World Journal of Pathology 2014, 3:12
Desmoid-type fibromatosis is fibroblastic proliferation occurring in the deep soft tissues and is characterized by infiltrative growth and tendency towards local recurrence but does not metastasize. It represents 3.5% of fibrous tumors and 0.03% of all neoplasms. Desmoid fibromatosis are rare in children, may occur in head and neck region but are extremely rare in the mediastinum with only five cases being reported in the literature till date. This case is being presented for its rarity
Three year old male child presented with a gradually progressive, firm 17x10cm swelling, fixed to the chest wall with dilated veins. Radiological imaging revealed possibility of soft tissue sarcoma/Ewing’s sarcoma. Cytological diagnosis of spindle cell tumor of intermediate grade, possibly of neuronal origin was made. Thoracotomy and excision of tumor was performed. Grossly there was a single, partially skin covered, grey white mass measuring 17x10x5cm, C/s showing grey white areas of whorling and cartilage projections. Microscopically interlacing fascicles of bland spindle cells in a collagenous background were seen with fusiform nuclei, uniform chromatin and inconspicuous nucleoli. No pleomorphism, mitoses were seen. Surrounding adipose tissue and bone was infiltrated by tumor. On Immunohistochemistry the tumor showed diffuse positivity for vimentin, focal positivity for S-100 and was negative for desmin. A diagnosis of mediastinal fibromatosis was made. However, the Patient succumbed to intraoperative cardiac arrest.
Fibromatosis should be considered in differential diagnosis of any spindle cell tumor of anterior mediastinum. It is impossible to give a preoperative definite diagnosis of fibromatosis on FNAC but with a core needle biopsy and IHC, the differential diagnoses can be narrowed.
Fibromatosis, Infantile, Mediastinum, Desmoid Tumor