Acknowledgements:
I would like to express my very great appreciation to Dr Monique Kafle for his valuable and constructive suggestions during the planning and development of this research work. His willingness to give time so generously has been very much appreciated.
I would also like to thank the Department of Pathology of the Civil Service Hospital for their constant support.
Case Report:
BACKGROUND
DermatoFibroSarcomaProtuberans (DFSP) is a rare, slow growing, recurrent tumor arising mostly from the dermis and subcutaneous fat. Patients present with slow growing multinodular solitary growth in trunk with recurrence post treatment.1 DFSP is a CD34 positive tumor with predominance of spindle cells in the histopathological sections. The etiological association with the COL1A1-PDGFB has led to the dramatic response to the new therapeutic lineage of tyrosine kinase inhibitor- imatinib mesylate.2 Given the lack of availability of the FDA approved targeted therapy, imatinib mesylate for recurrent DFSP in resource poor setting like ours, priority should be given towards wide local excision with 2-4cm margin in its treatment. The median age of onset of disease is around 40 years with male predominance.3 The histopathology of DFSP which is the confirmatory test, shows storiform collagenoma with pleomorphism of the spindle cells of the dermis.4 The mitotic rate in the spindle cells co-relate with the metastasis, which is rare.5 DFSP is difficult for the clinicians to diagnose and treat because of the rarity of the disease, non- specific presentation and high degree of recurrence. Here, we report a rare case report of DFSP with complete cure post wide local excision.
OBSERVATION
A 64-year-old male patient from Kathmandu, retired serviceman presented with asymptomatic to occasionally painful slow growing lesion over upper back, left scapular region for 16 years. Initially, single pinhead sized, firm, raised lesion with red color was noted over left upper back that increased in size and number with largest one showing multinodular appearance and firm consistency. Lesions evolved over period of years with 3 in number and larger plaque progressed to form indurated infiltrated plaque with surrounding redness and prominent overlying solitary papule in the other small plaque. On examination, 3 plaques were noted with the largest plaque 4 * 3 cm in size roughly oval in shape over the left upper back in the scapular region, approx. 5 cm from mid-vertebral line of the spine (Figure 1). Biopsy was done which showed storiform collagenoma of the spindle cells with whorled pattern. Wide local excision with 2-4 cm margin was done along with reconstruction with Z-plasty flaps.(Figure 2) The excision site with reconstruction healed over time with minimal scarring. (Figure 3) There was complete cure of the disease post treatment with no recurrence till date.