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.