A Rare Case of Ewing's-like Adamantinoma of Tibia Managed by Limb Salvage Surgery Using Long Segment Ilizarov Bone Transport: A Case Report and Review of Literature.

INTRODUCTION

Adamantinoma is a rare low-grade malignant tumor in young adults. Recent advances in molecular techniques have shown different variants with discordance between genotype and phenotype. This poses a challenge in diagnosis and management. Ewing's-like adamantinoma is one such variant. The appropriate treatment protocol for this dedifferentiated tumor remains to be established. Here, we present a rare case of Ewing's-like Adamantinoma treated with limb salvage surgery using three step Ilizarov technique with good results.

CASE REPORT

A 38-year-old lady presented at our OPD complaining of recent onset pain and gradual increase in size over a long standing swelling in the right lower tibia. Radiologically was diagnosed as Ewing's but was not responsive to chemotherapy. A Tru-Cut biopsy showed histological picture of dedifferentiated adamantinoma. Immunohistochemistry showed CD99 positivity. FISH revealed (11;22) translocation confirming Ewing's-like adamantinoma. She was managed with wide excision followed by staged Ilizarov distraction-osteogenesis and bone transport to correct the 13 cm discrepancy in bone length. There have been no signs of recurrence on post-resection follow-up of 2 years. Patient attained full range of knee and ankle movements.

CONCLUSION

The controversy as to what constitutes the difference between adamantinoma-like Ewing's and Ewing's-like adamantinoma persists despite technological advances. The appropriate treatment protocol remains to be established. Ilizarov three step techniques provide a feasible alternative to amputation while circumventing the difficulties of large bone reconstruction in the distal tibia.