Myxoinflammatory Fibroblastic Sarcoma of the Hand.

ABSTRACT

Myxoinflammatory fibroblastic sarcoma is an ultra-rare tumor type with a prevalence of fewer than two per 100,000 people. The tumor poses a challenge because it can be misdiagnosed as a benign lesion in clinical and radiological investigations, causing serious morbidity in patients. We present the case of a 33-year-old patient who presented with painless hand swelling that was misdiagnosed as lymphaticovenous malformation based on magnetic resonance imaging. The patient underwent surgical excision, and myxoinflammatory fibroblastic sarcoma was diagnosed postoperatively. All surgical interventions failed to achieve negative margin. A decision to start radiotherapy was made, and tissue convergence was done temporally using acellular dermal matrix and split-thickness skin graft. On patient follow-up, the graft had taken well, and the patient was undergoing radiotherapy sessions with a plan for permanent hand reconstruction after negative margins are achieved. Based on this case report, we identified that magnetic resonance imaging is not yet a reliable method to diagnose myxoinflammatory fibroblastic sarcoma. Therefore, implementing a multidisciplinary team approach, a preoperative core needle biopsy, planned surgical intervention, and early involvement of radiotherapy is recommended to minimize morbidity. We strongly urge establishing a sarcoma specialized treatment center in the region to limit patient morbidity.