Myxofibrosarcoma of neck: A rare case

Abstract

Myxofibrosarcoma (MFS) is commonly seen among elderly patients, usually sixth to eighth decade of life. Most of these tumours arise in the extremities (lower limbs > upper limbs) and are less commonly seen on the Trunk, Head and Neck, Retroperitoneum and Pelvic areas. The World Health Organization (WHO) defines MFS as the malignant fibroblastic neoplasm characterized by cellular pleomorphism, variably prominent myxoid stroma, and prominent elongated, thin-walled stromal blood vessels. Head Neck MFS is rare site of occurrence with 19 cases reported worldwide till date, described subsites being Maxillary sinus, Infra Temporal fossa, Pterygopalatine fossa, or Parotid gland. The Surgical wide local excision is the main modality of treatment for Non metastatic stage followed by Adjuvant Radiotheraphy.We are presenting a young adult male, presenting with Asymptomatic progressive neck mass, predominantly in posterior triangle distorting upper Aerodigestive tract anatomy. The Diagnosis of MFS done with MRI imaging of neck, Trucut biopsy and CECT scan thorax. With the anticipation of difficult intubation, Broncoscopic directed intubation is planned and done. The surgical wide resection amounted in excision of Sternomastoid, IJV, SAN and part of posterior triangle bed muscles for getting margin free status. The Histopathology showed spindle cells with Myxoid stroma, areas of necrosis with circumferential free margins, along with IHC showed positive for vimentin and CD34 with diagnosis as High Grade Myxofibrosarcoma. Post op patient was adviced Adjuvant Radiotheraphy.