Presentation
Palpable neck lump.
Patient Data
Large approximately 10 cm neck mass with heterogeneous echotexture and internal color Doppler signal.
Large left neck mass centered in the left retropharyngeal space, displacing the carotid artery and internal jugular vein laterally and pharynx medially. It is predominantly hypodense, appearing heterogeneous superiorly.
Large heterogeneous mass centered in the retropharyngeal/prevertebral space, extending from skull base to C6 level. Pharynx and parapharyngeal space is displaced anteriorly, and the carotid space is displaced laterally, with enhancement surrounding the internal carotid artery. Longus colli appears involved. It is mildly heterogeneous with an overall high T2 signal with low T2 septae, and vivid post-contrast enhancement.
Histopathology
MACROSCOPIC DESCRIPTION: An oval well-circumscribed nodule 25 x 25 x 20 mm with a gelatinous cut surface.
MICROSCOPIC DESCRIPTION: Sections show a variably cellular tumor comprising vaguely nodular arrangements of spindle cells within a prominent myxoid background. Tumor cells have eosinophilic processes and elongated hyperchromatic, pleomorphic nuclei. Occasional large multinucleated cells with vacuolated cytoplasm are seen. The background contains numerous thin-walled curvilinear blood vessels, scattered collagen fibers and a patchy chronic inflammatory cells infiltrate. Several highly cellular solid areas are present with markedly atypical cells. Mitoses are present, up to 3 per 10 HPF. There is no necrosis. Tumor extensively abuts the inked margins. Vessels and nerves are present within the tumor but lymphovascular and perineural invasion are not seen. The myxoid stroma and vacuolated cytoplasm within tumor cells stains with Alcian blue. Tumor cells stain with Bcl2, CD99 and weakly for CD31 and CD34, while negative for AE1/3, CAM5.2, SMA, desmin, myogenin, calponin, S100, SOX10 and NFP. Ki67 proliferative index is 5-10%.
DIAGNOSIS: High-grade myxofibrosarcoma.
Case Discussion
Myxofibrosarcoma are rare soft tissue tumors that most commonly occur in the lower limbs but rarely occur in the head and neck.
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