Post radiation myofibrosarcoma of hypopharynx
A 67-year-old man presented to our ENT department with history suggestive of globus feeling in the throat. The patient had received a full course of radiotherapy to the larynx for well-differentiated T1N0M0, squamous cell carcinoma of the larynx. There was no past history of hereditary conditions predisposing to sarcomas, including Recklinghausen's disease, hereditary forms of retinoblastoma and Wilms tumour. Direct laryngoscopic examination revealed a postcricoid tumour extending to the oesophagus. Biopsy was reported as a grade 2 spindle cell sarcoma. Computed tomographic scans demonstrated a post-cricoid lesion extending into the cervical oesophagus (Fig. ). No cervical lymphadenopathy or distant metastases were noted.\nThe patient underwent a total laryngopharyngectomy with flap repair. Macroscopically, the hypopharynx showed a polypoidal tumour measuring 50 × 32 × 21 mm in dimensions. Microscopy showed spindle cells arranged in short fascicles and sheets. Individual cells showed short spindle and elongated hyper-chromatic nuclei with mild-to-moderate amount of pale to eosinophilic cytoplasm (Fig. ). There was no evidence of squamous or glandular differentiation. There was no dysplasia within the adjacent squamous epithelium. On immunohistochemistry, diffuse staining for smooth muscle actin (SMA) in a tram track pattern was noted. In addition, a minor population of tumour cells stained for desmin (Fig. ). There was no staining for AE1 AE3, pancytokeratin, H-caldesmon, myogenin, CK 5/6, CK14, p63, 34BE12, CD34, CK7, CD56, EMA, S100, melan A, c-KIT and CD99. The final diagnosis of a myofibrosarcoma FNCLCC grade 2 was made. The surgical margins were free of tumour.