Abstract
Low grade fibromyxoid sarcoma (LGFMS) is an uncommon variant of fibrosarcoma with high risk of local recurrence, immense metastatic potential and frequently protracted period between tumour presentation and metastasis. This unusual malignancy rarely affects the region of the head and neck which makes cases of laryngeal LGFMS extremely infrequent. To date, LGFMS of the larynx has been scatteredly mentioned in the literature. Neither incidence nor causes and risk factors for laryngeal LGFMS have been clarified so far. To the authors' knowledge, this is the first case report that discusses the clinical course, imaging diagnosis, histopathological evaluation and surgical approach to radiation-induced laryngeal LGFMS.We present a case of a 70-year-old man who developed a LGFMS after previous radiotherapy (RT) for squamous cell carcinoma (SCC) of the larynx. The latency period between the time of radiation exposure and the diagnosis of LGFMS was twenty-seven months. After re-confirming the diagnosis with second biopsy and extensive imaging evaluation the patient was subjected to an open partial resection of the larynx. Owing to the rarity of the tumour, there is no established protocol with follow-up recommendations.This case highlights the importance of considering the RT history of the patient in order to monitor radiotherapy-related complications, including the occurrence of LGFMS.
Highlights
Low grade fibromyxoid sarcoma (LGFMS) is a rare variant of fibrosarcoma
We present a case of a 70-year-old man who developed a LGFMS after previous radiotherapy (RT) for squamous cell carcinoma (SCC) of the larynx
It is characterized by a high risk of local recurrence, an immense metastatic potential and protracted period between tumour presentation and metastasis.[1,2]
Summary
Low grade fibromyxoid sarcoma (LGFMS) is a rare variant of fibrosarcoma. It is characterized by a high risk of local recurrence, an immense metastatic potential and protracted period between tumour presentation and metastasis.[1,2] LGFMS is a well-described but sporadically observed neoplasm which makes it misdiagnosed; it is with undetermined incidence rate.[3,4] Usually, middle aged adults are affected.[5,6] Laryngeal LGFMS cases have been scatteredly mentioned in the literature with only a few contributable to previous radiation therapy.[3,6,7,8] We present a case of radiation-induced LGFMS of the larynx. Histopathology following endoscopic cold-steel excision revealed moderately differentiated squamous cell carcinoma (SCC) with no signs of regional or distant spread (T1N0M0). Following the endoscopic debulking of the tumour, the patient was soon discharged. Histopathology revealed lesions lined by keratinized stratified squamous epithelium surrounded by myxoid areas with curvilinear blood vessels (Fig. 2A, 2B), foci of necrosis and mitotic figures. Immunoreactivity with MUC4 revealed a characteristic moderate-to-strong and diffuse granular cytoplasmic expression (Figs 3E, 3F).
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