Title: Undifferentiated Pleomorphic Sarcoma of Larynx: A Rare Entity with Review of Literature
Authors: Himanshu Mishra, MadhupRastogi, Ritusha Mishra, Anurag Gupta, R K Srivastava, Anoop Srivastava
DOI: http://dx.doi.org/10.18535/jmscr/v3i9.59
Sarcomas of the larynx are rare neoplasms that constitute less than 1% of laryngeal malignancies. We describe a rare case of undifferentiated pleomorphic sarcoma of larynx of a 62-year- old male who presented with hoarseness of voice. Laryngoscope showed growth over glottic region of larynx and biopsy revealed poorly differentiated carcinoma. Patient was treated with radical radiotherapy (RT). After 3 months of completion of RT, laryngoscopy and CECT revealed residual lesion. Cordectomy was done. Post- cordectomy histopathology report (HPR) and review of pre RT biopsy specimen showed tumour composed of highly pleomorphic spindle cells with moderate eosinophilic cytoplasm, vesicular nuclei with prominent eosinophilic nucleoli, frequent mitoses and occasional multinucleate cells. IHC showed tumour cells were positive for vimentin and focal positive for smooth muscle actin but were negative for Pan-CK, CK- 5/6, S-100, CD-34 and desmin. The overall features were consistent with undifferentiated pleomorphic sarcoma. Post- cordectomy PET-CT showed metastatic disease. Patient was further managed with 6 cycles of Ifosfamide and Epirubicin based palliative chemotherapy (CT) and after 6 months of CT completion, patient is symptomatically better. The characteristic clinical, histopathological features and management of this rare case are described with a literature review. Keywords : Larynx, undifferentiated pleomorphic sarcoma, metastases
Abstract
