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 1. E. M. Sturgis and B. O. Potter, “Sarcomas of the head and neck region,” Current Opinion in Oncology, vol. 15,no. 3, pp. 239–252,2003. 2. Farhood AI, Hajdu SI, Shiu MH, Strong EW. Soft tissue sarcomas of the head and neckin adults. Am J Surg1990;160:365–9. 3. Resta l., Pennella A., Siore M. G., Gotticella M. A.,Malignant fibrous histiocytoma of the larynx afterradiotherapy for squamous cell carcinoma, Eur ArchOtorhinolaryngol, 2000, 257(5):260–262. 4. Weiss S. W., EnzingerF. M., Malignant fibroushistiocytoma: an analysis of 200 cases, Cancer, 1978,41(6):2250–2266 5. Barnes L., KanbourA., Malignant fibrous histiocytoma ofthe head and neck. A report of 12 cases, Arch OtolaryngolHead Neck Surg, 1988, 114(10):1149–1156. 6. Barnes L., Ferlito A., Soft tissue neoplasms. In:FERLITO A. (ed), Neoplasms of the larynx, Churchill Livingstone, Edinburgh, 1993, 265–304. 7. Saleem M., Mcarthur P. D., Hainau B., Velagapudi S. B.,Fibrous histiocytoma of the larynx, J LaryngolOtol, 1998,112(12):1205–1207 8. Godoy J, Jacobs JR, Crissman J. Malignant fibrous histiocytoma of the larynx. J SurgOncol 1986;31:62-5. 9. Barbosa F.H., De Oliveira Rangel M. Coelho S. R.,patrocínio J. A., PatrocínioL. G., Malignant fibroushistiocytoma of the larynx, Intl Arch Otorhinolaryngol, São Paulo, 2006, 10(3):242–244. 10. Pellitteri PK, Ferlito A, Bradley PJ. Management of sarcomas of the head and neck in adults. Oral Oncol2003;39:2–12Abstract
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