Acta Scientific Otolaryngology (ASOL)

Case Report Volume 2 Issue 2

A Rare Case of Biphenotypic Sino-Nasal Sarcoma - Case Report and Review of Literature

George MV1*, C S Sakunthalabhai2 and Lina Theresa3

1ENT Department, Jubilee Mission Medical College, Thrissur, Kerala, India
2Professor of Pathology, Jubilee Mission Medical College, Thrissur, Kerala, India
3Junior Resident in ENT, Jubilee Mission Medical College, Thrissur, Kerala, India

*Corresponding Author: George MV, ENT Department, Jubilee Mission Medical College, Thrissur, Kerala, India.

Received: January 26, 2020; Published: January 30, 2020

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Abstract

  Biphenotypic sinonasal sarcoma is a newly described tumour of nose and paranasal sinuses containing neural and muscle tissue together in the tumour tissue. There are almost 68 different types of tumours in the Sinonasal tract. This tumour is in addition to that. A 60 year old female with history of nose block of both sides of 8 years duration, and a visible mass in the right nasal cavity, since 2 years presented to us one month back. There was no obvious features of malignancy. CT PNS showed large heterogeneous calcified mass showing enhancement in the right nasal cavity eroding the nasal septum and reaching left side pushing it. Histopathology showed a neoplasm covered partly by partly by psuedostratified columnar epithelium and partly by squamous epithelium. Neoplasm was cellular and composed of spindle cells with spindle/oval/wavy nuclei arranged in in sheets It was seen invading the adjacent bone. Histopathology was suggestive of low grade sarcoma possibly Biphenotypic sinonasal sarcoma. Immunohistochemistry showed vimentin and S100 positivity, SMA was focally positive. Betacatenin showed cytoplasmic granular positivity, Ki 67 percentage of positive nuclei 2-3%, MyoD1 Negative and the final report was Biphenotypic sinonasal sarcoma. This neoplasm contained myogenic and nervous tissue together. Endoscopic excision of the tumour was done. She was advised Craniofacial resection for this low grade malignancy as it was dangerous to remove it endoscopically due to the close proximity with the cribriform plate.

Keywords: Biphenotypic Sinonasal Sarcoma; New Entities of Sinonasal Tract Tumours

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References

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  2. Stelow EB and Bishop JA. “Update from the 4th edition of the World Health Organization classification of head and neck tumours: tumors of the nasal cavity, paranasal sinuses and skull base”. Head and Neck Pathology 11.1 (2017): 3-15.
  3. Lewis JT., et al. “Low-grade sinonasal sarcoma with neural and myogenic features: a clinicopathologic analysis of 28 cases”. The American Journal of Surgical Pathology 36.4 (2012): 517-525.
  4. Lin Y., et al. “Biphenotypic sinonasal sarcoma with diffuse infiltration and intracranial extension: a case report”. International Journal of Clinical and Experimental Pathology 10.12 (2017): 11743-11746.
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  6. Al Zamel H., et al. “Nasal Biphenotypic Sino-Nasal Sarcoma in a Young Female-A Diagnostic Dilemma”. Global Journal of Medical Research (2018).
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  8. Knowles KJ., et al. “Biphenotypic Sinonasal Sarcoma Successfully Treated with Complete Excision and Facial Reconstruction with Flap: Case Report and Review of the Literature”. American Journal of Otolaryngology and Head and Neck Surgery 2 (2019). 
  9. Hermsen MA., et al. “Molecular Pathology of Sinonasal Tumors”. In: Franchi A. (eds) Pathology of Sinonasal Tumors and Tumor-Like Lesions. Springer, Cham. (2012).
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Citation

Citation: George MV., et al. “A Rare Case of Biphenotypic Sino-Nasal Sarcoma - Case Report and Review of Literature". Acta Scientific Otolaryngology 2.2 (2020): 24-26.




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