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Small Round Blue Cell Tumours of the Sinonasal Area: Our 5 year Experience in a Tertiary Care Centre in India

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Indian Journal of Otolaryngology and Head & Neck Surgery Aims and scope Submit manuscript

Abstract

Purpose: The main purpose of this study is to understand the characteristics and management of sinonasal small round blue cell tumors and also to emphasise the role of immunohistochemistry in their diagnosis and on the outcomes after endoscopic/open excision in these patients. Methods: This is a retrospective study conducted at a tertiary care referral centre in India which included 38 patients with sino nasal for a period of 5 years. All the patients were evaluated clinically and radiologically. All cases were confirmed diagnostically with histopathological examination and immunohistochemistry following surgical excision either by endoscopic or open approach. Some of the cases underwent post operative radiotherapy. Results: In our study, among 176 cases diagnosed with Sino nasal malignancies, 38 (21.6%) cases were diagnosed with sinonasal small round blue cell tumors with male to female ratio 1.4:1. Most common histopathological type among all the sinonasal small round blue cell tumors that presented to us was esthesioneuroblastoma i.e., 8 (21%) patients followed by pituitary macroadenoma in 7(8.4%) patients. Other types are undifferentiated squamous cell carcinoma 10(13.1%), craniopharyngioma 8(10.5%), lymphoma 3(7.9%), synovial/spindle cell sarcoma, malignant melanoma and adenocarcinoma 1(2.6%) each. Schwannoma, rhabdomyosarcoma, neuroendocrine carcinoma and neurofibroma 2 (5.2%) each. Conclusion: Sinonasal small round blue cell tumors are extremely rare tumours. Histopathological diagnosis with immunohistochemistry is characteristic of various tumors and is conclusive for diagnosis. Knowledge of these tumor entity is essential as early diagnosis helps in further management in preventing spread to vital structures and improving outcome. Most of the tumors have a multimodality treatment approach which includes surgical excision, radiotherapy and chemotherapy.

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References

  1. Jemal A, Siegel R, Ward E, Hao Y, Xu J, Thun MJ (2009). Cancer statistics. CA Cancer J Clin. 59(4):225–249. https://doi.org/10.3322/caac.20006

    Article  PubMed  Google Scholar 

  2. WatkinsonJC,ClarkeRW,(2018)Scott-Brown’s Otorhinolaryngology and Head and Neck surgery: 3 volume set.CRC Press;Jul17.

  3. Thompson LD (2017). Small round blue cell tumors of the sinonasal tract: a differential diagnosis approach. Mod Pathol. 30(s1):S1-S26. https://doi.org/10.1038/modpathol.2016.119

    Article  PubMed  Google Scholar 

  4. Bridge JA, Bowen JM, Smith RB (2010). The small round blue cell tumors of the sinonasal area. Head Neck Pathol. 4(1):84–93. https://doi.org/10.1007/s12105-009-0158-6

    Article  PubMed  PubMed Central  Google Scholar 

  5. Das, R., Sarma, A., Sharma, J. D., Singh, D., Rahman, T., & Kataki, A. C (2018) Clinico-histopathological study of sinonasal malignant tumours - a 5 years experience at a tertiary cancer institute. Headache. 6(11):48–59. https://doi.org/10.18535/jmscr/v6i11.56

    Article  Google Scholar 

  6. Cohen ZR, Marmor E, Fuller GN, DeMonte F(2002). Misdiagnosis of olfactory neuroblastoma. Neurosurg Focus. 12(5):e3. https://doi.org/10.3171/foc.2002.12.5.4

    Article  PubMed  Google Scholar 

  7. Mills SE, Frierson HF Jr (1985). Olfactory neuroblastoma. A clinicopathologic study of 21 cases. Am J Surg Pathol. 9(5):317–327.

    Article  CAS  PubMed  Google Scholar 

  8. Kadish S, Goodman M, Wang CC (1976). Olfactory neuroblastoma. A clinical analysis of 17 cases. Cancer. 37(3):1571–1576. https://doi.org/10.1002/1097-0142(197603)37:3<1571::aid-cncr2820370347>3.0.co;2-l

    Article  CAS  PubMed  Google Scholar 

  9. Iezzoni JC, Mills SE (2005). “Undifferentiated” small round cell tumors of the sinonasal tract: differential diagnosis update. Am J Clin Pathol. 124 Suppl:S110-S121. https://doi.org/10.1309/59RBT2RK6LQE4YHB.

  10. Wenig BM (2009). Undifferentiated malignant neoplasms of the sinonasal tract. Arch Pathol Lab Med. 133(5):699–712. https://doi.org/10.5858/133.5.699

    Article  PubMed  Google Scholar 

  11. Carey RM, Godovchik J, Workman AD, et al (2017). Patient, disease, and treatment factors associated with overall survival in esthesioneuroblastoma. Int Forum Allergy Rhinol. 7(12):1186–1194. https://doi.org/10.1002/alr.22027

    Article  PubMed  Google Scholar 

  12. Duncavage JA, Campbell BH, Hanson GA, et al (1983). Diagnosis of malignant lymphomas of the nasal cavity, paranasal sinuses and nasopharynx. Laryngoscope. 93(10):1276–1280. https://doi.org/10.1002/lary.1983.93.10.1276

    Article  CAS  PubMed  Google Scholar 

  13. Fajardo-dolci G, Magaña RC, Bautista EL, Huerta D. Sinonasallymphoma (1999).Otolaryngol Head Neck Surg.121(3):323–326. https://doi.org/10.1016/S0194-5998(99)70192-0

  14. Fletcher CD (2019) Diagnostic histopathology of tumors E-Book. Elsevier Health Sciences.

  15. Thompson L (2006). World Health Organization classification of tumours: pathology and genetics of head and neck tumours. Ear Nose Throat J. 85(2):74.

    Article  PubMed  Google Scholar 

  16. Goel R, Ramalingam K, Ramani P, Chandrasekar T (2012). Sino nasal undifferentiated carcinoma:a rare entity. J Nat Sci Biol Med. 3(1):101–104. https://doi.org/10.4103/0976-9668.95986

    Article  PubMed  PubMed Central  Google Scholar 

  17. Frierson HF Jr, Mills SE, Fechner RE, Taxy JB, Levine PA (1986). Sinonasal undifferentiated carcinoma. An aggressive neoplasm derived from schneiderian epithelium and distinct from olfactory neuroblastoma. Am J Surg Pathol. 10(11):771–779.

    Article  PubMed  Google Scholar 

  18. Sarangi S, Khera S, Vishwajeet V, Meshram V, Setia P, Malik A (2020). A rare presentation of Sinonasal Undifferentiated Carcinoma with brain metastasis and para-aortic mass. Autops Case Rep.10(4):e2020222. Published 2020 Nov 20. https://doi.org/10.4322/acr.2020.222

    Article  PubMed  PubMed Central  Google Scholar 

  19. Ejaz A, Wenig BM (2005). Sinonasal undifferentiated carcinoma: clinical and pathologic features and a discussion on classification, cellular differentiation, and differential diagnosis. Adv Anat Pathol.12(3):134–143.https://doi.org/10.1097/01.pap.0000163958.29032.56

    Article  PubMed  Google Scholar 

  20. Jeng YM, Sung MT, Fang CL, et al (2002). Sinonasal undifferentiated carcinoma and nasopharyngeal-type undifferentiated carcinoma: two clinically, biologically, and histopathologically distinct entities. Am J Surg Pathol. 26(3):371–376. https://doi.org/10.1097/00000478-200203000-00012

    Article  PubMed  Google Scholar 

  21. RichardG.Ellenbogen,Laligam N.Sekhar,Neil D.Kitchen,Harley Brito da Silva (2018)(4th eds) Craniopharyngiomas. Principles of neurological surgery,Elsevier,Pages 204–218.e3,<background-color:#CFBFB1;udirection:rtl;>https://doi.org/10.1016</background-color:#CFBFB1;udirection:rtl;> B978-0-323- 43140-8.00012-3.

  22. Fahlbusch R, Honegger J, Paulus W, Huk W, Buchfelder M (1999). Surgical treatment of craniopharyngiomas: experience with 168 patients. J Neurosurg. 90(2):237–250. https://doi.org/10.3171/jns.1999.90.2.0237

    Article  CAS  PubMed  Google Scholar 

  23. Smith MH, Atherton D, Reith JD, Islam NM, Bhattacharyya I, Cohen DM (2017). Rhabdomyosarcoma, Spindle Cell/Sclerosing variant: a clinical and histopathological examination of this rare variant with three new cases from the oral cavity. Head Neck Pathol. 11(4):494–500. https://doi.org/10.1007/s12105-017-0818-x

    Article  PubMed  PubMed Central  Google Scholar 

  24. Gallego Melcón S, Sánchez de Toledo Codina J (2005). Rhabdomyosarcoma: present and future perspectives in diagnosis and treatment. Clin Transl Oncol. 7(1):35–41. https://doi.org/10.1007/BF02710026

    Article  PubMed  Google Scholar 

  25. Parham DM, Barr FG(2013). Classification of rhabdomyosarcoma and its molecular basis. Adv Anat Pathol. 20(6):387–397. https://doi.org/10.1097/PAP.0b013e3182a92d0d

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Moreno MA, Roberts DB, Kupferman ME, et al (2010). Mucosal melanoma of the nose and paranasal sinuses, a contemporary experience from the M. D. Anderson Cancer Center. Cancer. 116(9):2215–2223. https://doi.org/10.1002/cncr.24976

    Article  PubMed  Google Scholar 

  27. Williams MD (2017). Update from the 4th Edition of the World Health Organization classification of Head and Neck Tumours: mucosal melanomas. Head Neck Pathol. 11(1):110–117. https://doi.org/10.1007/s12105-017-0789-y

    Article  PubMed  PubMed Central  Google Scholar 

  28. Xavier Júnior JCC, Ocanha-Xavier JP (2018). What does the 4th edition of the World Health Organization classification of Head and Neck Tumors bring new about mucosal melanomas?. An Bras Dermatol. 93(2):259–260. https://doi.org/10.1590/abd1806-4841.20187206

    Article  PubMed  PubMed Central  Google Scholar 

  29. Gallia GL, Sciubba DM, Hann CL, et al (2005). Synovial sarcoma of the frontal sinus. Case report. J Neurosurg. 103(6):1077–1080. https://doi.org/10.3171/jns.2005.103.6.1077

    Article  PubMed  Google Scholar 

  30. Perez-Ordonez B, Caruana SM, Huvos AG, Shah JP (1998). Small cell neuroendocrine carcinoma of the nasal cavity and paranasal sinuses. Hum Pathol. 29(8):826–832. https://doi.org/10.1016/s0046-8177(98)90452-x

    Article  CAS  PubMed  Google Scholar 

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Acknowledgements

The authors would like to gratefully acknowledge and thank medical supernindent, faculty, postgraduates, patients and attendants for accepting for publication.

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  1. Department of Otorhinolaryngology, Head and Neck Surgery, Gandhi Medical College and Hospital, Secunderabad, India

    Mounika Reddy Y, Sourabh Padmanabhan, Shobhan Babu A, Sai Swarna B &  Nagaraju

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Reddy Y, M., Padmanabhan, S., Babu A, S. et al. Small Round Blue Cell Tumours of the Sinonasal Area: Our 5 year Experience in a Tertiary Care Centre in India. Indian J Otolaryngol Head Neck Surg 75, 2177–2184 (2023). https://doi.org/10.1007/s12070-023-03840-z

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