Aim and objective: To describe small round blue cell tumors (SRBCT) of the sinonasal cavity which are tumors that are likely to be misdiagnosed due to their presenting features that often mimic each other. Background: Small round blue cell tumors clinically tend to cause symptoms of nasal obstruction and epistaxis. They have overlapping histologic and immunophenotypic findings; hence, a proper workup is required to reach the proper diagnosis and for further management of each case. Case description: We present two cases of SRBCT, where the patients presented with a 1-month history of nasal obstruction and episodes of epistaxis, along with ophthalmologic symptoms in one of the cases. The patients were diagnosed with histopathology and immunohistochemistry and responded well to surgical treatment and chemoradiotherapy. Conclusion: Sinonasal masses with erosive potential can have a wide differential diagnosis. Small round blue cell tumors respond well to surgical debridement and combined chemoradiotherapy, which can effectively prevent tumor recurrence in many cases. Clinical significance: Early diagnosis and proper management of the tumors effectively improve the treatment outcome and lead to lowering of mortality and morbidity for the patient.
Curado MP, Edwards B, Shin HR, et al. Cancer incidence in five continents: Volume IX, Vol IARC Scientific Publications No. 160, Lyon, France: International Agency for Research on Cancer; 2007.
Weber AL, Stanton AC. Malignant tumors of the paranasal sinuses: radiologic, clinical, and histopathologic evaluation of 200 cases. Head Neck Surg 1984;6(3):761–776. DOI: 10.1002/hed.2890060310.
Simons SA, Bridge JA, Leon ME. Sinonasal small round blue cell tumors: an approach to diagnosis. In: Seminars in diagnostic pathology, vol. 33, No. 2. WB Saunders; 2016. pp. 91–103.
Smorenburg CH, van Groeningen CJ, Meijer OW, et al. Ewing's sarcoma and primitive neuroectodermal tumour in adults: single-centre experience in The Netherlands. Neth J Med 2007;65:132–136.
Iriz A, Albayrak L, Eryilmaz A. Extraskeletal primary Ewing's sarcoma of the nasal cavity. Int J Pediat Otorhinolaryngol Extra 2007;2(3):194–197. DOI: 10.1016/j.pedex.2007.05.008.
Fertillo A. Primary ewings sarcoma of the maxilla: a clinico-pathological study of four cases. J Laryngol Otol 1978;94:1007–1024.
Böör A, Jurkovič I, Friedmann I, et al. Extraskeletal Ewing's sarcoma of the nose. J Laryngol Otol 2001;115(1):74–76. DOI: 10.1258/0022215011906885.
Mills SE, Fechner RE. Undifferentiated neoplasms of the sinonasal region: differential diagnosis based on clinical LM, EM and immunohistochemical. Semin Diagn Pathol 1989;6:316–328.
Toda T, Atari E, Sadi AM, et al. Primitive neuroectodermal tumor in sinonasal region. Auris Nasus Larynx 1999;26(1):83–90. DOI: 10.1016/S0385-8146(98)00051-0.
Thompson LD. Small round blue cell tumors of the sinonasal tract: a differential diagnosis approach. Mod Pathol 2017;30(s1):S1. DOI: 10.1038/modpathol.2016.119.
La TH, Meyers PA, Wexler LH, et al. Radiation therapy for Ewing's sarcoma: results from memorial Sloan-Kettering in the modern era. Int J Radiat Oncol Biol Phys 2006;64(2):544–550. DOI: 10.1016/j.ijrobp.2005.07.299.
Raney RB, Asmar L, Newton WA, et al. Ewing's sarcoma of soft tissues in childhood: a report from the Intergroup rhabdomyosarcoma study, 1972 to 1991. J Clin Oncol 1997;15(2):574–582. DOI: 10.1200/JCO.19126.96.36.1994.