Sinonasal teratocarcinosarcoma: report of a case with review of literature and treatment outcome

Sinonasal Teratocarcinosarcoma: A Rare Clinical Entity managed by Medial Maxillectomy and Adjuvant Chemoradiation

“…6 Incidence is seven times higher in males. 15,16 No major meta-analysis is done on the tumor studies as most literature are case reports and series where the authors widely vary clinical features, management techniques and biological outcomes. 9,17 Clinical behavior and treatment modalities are not stressed in these studies and more of a histopathology, differential diagnosis and tissues of origin were remarked.…”

Ethmoid Sinus Mass

“…The prognosis of SNTCS is poor. Survival rates are reported to be 30% to 54% at 3 years and 20% at 5 years, 2,8 with a mean survival of 1.7 to 1.9 years. 3,6 Recurrence and metastasis rates are high, occurring in 67% of patients treated with surgery alone 2 and 45.7% of patients treated with surgery and radiation.…”

“…5 Primitive neuroectodermal or blastemal components are characterized by small, hyperchromatic cells that may be organized in nests, sheets, or rosettes, and stain positively for neuroendocrine markers, such as synaptophysin, chromogranin A, and CD99. 2 Staining of the epithelial and mesenchymal elements depends on the particular components of a given tumor. Given their histologic heterogeneity and rarity, SNTCS is commonly misdiagnosed, especially when inadequately sampled during biopsy, with an initial identification rate of roughly 50%.…”

Sinonasal teratocarcinosarcoma: is minimally invasive resection followed by adjuvant histology directed chemo-radiation a better alternative to radical excision? Case report

“…[4][5][6] Immunohistochemical studies clearly demonstrated SNTCS usually occur in elderly males with an average age at presentation of 51.75 years. 4,7 It rarely occurs at a younger age. Only 4 cases below the age of 25 years reported till date, all of them coming from Indian subcontinent; a fact possibly pointing towards a common environmental or genetic link predisposing to early onset of this tumour.…”

“…Sixty percent (60%) of the patients received adjuvant radiotherapy and 12% underwent adjuvant chemoradiation. 2,5,7 In view of its aggressive nature, an extensive radical excision of the tumour with or without chemoradiation is advocated as the optimum treatment. However local recurrence of SNTCS after excision has been reported as high as 45% with a mean recurrence time of 21.3 months.…”