A teenage rarity: Sinonasal teratocarcinosarcoma in an adolescent and its clinical course

Introduction

Sinonasal teratocarinosarcoma (SNTCS) is a rare and highly aggressive malignancy of the anterior skull base typically affecting the paranasal and ethmoidal sinuses. Histologically, SNTCS exhibits unique heterogeneous features having mixed characteristics of teratoma and carcinosarcoma. This complexity makes diagnosis challenging, often leading to delays in treatment. The first case of SNTCS was described in the literature in 1966 by Patchefsky et al. However the term “teratocarcinosarcoma” was coined 16 years later by Heffner and Hyams who outlined the unique heterogenic pathology of the disease in their analysis of 20 cases. The overall incidence of this tumor remains extremely low with less than 150 cases reported in the literature, primarily consisting of single case reports or case series.

Case report

This case report details a rare instance of sinonasal teratocarcinosarcoma in a 15-year-old male who presented with nasal swelling. Imaging revealed polypoidal thickening in the sinuses, leading to surgical resection. Pathology indicated undifferentiated carcinoma with positive markers for teratocarcinosarcoma. After a year in remission, a local recurrence was noted, prompting additional surgery and adjuvant chemoradiotherapy. The patient completed radiotherapy and chemotherapy with no residual disease on follow-up imaging. He currently remains in remission under regular surveillance.

Conclusion

Sinonasal teratocarcinosarcoma (SNTCS), is known to be aggressive in nature leading to a poor prognosis. Aggressive tri-modality treatment is currently the most effective treatment with acceptable toxicity. The rarity of the condition underscores the need for further research to understand the genetic or environmental influences that may contribute to such atypical cases.