Teratocarcinosarcoma of the nasal cavity: challenges in the clinico-pathologic perspectives

Clinicopathologic illustration of sinonasal teratocarcinosarcoma (SNTCS) in a middle-aged man, highlighting the difficulties and challenges encountered during surgical intervention, histopathologic diagnosis, and its overall management.

Case report and literature review.

A 40-year-old man having recurrent epistaxis for three months presented with a dark-colored protruding polypoid nasal mass. Magnetic resonance imaging revealed a large, heterogeneous gadolinium-enhanced infiltrative lesion in the left nasal cavity with a T2-hypointense trans-septal zone. The mass abutted the cribriform plate, lamina papyracea, and septum, involved the inferior and middle turbinates, and blocked the choanae through the nasopharynx. There was profuse hemorrhage when an endoscope-assisted biopsy was attempted. Histopathology from the debulked specimen suggested SNTCS. The diagnosis was confirmed on immunohistochemistry. A metastatic search turned negative. However, at three weeks, the patient returned with a recurrence. A repeat debulking was done, and he was thereafter put on adjuvant chemotherapy. A second recurrence/residual lesion was noted midway through the chemotherapy regimen. However, it resolved after completion of the chemotherapy and subsequent image-guided radiotherapy, and the patient continued to be disease-free till the last follow-up at six weeks postradiotherapy.

SNTCS is an extremely aggressive malignancy that is seldom encountered in routine otolaryngology and pathology practice, with only a few reports of SNTCS documented. It is histologically composed of epithelial, mesenchymal, and primitive neuroectodermal elements with areas of undifferentiation. They are notorious for troublesome intra-operative bleeding, making complete surgical excision difficult, and the absence of a tumor-free margin often results in recurrence. Owing to their variegated and heterogeneous tissue composition, histopathologic diagnosis is enormously challenging without a representative tissue sample and immunohistochemistry. In spite of prompt and energetic multimodality treatment, survival rate is dismal.