A case of a Beta-human chorionic gonadotropin secreting sinonasal teratocarcinosarcoma

Background and Importance Sinonasal teratocarcinosarcoma (SNTCS) is an unusual and aggressive neoplasm characterized by the combination of teratoma and carcinosarcoma features that is often misdiagnosed based on a biopsy sample. Approximately 60 cases have been reported in the English literature, but none have been associated with serum tumor markers. We report a case of SNTCS with elevation of serum β-human chorionic gonadotropin (hCG) level. Clinical Presentation The patient was a 22-year-old pregnant woman at diagnosis, and her β-hCG level was appropriately elevated. Her initial treatment was a surgical resection followed by radiation therapy (RT). Two years later, the patient had a pregnancy work-up after a sexual assault. Her serum β-hCG was elevated, but a nongravid uterus was seen on ultrasound. Magnetic resonance imaging (MRI) of the head revealed an intracranial tumor recurrence in the right frontal lobe. During salvage RT, β-hCG decreased rapidly to normal levels, with a complete tumor response on MRI. Retrospective examination of the initial surgical specimen showed expression of β-hCG associated with the anaplastic mesenchymal component. Conclusion This is the first reported case of a β-hCG secreting SNTCS. The source of β-hCG production was traced to a neoplastic smooth muscle component of the tumor.