A Case of a Beta-Human Chorionic Gonadotropin Secreting Sinonasal Teratocarcinosarcoma

Abstract

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.

Sinonasal teratocarcinosarcoma: a case report and literature review

“… 6 , 7 The incidence is relatively low, with fewer than 150 cases reported worldwide and only ∼10 cases in China. We reviewed the literature from 1968 to 2020, and 107 SNTCS cases were identified during this period ( Table 1 ) 5 , 8 – 22 .…”

A rare case of intracranial teratocarcinosarcoma: Case report and review of literature

“…A review of literature revealed 11 cases of TCS with intracranial invasion [ Table 1 ]. [ 3 6 7 8 9 16 17 19 20 21 ] Although the documented cases with predominant intracranial involvement are scarce, treatment with surgical resection with or without adjuvant treatments of chemotherapy and radiation therapy is the most widely accepted with goal for gross total resection. Despite few intracranial cases described in the literature, patients undergoing gross total resection fare better than those with subtotal resection.…”