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.

Keywords

teratocarcinosarcoma - β-hCG - gonadotropin - serum - intracranial

• References

• 1 Heffner DK, Hyams VJ. Teratocarcinosarcoma (malignant teratoma?) of the nasal cavity and paranasal sinuses A clinicopathologic study of 20 cases. Cancer 1984; 53 (10) 2140-2154
• 2 Cardesa A, Luna MA. Germ cell tumours. In: Barnes L, Eveson JW, Reichart P, Sidransky D, , eds. World Health Organization Classification of Tumours: Pathology & Genetics—Head and Neck Tumours. Lyon, France: IARC; 2005: 76-77
• 3 Wei S, Carroll W, Lazenby A, Bell W, Lopez R, Said-Al-Naief N. Sinonasal teratocarcinosarcoma: report of a case with review of literature and treatment outcome. Ann Diagn Pathol 2008; 12 (6) 415-425
• 4 Kleinschmidt-DeMasters BK, Pflaumer SM, Mulgrew TD, Lillehei KO. Sinonasal teratocarcinosarcoma (“mixed olfactory neuroblastoma-craniopharyngioma”) presenting with syndrome of inappropriate secretion of antidiuretic hormone. Clin Neuropathol 2000; 19 (2) 63-69
• 5 Kane SV, Karpate AA, Bal M, Juvekar SL, Pai PS. Chemotherapy-induced neuronal maturation in sinonasal teratocarcinosarcoma—a unique observation. Head Neck Pathol 2009; 3 (1) 31-36
• 6 Pai SA, Naresh KN, Masih K, Ramarao C, Borges AM. Teratocarcinosarcoma of the paranasal sinuses: a clinicopathologic and immunohistochemical study. Hum Pathol 1998; 29 (7) 718-722
• 7 Shemen L, Galantich P, Murali R. Malignant teratocarcinosarcoma of the sphenoid sinus. Otolaryngol Head Neck Surg 1995; 112 (3) 496-500
• 8 Liang S, Stone G, Chalas E, Pearl M, Callan F, Zheng W. A high-grade uterine leiomyosarcoma with human chorionic gonadotropin production. Int J Gynecol Pathol 2006; 25 (3) 257-261
• 9 Kelley BC, Arnold PM, Grant JA, Newell KL. Primary intracranial β-human chorionic gonadotropin-producing leiomyosarcoma in a 2-year-old immunocompetent child. J Neurosurg Pediatr 2012; 10 (2) 121-125
• 10 Froehner M, Gaertner HJ, Manseck A, Oehlschlaeger S, Wirth MP. Retroperitoneal leiomyosarcoma associated with an elevated beta-HCG serum level mimicking extragonadal germ cell tumor. Sarcoma 2000; 4 (4) 179-181