Citation Information :
Mitra S, Nandeesh BN. Sinonasal Teratocarcinosarcoma, a Rare Neoplasm with Potential Diagnostic Pitfalls: A Report of Three Cases. Int J Head Neck Surg 2023; 14 (4):71-76.
Background: Sinonasal teratocarcinosarcoma (SNTCS) is a very rare aggressive neoplasm of the sinonasal tract and affects older individuals. The tumor accounts for <1% of all sinonasal neoplasm and shows a striking male predominance. The admixture of epithelial, mesenchymal, and primitive neuroepithelial elements is essential for the diagnosis of this tumor. However, the morphological differential diagnosis of SNTCS is wide and can lead to potential diagnostic error if any one of the three elements predominates in a biopsy.
Case description: We report three cases of SNTCS from a single center. All three patients were middle-aged adults, including one male and two female patients. The age range was between 42 and 46 years. All of the three patients presented with nasal obstruction, pain, and nasal bleeding. The imaging study revealed a direct intracranial extension of the tumor in one of the patients. Two patients underwent frontal craniotomy, and the third patient underwent transnasal decompression of the tumor. On light microscopic examination, all three cases revealed similar morphology. The tumor tissue was composed of three distinct components, including malignant epithelial, mesenchymal, and primitive neuroepithelial elements. Immunohistochemistry (IHC) showed strong pan-cytokeratin (AE1/AE3) positivity in the epithelial component. The mesenchymal component showed varied differentiation, including osseous and rhabdomyoblastic differentiation. The primitive neuroepithelial element showed positivity for cluster of differentiation 99 (CD99) and synaptophysin IHC.
Conclusion: Sinonasal teratocarcinosarcoma (SNTCS) is often underdiagnosed because of the rarity of this tumor and its histomorphological overlap with a wide range of sinonasal malignancies. A careful morphological assessment of the biopsy sample and a battery of ancillary investigations, including IHC, confirm the diagnosis.
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. DOI: 10.1002/1097-0142(19840515)53:10<2140::aid-cncr2820531025>3.0.co;2-y
Misra P, Husain Q, Svider PF, et al. Management of sinonasal teratocarcinosarcoma: a systematic review. Am J Otolaryngol 2014;35(1):5–11. DOI: 10.1016/j.amjoto.2013.04.010
Contrera KJ, Woody NM, Rahman M, et al. Clinical management of emerging sinonasal malignancies. Head Neck 2020;42(2):2202–2212. DOI: 10.1002/hed.26150
Smith SL, Hessel AC, Luna MA, et al. Sinonasal teratocarcinosarcoma of the head and neck: a report of 10 patients treated at a single institution and comparison with reported series. Arch Otolaryngol Head Neck Surg 2008;134(6):592–595. DOI: 10.1001/archotol.134.6.592
Thompson L. World Health Organization classification of tumors: pathology and genetics of head and neck tumors. Ear Nose Throat J 2006;85(2):74. PMID: 16579185.
Chapurin N, Totten DJ, Morse JC, et al. Treatment of sinonasal teratocarcinosarcoma: a systematic review and survival analysis. Am J Rhinol Allergy 2021;35(1):132–141. DOI: 10.1177/1945892420959585
Agrawal N, Chintagumpala M, Hicks J, et al. Sinonasal teratocarcinosarcoma in an adolescent male. J Pediatr Hematol Oncol 2012;34(7):e304–e307. DOI: 10.1097/MPH.0b013e318266baa8
Budrukkar A, Agarwal JP, Kane S, et al. Management and clinical outcome of sinonasal teratocarcinosarcoma: single-institution experience. J Laryngol Otol 2010;124(7):739–743. DOI: 10.1017/S0022215109992866
Rotenberg B, El-Hakim H, Lodha A, et al. Nasopharyngeal teratocarcinosarcoma. Int J Pediatr Otorhinolaryngol 2002;62(2):159–164. DOI: 10.1016/s0165-5876(01)00575-4
Wei S, Carroll W, Lazenby A, et al. Sinonasal teratocarcinosarcoma; report of a case with review of literature and treatment outcomes. Ann Diagn Pathol 2008;12(6):415–425. DOI: 10.1016/j.anndiagpath.2007.05.003
Pai SA, Naresh KN, Masih K, et al. Teratocarcinosarcoma of the paranasal sinuses: a clinicopathologic and immunohistochemical study. Hum Pathol 1998;29(7):718–722. DOI: 10.1016/s0046-8177(98)90281-7
Ling FT, Gerin-Lajoie J, Wang D. Sinonasal teratocarcinosarcoma. J Otolaryngol 2004;33(3):203–206. DOI: 10.2310/7070.2004.03012
Shimazaki H, Aida S, Tamai S, et al. Sinonasal teratocarcinosarcoma: ultrastructural and immunohistochemical evidence of neuroectodermal origin. Ultrastruct Pathol 2000;24(2):115–122. DOI: 10.1080/01913120050118602
Thomas J, Adegboyega P, Iloabachie K, et al. Sinonasal teratocarcinosarcoma with yolk sac elements: a neoplasm of somatic or germ cell origin? Ann Diagn Pathol. 2011 Apr;15(2):135–139. DOI: 10.1016/j.anndiagpath.2010.01.004
Vranic S, Caughron SK, Djuricic S, et al. Hamartomas, teratomas and teratocarcinosarcomas of the head and neck: report of 3 new cases with clinico-pathologic correlation, cytogenetic analysis, and review of the literature. BMC Ear Nose Throat Disord 2008;8:8. DOI: 10.1186/1472-6815-8-8
Birkeland AC, Burgin SJ, Yanik M, et al. Pathogenetic analysis of sinonasal teratocarcinosarcomas reveal actionable β-catenin overexpression and a β-catenin mutation. J Neurol Surg B Skull Base 2017;78(4):346–352. DOI: 10.1055/s-0037-1601320
Rooper LM, Uddin N, Gagan J, et al. Recurrent Loss of SMARCA4 in Sinonasal Teratocarcinosarcoma. Am J Surg Pathol 2020;44(10):1331–1339. DOI: 10.1097/PAS.0000000000001508
Fernandez PL, Cardesa A, Alos L, et al. Sinonasal teratocarcinosarcoma: an unusual neoplasm. Pathol Res Pract 1995;191(2):166–171; discussion 172–173. DOI: 10.1016/S0344-0338(11)80567-4
Ogawa T, Ikeda K, Watanabe M, et al. A case report of sinonasal teratocarcinosarcoma. Tohoku J Exp Med 2000;190(1):51–59. DOI: 10.1620/tjem.190.51
Takasaki K, Sakihama N, Takahashi H. A case with sinonasal teratocarcinosarcoma in the nasal cavity and ethmoid sinus. Eur Arch Otorhinolaryngol 2006;263(6):586–591. DOI: 10.1007/s00405-006-0014-1
Fatima SS, Minhas K, Din NU, et al. Sinonasal teratocarcinosarcoma: a clinicopathologic and immunohistochemical study of 6 cases. Ann Diagn Pathol 2013;17(4):313–318. DOI: 10.1016/j.anndiagpath.2013.01.003
Leelamma JP, Mohan BP, Srinivasan A. Sinonasal teratocarcinosarcoma- a rare tumour not so rarely misdiagnosed. Iran J Pathol 2018;13(1):85–88. PMID: 29731800.
Jin W, Teng Y, Zhao P, et al. Sinonasal teratocarcinosarcoma masquerading as olfactory neuroblastoma. Int J Clin Exp Pathol 2018;11(2):910–915. PMID: 31938183.
Compton ML, Lewis JS Jr, Faquin WC, et al. SALL-4 and beta-catenin expression in sinonasal teratocarcinosarcoma. Head Neck Pathol 2022;16(1):229–235. DOI: 10.1007/s12105-021-01343-3
Tchoyoson Lim C C, Thiagarajan A, Sim C S, et al. Craniospinal dissemination in teratocarcinosarcoma. J Neurosurg 2008;109(2):321–324. DOI: 10.3171/JNS/2008/109/8/0321
Carrizo F, Pineda-Daboin K, Neto AG, et al. Pharyngeal teratocarcinosarcoma: a review of the literature and report of two cases. Ann Diagn Pathol 2006;10(6):339–342. DOI: 10.1016/j.anndiagpath.2006.03.006
Naito T, Umemura S, Nakamura H, et al. Successful treatment with nivolumab for SMARCA4-deficient non-small cell lung carcinoma with a high tumor mutation burden: a case report. Thorac Cancer 2019;10(5):1285–1288. DOI: 10.1111/1759-7714.13070
Zaman GJR, de Roos JADM, Libouban MAA, et al. TTK inhibitors as a targeted therapy for CTNNB1(β-catenin) mutant cancers. Mol Cancer Ther 2017;16(11):2609–2617. DOI: 10.1158/1535-7163.MCT-17-0342