Tomotherapy for sinonasal teratocarcinosarcoma with SMARCA4 deletion and CTNNB1 mutation: A case report and literature review

Abstract

Sinonasal teratocarcinosarcoma (SNTCS) is a rare and aggressive malignant tumor that is difficult to diagnose and lacks a standard therapeutic strategies. Here, we report a case of SNTCS harboring a SMARCA4 deletion and a CTNNB1 mutation. Following incomplete surgical resection, the patient received synchronous postoperative chemotherapy with tomotherapy (TOMO) radiation and achieved a complete response lasting for > 47 months with tolerated side effects. This case suggests that SMARCA4 deletion and CTNNB1 mutation may be key molecular events in the development of SNTCS, potentially providing valuable clues for its diagnosis. Moreover, to the best of our knowledge, this is the first case demonstrating that TOMO technology can be a beneficial option for SNTCS radiotherapy.

Keywords

CTNNB1 mutation / radiotherapy / sinonasal teratocarcinosarcoma / SMARCA4 deletion / tomotherapy

References

[1] Misra P, Husain Q, Svider PF, Sanghvi S, Liu JK, Eloy JA. Management of sinonasal teratocarcinosarcoma: a systematic review. Am J Otolaryngol. 2014;35(1):5-11.

[2] Su YY, Friedman M, Huang CC, Wilson M, Lin HC. Sinonasal teratocarcinosarcoma. Am J Otolaryngol. 2010;31(4):300-303.

[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] Rooper LM, Agaimy A, Gagan J, et al. Comprehensive Molecular Profiling of Sinonasal Teratocarcinosarcoma Highlights Recurrent SMARCA4 Inactivation and CTNNB1 Mutations. Am J Surg Pathol. 2023;47(2):224-233.

[5] Rooper LM, Uddin N, Gagan J, et al. Recurrent Loss of SMARCA4 in Sinonasal Teratocarcinosarcoma. Am J Surg Pathol. 2020;44(10):1331-1339.

[6] 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.

[7] Contrera KJ, Woody NM, Rahman M, Sindwani R, Burkey BB. Clinical management of emerging sinonasal malignancies. Head Neck. 2020;42(8):2202-2212.

[8] Sharma HS, Abdullah JM, Othman NH, Muhamad M. Teratocarcinosarcoma of the nasal cavity and ethmoid. J Laryngol Otol. 1998;112(7):682-686.

[9] Wassef SN, Kapur P, Barnett SL, Myers LL. Sinonasal teratocarcinosarcoma with intracranial extension: case report and literature review. Ear Nose Throat J. 2012;91(12):536-539.

[10] Yoon SY, Park KS, Hwang JH, Park SH, Han MH. Sinonasal Teratocarcinosarcoma, a Rare Tumor Involving Both the Nasal Cavity and the Cranial Cavity. Brain Tumor Res Treat. 2020;8(1):57-61.

[11] 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.

[12] Sobani ZA, Akhtar S, Junaid M, Salahuddin I. Sinonasal teratocarcinosarcoma. J Pak Med Assoc. 2012;62(6):633-635.

[13] Morin PJ, Sparks AB, Korinek V, et al. Activation of beta-catenin-Tcf signaling in colon cancer by mutations in beta-catenin or APC. Science. 1997;275(5307):1787-1790.

[14] Zhang X, Wang L, Qu Y. Targeting the β-catenin signaling for cancer therapy. Pharmacol Res. 2020;160:104794.

[15] Zhang Y, Wang X. Targeting the Wnt/β-catenin signaling pathway in cancer. J Hematol Oncol. 2020;13(1):165.

[16] Dev A, Vachher M, Prasad CP. β-catenin inhibitors in cancer therapeutics: intricacies and way forward. Bioengineered. 2023;14(1):2251696.

[17] Zhao H, Ming T, Tang S, et al. Wnt signaling in colorectal cancer: pathogenic role and therapeutic target. Mol Cancer. 2022;21(1):144.

[18] 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.

[19] Wang Q, Qin J, Cao R, et al. Comparison of Dosimetric Benefits of Three Precise Radiotherapy Techniques in Nasopharyngeal Carcinoma Patients Using a Priority-Classified Plan Optimization Model. Front Oncol. 2021;11:646584.

[20] Feliciani G, Licciardello T, Guidi C, et al. Comparison of HDR-brachytherapy and tomotherapy for the treatment of non-melanoma skin cancers of the head and neck. Radiother Oncol. 2025;204:110703.

[21] Zhang Y, Rong L, Wang Z, Zhao H. The top 100 most cited articles in helical tomotherapy: a scoping review. Front Oncol. 2023;13:1274290.

[22] Chuang EY, Hou PY, Shueng PW, et al. Improved quality of life in head and neck cancer patients treated with modern arc radiotherapy techniques - A prospective longitudinal analysis. Front Oncol. 2024;14:1424034.

[23] Peng G, Ke Y, Wang T, Feng Y, Li Y, Wu G. Intensity-modulated radiotherapy for sinonasal teratocarcinosarcoma. J Huazhong Univ Sci Technolog Med Sci. 2011;31(6):857-860.

RIGHTS & PERMISSIONS

2025 The Author(s). Precision Radiation Oncology published by John Wiley & Sons Australia, Ltd on behalf of Shandong Cancer Hospital & Institute.