Brain Tumor Res Treat.  2020 Apr;8(1):57-61. 10.14791/btrt.2020.8.e2.

Sinonasal Teratocarcinosarcoma, a Rare Tumor Involving Boththe Nasal Cavity and the Cranial Cavity

• Yoon SY
• Park KS
• Hwang JH
• Park SH
• Han MH

• KMID: 2500123
• DOI: http://doi.org/10.14791/btrt.2020.8.e2

Abstract

Sinonasal teratocarcinosarcoma (SNTCS), a very rare tumor, is known to be a heterogeneous with epithelial, mesenchymal, and neuroepithelial components and shows a very aggressive clinical course. Due to the heterogeneity of this tumor, it is often misdiagnosed. No definitive treatment modality has been reported because it is a very rare tumor. A 44-year-old man presented to a rhinologist with headache and nasal obstruction, and an intranasal tumor was found that invaded into the cranial cavity. He underwent combined surgery with a rhinologist and a neurosurgeon following cognitive decline that worsened after a transnasal biopsy. The patient was diagnosed with SNTCS and underwent radiotherapy. However, residual tumor was found during radiotherapy and additional chemotherapy was administered. Follow-up brain MRI revealed no remnant or recurrent lesion. SNTCS is a tumor that has not yet been well researched and should be further investigated for proper treatment.

Keyword

Teratocarcinosarcoma; Brain tumor; Chemotherapy; Radiotherapy

Figure

• 

  Fig. 1 Brain MRI with gadolinium enhancement shows large mass involving right nasal cavity with marked intracranial extension.

• 

  Fig. 2 Brain CT enhancement shows an intracranial extended nasal mass with erosion of cribriform plate.

• 

  Fig. 3 Preoperative transnasal endoscopy shows purplish nodular mass.

• 

  Fig. 4 Postoperative MRI with gadolinium enhancement shows gross total mass removal.

• 

  Fig. 5 Histopathological finding with hematoxylin and eosin staining. A: Microscopically, epithelial component and mesenchymal components are shown in neurofibrillary background (original magnification ×100). B: Stratified squamous epithelium with clear cell change are admixed with malignant spindle cells (original magnification ×200). C: Adenocarcinoma is surrounded by immature neuroepithelial cells (original magnification ×200). D: Malignant spindle cells with myxoid stroma about the adenocarcinoma (original magnification ×200).

• 

  Fig. 6 Histopathological finding with immunohistochemistry. A: Epithelial component is diffuse positive for cytokeratin (original magnification ×100). B, C: Immature neuroepithelial cells are positive for chromogranin and synaptophysin (original magnification ×200).

Reference

1. El-Naggar AK, Chan JKC, Grandis JR, Takata T, Slootweg PJ. WHO classification of head and neck tumours. 4th ed. Lyon: International Agency for Research on Cancer;2017. p. 267.

2. Heffner DK, Hyams VJ. Teratocarcinosarcoma (malignant teratoma?) of the nasal cavity and paranasal sinuses a clinicopathologic study of 20 cases. Cancer. 1984; 53:2140–2154. PMID: 6322958.

3. Misra P, Husain Q, Svider PF, Sanghvi S, Liu JK, Eloy JA. Management of sinonasal teratocarcinosarcoma: a systematic review. Am J Otolaryngol. 2014; 35:5–11. PMID: 23731851.

4. 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; 12:415–425. PMID: 18995206.

5. Yang S, Sun R, Liang J, Zhou Z, Zhou J, Rui J. Sinonasal teratocarcinosarcoma: a clinical and pathological analysis. Int J Surg Pathol. 2013; 21:37–43. PMID: 22923779.

6. Sweety Vijay S, Kumar TD, Srikant B, Vithal SH, Vijay KS, Gurunath P. Intracranial presentation of teratocarcinosarcoma. J Clin Neurosci. 2010; 17:1347–1349. PMID: 20655234.

7. Kim JH, Maeng YH, Lee JS, Jung S, Lim SC, Lee MC. Sinonasal teratocarcinosarcoma with rhabdoid features. Pathol Int. 2011; 61:762–767. PMID: 22126386.

8. Nitsche M, Hermann RM, Christiansen H, Berger J, Pradier O. Rationale for individualized therapy in Sinonasal Teratocarcinosarcoma (SNTC): case report. Onkologie. 2005; 28:653–656. PMID: 16330889.

9. Casali PG, Abecassis N, Bauer S, et al. Soft tissue and visceral sarcomas: ESMO-EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2018; 29 Suppl 4:iv51–iv67.

10. 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. PMID: 19025657.

11. Carrizo F, Pineda-Daboin K, Neto AG, Luna MA. Pharyngeal teratocarcinosarcoma: review of the literature and report of two cases. Ann Diagn Pathol. 2006; 10:339–342. PMID: 17126251.