Sinonasal Teratocarcinosarcoma: A Case Report of a Rare Entity

Introduction: Sinonasal teratocarcinosarcoma (SNTCS) is a rare, highly aggressive malignancy that arises in the paranasal sinuses. The diagnosis requires pathological examination that reveals malignant epithelial components along with at least two malignant mesenchymal tissues types, such as cartilage, bone, muscle, or fibroblasts. Due to the rarity of this tumor and the heterogenicity of the tissue sample, diagnosis of SNTCS can be challenging.

Case Presentation: A 70-year-old female presented to the emergency department with one day of rapid onset, progressive right vision loss associated with severe headache and vomiting. She also complained of several months of left-sided nasal obstruction. Nasal endoscopy revealed a large mass with pushing borders within the left nasal cavity. No lymphadenopathy was appreciated. CT and MRI revealed an invasive sinonasal mass involving the septum, cribriform plate, lamina papyracea, and orbital apex with right optic nerve compression from mass effect ([Fig. 1]). Preoperative ophthalmologic exam revealed a right relative afferent pupillary defect and only light perception on visual acuity testing. The patient was taken emergently to the operating room for a planned partial resection of tumor to decompress the orbital apex. On postoperative day 1, the patient had improvement in visual acuity with eventual return to normal vision. Residual tumor along the skull base was resected in a staged manner one week from the initial resection. Skull base reconstruction was performed in a multi-layered fashion using tensor fascia lata, nasal septal bone, and bilateral nasoseptal mucosal flaps. She is scheduled to receive adjuvant chemoradiation at the recommendation of the multidisciplinary tumor board.

Pathological examination displays a carcinomatous component of focal squamous differentiation with keratinization ([Fig. 2]), clusters of glandular formations, cells with eosinophilic cytoplasm and high-grade, enlarged central nuclei ([Fig. 3]), and immunohistochemical staining for synaptophysin consistent with rhabdomyosarcomatous differentiation. Immunochemical testing for nuclear protein in testis (NUT) carcinoma was negative. The diagnosis of sinonasal teratocarcinosarcoma was confirmed.

Discussion: Sinonasal teratocarcinosarcoma (SNTCS) is a rare and highly malignant sinonasal tumor that exhibit epithelial, mesenchymal, and neural tissue components. SNTCS is more common in adults, with an average age reported between 50–54.5 years old and male patients with a strong male predominance (83–87%). The most common clinical symptoms are nasal obstruction and recurrent epistaxis. Only 11 other cases of SNTCS have been reported to present with ocular manifestations, and only 8 cases reported involving the anterior cranial fossa, as in this case.

The prognosis of SNTCS is poor, even with multimodal treatment. Surgical resection with adjuvant radiotherapy is usually recommended with or without chemotherapy. It remains unclear whether addition of adjuvant chemotherapy significantly prolongs survival compared with surgery and radiation. Chapurin et al. demonstrated a two-year survival rate of 55% with a recurrence rate of 38%.

Fig. 1 T1 Post-contrast MRI: mass involving bilateral posterior nasal cavity, cribriform plate, and right orbital apex.

Fig. 2 Focal squamous differentiation with keratinization.

Fig. 3 Round to polygonal tumor cells with eosinophilic cytoplasm and high-grade, enlarged central nuclei, consistent with rhabdomyosarcomatous differentiation.

Publication History

Article published online:
15 February 2022