Sinonasal Sarcomatoid Carcinoma: A Case Report

Introduction: Sarcomatoid carcinomas are biphasic tumors displaying epithelial and mesenchymal elements. It has been reported to arise from several sites throughout the body, but head and neck involvement is rare. There are only a few case reports of involvement of the nasal cavity. To our knowledge, this is the first report of sarcomatoid carcinoma arising from within the sphenoid sinus.

Case Presentation: A 26-year-old male was referred for evaluation of a nasal mass. He complained of a 6-week history of severe bilateral nasal obstruction, accompanied by epistaxis, nasal drainage, and facial pressure. His surgical history is significant for septoplasty and inferior turbinate reduction 1 year prior for nasal obstruction. His medical, social, and family history is otherwise noncontributory. His initial CT scan ([Fig. 1]) revealed a large mass emanating from the right sphenoethmoidal recess extending into the nasopharynx. The skull base overlying the mass appeared thin, there was no obvious orbital erosion, and no expansion of the pterygopalatine fossa. Preoperative MRI also confirmed these results with no obvious intraorbital or intracranial extension.

Intraoperatively, the mass was soft and pedicled in two areas: the planum sphenoidale and along the Vidian canal. These areas had hyperostotic bone which was drilled down achieve gross total resection. Histological evaluation showed a cellular myxoid and spindle cell lesion permeating the submucosa in a variably “loose and dense” pattern. Mitotic activity is numerous with strong expression of pankeratin, and negative for all other markers including CD34, CD31, β-catenin, Sox10, S-100 protein, desmin, myogenin, EMA, and GFAP. The diagnosis of sarcomatoid carcinoma was made given its foci of strong pancytokeratin expression.

A PET/CT did not reveal evidence of metastatic disease. A postoperative MRI revealed enhancement in the right Vidian canal ([Fig. 2]). The case was presented at the interdisciplinary tumor board and the recommendation was made for resection of the area of enhancement for gross total resection and adjuvant chemoradiation. Six weeks after the initial surgery, the patient was taken back to the operating room for dissection of the infratemporal fossa with sacrifice of the Vidian nerve ([Fig. 3]). Final pathology demonstrated no evidence of tumor within the nerve. He completed two cycles of high-dose cisplatin but developed tinnitus and did not receive the third dose. He completed 6 weeks of external beam radiotherapy.

Discussion: Sarcomatoid carcinomas are a dedifferentiated form of conventional squamous cell carcinomas and have been known by other nomenclature including carcinosarcoma, spindle cell squamous carcinoma, pseudosarcoma, and pleomorphic carcinoma. Patients typically present in the fifth to sixth decade of life, with a 3.7:1 predilection for males. Due to the rarity of the disease, there is no specific treatment protocol in place and variations of surgery, chemotherapy, and radiation therapy are described. After presentation at the interdisciplinary tumor board, the decision was made to pursue trimodal therapy with gross total resection and adjuvant chemoradiation. The patient has no evidence of local or distant recurrence to date.

Fig. 1

Fig. 2

Fig. 3 Vidian nerve with clip, coursing through the Vidian canal.

Publication History

Article published online:
12 February 2021