Biphenotypic Sinonasal Sarcoma: A Case Report and Review of Literature

Abstract

Objectives: Biphenotypic sinonasal sarcoma (BSNS), previously low-grade sinonasal sarcoma with neural and myogenic features, is a rare tumor of the sinonasal tract first described in 2012. Due to its rarity, limited literature is available in providing clinicians with a standardized treatment regimen, particularly in cases of positive surgical margins. This article aims to provide a clinical review of the currently available reported cases of BSNS, as well as presenting clinical, radiologic, and pathologic details of 2 novel cases. Methods: Online electronic databases include PubMed and Embase where queried for reports of biphenotypic sinonasal sarcoma or low-grade sinonasal sarcoma with neural and myogenic features. Two previously unpublished cases were included in the results. Data including clinical presentation, epidemiologic data, radiologic evaluation, intraoperative details, histopathology, treatment modality, and postoperative follow-up information were included. Results: A total of 100 previously published cases were identified in 12 prior articles. Mean age at presentation was 52.9 years. Extrasinonasal extension was observed in 27.4% of cases with most common site of extension being cribriform plate. Forty-seven cases included treatment details with surgical excision being the most common modality. Recurrence rates were identical for both surgical excision alone and surgical excision with adjuvant radiotherapy (33.3%). Conclusions: Biphenotypic sinonasal sarcoma is a slow-growing tumor that is amenable to surgical resection. Recurrence rates are similar between surgical excision and surgical excision with adjuvant radiation therapy, but limited data in reported cases preclude a determination of treatment superiority.

Biphenotypic Sinonasal Sarcoma: A Genetically Confirmed Case Showing Bone Invasion Accompanying a Non-neoplastic Respiratory Epithelium

“…Although some previous reports mentioned that β-catenin positivity in immunohistochemistry is useful for biphenotypic sinonasal sarcoma diagnosis, with 54% to 83.3% sensitivity, 11,12 the present case was negative for this marker. In such cases, the detection of fusion genes can be a diagnostic aid.…”

“…Local recurrence has been reported in approximately 30% of cases with no distant metastasis. 12,14 The patient in the present case was treated using surgery alone without adjuvant therapy, and the patient was free from disease 5 years after surgery.…”

A Rare Sinonasal Malignancy: Biphenotypic Sinonasal Sarcoma

“…The treatment approach is the surgical excision of the lesion. In a review published by Kominsky et al ( 3 ) in 2021, recurrence rates were found comparable between patients who underwent surgical excision only and patients who received radiotherapy after surgical excision. Therefore, the benefit of radiotherapy can be said to be controversial.…”

“…The rarity of the disease and the small number of cases described in the literature limit the assessment of treatment efficacy and more data is needed. Postoperative radiotherapy and chemotherapy options should be decided by discussing the risks and possible outcomes with the patient ( 3 ).…”

“…Patients usually present with non-specific symptoms related to the mass effect of the tumor, such as nasal obstruction, nasal congestion, recurrent sinusitis attacks, epistaxis, facial pain, and pressure sensation. Ophthalmologic symptoms such as diplopia, proptosis, and blurred vision can also be observed in patients with orbital spread ( 2 , 3 ).…”