Discussion:
Sinonasal sarcoma is described as a low grade carcinoma with neural and myogenic differentiation, that initially came into literature in 2012 with the efforts of Lewis et al [3]. The sarcomas of head and neck are rare and only accounting for 10% or less of overall head and neck malignancies [4]. The head and neck sarcomas are mostly originate from soft tissue accounting for about 80% of the cases and rest of the 20% belongs to either bone or cartilage related sarcomas [4]. The sarcoma arising from sinonasal cavities is rarely observed location for such kind of low grade sarcomas. The etiological association of sinonasal sarcoma is not clearly elucidated in the literature, however; the involvement of genetic factors is of paramount significance in the development of sinonasal sarcoma [5]. Association of sinonasal sarcoma with other diseases like, TP53 mutation, Gardner syndrome, Li-Fraumeni syndrome, tuberous sclerosis, Neurofibromatosis, and irradiation for pre-existing cancer and Epstein-Barr virus infection, have been observed in many studies [6].
The signs and symptoms of sinonasal sarcoma can vary depending on the location and size of the tumor. Common symptoms include nasal obstruction, congestion, epistaxis (nosebleeds), facial pain or pressure, and sinusitis-like symptoms such as facial swelling, headache, and nasal discharge. Other potential symptoms include vision changes, double vision, eye pain, and proptosis (bulging of the eye) [7][8]. In some cases, sinonasal sarcoma may also cause neurological symptoms such as numbness or weakness in the face, mouth, or tongue, or difficulty swallowing or speaking [8]. Because these symptoms are non-specific and can also be caused by other conditions such as sinusitis, they can sometimes lead to a delay in diagnosis. However, if a sinonasal sarcoma is suspected, further testing such as imaging studies and biopsy can help to confirm the diagnosis [9].
Detailed investigations like CT scan, MRI and PET can provide information of paramount significance in terms of size and location of the tumor, as well as whether it has spread to other parts of the body [10]. In addition, newer imaging techniques such as diffusion-weighted MRI and dynamic contrast-enhanced MRI may be useful for evaluating the extent of tumor invasion and vascularity [11]. Biopsy is the definitive diagnostic test for sinonasal sarcoma. A tissue sample is taken from the tumor and examined under a microscope to confirm the presence of cancer cells and determine the specific type of sarcoma [10]. Endoscopy may be used to visualize the tumor and obtain a tissue sample for biopsy [12]. In addition, advanced endoscopic techniques such as fluorescence-guided endoscopy may help to improve the accuracy of tumor detection and delineation [13].
The optimal treatment for sinonasal sarcoma depends on various factors such as the type and stage of the tumor, as well as the patient’s overall health and preferences. Surgery is the mainstay of treatment for sinonasal sarcoma, and the goal is to remove the entire tumor with negative margins. Depending on the location and extent of the tumor, different surgical approaches such as endoscopic resection, open craniofacial resection, or a combination of both may be used [14]. Adjuvant radiation therapy may also be given after surgery to reduce the risk of recurrence [15]. Radiation therapy may also be used as the primary treatment for sinonasal sarcoma in some cases, particularly for tumors that are not amenable to surgery or for patients who are not candidates for surgery due to advanced age or comorbidities. External beam radiation therapy and intensity-modulated radiation therapy (IMRT) are commonly used techniques [16]. However, radiation therapy alone may not be curative in all cases, and some patients may require additional surgery or systemic therapy [17]. Chemotherapy is generally not considered a standard treatment for sinonasal sarcoma due to its limited effectiveness, although it may be used in certain situations such as for unresectable or metastatic tumors. Some studies have shown promising results with targeted therapies such as tyrosine kinase inhibitors, which are drugs that block the signals that promote tumor growth [18].