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].