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\begin{document}

\title{A Rare Case of Sinonasal Sarcoma with Intracranial Extension: a Case
Report}



\author[1]{Ahmed   Hassan Ashfaq}%
\author[1]{Muhammad   Arshad }%
\author[1]{Nida Riaz}%
\author[1]{Salman Aslam}%
\author[2]{Faizan Fazal}%
\author[1]{Shahzaib Maqbool }%
\affil[1]{Benazir Bhutto Hospital}%
\affil[2]{Rawalpindi Medical University}%


\vspace{-1em}



  
  \date{June 10, 2024}


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\sloppy


\textbf{A rare case of Sinonasal sarcoma with intracranial extension: a
case report}

\textbf{Abstract:}

Herein, we are reporting a rare case of sinonasal sarcoma with
intracranial extension. Patient presented with complaints of nasal
obstruction and epistaxis. The investigations including
histopathological analysis were suggesting a highly aggressive sinonasal
sarcoma with intracranial extension also involving the internal carotid
artery in circumferential manner. Tumor was unresectable due to extent
of invasion and patient was referred to radiotherapy department for
reduction of tumor size with radiotherapy.

\textbf{Keywords:}

Sinonasal sarcoma, Intracranial extension, Spindle cells, Fascicular
pattern.

\textbf{Introduction:}

Malignant spindle cell tumors account for 1\% of the total head and neck
cancers and of all 5-10\% are classified as sarcomas in adult population
{[}1{]}. According to World Health Organization (WHO) classification in
2017, the head and neck sarcomas are divided into eight histological
subtypes: undifferentiated pleomorphic sarcoma, fibrosarcoma,
angiosarcoma and malignant peripheral nerve sheath tumor (MPNST)
{[}1{]}. Due to diverse histopathology variants, the diagnosis of head
and neck sarcomas poses significant challenges towards diagnosis and
management. Sinonasal sarcoma is one of the unique tumor now presented
by WHO and has been added in the classification of head and neck
malignancies recently {[}2{]}.

Herein we are reporting a rare case of sinonasal sarcoma in 38-years old
female presented to department of otolaryngology and head and neck
surgery, Benazir Bhutto Hospital, Rawalpindi.

\textbf{Case Report:}

A 38-years old female presented to Outpatient department of
otolaryngology and head and neck surgery, Benazir Bhutto Hospital,
Rawalpindi with complaints of bilateral complete severe nasal
obstruction from 3 months and intermittent epistaxis from 3 days.
Patient also has history right sided nasal obstruction 13 years back
with epistaxis for which intranasal polypectomy was done and symptoms of
nasal obstruction and epistaxis were resolved. But now patient again
presented with symptoms of B/L nasal obstruction and epistaxis. At
presentation patient was vitally stable and laboratory analysis was also
normal with no significant abnormalities except low hemoglobin (8.1
g/dl) for which 3 pints of transfusion was done with improvement in
hemoglobin levels.

On external nasal examination a deformity (expanded vestibule was
observed) with increased inter-canthal and inter-pupillary distance but
Normal eye movement and reflexes, on anterior rhinoscopy B/L completely
obstructing nasal mass was observed that was bleeding with probing. On
palpation, the mass was painful and tender sinuses were present. Nasal
patency was absent bilaterally with anosmia bilaterally. On probing mass
originating from lateral aspect of nasal cavity was observed. No
neurological dysfunction was present at the time of presentation.
Posterior rhinoscopy was insignificant and there were no significant
findings in throat and ear examination. Cervical level II lymph nodes on
left side of the neck were palpable.

On X ray (water's view) of nose and paranasal sinuses, homogeneous haze
in B/L maxillary and frontal sinuses and nasal cavity was observed
suggesting polypoidal mass formation as shown in \textbf{figure 1.} On
CECT aggressive looking polypoidal heterogeneously enhancing soft tissue
density mass measuring 9x6.3x5.6cm involving nasal cavity and all
paranasal sinuses causing their expansion, obliteration, and bony
erosions with intracranial extension was observed as shown
in\textbf{figure 2} . MRI scan was suggesting a highly neoplastic mass
with bony erosions with intracranial extension and involving right
cavernous sinus as well. Tumour was also encircling the internal carotid
artery covering 90\% of its circumstance validating the unresectable
mass with intracranial extension. On incisional biopsy, microscopy
showed low grade spindle cell proliferation in fascicular herring bone
pattern suggesting sinonasal sarcoma as shown in \textbf{figure 3.} On
immunohistochemistry analysis, S-100 was positive.

After all baseline and specific investigation and work up a final
diagnosis of sinonasal sarcoma was made and it was labelled as
unresectable mass due to intracranial extension and vascular invasions
and patient was referred for radiotherapy for reduction in mass volume.\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image1/image1}

\end{center}
\end{figure}

\textbf{Figure 1: Showing the X-ray PNS (Waters' view) that is
delineating the complete obstruction of nasal cavity and maxillary
sinuses with sinonasal sarcoma.}\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image2/image2}

\end{center}
\end{figure}

\textbf{Figure 2: Showing the presence of mass completely obstructing
sinonasal cavities and with bony erosions and aggressive looking
spread.}\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image3/image3}

\end{center}
\end{figure}\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image4/image4}

\end{center}
\end{figure}\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image5/image5}

\end{center}
\end{figure}\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image6/image6}

\end{center}
\end{figure}

\textbf{Figure 3: Histological presentation of biopsied sample depicting
fascicles of low grade, monotonous spindle cell proliferation in
herringbone pattern suggesting sinonasal sarcoma. (H\&E).}

\textbf{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{]}.

\textbf{Conclusion:}

It was a rare case of sinonasal sarcoma of aggressive nature that was
completely occupying nasal cavity and paranasal sinuses with bony
erosions. CT scan and MRI was suggesting a highly aggressive mass with
intracranial extension (extrdural). The mass was also abutting the
internal carotid artery with its 90\% circumference and was labelled as
an unresectable mass and patient was referred for radiotherapy.

\textbf{Conflict of interest:}

Authors have declared that they have no conflict of interest to
disclose.

\textbf{Financial statement:}

None

\textbf{Data sharing statement:}

The relevant data of this manuscript is given in conclusion.

\textbf{References:}

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