International Journal of Surgery Case Reports 114 (2024) 109160

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International Journal of Surgery Case Reports

journal homepage: www.elsevier.com/locate/ijscr

Case report

A rare nasal fibrosarcoma: A case report

Rina Masadah a, b, *, Nani Iriani Jufri b, c, Desy Ekamadayani Ahmad d, Muhammad Faruk e
a
Department of Pathology Anatomy, Faculty of Medicine, Hasanuddin University – Hasanuddin University Hospital, Makassar, Indonesia
b
Siloam Hospital, Makassar, Indonesia
c
Department of Otorhinolaryngology and Head and Neck Surgery, Faculty of Medicine, Hasanuddin University, Makassar, Indonesia
d
Department of Pathology Anatomy, Faculty of Medicine, Hasanuddin University, Makassar, Indonesia
e
Department of Surgery, Faculty of Medicine, Hasanuddin University – Hasanuddin University Hospital, Makassar, Indonesia

A R T I C L E I N F O A B S T R A C T

Keywords: Introduction: Fibrosarcoma is a malignant neoplasm, with nasal localization uncommon. Clinically, the main
Fibrosarcoma symptoms are epistaxis and nasal obstruction. Microscopically, it is characterized by a dense proliferation of
Epistaxis fibroblasts with a herringbone pattern and spindle-shaped cells with hyperchromatic nuclei.
Nasal obstruction
Presentation of case: We report a nasal fibrosarcoma of a 37-year-old woman with a recurrent history of epistaxis
Nasal cavity
Immunohistochemistry
and nasal obstruction for several years. A biopsy was performed, and the histopathological findings reported a
Case report dense proliferation of fibroblasts with a herringbone pattern and spindle-shaped cells with hyperchromatic
nuclei. The immunohistochemistry was positive for Ki67, P53, and vimentin staining. The final diagnosis was
nasal fibrosarcoma. The patient underwent functional endoscopic sinus surgery and extirpation of the tumor on
the wall of the right maxilla, right ethmoid sinus, and right frontal area. Thirteen months later, the patient
experienced a recurrence. An external approach was taken under general anesthesia via lateral rhinotomy with
medial maxillectomy. The patient underwent chemotherapy with a regimen of paclitaxel and cisplatin for six
cycles.
Discussion: We gave chemotherapy in this case because the patient experienced a recurrence, tumor-free margins
were not obtained, and a computed tomography scan showed bone involvement.
Conclusion: Nasal fibrosarcoma is a rare malignant neoplasm, which can be diagnosed by its histopathological
features and immunohistochemistry.

1. Introduction
Fibrosarcoma is a malignant neoplasm, with the main predilection
site being the extremities. Among all soft tissue sarcomas of the head and
neck, fibrosarcoma accounts for only 7–10 %. [1] The main symptoms
are epistaxis and nasal obstruction, which can occasionally be mis­
diagnosed as papillomas. [2] Rather than the mucosal connective tissue,
the periosteum appears to be the tissue origin of nasal fibrosarcoma. [3]
The etiology of fibrosarcoma is not completely understood. Several
factors are thought to be involved, such as radiation, trauma, and other
bone disease. [4] Nasal fibrosarcoma can be diagnosed by its histo­
pathological features and immunohistochemistry.
We report a 37-year-old woman with a rare nasal fibrosarcoma. To
our knowledge, this is the first report of nasal fibrosarcoma from
Indonesia. This report aligns with the updated consensus-based surgical
case report (SCARE) guidelines. [5]
2. Presentation of case
A 37-year-old Mandarese woman was referred to our hospital with a
complaint of recurrent epistaxis and nasal obstruction for several years.
She had no history of other disease, trauma, or radiation treatment. She
reported no consumption of tobacco or alcohol and no family history of
cancer. On clinical nasal endoscopic examination, lacerations and a
mass were observed on the right side of the nasal cavity. The pharyngo-
larynx, oral cavity, and nasopharynx exhibited no further abnormalities.
The lymph nodes in the neck were not palpable. Regular biochemical
and hematological testing were normal, except for hemoglobin at 8 g/dL

* Corresponding author at: Department of Pathology Anatomy, Faculty of Medicine, Hasanuddin University – Hasanuddin University Hospital, Jalan Perintis
Kemerdekaan KM 11, Makassar, South Sulawesi 90245, Indonesia.
E-mail addresses: r.masadah@med.unhas.ac.id (R. Masadah), irianidjufri@gmail.com (N.I. Jufri), desy.madayani@gmail.com (D.E. Ahmad), muhammadfaruk@
unhas.ac.id (M. Faruk).

https://doi.org/10.1016/j.ijscr.2023.109160
Received 21 September 2023; Received in revised form 6 December 2023; Accepted 8 December 2023
Available online 15 December 2023
2210-2612/© 2023 The Authors. Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY-NC-ND license
(http://creativecommons.org/licenses/by-nc-nd/4.0/).
R. Masadah et al. International Journal of Surgery Case Reports 114 (2024) 109160

Fig. 1. Microscopic findings (hematoxylin and eosin staining). A: A herringbone pattern (magnification x100), B: A herringbone pattern with mitoses (arrow;
magnification x400).

Fig. 2. Immunohistochemistry staining. A: Ki67 staining positive in nuclei (magnification x400), B: P53 staining positive in nuclei (magnification x400), C: Vimentin
staining positive in membrane and cytoplasm (magnification x400).

and slight leukocytosis (13.3 × 109/L). Axial- and coronal-view sphenoid sinuses. The orbital structures, cranial fossa, and other para­
computed tomography (CT) scans of the head revealed an isodense nasal sinuses were unaffected.
tumor of 4.4 × 3.3 × 2 cm without bone erosion, filling the anterior The patient underwent a blood transfusion of three bags. A biopsy
portion of the right nasal cavity and extending to the right ethmoid and was performed under local anesthesia. The histopathological findings

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R. Masadah et al. International Journal of Surgery Case Reports 114 (2024) 109160

Fig. 3. Axial (A and B) and coronal (C) view. CT scans of the head revealed an isodense tumor of 4.6 × 3.6 × 3 cm with bone erosion in the ethmoid and sphenoid
sinuses. The tumor filled the anterior portion of the right nasal cavity, extending to the right maxilla (blue arrow) and ethmoid (red arrow) and sphenoid sinuses
(yellow arrow). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

reported a dense proliferation of fibroblasts with a herringbone pattern.
The cells were spindle-shaped with hyperchromatic nuclei (Fig. 1). Mi­
toses were found at 1–2 per 10 high-power fields. Immunohistochem­
istry staining was performed, and the result was positive for Ki67, P53,
and vimentin (Fig. 2). Based on these results, the final diagnosis for this
rare neoplasm was nasal fibrosarcoma. The differential tumor diagnosis
included malignant and benign sinonasal-region disease.
The patient underwent functional endoscopic sinus surgery. Intra­
operatively, a lumpy mass on the wall of the right maxilla, right
ethmoid, and right frontal area appeared to bleed easily. Extirpation of
the tumor was carried out in these areas.
Thirteen months later, the patient experienced a recurrence, with
symptoms of epistaxis from the right nasal cavity. Axial- and coronal-
view CT scans of the head revealed an isodense tumor of 4.6 × 3.6 ×
3 cm, with bone erosion in the ethmoid and sphenoid sinuses. The tumor
filled the anterior portion of the right nasal cavity, extending to the right
maxilla and ethmoid and sphenoid sinuses. The orbital structures, cra­
nial fossa, and other paranasal sinuses were unaffected (Fig. 3). The
patient was then planned for tumor extirpation.
An external approach was taken via lateral rhinotomy with medial
maxillectomy under general anesthesia. A firm reddish mass was
discovered emerging from the lateral wall of the nose in the sphenoid
and ethmoidal regions, an abrupt contrast to its source from the
ethmoidal area. Subsequently, the tumor was freed from its lateral
connection and excised in its entirety. Posterior and anterior nasal
packing was used to control the bleeding after the procedure, and it was
removed 24 h later. The patient responded favorably to the surgical
procedure, and on the fourth postoperative day, she was discharged.
One month later, the patient began undergoing chemotherapy with a
regimen of paclitaxel and cisplatin for six cycles. No indications of tumor
recurrence existed at the 8-month follow-up. The patient is currently still
undergoing follow-up every month at our institution.
3. Discussion
Fibrosarcoma is a malignant neoplasm of fibroblasts. Nasal locali­
zation is uncommon. [1] Only 16 % of fibrosarcoma presents in the head
and neck, with the main predilection site being the extremities. [6,7]
Fibrosarcoma commonly presents in men in their third and fourth de­
cades. [3] The etiology of fibrosarcoma is not completely understood;
the most likely factor is radiation exposure. Several cases have reported
that other bone diseases such as osteomyelitis and Paget's disease also

3
R. Masadah et al.
increase the risk of fibrosarcoma.
Fibrosarcoma is commonly an unencapsulated, densely proliferating
spindle-cell tumor, invading bone by direct pressure. [1] Nasal fibro­
sarcoma can metastasize, shows variable collagen production, and does
not present differentiation in other types of tissue. Its histopathological
features can lead to confusion with other differential diagnoses such as
benign sinonasal polyps, fibroma, inflammatory myofibroblastic tumor,
Ewing's sarcoma, lymphoma, sinus cancer (adenocarcinoma and squa­
mous cell carcinoma), and desmoid fibrosarcoma. [3,4] Microscopically,
nasal fibrosarcoma shows hypercellular lesions. Proliferated spindle
cells form a fascicles-and-bundles pattern at various angles (called the
herringbone pattern), with nuclear atypia and mitoses. Immunohisto­
chemistry staining is positive for Ki67, P53, and vimentin. [2,3] Many
limitations exist regarding supporting examination where we cannot
perform further pathological examinations such as immunohistochem­
istry S-100, CD31, CD33, cytokeratins, human melanoma black-45,
smooth muscle actin, CD34, and reticulin, thus ruling out myogenic,
neurogenic, or epithelial tumors. The differential diagnosis of nasal
fibrosarcoma could include malignant and benign sinonasal-region
disease. The latter includes ossifying fibroma, benign sinonasal polyps,
desmoid-type fibromatosis, inverting papillomas, and inflammatory
myofibroblastic tumor. Malignant tumors in the sinonasal region should
be differentiated from paranasal sinus fibrosarcomas. These include
metastasis, sinonasal lymphoma, and paranasal sinus cancer (salivary
gland–type carcinoma, adenocarcinoma, and squamous cell carcinoma).
[2,3]
The main treatment for nasal fibrosarcoma is wide local excision.
Several cases have reported that endoscopic surgery can be performed
with less morbidity and better results than wide local excision. [8]
Endoscopic surgery can reduce morbidity and allow faster recovery. [9]
Some cases have reported that the treatment with the best outcome is
complete surgical resection, followed by reconstruction and post­
operative radiotherapy. [6] Radiotherapy has been performed as an
adjuvant therapy for positive microscopic margins or incomplete gross
excision. Chemotherapy has been performed as a neoadjuvant treatment
combined with radiotherapy as palliative therapy for inoperable pa­
tients. Its risk of recurrence is high, and its risk of distant metastases is
low. [1,6] In a systematic review by Bughrara of 109 patients, 49 (46 %)
underwent only surgical treatment, 41 (39 %) underwent radiation, 11
(10 %) underwent chemotherapy without radiation, and five (5 %) un­
derwent chemotherapy and radiation. [2] In this case, we gave
chemotherapy because the patient had experienced recurrence, tumor-
free margins were not obtained, and the CT scan showed bone
involvement.
The prognosis of nasal fibrosarcoma depends on the tumor size,
histopathological grade, and surgical treatment outcome. Nasal fibro­
sarcoma has a poor survival rate, ranging from 20 % to 35 %. [1] One
study of 52 cases of nasal fibrosarcoma that caused death reported that
nasal fibrosarcoma was associated with high mortality, with 18 cases
(35 %) of death at 1 year of treatment. [2] Currently, we perform routine
follow-up endoscopic and imaging examinations for the early detection
of recurrence.
4. Conclusion
To our knowledge, this is the first report of nasal fibrosarcoma from
Indonesia. Nasal fibrosarcoma is a rare malignant neoplasm, which can
be diagnosed by its histopathological features and
immunohistochemistry.
Consent
Written informed consent was obtained from the patient for
International Journal of Surgery Case Reports 114 (2024) 109160
publication of this case report and accompanying images. A copy of the
written consent is available for review by the Editor-in-Chief of this
journal on request.
Declaration of competing interest
N/A
Acknowledgment
None.
Ethical approval
The case report is exempt from ethnical approval in our institution. It
is only necessary to obtain the patient's consent.
Funding
N/A
Author contribution
RM and NIJ, study concept, and therapy for this patient. DEA and
MF: Data collection, Writing-Original draft preparation. RM, NIJ, DEA,
and MF: Editing, Writing. All authors read and approved the final
manuscript.
Guarantor
RM
Research registration number
Not applicable – single case report.
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