Eur Arch Otorhinolaryngol (2006) 263: 641–643

DOI 10.1007/s00405-006-0026-x

HEAD AND NECK ONCO L OGY

G. Plaza Æ J. Ferrando Æ F. Pinedo

Sinonasal fibrosarcoma: a case report

Received: 20 September 2005 / Accepted: 21 October 2005 / Published online: 9 March 2006

Springer-Verlag 2006

Abstract Sinonasal fibrosarcoma (SFS) is an infrequent The SFS usually presents as other sarcomas in this
malignant neoplasm. It usually presents as other sarco- region, with nasal obstruction and epistaxis [1, 10]. After
mas in this region, with nasal obstruction and epistaxis. imaging is done, the final diagnosis is based on the his-
The final diagnosis is based on the histopathology and topathology and immunohistochemistry. Histologically,
immunohistochemistry. We report the case of a 58-year- they are hypercellular lesions composed of thin, elongated
old man with an 8-month history of left proptosis, spindle cells, arranged in long fascicles and bundles
recurrent epistaxis and nasal obstruction. Nasal endos- intersecting at different angles, and hence are called the
copy confirmed a left nasal neoplasia. CT and MRI herringbone pattern [1, 3, 6]. Nevertheless, these tumors
showed the extension of the neoplasia, occupying the left are biologically diverse, depending on histological type
nasal fossa and ethmoid sinuses, and eroding the medial and grading [1, 6, 10].
wall of the orbit. Complete removal was achieved They are associated with a high risk of local recur-
through endoscopic sinus surgery, preserving the orbit. rence and low risk of distant metastases [1, 6]. Therefore,
SFS was found on histopathologic examination. After wide local excision with radical margins has been the
4 years of follow-up, nasal endoscopy, CT and MRI usual treatment of SFS [10, 11]. Orbital exenteration due
imaging show no sign of recurrence. to tumor invasion is sometimes required [1], but orbital
preservation has also been reported to have good results
Keywords Sinonasal fibrosarcoma Æ Nose Æ Endoscopic when feasible [4, 5]. Endoscopic removal of sinonasal
sinus surgery Æ Proptosis Æ Orbit malignant tumors has been thoroughly reported, with
good oncological results and less morbidity than radical
approaches [4, 8, 9].
Introduction We report a patient with SFS, which was managed by
endoscopic sinus surgery, avoiding damaging orbit
Sinonasal fibrosarcoma (SFS) is an infrequent malignant structures.
neoplasm [10]. These tumors only account for 7–10% of
all the sarcomas of the head and neck region [6]. Only a
few scattered cases have been published, although there Case report
are several comprehensive reviews on histopathological
findings and treatment results on SFS [1, 3, 6]. A 58-year-old man was referred to our hospital because
of severe epistaxis from the left nasal fossa. He had an
8-month history of nasal obstruction, recurrent epistaxis
and rhinorrhea, as well as hyposmia and frequent sinusal
G. Plaza (&)
Department of Otolaryngology, Fundación Hospital Alcorcón, cephalalgias.
Av Budapest 1, Alcorcon, 28922, Madrid, Spain On physical examination the left proptosis, palpebral
E-mail: [email protected] hematoma and orbital cellulitis were observed. The
Fax: +34-91-6219409 ophthalmologic examination confirmed preseptal orbi-
J. Ferrando
tal cellulitis and ophthalmoplegia. Nasal endoscopy
Department of Neuroradiology, Fundación Hospital Alcorcón, revealed a left nasal neoplasia fully occluding the nasal
Madrid, Spain cavity and the right chronic rhinosinusitis.
The coronal and axial CT images showed bilateral
F. Pinedo occupation of both nasal cavities. Bone erosion was
Department of Pathology, Fundación Hospital Alcorcón,
Madrid, Spain observed in the left papiraceous lamina, making the
642

Fig. 1 a Axial CT showed

bilateral occupation of both
nasal cavities and marked left
proptosis. Bone erosion was
observed in the left papiraceous
lamina, and the presence of an
infiltrative process affecting the
extraconal fatty reticulum and
preseptal space. b Coronal MR
T2-weighted confirmed the
presence of a neoplasm in the
left nasal cavity, and showed
how rhinosinusitis was the
cause of right side pathology

mass to contact with orbit fat (Fig. 1a). An MR with

contrast confirmed the presence of neoplasia in the left Discussion
nasal cavity, ethmoid sinuses and orbit, and showed how
rhinosinusitis was the cause of right side pathology SFS, a malignant tumor of fibroblasts, is an infrequent
(Fig. 1b). Under general anesthesia, complete removal neoplasm in the nasal cavity and paranasal sinuses [10]. It
of the neoplasia was achieved through endoscopic sinus usually presents in the third and fourth decades of life, and
surgery. The tumor involved the ethmoid cells and the more commonly on males. Clinical and histopathological
medial orbital fat. It was removed preserving the orbit findings of SFS are comprehensively reviewed in some
structures, and restoring ocular motility. short series on 13 [1], 18 [3] and 14 cases [6].
A low-grade fibrosarcoma was found on histopath- SFS demonstrates variable collagen production,
ological exam. It was a highly cellular tumor composed does not show differentiation into other types of tissue
of elongated spindle-shaped cells arranged in bundles, and has the ability to metastasize [1, 10]. These fea-
so-called herringbone pattern. The individual cells had tures allow the establishment of a differential diagnosis
relatively uniform, spindle-shaped or slightly ovoid between inflammatory myofibroblastic tumor, fibroma,
nuclei, which demonstrate little pleomorphism. A vari- desmoid fibromatosis and SFS [10]. In contrast to
able amount of collagen and moderate mitotic activity desmoid fibromatosis, SFS is more cellular, shows a
were observed (Fig. 2a, b). greater degree of pleomorphism and nuclear atypia,
After 48 months of follow-up, the patient remained with a larger number of mitoses, and may have areas
asymptomatic and the nasal endoscopy showed a of prominent necrosis [2]. As the tumor cells usually
normal left nasal cavity. The CT and MR exams show positive staining with vimentin, a muscle-specific
showed no sign of recurrence (Fig. 3c, b). Later, the actin, and fibronectin, the term myofibrosarcoma has
patient died because of a pancreas cancer, without any been proposed for this entity [7].
evidence of local or distant recurrence on the Surgery remains the mainstay for the treatment of
pathology reports. SFS. Wide local excision with radical margins is

Fig. 2 a A low-grade fibrosarcoma was found on histologic mitotic activity, arranged in fascicles and bundles with occasional
examination. Beneath a nasal mucosa lined by ciliated respira- ‘‘herringbone’’ pattern (hematoxylin and eosin stain, 40·). Immu-
tory-type epithelium, a hypercellular lesion can be observed nohistochemically, the cells are only positive for vimentin (not
(hematoxylin and eosin stain, 20·). b This is a low-grade sarcoma shown)
composed of spindle cells with rare pleomorphism and moderate
 643

Fig. 3 a After 30 months of

follow-up, coronal CT showed
wide resection of left sinonasal
structures, with no sign of
recurrence. b After 48 months
of follow-up, coronal MR T2-
weighted showing post-surgical
changes. There was no evidence
of residual/recurrent tumor.
Note the normalization of
extraconal fatty reticulum,
medial rectus and preseptal
space

generally recommended [10, 11]. Adjuvant radiotherapy 2. Gnepp DR, Henley J, Weiss S, Heffner D (1996) Desmoid
is also used in positive surgical margins or macroscopi- fibromatosis of the sinonasal tract and nasopharynx. A clini-
copathologic study of 25 cases. Cancer 78:2572–2579
cally incomplete excision [6]. As shown in the present 3. Heffner DK, Gnepp DR (1992) Sinonasal fibrosarcomas,
case, complete removal can be achieved by endoscopic malignant schwannomas, and ‘‘Triton’’ tumors. A clinico-
sinus surgery, avoiding damaging the orbit and restoring pathologic study of 67 cases. Cancer 70:1089–1101
ocular motility [4]. The greatest advantage of endoscopic 4. Hosemann WG, Weber RK, Keerl RE, Lund V (2000) Mini-
mally invasive endonasal sinus surgery, 1st edn. Thieme,
surgery for malignant tumors is the reduction in mor- Stuttgart, pp 72–80
bidity. However, it does not allow for an en bloc resec- 5. Imola MJ, Schramm VL Jr (2002) Orbital preservation in
tion with radical margins. Instead, it usually results in a surgical management of sinonasal malignancy. Laryngoscope
piecemeal excision of the neoplasia, and sometimes re- 112:1357–1365
quires frozen section pathology to obtain adequate safe 6. Koka V, Vericel R, Lartigau E, Lusinchi A, Schwaab G (1994)
Sarcomas of nasal cavity and paranasal sinuses: chondrosar-
margins [8, 9]. coma, osteosarcoma and fibrosarcoma. J Laryngol Otol
SFS is usually associated with a high risk of local 108:947–953
recurrence, and low risk of distant metastases [1, 6, 10]. 7. Kondo S, Yoshizaki T, Minato H, Horikawa I, Tatsumi A,
The biological behavior of the tumor in this region is Furukawa M (2001) Myofibrosarcoma of the nasal cavity and
paranasal sinus. Histopathology 38:482–484
very similar to that of carcinoma, and death may result 8. Kuhn FA, Javer AR (2003) Low-grade fibrosarcoma of the
from intracranial extension of a persistent or recurrent anterior skull base: endoscopic resection and repair. Am J
tumor [1]. Frequent endoscopic and imaging controls Rhinol 17:347–350
are very useful to achieve early detection of recurrences 9. Lin HL, Huang CC, Lee TJ (2004) Endoscopic sinus surgery
treatment for a huge sinonasal fibroma. Chang Gung Med J
that can also be treated endoscopically [4]. 27:233–237
10. Pérez-Ordóñez B, Huvos AG (2001) Nonsquamous lesions of
nasal cavity, paranasal sinuses, and nasopharynx. In: Gnepp
References DR (ed) Diagnostic surgical pathology of the head and neck.
W.B. Saunders Company, Philadelphia, pp 79–139
11. Shah JP (1977) Fibrosarcoma of nasal cavity. N Y State J Med
1. Fu YS, Perzin KH (1976) Nonepithelial tumors of the nasal 77:983–984
cavity, paranasal sinuses, and nasopharynx. A clinicopatho-
logic study. VI. Fibrous tissue tumors (fibroma, fibromatosis,
fibrosarcoma). Cancer 37:2912–2928