Case Report

Benign Extrapleural Solitary Fibrous Tumor of the Head and

Neck
By: Princess C. Manieda, M.D., Emmanuel Tadeus S. Cruz, M.D.
ABSTRACT
Introduction: Presented is a rare case of a benign Extrapleural Solitary Fibrous Tumor, its’ differential diagnoses,
diagnostics, management and prognosis.
Case: A 58-year old female was admitted because of a 3-year history of bulging mass on the left cheek due to a slowly
growing palatal mass. Physical examination showed a gross facial deformity due to a bulging mass on the left cheek with
submandibular extension. A rubbery, well circumscribed mass at the left soft palate extending to the oropharynx and left
retromolar trigone was also seen. The overlying skin and oral mucosa were normal. There was no facial paralysis nor
cervical lymphadenopathy present. The contrast-enhanced CT scan of the neck revealed a large 7.0cm x 7.6cm x 6.1cm
enhancing soft tissue mass at the left masticator space. The incisional biopsy revealed spindle cell neoplasm so she
underwent wide surgical resection of the mass. The histopathologic diagnosis was Extrapulmonary Solitary Fibrous Tumor
(SFT) with immunoreactivity to CD34 and S100.
Discussion/Conclusion: This is a rare case of an extrapulmonary SFT of mesenchymal origin that can be difficult to
distinguish from other fibroblastic or myofibroblastic tumor and it was only diagnosed on immunohistochemistry.
Keywords: soft tissue neoplasm, oral neoplasms, solitary fibrous tumor

*From the Department of Otorhinolaryngology – Head and Neck Surgery, Manila Central University, Filemon D. Tanchoco Medical
Foundation and Hospital
3rd Prize: 36th Case Report Oral Contest, January 29, 2017, Tanchoco Auditorium
Correspondence: Princess C. Manieda, MD ● Email address: princessceninmanieda@yahoo.com ● Department of Otorhinolaryngology –
Head and Neck Surgery, MCU-FDTMF Hospital, Samson Road, EDSA, Caloocan City Tel: 09333183106

INTRODUCTION enlarging palatal mass of 3 years duration. There was

Extrapleural solitary fibrous tumors (SFT) are

uncommon mesenchymal neoplasm1 and can occur
in any location in the head and neck. However, there
are few cases of SFT reported.1-3 The histological
spectrum of SFT is quite broad and can be mistaken
for other benign or malignant soft tissue tumors,
thus contributing to diagnostic difficulties.

The objective of this paper is to present a case

of an Extrapulmonary Solitary Fibrous Tumor located in
the oral cavity, specifically the soft palate and assess
the clinical and histological features,
immunohistochemical workup available to aid in the
diagnosis, management, and prognosis.

CASE PRESENTATION

A 58-year old female was referred to the

Department of Otorhinolaryngology for a gradually
Philippine Scientific Journal Vol. 50 • No. 1
2
no history of trauma, feeding difficulty nor weight loss.
Past medical, family, personal social histories were
unremarkable.

Physical examination showed a gross facial

deformity due to a bulging mass on the left cheek with
submandibular extension (Figure 1). A rubbery, fixed,
well circumscribed, non-ulcerating intraoral mass at
the left side of the soft palate was seen. It extended
medially obstructing the oropharynx and left retromolar
trigone (Figure 2). The skin of the cheek and oral
mucosa were normal. There was no bruit nor pulsation
noted. There was no facial paralysis nor cervical
lymphadenopathy. Nasal endoscopy and flexible
laryngeal videoendoscopy showed a bulging mass in
the entire nasopharynx extending inferiorly to the
oropharynx and epiglottis (Figures 3-4). Vocal folds
were symmetrical and mobile. Contrast-enhanced CT
scan of the neck revealed a large enhancing soft tissue
mass at the left masticator space measuring 7.0 cm x
7.6 cm x 6.1cm (Figure 5). Pre-operative incisional
biopsy was done via a transoral approach and revealed
spindle cell neoplasm on histopathology.

2 Philippine Scientific Journal Vol. 50 • No.

2

The patient underwent tracheostomy prior to
excision of the mass. The lesion was accessed thru a lip
split incision with mandibulotomy. Complete removal
of the mass was carried out with a wide surgical
resection. Intraoperative finding was a firm, well
circumscribed mass in the left buccal space, posterior to
the left mandibular ramus. This extended to the hard and
soft palate junction superiorly and left submandibular
area inferiorly (Figure 6). Grossly, the mass excised
measured
9.0 x 5.5 x 5.1 cm. Cut section of the specimen revealed
a tan-yellow, fleshy, lobulated surface with areas of
haemorrhage, necrosis and cystic degeneration (Figure
7). Microscopically, there was presence of a patternless
architecture with combination of hypo and hypercellular
areas of proliferating fibroblasts separated by thick
bands of hyalinized tissue. There were staghorn-like
branching of the thin-walled vessels (Figure 8). Tumor
cells were ovoid and vesicular with scanty, pale
cytoplasm (Figure 9). Tumor necrosis were mini-
mal. Mitotic figures were 0-2 per HPF. diagnosis was
Extrapulmonary Solitary Fibrous Tumorw ith strong
immunoreactivity to CD34 and a focal reactivity S100.
Further immunohistochemical staining for CD99
was requested.
The patient was discharged well on the fifth
postoperative day. She followed up on the 7th day
for the removal of sutures and nasogastric tube.
Postoperative appearance of the surgical site was a
widened oropharyngeal area with good wound healing.
Repeat nasal endoscopy and laryngeal video endoscopy
were normal. She was seen at the OPD every month for
follow up with no evident signs of tumor recurrence.
DISCUSSION
Among the common benign tumors which
are usually seen in the head and neck region
include vascular tumors, salivary gland neoplasm,
neurofibroma or schwannoma and others.
Vascular tumours such as hemangioma is
encountered particularly in the oral cavity. These are
the most common benign mesenchymal neoplasm,
accounting for almost 40% of the benign tumours in
general. However, vascular tumors were excluded
due to age predilection, which is commonly seen in
the first decade, gross clinical appearance of the mass
Benign Extrapleural Solitary Fibrous Tumor of the Head and Neck
which is non hyperemic with no pulsation and the
histopathology report showed absence of prominent
blood vessels present in vascular tumours.
Salivary gland tumors are frequently encountered
benign tumor of the minor salivary glands. Most common
of which is pleomorphic adenoma commonly seen in the
hard palate, lip, buccal mucosa, alveolar ridge, floor of
the mouth, and tongue in decreasing order of frequency. 4
Also it appeared as smooth and relatively slow growing.
Histologic features of pleomorphic adenoma has
epithelial elements that resembles ductal cells or
myoepithelial cells that are arranged in duct formations,
acini, irregular tubules, strands, or sheets of cells which
differs from a solitary fibrous tumor that has a patternless
architecture characterized by a combination of hypo
and hypercellular areas, separated by thick bands of
hyalinized, sometimes keloidal, collagen and thin-walled
branching haemangiopericytoma-like vessels with ovoid
to spindle-shaped cells and a limited pale cytoplasm
which is therefore, excluded as a differential.
Another benign tumor which may occur in
the oral cavity are peripheral nerve sheet tumors (eg.
Neurofibromaand Schwannoma). Neurofibroma is the
most common peripheral nerve sheet tumor which is
typically diagnosed in adolescents and young adults.
Solitary neurofibromas of the oral cavity usually involve
the tongue or the buccal or labial mucosa and present as
soft, painless, slow-growing masses that are tender to
pressure or palpation.4. It is quite difficult to distinguish
this tumor from other soft tissue tumor histologically
without the aid of immunohistochemistry.
Neurofibromas and schwannoma are both diffusely and
strongly positive for S100 protein and are negative for
CD34. As with this case, the tumor exhibits a diffuse
immunoreactivity to CD34 and a focal reactivity S100.
Among malignant neoplasms of the oral cavity,
squamous cell carcinoma amount to more than 90% 1 of
malignant tumours of the oral cavity and oropharynx
which was ruled out. Another malignant neoplasm that
are found in the head and neck region due to the
abundant lymphatic supply, are lymphomas. Although
the palatal mass in this case was huge, it was well
confined and delineated, no cervical lymphadenopathy,
and the final histopathology report showed an
Extrapulmonary SFT.
Soft tissue tumors account for less than 1% of all
tumors. The annual incidence of soft tissue tumors are
Philippine Scientific Journal Vol. 50 • No. 3
2

approximately 300 per 100,000 people in the general
population, and are mostly benign.5 Solitary fibrous
tumours (SFT) in particular, are rare neoplasms
of mesenchymal origin that account for less than 2%
of all soft tissue tumours.6
Solitary fibrous tumor was first described in
the pleura, by Klemperer and Rabin in 1931 7. Early
reports uniformly regarded SFT as a tumor of
mesothelial in origin. Stout and Murray in 1942,
described these tumors as “vascular tumors arising
from Zimmerman’s pericytes” and suggested that these
tumors be called hemangiopericytomata. Nowadays, it
has been suggested that SFT arises from pluripotential
mesenchymal cells located in the connective tissue 8,
and the World Health Organization (WHO)/
International Agency for Research on Cancer (IARC)
currently classified this tumor as fibroblastic/
myofibroblastic tumor9. Approximately 76% of
fibroblastic and myofibroblastic tumors are regarded
as benign, 13% as intermediate, and 11% as malignant,
wherein SFTs are classified as intermediate.
Solitary fibrous tumor are mostly found in the
pleura and are rarely found in the head and neck. In the
head and neck, 40 percent are found in the subcutaneous
tissue while others arise in deep soft tissue 1. Other sites
that were reported includes the cheek, posterior trigone,
palate 10, parotid10, upper lip10,v entral tongue11, and
sublingual gland12. Extrapleural solitary fibrous tumsors
(SFTs) are most commonly seen in middle-aged adults
aged 20-70 years. Males and females are affected
equally. Our patient, is a 58 year old, female and the
tumor originated from the soft palate, obstructing the
oropharynx.
SFTs found in the head and neck present as
well- delineated, slow growing painless masses1. Most
are well circumscribed, measuring between 1 and 25 cm
(median, 5-8cm), generally smaller in the head and neck
region. The mass of our patient measured 9 cm in its
largest diameter was well circumscribed and slowly
growing. Though it was large enough to affect the
oropharyngeal phase of swallowing because of its
location, she did not experience difficulty in feeding.
She opted to undergo surgery for cosmetic reasons
because of the bulge on her cheek.
Surgical management with wide surgical
resection is the treatment of choice because of the high local
recurrence rate of 21% to 47%.4 In this case, the tumor
Benign Extrapleural Solitary Fibrous Tumor of the Head and Neck
was accessed via a lip split incision with mandibulotomy
and resected with wide surgical margins.
Intraoperatively, the mass was well circumscribed, firm,
and located posterior to the left mandibular ramus that
extends to the hard and soft palate junction superiorly
and inferiorly to the left submandibular area (Figure 6).
SFTs have multinodular, whitish, and firm appearance on
cut section. In our patient, the mass showed a lobulated,
whitish surface with areas of haemorrhage, necrosis and
cystic degeneration (Figure 7).
Microscopically, SFTs have patternless
architecture characterized by a combination of hypo
and hypercellular areas, separated by thick bands of
hyalinized, sometimes keloidal, collagen and
thin-walled branching haemangiopericytoma-like
vessels. Tumor cells are ovoid to spindle-shaped with
limited pale cytoplasm having indistinct borders and
dispersed chromatin within vesicular nuclei. Mitoses are
generally scarce, rarely exceeding 3 mitoses per 10 HPF.
The histology of SFT may resemble some benign soft
tissue tumors (eg, spindle cell lipoma, and cellular
angiofibroma) and some malignant soft tissue tumors
(eg. Fibrosarcoma, rhabdomyosarcoma, peripheral nerve
sheath tumour,etc). In our patient, the tumor showed a
hypo and hypercellular areas of proliferating fibroblasts
separated by thick bands of hyalinized tissue and
staghorn-like, branching, thin walled blood vessels.
Tumor cells are ovoid, vesicular with scanty, pale
cytoplasm (Figure 8). Tumor necrosis are rare and
mitotic figures are 0-2 per HPF, signifying that this tumor
is benign. Malignant SFTs are usually hypercellular
lesions, showing increased mitoses (>4 mitoses per10
HPF), variable cytological atypia, tumor necrosis, and/or
infiltrative margins, of which mitoses seem to be most
prognostic.1 Further immunohistochemistry can be done
in differentiating this tumor from other malignant as well
benign soft tissue tumor.
The immunohistochemical profile of SFT using
conventional markers is relatively nonspecific, with
CD34 expression being the most consistent and the most
reliable finding reported in SFTs to date which is present
in 95% of cases. CD34 (Q BEND 10) is a marker that
stains normal and neoplastic endothelial cells, as well as
a variety of soft tissue neoplasms.13 Aside from SFTs
being immunoreactive to CD34, benign and malignant
soft tissue tumors that are immunoreactive to CD34
includes Giant Cell Angiofibroma, Lipofibromatosis,
Myofibrosarcoma, etc. In our case, the
specimen exhibits diffuse
Philippine Scientific Journal Vol. 50 • No. 39
2
Benign Extrapleural Solitary Fibrous Tumor of the Head and Neck
immunoreactivity to CD34 with focal reactivity to
S100. Immunoreactivity to CD99 in 70% of cases
and EMA in 20-30% of cases were also seen in cases
of SFTs.14,15 Virtually all SFTs are positive for
BCL2,14,15,16 but also a nonspecific finding, as expression
is also observed in a variety of other mesenchymal
neoplasms.21 Focal and limited reactivity to S100,
keratins and/or desmin has occasionally been reported.
In a study done by Doyle,et al,17 231 tumors
were evaluated, including 60 cases of SFT as well as
other benign and malignant mesenchymal neoplasms
and sarcomatoid mesotheliomas. Fifty-nine of 60
SFT cases (98%) usually showed diffuse and intense
nuclear expression of STAT6. All the remaining
tumor types were negative for STAT6, except for three
dedifferentiated liposarcomas and one deep fibrous
histiocytoma, which showed weak staining for STAT6.
Schweizeret al18 also identified an
overexpression of nuclear STAT6 protein in meningeal
SFTs. Therefore, STAT6 is a highly sensitive and
almost perfectly specific immunohistochemical
marker for SFT and can be helpful to distinguish this
tumor type from histologic mimics.17 However,
immunohistochemical marker, STAT6, is not
available in the country.
Post-operative management of benign SFTs
include a close long-term follow up, although, most
histologically benign SFTs prove to be non-recurring
and non-metastasizing lesions.1 Ten percent of
malignant SFTs with a size of more than 10 cm with
positive tumor margins indicates poor prognosis
and may have local or distant recurrence after primary
resection. In cases of malignant SFTs, metastases
are most frequently observed in the lungs, bone, and
liver.1 SFTs that are advanced, recurrent, and
metastatic may benefit from post-operative radiation
and chemotherapy.19,20 In this case, the tumor has a
benign histology, therefore, no need for chemotherapy/
radiotherapy procedure postoperatively22.
CONCLUSION
Presented is a rare case of an extrapulmonary
SFT of mesenchymal origin that can be distinguished
from other fibroblastic or myofibroblastic tumors
by immunohistochemistry.
40 Philippine Scientific Journal Vol. 50 • No.
2
REFERENCES
1. Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F.
WHO/IARC Classification of Tumours, 4th ed, vol.5,
2013.
2. De Oliveira D, et al. Large Solitary Fibrous Tumor of the
Oral Cavity--Report of a Case. Pathology-- Research and
Practice. 210(12),1064-1067,2014
https://doi.org/10.1016/j.prp.2014.09.018
3. Lee JH, Seok-Lee E, Kwon SY, Kim YS. Comparison of
Sclerotic Fibroma and Solitary Fibrous Tumor in the Oral
Cavity. Yonsei Med J. Jun 07 2007; 48(3): 535-539.
4. Flint P, Haughey B., Lund V, Niparko J, Robbins K,
Thomas JR, Lesperance M. W. Cummings Otolaryngology
Head and Neck Surgery. Sixth edition, Philadelphia, PA:
Mosby/Elsevier, December 2014.
5. Razek AA, Huang B. Soft Tissue Tumors of the Head
and Neck: Imaging-based Review of the WHO
Classification. Radiographics J. Nov-Dec 2011; 31
(17):1923-1952
6. Lee CUC, Glockner J. Mayo Clinic Body MRI Case
Review. 2014: 626-27
7. Klemperer P., Rabin CB. Primary neoplasms of the pleura:
a report of five cases. Arch Pathol. 11: 385-412, 1931.
8. Tenna S, Poccia I, Cagli B, Aveta A, Manzo MJ, Persichetti
P. A locally aggressive solitary fibrous tumor of the leg:
case report and literature review, Int. J.Surg. Case Rep.
2012: 3(5):177–180.
9. Guillou L, Fletcher JA, Fletcher CDM, Mandhal N.
Extrapleural solitary fibrous tumour and haemangiomp
ericytoma, Pathology and Genetics of Tumors of Soft
Tissue and Bone (WHO). IARC Press, Lyon, 2002. pp.
86–90
10. Perez-Ordonez B, Koutlas JG, Strich E, Gilbert RW,
Jorda RCK. Solitary fibrous tumor of the oral cavity: an
uncommon location for a ubiquitous neoplasm. Oral Surg
Oral Med Oral Pathol Oral Radiol Endod . 1999;87:589–93.
11. Piattelli A, Fioroni M, Rubini C. Solitary fibrous
tumor of the tongue. Oral Oncol 1998; 34:431–4.
12. Gunhan O, Yildiz FR, Celasum B, Onder T, Finci
R. Solitary fibrous tumor of the sublingual gland: report
of a case. J Laryngol Otol. 1994;108:998–1000.
13. Rosai J. Rosai and Ackerman Surgical Pahology.
10th ed, Mosby; June 2011.
 Benign Extrapleural Solitary Fibrous Tumor of the Head and Neck

14. Rao N, Colby TV, Falconieri G, et al. Intrapulmonary

solitary fibrous tumors: clinicopathologic
and

Philippine Scientific Journal Vol. 50 • No. 41

2
Benign Extrapleural Solitary Fibrous Tumor of the Head and Neck

immunohistochemical study of 24 cases. Am

J Surg Pathol. 2013;37:155–166.

15. Renshaw AA. O13 (CD99) in spindle cell tumors:

reactivity with hemangiopericytoma, solitary fibrous tumor,
synovial sarcoma, and meningioma but rarely with
sarcomatoid mesothelioma. Appl Immunohistochem.
1995;3:250–256.

16. Chilosi M, Facchettti F, Dei Tos AP, et al. Bcl-2

expression in pleural and extrapleural solitary
fibrous tumours. J Pathol. 1997;181:362–367

17. Doyle L, Vivero M, Fletcher CD, Mertens F, Hornick JL..

Nuclear expression of STAT6 distinguishes solitary fibrous
tumor from histologic mimics. Mod Pathol. 2014;
27(3): 390–395.

18. Schweizer L, Koelsche C, Sahm F, Piro RM, Capper D,

Reuss DE, Pusch S, Habel A, et al. Meningeal
hemangiopericytoma and solitary fibrous tumors carry the
NAB2-STAT6 fusion and can be diagnosed by nuclear
expression of STAT6 protein. Acta Neuropathol
2013;125(5):651–658.

19. Gold JS, Antonescu CR, Hajdu C, Ferrone CR, Hussain

M, Lewis JJ, Brennan MF, Coit DG. Clinicopathologic
correlates of solitary fibrous tumors. Cancer. 2002;94:
1057–68.

20. Gao C, Yong Z, Yu YH. Postoperative Radiotherapy for

the Treatment of Solitary Fibrous Tumor with Malignant
Transformation of the Pelvic. Radiotherapy for Malignant
Solitary Fibrous Tumor. Medicine (Baltimore).
Jan 2016; 95(2):1-5.

21. Suster S, Fisher C, Moran CA. Expression of bcl-2

oncoprotein in benign and malignant spindle cell
tumors of soft tissue, skin, serosal surfaces, and
gastrointestinal tract. Am J Surg Pathol.1998;22:863–872.

22. Cox DP, Daniels T, Jordan RC. Solitary fibrous tumor

of the head and neck, Oral Surg. Oral Med. Oral Pathol.
Oral Radiol. Endodontol. Jul 2010;110 (1): 79–84.

42 Philippine Scientific Journal Vol. 50 • No.

2