REVIEW ARTICLE

Solitary Fibrous Tumor/Hemangiopericytoma Dichotomy

Revisited: A Restless Family of Neoplasms in the CNS
Can Ege Yalcin, MD and Tarik Tihan, MD, PhD

Abstract: Solitary brous tumor (SFT) and hemangiopericytoma

(HPC) both entered the literature as separate entities in the early to
mid 1900s. In contrast to their central nervous system (CNS)
counterparts, there has been a tendency to consider these 2 entities
as 1 since the early 1990s, as soft tissue SFT gradually included the
tumors previously diagnosed as HPC. The most recent World
Health Organization (WHO) classication of the tumors of soft
tissue considered the term HPC obsolete, and places all such
tumors within the extrapleural SFT category. In contrast, CNS
SFT and HPC continue to be regarded as dierent entities in the
latest version of the WHO CNS tumor classication. A change in
this approach is currently being considered for the upcoming
revision of the WHO scheme, but it is not quite clear whether such
a change will be as drastic as the one adopted by the soft tissue and
bone tumor working group. This article focuses on the historical
evolution of these 2 labels as primary CNS neoplasms, and reviews
their dierences and similarities in terms of clinical, pathologic, and
molecular features.
Key Words: hemangiopericytoma, solitary brous tumor, CNS
tumors, mesenchymal tumor

(Adv Anat Pathol 2016;23:104111)

INTRODUCTION AND HISTORICAL PERSPECTIVE

In the modern era of pathology, the report by Klemperer and Rabin1 is considered the rst that describes the
solitary brous tumor (SFT) as a primary localized neoplasm of the visceral pleura. According to this report, the
slow-growing tumors did not become symptomatic until
they were large enough to cause mass eect. They were
well-encapsulated spindle cell tumors resembling brosarcoma. Yet, unlike brosarcomas, these tumors did not
show any sign of metastasis or inltration.1 It was originally suggested that SFTs were derived from the mesothelial cells of the visceral pleura.1,2 However, there was
increasing evidence for a mesenchymal origin of these
tumors,3 especially following studies that reported these
neoplasms in extrapleural sites.4,5 Some of these tumors
were also recognized to be associated with systemic hypoglycemia.6,7 SFT in the central nervous system (CNS) was
rst described by Carneiro et al,8 as an entity distinct from
the brous meningioma, which may have been how SFTs
were classied in the CNS before 1996.9 Many additional
observations have led to the reclassication of SFTs under
the mesenchymal, nonmeningothelial tumors in the latest
From the Department of Pathology, Neuropathology Division, University of California San Francisco, San Francisco, CA.
The authors have no conicts of interest or NIH funding to report.
Reprints: Tarik Tihan, MD, PhD, Department of Pathology, Neuropathology Division, University of California San Francisco, Room
M551, 505 Parnassus Avenue, San Francisco, CA 94143-0102
(e-mail: tarik.tihan@ucsf.edu).
All gures can be viewed online in color at http://www.anatomic
pathology.com.
Copyright r 2016 Wolters Kluwer Health, Inc. All rights reserved.

WHO CNS classication.10 This classication scheme

dened SFT as a neoplasm that aects both cranial and
spinal meninges composed of spindle cells that are disposed
in fascicles between prominent, eosinophilic bands of
collagen.
Hemangiopericytoma (HPC) was initially described by
Stout and Murray,11 and was thought to originate from the
pericapillary cells called pericytes that were described earlier by Zimmermann.12 HPCs were dicult to dierentiate
from other stromal and especially vascular tumors, and
the main unique morphologic feature was the so-called
staghorn vessels that gave rise to the term hemangiopericytoma-like vasculature. Stout and Murray11 considered these tumors as primarily vascular neoplasms
composed of haphazardly arranged tubules of endothelial
cells surrounded by pericytic cells with a supporting
meshwork of reticulin bers. Stout and Murray expanded
their initial denition with 25 more examples, broadening
the spectrum of HPC in terms of location, histologic
appearance, and clinical behavior. In addition to soft tissue
examples, this study included 1 tumor involving the
meninges.13 Following the report by Stout and Murray,
many studies highlighted the clinicopathologic spectrum of
HPCs in the CNS.1420 In 2007, the revised WHO classication of CNS tumors included a separate chapter for
HPC, and this entity was dened as highly cellular and
vascularized tumor, exhibiting a characteristic monotonous
appearance and a well-developed, variably thick-walled,
branching staghorn vasculature; almost always attached
to the dura and having a high tendency to recur and to
metastasize outside the CNS.10
The major challenges for the WHO CNS tumor classication are twofold; rst, there is acknowledgment of the
confusion and overlap with SFT in the section for HPC, but
little acknowledgment of the fact that these tumors were
already lumped into the same entity by the WHO soft tissue
tumor classication scheme. Second, there is much uncertainty about grading of both tumors; HPC is either WHO
grade II or III, and SFT is not assigned a specic WHO
grade.10 The presence of tumors that fell in between the 2
ends of the SFT/HPC spectrum is also recognized.21 There
was a presumption that the CNS examples of these tumors
were somewhat dierent, contrary to the assertions in the
revised WHO soft tissue classication.22,23

CLINICOPATHOLOGIC FEATURES
SFTs have been reported virtually everywhere along
the neuraxis. Fargen et al24 provide a comprehensive review
of studies on SFT in the CNS since the initial report by
Carneiro et al in 1996.8 This review suggests that intracranial SFTs were more common than intraspinal tumors
with an equal male to female ratio.24 Most intracranial
SFTs were dura-based masses and the median age was

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between the fth and the sixth decade. Rarely, SFTs were
found in other locations such as cranial nerves,25 ventricles,21,26,27 or without any connection to dura mater.28,29
In contrast to the intracranial SFTs, there was a slight male
preponderance among intraspinal SFTs (56.1%), with a
slightly lower median age at diagnosis.
HPCs reportedly have a slightly higher preponderance
among male individuals, and patients most often present
with headaches. The median age at presentation is typically
during the fourth decade.16 The majority of reported HPCs
were supratentorial, but the tumors were found anywhere
along the neuraxis.15,16,20,3033 Additional presenting
symptoms included papilledema, hemiparesis, gait disturbance, and ataxia, depending on the localization of the
tumor.15
There is a signicant overlap between clinical and
demographic features of patients with SFT and HPC, and
the reported dierences in median age and male/female
ratio may simply reect the limited size of dierent cohorts.
Thus, clinical and demographic features do not allow a
distinction between SFT and HPC.

Imaging
Radiologic features of both SFT and HPC may be
found in the earlier reports of angioblastic meningioma, a
term that was often used before SFT and HPC were recognized as distinct entities.34,35 Magnetic resonance imaging of the lesions reveals lobular, extraexial masses frequently attached to dura and occasionally showing skull
erosion. The tumors often show a broad attachment to
dura, and the contrast enhancement is often marked and
diuse (Fig. 1A), whereas at other times enhancement can

SFT/HPC Dichotomy in CNS

be heterogenous. The tumors are typically isointense to

gray matter on T1-weighted and T2-weighted images but
they may be heterogenous on both modalities.17,36 Local
mass eect, peritumoral edema, and dural tail sign may be
observed in many examples, and these features may suggest
a meningioma (Fig. 1B). On computerized tomography,
HPCs appear as lytic, dura-based lesions without associated
hyperostosis or calcications.17 Magnetic resonance images
are often similar to those described above with avid contrast enhancement.17,36,37 As highly vascular tumors, HPCs
have been reported to demonstrate corkscrew-like vessels
on angiography.38 They may have nodular or smooth
margins, which are clearly demarcated from the surrounding tissue.
Although the radiologic appearance of tumors
reported as HPC is better documented in the CNS, there
seems to be no signicant distinction between SFT and
HPC in terms of radioimaging.

Outcome and Management

One of the perceived dierences between SFTs and
HPC has been the biological behavior of these tumors in
the CNS. Earlier studies have reported indolent growth,
low rate of recurrence, and almost no extracranial metastases for SFTs, whereas HPCs were considered highly
aggressive tumors with a high rate of local recurrence and a
risk of developing extracranial metastases.21 However, as
some tumors with the histologic appearance of one could
transform into the other entity on recurrence, the differences in biological behavior is partly dependent on how
the tumors that straddle the boundary between SFT and
HPC were classied.21,39,40 Nevertheless, many studies,

FIGURE 1. Typical radiologic appearance of SFT/HPC: the radiologic appearance fails to identify any difference between tumors
reported as SFT and HPC. All such tumors were solid, diffusely enhancing, and dura-based masses. A, Axial contrast-enhanced
T1-weighted MRI of an SFT in an older male involving the posterior fossa with modest mass effect. B, Coronal contrast-enhanced
T1-weighted MRI from a middle-aged woman with HPC. Note the typical dural-tail sign (arrow) that is often considered typical
for meningiomas. HPC indicates hemangiopericytoma; MRI, magnetic resonance imaging; SFT, solitary fibrous tumor.

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Yalcin and Tihan

recent or remote, suggest that the histologic pattern typical

of SFT, which is often seen without features of a high-grade
sarcoma (ie, mitoses or necrosis), seems to correlate with a
more indolent course, whereas the typical histologic
appearance of HPC, even without a high mitotic rate or
necrosis, often implies a more aggressive course.21 Other
studies also suggested that SFT behaved mostly in an
indolent manner, with a fair possibility of recurrence for the
partially resected tumors.24,29,41,42 The main goal of treatment for SFT has been reported as gross total resection.
Although only 14% of grossly resected SFTs recur, this
ratio increases for partially removed tumors.24 The number
of cases belonging to the anaplastic (8%) or malignant
(6%) category within the SFT diagnosis is very small, but
they have been reported.43,44 Rare, metastatic examples of
CNS SFTs were also published in the literature.4547
As in SFT, gross total resection of HPCs is the most
benecial and frequently performed initial treatment.48
However, unlike the typical clinical course of tumors with
the classical histologic features of SFT, local recurrence and
distant metastases are more common in tumors reported in
the HPC category.1921,37 Hence, adjuvant radiotherapy is
performed to eliminate the risk of recurrence. Recent
studies suggest an approximate rate of 60% for local
recurrence and 20% chance of metastatic disease even with
suboptimal follow-up periods.20
The operational denition of tumors in the SFT/HPC
category may vary and there may be some bias in classication. Yet, the CNS tumors with the traditional morphologic features of SFT seem to behave in an indolent
manner, whereas the typical features of HPC are associated
with a more aggressive course. Thus these 2 CNS tumors
have been interpreted as either tumors at the opposite end
of a single entity or distinct entities with numerous overlapping characteristics.18,21,23,49

Pathologic Features
Macroscopically, SFT appears as a well-circumscribed,
rubbery mass with clear evidence of brotic qualities, and the
surgeons intraoperative impression is often that of a brous

Adv Anat Pathol

Volume 23, Number 2, March 2016

meningioma (Fig. 2A). Although HPC can have a similar

macroscopic appearance, the typical example is also a wellcircumscribed, slightly rubbery mass with a so-called shesh look, implying a cellular tumor (Fig. 2B). Many
examples of this spectrum of neoplasms have macroscopic
features indistinguishable from meningiomas.
Histologically, SFT is a prominently collagenous
tumor composed of layers of eosinophilic collagen dispersed in between fascicular spindle to oval cells (Figs. 3A,
C). In contrast to tumors reported as HPC, SFTs have a
poor reticulin network, and special stains dene abundant
collagen bers (Fig. 3E) instead of the rich reticulin network around clusters of tumor cells in HPC (Fig. 3F).
In contrast to meningiomas, there is near absence of
psammoma bodies, whorls, epithelial features, nuclear
grooves, and inclusions. The typical vascular pattern in
many SFTs is very much like those reported as HPC. The
staghorn or HPC-like thin-branching vessels, which are
not seen in every example, impart a typical recognizable
appearance.21
Immunohistochemical studies show strong positivity
with vimentin antibodies, as well as CD34, which recognize
a transmembrane glycoprotein expressed in a diverse type
of cells.4,8,21,50 SFTs are also strongly positive with the Bcl2 and factor XIIIa antibodies (Figs. 4A, B). In addition to
desmin negativity, SFTs are negative for EMA and S-100,
which are helpful to dierentiate it from brous meningioma and schwannoma, respectively.18 Proliferation indices
as detected with the Ki-67 antibody show a range from 1%
to 10%, with a median labeling index of 3%.21
Microscopically, HPCs are composed of cells with
oval to spindle-shaped nuclei distributed unequally among
the tumor causing a biphasic pattern hypercellular and
hypocellular areas. Many reports and descriptions of HPC
use terms such as jumbled or patternless to underscore
the haphazard arrangement of individual cells or small
groups of cells (Figs. 3B, D) that are invested in a rich
network of reticulin bers.16,20 This feature was suggested
as an important distinguishing feature of HPCs, even
though tumors may vary in their reticulin network content

FIGURE 2. Macroscopic features of SFT/HPC. A, Cross-section of a 5.4 cm frontal lobe SFT demonstrating a fibrotic, firm to rubbery
mass with alternating white to tan areas, reminiscent of a fibroma, or fibrous meningioma. B, Gross appearance of a 2 cm HPC from an
older female. HPC indicates hemangiopericytoma; SFT, solitary fibrous tumor. Please see this image in color online.

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SFT/HPC Dichotomy in CNS

FIGURE 3. Histologic features of typical examples of SFT/HPC. A, SFT with abundant collagen in a hypercellular area (original
magnification 100). B, HPC with typical staghorn or HPC-like vasculature and an indistinct patternless pattern (original magnification  100). C, Higher-power magnification of SFT with bland spindle cells and abundant collagen (original magnification 200).
D, HPC showing tumor cells with round to oval nuclei and a jumbled arrangement in a rich vascular background (original magnification 200). E, Trichrome stain in SFT demonstrating a rich collagen background within the tumor and thin HPC-like vasculature
(original magnification  200). F, Reticulin staining in an HPC shows a rich network of reticulin fibers invested around individual and
small groups of cells (original magnification 100).

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FIGURE 4. Immunohistochemical features of SFT (AC) and HPC (DF). A, Staining for CD34 showing diffuse positivity in tumors cells in
SFT (original magnification 100). B, Staining for bcl-2 in SFT showing diffuse positive staining (original magnification 200). C,
STAT6 nuclear positivity in SFT (original magnification  150). D, CD34 staining in HPC demonstrating positivity limited to vasculature
(original magnification  100). E, Staining for bcl-2 in HPC showing diffuse positive staining (original magnification 200). F, STAT6
nuclear positivity in HPC (original magnification  200). Please see this image in color online.

(Fig. 3F). The tumor cells lack pseudo-inclusions, grooves,

psammoma bodies or calcications, further distinguishing
HPC from meningioma. The staghorn vascular pattern is
often prominent, giving the tumor a richly vascularized
appearance.15,16,21,37 This is a challenging aspect of the
diagnosis, as many spindle cell tumors demonstrate a
staghorn vascular pattern, but can be readily classied
under dierent unique entities.22,39,51
HPCs are diusely reactive for vimentin, bcl-2
(Fig. 4E), and factor XIII.18 The tumor cells often do not
show considerable CD34 positivity (Fig. 4D), but weak and
patchy positivity has been observed for both CD34 and
Leu-7 antibodies. Similar to SFTs, the tumors are negative
for S-100 and EMA. HPCs often exhibit a high Ki-67
labeling, but the range is between 1% and 12%, with a
median of around 4% in most cases.21
Anaplastic, that is, WHO grade III, HPCs dier from
lower-grade tumors in terms of increased mitotic activity,
necrosis, hemorrhage, nuclear atypia, and hypercellularity.16 Among these features, high mitotic rate and
necrosis was historically regarded as independent predictor
or aggressive behavior.16 The grading criteria for anaplastic
tumors require a reassessment with the current understanding of these tumors, rather than the original grading
scheme used by the current WHO classication.16
The discussion of histologic dierences between SFT and
HPC is rendered quite dicult, as the tumors are named on
the basis of their histologic appearance, and trying to assert

histologic dierences becomes a self-fullling prophecy.39,51

Therefore, asserting a distinction on the basis of histologic
dierences, although quite a valid method for many other
entities and variants, requires some other form of validation
before these 2 names are considered as dierent or similar in
nature. Thus, a genetic signature discovered in recent studies
provides a strong link in attempting to unify these entities.

TOWARDS A UNIFYING CONCEPT

Clinically, radiologically, and partly on histologic
grounds, SFTs and HPCs have more overlapping features
than dierences, which were reected in the debates
regarding their classication.22,39,5153 Because these tumors
also show ultrastructural and immunohistochemical similarities, the nal diagnoses for many such tumors remain
controversial, and thus the conclusions of earlier studies on
either tumor category can be called to question. To complicate the matters further, it is quite possible that tumors
previously reported as HPC could have been tumors
belonging to other categories.1416,54 These issues have led
to a diagnostic dilemma and the question as to whether one
can justify the existence of these tumors as distinct
entities.39
Since their incorporation into the WHO Classication
of CNS Tumors, SFT and HPC have been considered distinct entities. SFT was classied as a tumor with a benign
course (ICD-O 8815/0), and HPC was considered locally

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Adv Anat Pathol

Volume 23, Number 2, March 2016

aggressive (9150/1).55 This approach remained unchanged

in the 2007 revision, and HPCs were further divided into
WHO grade II (9150/1) and grade III (9150/3) categories.56
The criteria for anaplasia were adopted from the earlier
studies underscoring the importance of mitoses and
necrosis.10 A clear grading scheme for low-grade and highgrade SFT and HPC still requires more elaborate studies
and a decision as to whether a single entity (SFT/HPC) or 2
distinct entities are analyzed.
Since their initial denitions, the cell of origin for both
SFT and HPC has been debated. HPCs were thought to
arise from the pericytes, whereas SFT was initially considered a mesothelial neoplasm.1,11 However, after the recent
WHO publications, both were classied as broblastic
neoplasms.53 This allowed a reclassication of SFT and
HPC under the same category as extrapleural SFT.57 With
this change in classication, the thin line between HPC and
SFT was abolished, and soft tissue HPC was no longer
recognized as a separate entity.22,23 This idea was further
support with the discovery of a specic fusion protein and
nuclear presence of STAT6 that characterized all tumors
previously recognized as either SFT or HPC.5860
Chmielecki et al60 and Robinson et al58 reported a
fusion on chromosome 12q13 between NGFI-A-binding
protein 2 (NAB2), a polyadenosine-RNA binding protein,
and STAT6, a member of the signal transducer and activation of transcription family. NAB2, an intranuclear
protein, functions as a transcriptional modulator of Zn
nger transcription factors EGR1, 2, and 3.61 STAT6 is
localized to the cytoplasm and is a member of the STAT
family of transcriptional regulators. Activation of Janus
kinases by STAT family members leads to tumorigenesis
through various mechanisms that include angiogenesis,
enhanced survival, proliferation, and immunosuppression.62 Chmielecki et al60 postulate in their study that
NAB2/STAT6 fusion protein translocates into the nucleus
through dimerization and results in STAT6-dependent gene
expression. As both wild-type proteins are localized in
dierent compartments of the cell, the chromosomal rearrangement results in the nuclear relocation of STAT6 initiating a cascade of events leading to transformation of the
repressor nature of NAB2 to an activator leading to
tumorigenesis. This translocation can be demonstrated with
the presence of strong nuclear STAT6 positivity (Figs. 4C,
F) in tumors with the NAB2/STAT6 fusion.58,60 NAB2/
STAT6 fusion has been identied in both SFT and HPC,
and the STAT6 immunostaining has been successfully used
to distinguish these 2 neoplasms from other sarcomas.52,63
The discovery of a common fusion event has provided
further support for those who advocated combining these 2
terms under the same entity. Combined with the similarities
in clinical and pathologic features, this common genetic
alteration was the nal piece of support for combining these
names under the same entity, even though the possibility of
a pericytic neoplasm being a distinct entity was left open for
discussion.23

A FORK ON THE ROAD?

As we all expected that the common fusion event should
put an end to the distinction of SFT from HPC, Barthelme
et al64 reported that the phenotype and the clinical behavior of
SFT changed according to the nature of the NAB2/STAT6
fusion that were discovered in some tumors. In their study of
52 such tumors, the authors identied NAB2ex4-STAT6ex2/3
Copyright

SFT/HPC Dichotomy in CNS

and NAB2ex6-STAT6ex16/17 as the most common fusion

variants. These 2 dierent fusion variants occurred in
patients with quite dierent clinical and pathologic characteristics.64 Tumors with the NAB2ex4-STAT6ex2/3
fusion corresponded more to a thoracic localization, older
age, greater size, and indolent behavior. In contrast, tumors
expressing NAB2ex4-STAT6ex2/3 fusion were associated
with a younger age, smaller size, higher mitotic counts and
more aggressive biology. In addition, the histologic features
of the NAB2ex4-STAT6ex2/3 genotype were typical for the
classic SFT pattern, whereas most of the NAB2ex6STAT6ex16/17 phenotypes demonstrated the classical HPC
features.

FUTURE DIRECTIONS
As we were writing these lines, the debate between the
lumpers and the splitters was still in full swing, and even
though SFT and HPC have come closer to one another,
there are still questions as to whether there are sucient
dierences to continue asserting that dierence for CNS
tumors. There are many aspects of these tumors suggesting
that at the very least, they belong to the same family of
neoplasms, a term that has been used by many others to
describe the similarity of these 2 categories. Yet, since the
beginning of the reports of SFT and HPC in the CNS,
many have noticed certain dierences in clinical behavior
and histologic features as well. The nding of the same
fusion partners strengthens the former view and gives
support to the lumpers. Yet, the dierent fusion types,
which also correspond to certain clinical and histologic
prototypes, give hope to the splitters. It is still unclear
whether the dierent fusion patterns of the NAB2 and
STAT6 genes described in soft tissue examples will apply to
the CNS tumors. We presume that the picture will not be as
clear and denitive as one would expect in the type of
NAB2/STAT6 fusion in these tumors, and the uncertainty
may prevail. Unless a clear line can be drawn between these
tumors in genetic terms, we will need to get used to consolidate 2 CNS entities into 1.

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