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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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Adv Anat Pathol Volume 23, Number 2, March 2016
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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
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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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
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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Copyright r 2016 Wolters Kluwer Health, Inc. All rights reserved.
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.
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Copyright r 2016 Wolters Kluwer Health, Inc. All rights reserved.
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.
REFERENCES
1. Klemperer P, Rabin CB. Primary neoplasms of the pleura.
A report of five cases. Arch Pathol. 1931;11:385412.
2. Stout AP. Localized pleural mesothelioma. Investigation of its
characteristics and histogenesis by the method of tissue culture.
Arch Pathol. 1942;34:951964.
3. Dervan P, Tobin B, OConnor M. Solitary (localized) fibrous
mesothelioma: evidence against mesothelial cell origin. Histopathology. 1986;10:867875.
4. Westra WH, Gerald WL, Rosai J. Solitary fibrous tumor
consistent CD34 immunoreactivity and occurrence in the orbit.
Am J Surg Pathol. 1994;18:992998.
5. Hanau CA, Miettinen M. Solitary fibrous tumor: histological
and immunohistochemical spectrum of benign and malignant
variants presenting at different sites. Hum Pathol. 1995;26:
440449.
6. Chamberlain MH, Taggart DP. Solitary fibrous tumor
associated with hypoglycemia: an example of the Doege-Potter
syndrome. J Thorac Cardiovasc Surg. 2000;119:185187.
7. Doege KW. Fibro-sarcoma of the mediastinum. Ann Surg.
1930;92:955960.
8. Carneiro S, Scheithauer B, Nascimento A, et al. Solitary
fibrous tumor of the meninges: a lesion distinct from fibrous
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9.
10.
11.
12.
13.
14.
15.
16.
17.
18.
19.
20.
21.
22.
23.
24.
25.
26.
27.
28.
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60.
61.
62.
63.
64.
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