Imaging of Solitary Fibrous Tumor/ - PowerPoint Presentation

Slide1

Imaging of Solitary Fibrous Tumor/Hemangiopericytoma Spectrum in Brain, Head & Neck, and Spine—Pathological Correlations

Kwofie

M

1

,

Moritani

T

1

,

Vijapura

C

1

,

Kademian

J

1

, Kirby

P

2

1: Department of Diagnostic Radiology, University of Iowa Hospitals and Clinics, Iowa City, IA

2. Department of Pathology, University of Iowa Hospitals and Clinics, Iowa City, IASlide2

INTRODUCTIONIntracranial solitary fibrous tumors (SFT) and

hemangiopericytomas

(HPC) are generally part of a histologic spectrum of fibroblastic-type mesenchymal

neoplasms, unlike soft tissue counterpart

Based on recent genetic analysis (

NAB2-STAT6

fusion gene), intracranial SFT/HPC are considered a true counterpart of soft tissue SFT/HPC

HPC

of the CNS

have a

recurrence rate reaching >92

% compared to soft tissue SFTSlide3

INTRODUCTIONAccurate diagnosis on imaging has implications for management

Intracranial HPCs are rare as they represent 2 to

4% of

meningeal

tumours

in large series, thus

comprising less

than 1% of all intracranial

tumours

histogenesis

of

intracranial HPC has

been a matter

of controversy

for a long timeSlide4

INTRODUCTIONIntracranial HPC frequently misdiagnosed as meningiomaGeneral agreement

that

intracranial HPC

is more aggressive than

meningioma (greater local recurrence,

extraneural

metastases, irregular or

polylobulated

borders, bone

erosion)Slide5

PURPOSE OF EXHIBITTo describe imaging findings of SFT/HPC in the brain, head & neck, and spine

To describe pathologic correlations

To discuss differential diagnosisSlide6

APPROACHReview SFT/HPC cases at UIHC from 2012 to 2016Review CT and MRI of cases

Review pathology of cases

Compare to recent literature reviewSlide7

CASES: EPIDEMIOLOGY

Sample: living cases SFT/HPC from 2012 to 2016 at UIHC

N: 15

# Male: 6

# Females: 9

Mean Age: 58

Age range: 36-89

# still alive: 14Slide8

CASES: SYMPTOMS AND LOCATION

At least 3 cases were recurrent diseaseSlide9

CASES: PATHOLOGY

Cases 8-11 diagnosed as SFT/HPC spectrumSlide10

HISTOPATHOLOGYSFTs are typically composed

of juxtaposed hyper- and

hypocellular

spindle

cell

proliferation

, dense

collagenous

stroma

, and numerous thin-walled blood vessels with

a staghorn configuration, a histologic

hallmark of

hemangiopericytoma

or

SFTSlide11

HISTOPATHOLOGYSFT have

cellular

component (monotonous appearance and thin-walled branching vessels),

fibrous

component (alternating fibrous areas and

hyalinized

thick-walled vessels), or

both

components with variable

degrees

cellular variant of SFT is indistinguishable from

hemangiopericytomaSlide12

IMMUNOHISTOCHEMISTRYFibrous SFTStrong CD34 positivity

Bcl-2

and vimentin positivity

and

S100

, actin, and keratin

negativity

cellular

variant of

SFT/HPC

weaker CD34 positivitySlide13

CASES: IMAGING FINDINGOn MRI,

low

T2 signal correlates with fibrous content, and

high

T2 correlates with cellular component

Multicystic

changes and flow voids may be seen

Signal

characteristics on diffusion-weighted imaging (DWI) and low apparent diffusion coefficient (ADC) correlate with

cellularity.

Perfusion-weighted

image shows an early enhancement pattern. Slide14

CASES: IMAGING FINDINGOn CT, SFT/HPC are

generally

mildly or moderately

hyperdense

and

enhancing

corresponding to cellular or fibrous componentSlide15

CASES

54-year-old female with dizziness.

Left temporal solitary fibrous tumor WHO grade 2.

(A) Axial CT showing a

hyperdense

medial portion of the lesion

.

(B) Coronal T2 shows

variable high

T2 signal in the lateral portion of the lesion consistent with

cellular components and cystic

changes. (C) and (D)

Axial

and sagittal T1 post contrast, respectively show enhancement. (E) and (F) DWI and ADC, respectively, show focal area of diffusion restrict in the medial aspect of lesion

.Slide16

CASES

54-year-old female with dizziness.

Left temporal solitary fibrous tumor WHO grade 2

.

Tissue

fragments show a cellular proliferation of oval to

spindled, relatively

uniform cells in a swirling pattern, separated by

parallel bundles

of collagen. Occasional thin-walled, dilated vessels

are identified

within the cellular proliferation.  The neoplastic cells

stain positively

for vimentin, CD34, CD99, F13a and BCL-2, and negatively

for

synaptophysin

, chromogranin, CD45, CD20, CD3, EMA,

pankeratin

and

S100. Slide17

CASES55 year-old female with history of headaches and depression

. Left temporal anaplastic

hemangiopericytoma

, WHO grade 3.

(A) Axial T2 show lesion with

multicystic

changes. (B) Axial T1 with low signal in areas of cystic changes. (C) S

agittal

T1 post contrast enhancement of the solid components. (D) and (E) DWI and ADC, respectively, with focal area of diffusion restriction in the posterior aspect of lesion

.Slide18

CASES55 year-old female with history of headaches and depression

. Left temporal anaplastic

hemangiopericytoma

, WHO grade 3.

Sections show fragments of cellular tissue containing

numerous vascular

spaces of varying size and shape with occasional vascular

spaces demonstrating

a staghorn-type architecture.  The intervening stroma

is densely

cellular, demonstrating spindled to ovoid cells with occasional

pleomorphic nuclei with a haphazard orientation.  

Focal

areas

demonstrate a

more solid appearance containing numerous small vascular spaces

and there

are other areas that have greater fibrous stroma associated with

the cells

.  Dense fibrous tissue is present along the edge of some fragments

, consistent

with dura.  Only rare bits of brain parenchyma are seen along

the edges of some fragments, but no brain invasion per se is identified

. Mitoses

are greater than 5 per 10 high power fields in some areas.

There are

focal aggregates of lymphocytes and a few aggregates of

foamy macrophages

.  At the edge of one fragment, there is focal necrosis

associated with hemorrhage.  Slide19

CASES55 year-old female with history of headaches and depression

. Left temporal anaplastic

hemangiopericytoma

, WHO grade 3.

 The

reticulin

stain shows varying degrees

of

reticulin

deposition throughout the tumor.  Vimentin demonstrates

diffuse positivity

.

Immunohistochemical

stains for CD31 and CD34 highlight

the previously

described vascular spaces.  In addition, CD34 is

focally positive

within a minority of tumor cells.  Factor

XIIIa

demonstrates scattered

positivity within tumor cells and CD57 stain demonstrates

rare positivity

within tumor cells.  Staining for EMA shows minimal

weak staining

and an

immunostain

for progesterone receptor shows a very

rare weakly

positive nuclei. Staining for S100, SMSA and

pankeratin

stains

are negative

within the cells of interest. Slide20

CASES43 year-old male with bipolar affective disorder and suicide attempts.

Intraventricular solitary fibrous tumor WHO grade 2

. (A) Axial CT shows

hyperdense

periphery of the lesion. (B) Axial T2 with low and high T2 components corresponding to fibrous and cellular components within the lesion. (C) Axial T1 shows flow voids within lesion. (D) Axial T1 post contrast shows more enhancement of the cellular components compared to the fibrous component. (E)

DWI showed partially restricted diffusion with decreased ADC (ADC not shown).

(F) MR spectroscopy showing low NAA (N-acetyl aspartate

) peak consistent

with tumor of non neuronal

origin,

and

increase

MI

peak which may help

distinguish SFT/HPC from meningioma. MI:

myo

-inositol;

chol

: choline.Slide21

CASES: IMAGING FINDING43 year-old male with bipolar affective disorder and suicide attempts.

Intraventricular solitary fibrous tumor WHO grade 2

.

Sections show

monomorphous

tumor composed of closely packed, randomly

oriented

spindle cells with intervening

fibrocollagenous

to

hypocellular

stroma with interspersed staghorn-type blood vessels. The nuclei are oval

to

elongated with moderately dense chromatin and inconspicuous nucleoli.

The

mitotic activity is up to 1 mitosis/10 high power field. No necrosis,

atypia

or increased cellularity is seen. There is no bone invasion. Slide22

CASES: IMAGING FINDING89 year-old female with a mass in the right brow and diplopia.

Right

sinonasal

malignant solitary fibrous tumor/

hemangiopericytoma

intermediate grade (FNCLCC grade 2/3).

(A) Coronal CT shows erosion of adjacent bone. No calcification within lesion. (B), (C) Coronal T2 andT1 show high and low signals within lesion. (D) Coronal T1 post contrast shows relative enhancement of the periphery of the lesion. (E) DWI shows high signals in the T2 high component and low in fibrous component due to T2 dark through. (F) and (G) H&E stain showing fibrous (F) and cellular components (G), respectively

. Slide23

CASES89 year-old female with a mass in the right brow and diplopia.

Right

sinonasal

malignant solitary fibrous tumor/

hemangiopericytoma

intermediate grade (FNCLCC grade 2/3).

The

mitotic count is 14/10 HPFs.  No necrosis is identified.

Immunohistochemical

studies reveal the precursor solitary fibrous tumor to be strongly positive for CD34, with loss of CD34 expression in the more cellular component, consistent with the above diagnosis.  

Desmin

is focally positive while

pankeratin

is negative.Slide24

CASES56 year-old male with L3 spinal tumor.

Benign solitary fibrous tumor

. (A) Axial CT lumbar spine at L3 level showing

isodense

mass with remodeling of the left L3 poster elements. (B) Axial T2 shows predominantly low T2 signal within lesion. (C)

Coronal

T1 shows normal bone marrow signal. (D)

Axial

T1 post contrast shows enhancement within lesion

.Slide25

CASES56 year-old male with L3 spinal tumor.

Benign solitary fibrous tumor

.

The

tumor focally

expressed

CD34 with no significant expression of

S100,

neurofilament

, SMSA, SMM, EMA, MUC4, beta-catenin (nuclear), or KIT.Slide26

DIFFERENTIAL DIAGNOSISMeningioma (has calcifications, hyperostosis

, broader

dural

tail favors meningioma)

metastasis

, and

other

primary benign or malignant tumorsSlide27

DISCUSSION AND CONCLUSIONIntracranial SFT

and HPC continue to be regarded as different entities

while soft tissue HPC is considered to be SFT in the latest

version of the WHO CNS tumor classification

.

Intracranial

HPC have a recurrence rate reaching >92% compared to soft tissue SFT

possibly secondary to

genetic difference in NAB

2-STAT6

Intracranial

SFT/HPC form

a histopathologic spectrum

Imaging findings such as l

ow T2 and high T2 signals

correlates with

fibrous, cellular and cystic content of SFT/HPC and are helpful in diagnosisSlide28

REFERENCES1. Shanbhogue

AK, Prasad SR, Takahashi N, et al. Somatic and visceral solitary fibrous tumors in the abdomen and pelvis: cross-sectional imaging spectrum.

Radiographics

2011;31:393-408

2. Wu W, Shi JX, Cheng HL, et al.

Hemangiopericytomas

in the central nervous system.

J

Clin

Neurosci

2009;16:519-523

3.

Maekawa

A,

Kohashi

K, Yamada Y, et al. A case of intracranial solitary fibrous tumor/

hemangiopericytoma

with dedifferentiated component.

Neuropathology

2015;35:260-265

4.

Chiechi

MV,

Smirniotopoulos

JG, Mena H. Intracranial

hemangiopericytomas

: MR and CT features.

AJNR Am J

Neuroradiol

1996;17:1365-1371

5.

Fargen

KM,

Opalach

KJ, Wakefield D, et al. The central nervous system solitary fibrous tumor: a review of clinical, imaging and pathologic findings among all reported cases from 1996 to 2010.

Clin

Neurol

Neurosurg

2011;113:703-710

6. Yuzawa

S, Nishihara H, Wang L,

Tsuda

M, et al. Analysis of NAB2-STAT6 Gene Fusion in 17 Cases of Meningeal Solitary Fibrous Tumor/

Hemangiopericytoma

. Review of Literature.

Am J

Surg

Pathol

.

2016 Feb 26. [

Epub

ahead of print

]

7.

Yalcin

CE,

Tihan

T. Solitary

Fibrous Tumor/

Hemangiopericytoma

Dichotomy Revisited: A Restless Family of Neoplasms in the CNS.

Adv

Analt

Pathol

.

2016

Mar;23(2):104-11