AFIP ARCHIVES 759

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From the Archives of the AFIP

Localized Fibrous Tumors of the Pleura1

CME FEATURE Melissa L. Rosado-de-Christenson, MD ● Gerald F. Abbott, MD

See accompanying H. Page McAdams, MD ● Teri J. Franks, MD ● Jeffrey R. Galvin, MD
test at http://
www.rsna.org
/education Eighty-two localized fibrous tumors of the pleura (LFTP) were re-
/rg_cme.html
viewed retrospectively for the clinical, pathologic, and radiologic find-
ings. Forty-four women and 38 men ranged in age from 17 to 78 years
LEARNING
OBJECTIVES (mean, 54.7 years). Sixty-four benign LFTP ranged in size from 2 to
FOR TEST 6 30 cm (mean, 13.2 cm), and 18 malignant tumors ranged from 3 to 23
After reading this cm (mean, 14.4 cm). Forty-eight patients (60%) presented with symp-
article and taking toms. Radiographs of 76 patients demonstrated solitary masses occu-
the test, the reader
will be able to: pying or extending into the inferior hemithorax (79%). Computed
䡲 Describe the clini- tomography (CT) of 78 lesions demonstrated lobular masses (83%)
cal presentation of
patients with LFTP. that formed at least one acute angle (96%) or only acute angles (65%)
䡲 List the pathologic with the adjacent pleura. Heterogeneous lesion attenuation was docu-
features of LFTP and
address difficulties
mented in 88% of enhanced and in 68% of unenhanced CT scans.
and controversies Contrast enhancement was common (62% of cases). Magnetic reso-
about their histologic nance (MR) imaging of 18 lesions demonstrated heterogeneous sig-
diagnosis.
䡲 Define the radio-
nal intensity on both T1- and T2-weighted images (78% and 83%, re-
graphic and cross- spectively). Multiplanar MR imaging allowed visualization of the dia-
sectional imaging
characteristics of
phragm and documentation of an intrathoracic mass in all cases. LFTP
LFTP. are solitary lobular heterogeneous masses that occur in symptomatic
䡲 Discuss existing adults and often affect the inferior hemithorax. Malignant lesions are
difficulties in deter-
mining the pleural radiologically indistinguishable from those with benign histologic char-
origin of these in- acteristics. Radiographic and CT features characteristic of pleural loca-
trathoracic lesions
based on imaging
tion are typically absent.
features.

Abbreviations: AFIP ⫽ Armed Forces Institute of Pathology, H-E ⫽ hematoxylin-eosin, LFTP ⫽ localized fibrous tumors of the pleura, PA ⫽ pos-
teroanterior

Index terms: Lung neoplasms, 66.317, 66.3254 ● Pleura, neoplasms, 66.317, 66.3254

RadioGraphics 2003; 23:759 –783 ● Published online 10.1148/rg.233025165

1From the Department of Radiology and Nuclear Medicine, Uniformed Services University of the Health Sciences, Bethesda, Md (M.L.R.); Depart-
ment of Diagnostic Imaging, Brown Medical School, Rhode Island Hospital, Providence, RI (G.F.A.); Department of Radiology, Duke University
Medical Center, Durham, NC (H.P.M.); and Departments of Pulmonary and Mediastinal Pathology (T.J.F.) and Radiologic Pathology (J.R.G.),
Armed Forces Institute of Pathology, Washington, DC. Received November 19, 2002; revision requested January 13, 2003 and received February 10;
accepted February 13. Address correspondence to M.L.R., 7948 Creek Hollow Rd, Blacklick, OH 43004 (e-mail: rosado@insight.rr.com).

The opinions and assertions contained herein are the private views of the authors and are not to be construed as official nor as reflecting the views of
the Departments of the Air Force, Navy, Army, or Defense.
 760 May-June 2003
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Introduction
Localized fibrous tumors are rare mesenchymal
neoplasms that most commonly affect the pleura
but have also been described in a number of other
locations including the mediastinum and the
lung. Extrathoracic localized fibrous tumors have
been reported in the abdomen, the head and
neck, and the central nervous system. Many
names have been used to designate this neoplasm.
The inconsistent nomenclature that appears in
the published literature emphasizes controversies
regarding the precursor cell for localized fibrous
tumors and their variable microscopic appearance
and unpredictable biologic behavior.
Patients with localized fibrous tumors of the
pleura (LFTP) are typically adults who may
present with symptoms related to local or sys-
temic effects produced by the neoplasm or who
may be entirely asymptomatic. Benign and malig-
nant subtypes of LFTP are recognized. At gross
examination, these are lobular soft-tissue masses,
which are often described as pedunculated lesions
arising from the visceral pleura. Radiologically,
they are intrathoracic masses of variable sizes,
which may not exhibit the classic imaging features
described in extraparenchymal lesions. On cross-
sectional images, they are well-defined lobular
heterogeneous masses. Excision is curative in the
majority of patients, although a small but signifi-
cant number of lesions recur, undergo malignant
transformation, or metastasize.
Although the most common primary pleural
neoplasm is malignant mesothelioma, radiologists
should also be able to identify the much rarer
LFTP, as these two neoplasms have radically dif-
ferent prognoses. To help familiarize radiologists
with the spectrum of radiologic features of LFTP,
we review a large series of LFTP, with emphasis
on the radiographic appearance of these lesions
and their findings at computed tomography
(CT), magnetic resonance (MR) imaging, an-
giography, and ultrasonography (US). We also
describe the clinical presentation of patients with
LFTP and the pathologic characteristics of these
tumors and discuss their therapy and prognosis.
Materials and Methods
A retrospective review of 101 cases of localized
fibrous tumors referred to the Pulmonary and
Mediastinal Section of the Department of Radio-
logic Pathology at the Armed Forces Institute of
Pathology (AFIP) between 1987 and 2001 was
performed. Ten multifocal or recurrent LFTP
and nine extrapleural localized fibrous tumors
occurring in the mediastinum (n ⫽ 6) and in the
lung (n ⫽ 3) were excluded. The remaining 82
LFTP form the basis of this review. Seventy-six
RG f Volume 23 ● Number 3
lesions were evaluated with chest radiography, 78
with chest CT, 18 with MR imaging, 10 with an-
giography, and nine with US. Fifty-five patients
were imaged with chest radiography and CT; 17
with chest radiography, CT, and MR imaging;
and one with chest radiography and MR imaging.
Six patients were evaluated only with CT, and
three with only chest radiography.
For 80 patients, detailed clinical histories were
available and were reviewed for age, gender, and
clinical presentation. Surgical and pathology re-
ports were reviewed to determine tumor size, lo-
cation, pleural surface of origin, presence or ab-
sence of a pedicle, as well as gross and micro-
scopic findings. Results of needle biopsies, when
performed, were also noted. The diagnosis of lo-
calized fibrous tumor was confirmed in every case
through the microscopic evaluation of glass slides
prepared from the resected tissues and reviewed
by an experienced thoracic pathologist in the De-
partment of Pulmonary and Mediastinal Pathol-
ogy at the AFIP. The lesions were classified as
benign or malignant based on established histo-
logic criteria, particularly the presence of more
than four mitotic figures per 10 high-power fields.
All chest radiographs and CT studies were re-
viewed by two thoracic radiologists (M.L.R.,
G.F.A.), and MR imaging studies were reviewed
by three thoracic radiologists (M.L.R., G.F.A.,
H.P.M.). Findings were recorded by consensus.
Chest radiographs were evaluated to determine
lesion size and location within the thorax as well
as border characteristics and presence or absence
of associated findings including pleural effusion
and mass effect.
CT studies were performed at multiple institu-
tions with a variety of scanners and variable scan-
ning techniques. Sixty-six studies were performed
after the administration of intravenous contrast
material, and 25 were performed without contrast
material (13 of these studies were performed be-
fore and after intravenous contrast material ad-
ministration). Lesions were evaluated to deter-
mine location, mobility within the thorax, border
characteristics, attenuation, presence or absence
of calcification, and patterns of contrast enhance-
ment.
MR imaging studies were performed on a vari-
ety of MR imaging equipment, with various com-
binations of axial, coronal, and sagittal planes.
Fifteen lesions were imaged with T1-weighted
and T2-weighted or cine gradient recalled echo
sequences, and three lesions were imaged with
T1-weighted sequences only. Six lesions were
imaged before and after administration of intrave-
nous gadolinium. All lesions were evaluated to
determine morphologic features, signal intensity
characteristics, and patterns of enhancement and
to exclude involvement of adjacent structures.
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Table 1
Clinical Data for 80 Patients with LFTP

Sign or Symptom Benign (n ⫽ 62) Malignant (n ⫽ 18) Total (n ⫽ 80)

Dyspnea 18 (29) 4 (22) 22 (28)
Chest pain 10 (16) 5 (28) 15 (19)
Cough 6 (10) 2 (11) 8 (10)
Upper respiratory infection 2 (3) 1 (6) 3 (4)
Hemoptysis 1 (2) 0 (0) 1 (1)
Sweats 2 (3) 0 (0) 2 (3)
Weakness and fatigue 2 (3) 2 (11) 4 (5)
Lethargy 2 (3) 0 (0) 2 (3)
Confusion 1 (2) 0 (0) 1 (1)
Superior vena cava syndrome 0 (0) 1 (6) 1 (1)
Hypoglycemia 6 (10) 0 (0) 6 (8)
Clubbing 3 (5) 0 (0) 3 (4)
Weight loss 6 (10) 0 (0) 6 (8)
Asymptomatic 27 (44) 5 (28) 32 (40)
Note.—Twenty-one patients had multiple signs or symptoms. Numbers in parentheses are percentages.

Figure 1. Benign LFTP. Photograph of Figure 2. Benign LFTP. Intraoperative photo-

a resected, ovoid LFTP, which arose from
the parietal pleura, shows prominent blood
vessels over the thin serosal lining of the
tumor. The tumor was excised en bloc
with a portion of the adjacent chest wall.
Results
graph shows a pedunculated LFTP, which arises
from the visceral pleura. The surgical forceps
hold the adjacent partially collapsed lung.
through eighth decades of life. Clinical data
(available in 80 of 82 patients) are presented in
Table 1.

Patients and Clinical Presentation Operative Findings

There were 44 women and 38 men who ranged
in age from 17 to 78 years (mean, 54.7 years).
Sixty-four patients had benign LFTP (35 women,
29 men), and 18 had malignant tumors (nine
women, nine men). The patients with benign
LFTP ranged in age from 25 to 78 years (mean
age, 55.9 years), and those with malignant LFTP
ranged in age from 17 to 75 years (mean age, 52.5
years). Eighty-four percent of benign and 78% of
malignant LFTP affected patients in the fifth
Operative reports were available in 78 cases (60
benign, 18 malignant). Complete surgical exci-
sion was performed in all but one lesion. Thirty-
two benign LFTP (53%) arose from the parietal
pleura (Fig 1), and 27 (45%) from the visceral
pleura (Fig 2); in one case, the pleural surface of
origin was not stated. A pedicle connecting the
lesion to the pleura was described in 30 (50%)
 762 May-June 2003 RG f Volume 23 ● Number 3
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Figure 3. Benign
LFTP. Photograph
of a cut section of a
pedunculated LFTP
demonstrates an
ovoid lobular mass
with a thin pedicle
(arrow) by which it
was attached to the Figure 4. Benign LFTP. Photograph of
visceral pleura. Note a benign LFTP demonstrates a spherical
the firm, yellow-tan lobular mass. Note the site of prior broad
appearance of the attachment (arrows) of the lesion to the
tumor. parietal pleura.

lesions (Figs 2, 3); 19 (32%) had a broad attach-

ment to the pleura (Fig 4); and in 11 cases, the
presence or absence of a pedicle was not stated in
the surgical report.
Twelve malignant LFTP (67%) were attached
to the parietal pleura and six (33%) to the visceral
pleura. Eight (44%) lesions had a pedicle, and
eight (44%) did not. In two cases, the nature of
the pleural connection was not specified.

Pathologic Findings
Needle biopsies were performed in 27 lesions (22
benign, five malignant). The diagnosis of LFTP
was established in seven benign and in one malig-
nant LFTP based on the microscopic examina-
tion of the biopsy specimens. The remaining bi-
opsies were inconclusive. There were 64 benign
and 18 malignant LFTP. Pathology reports were
available in 81 cases. Forty-nine LFTP (60%)
were located in the right hemithorax, and 33
(40%) in the left. Benign lesions ranged in size
from 2 to 30 cm (average size, 13.2 cm), and ma-
lignant lesions ranged in size from 3 to 23 cm (av-
erage size, 14.4 cm). Macroscopic descriptions of
63 benign LFTP noted gross evidence of hemor-
rhage in 27 (43%), cystic change in 14 (22%),
and necrosis in three (5%) (Fig 5). Seven malig-
nant LFTP (39%) exhibited hemorrhage, four
Figure 5. Benign LFTP. Photograph of a cut section
of a benign LFTP demonstrates an ovoid lobular mass
with extensive internal hemorrhage and necrosis.
(22%) had necrosis, and cystic change was found
in three (17%).
Imaging Findings
Chest Radiography.—Seventy-six LFTP (59
benign, 17 malignant) were imaged with radiog-
raphy. Sixty (79%) lesions extended into or occu-
pied the inferior hemithorax (Fig 6). Twenty-one
of these lesions (18 benign, three malignant)
abutted the ipsilateral hemidiaphragm, con-
formed to its shape, and simulated diaphragmatic
elevation or eventration (Fig 7). The majority of
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Figure 6. Benign LFTP in an asymptomatic 29-year-old woman. Posteroanterior (PA) (a) and lateral (b)
chest radiographs demonstrate an ovoid, slightly lobular mass in the left inferior hemithorax that abuts the left
hemidiaphragm.

Figure 7. Benign LFTP in a 62-year-old woman with right-sided chest pain. PA (a) and lateral (b) chest ra-
diographs demonstrate a rounded well-defined mass of the right inferior hemithorax that conforms to the shape
of the diaphragm and mimics diaphragmatic elevation. The LFTP was discovered at abdominal CT.
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Figure 8. Benign LFTP in a 73-year-old woman with dyspnea,

weight loss, and hypoglycemia. (a, b) PA (a) and lateral (b)
chest radiographs demonstrate a large mass that occupies more
than half of the right hemithorax. The superior border of the le-
sion is lobular and well defined. (c) Photograph of a cut section
of the resected mass demonstrates a lobular contour and a
whorled nodular fibrous appearance. Scale is in centimeters.

the lesions that produced this finding (19 cases)

occurred in the right hemithorax. Sixty-nine
lesions (91%) had at least one well-defined bor-
der (Figs 6 – 8). Differential visualization of the
lesion’s borders, a radiographic finding de-
scribed as characteristic of pleural mass lesions,
was noted in 25 (33%) cases (Fig 9). Radio-
graphic demonstration of tumor extension into
the fissure was seen in three (4%) benign le-
sions (Fig 9).

Figure 9. Benign LFTP in an asymptomatic 54-year-old man. (a, b) PA (a) and lateral (b) chest radiographs ‹
demonstrate an ovoid mass in the left hemithorax. The inferior border of the lesion is well defined, but its superior
border is ill defined (a). The lesion is aligned along the course of the major fissure on the lateral radiograph (b).
(c, d) PA (c) and lateral (d) chest radiographs obtained 6 years later demonstrate interval growth of the lesion and a
change in its position within the thorax that confirms its pleural location. (e) Contrast-enhanced chest CT scan (lung
window) shows the superior border of the lesion, its extension into the fissure, and a pedicle (arrow) that connected
the mass to the fissural visceral pleura. (f) Contrast-enhanced chest CT scan (mediastinal window) demonstrates the
heterogeneously enhancing lobular mass, which forms obtuse and acute angles with the adjacent pleura. Note associ-
ated ipsilateral pleural thickening or fluid.
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Figure 10. Benign LFTP in a 72-year-old man

with dyspnea. (a, b) PA (a) and lateral (b) chest
radiographs demonstrate an enormous mass that
occupies almost the entire right hemithorax and
produces mass effect on the mediastinum. Note the
well-defined lobular superior border of the lesion
and the right pleural effusion. (c) Contrast-en-
hanced chest CT scan (mediastinal window) dem-
onstrates the large heterogeneously enhancing mass
in the right hemithorax, which produces mass ef-
fect on the mediastinum as well as atelectasis of the
adjacent lung. Note geographic areas of low attenu-
ation within the lesion.

Twenty (34%) benign and five (29%) malig-

nant LFTP occupied more than half a hemitho-
rax (Figs 8, 10), and only two lesions (one be-
nign, one malignant) filled the entire hemithorax.
Seventeen (29%) benign tumors and six (35%)
malignant lesions produced mass effect on the
adjacent structures (Fig 10). Twelve (20%) be-
nign and four (24%) malignant LFTP were asso-
ciated with an ipsilateral pleural effusion (Fig 10).
Chest wall involvement manifesting as osseous
changes in adjacent ribs was seen in only two
(3%) cases (one benign, one malignant).
Computed Tomography.—Seventy-eight
LFTP (61 benign, 17 malignant) were evaluated
with CT, and morphologic features are summa-
rized in Table 2. Twenty benign and five malig-
nant LFTP were imaged before the administra-
tion of intravenous contrast material. Twelve be-
nign tumors (60%) and all five malignant lesions
(100%) exhibited heterogeneous attenuation ei-
ther due to intrinsic areas of low attenuation (Figs
10, 11) or because of intralesional calcification
(Fig 11). In seven benign LFTP (35%), heteroge-
neity was characterized as geographic (n ⫽ 6),
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Table 2
CT Morphologic Features of 78 LFTP

Morphologic Feature Benign (n ⫽ 61) Malignant (n ⫽ 17) Total (n ⫽ 78)

Lobular borders 51 (84) 14 (82) 65 (83)
Smooth borders 10 (16) 3 (18) 13 (17)
Only acute angles 40 (66) 11 (65) 51 (65)
At least one acute angle 58 (95) 17 (100) 75 (96)
At least one obtuse or right angle 20 (33) 6 (35) 26 (33)
Only obtuse or right angle 2 (3) 0 (0) 2 (3)
Smoothly tapered border 20 (33) 6 (35) 26 (33)
Fissural extension 11 (18) 1 (6) 12 (15)
Pedicle 1 (2) 0 (0) 1 (1)
Change in position 2 (3) 2 (12) 4 (5)
Calcification 14 (23) 6 (35) 20 (26)
Punctate 9 (64*) 5 (83*) 14 (70*)
Linear 6 (43*) 1 (17*) 7 (35*)
Coarse 5 (36*) 1 (17*) 6 (30*)
Mass effect on mediastinum 22 (36) 10 (59) 32 (41)
Atelectasis 40 (66) 13 (76) 53 (68)
Pleural effusion 21 (34) 8 (47) 29 (37)
Nodular attenuation 16 (26) 6 (35) 22 (28)
Chest wall involvement 3 (5) 3 (18) 6 (8)
Rib sclerosis 3 (100†) 0 (0†) 3 (50†)
Soft-tissue involvement 0 (0†) 3 (100†) 3 (50†)
Note.—Angles could not be determined in one case of supradiaphragmatic LFTP. Numbers in parentheses are
percentages.
*Denotes relative percentages of different types of calcification.
†Denotes relative percentages of different types of chest wall involvement.

Figure 11. Benign LFTP in an asymptomatic 39-year-old man. Unenhanced chest CT scans (mediastinal
window) demonstrate a soft-tissue mass of the left inferior hemithorax with well-defined lobular borders. The
mass forms acute angles with the adjacent pleural surface and contains a geographic area of low attenuation
(a) and multifocal coarse calcifications (b).
 768 May-June 2003 RG f Volume 23 ● Number 3

RadioGraphics Figure 12. Malignant LFTP in a 30-year-old man with chest pain. (a) PA chest radiograph demon-
strates a well-marginated rounded mass in the right paravertebral inferior hemithorax. (b) Unenhanced
chest CT scan (mediastinal window) demonstrates a well-defined heterogeneous mass with focal areas of
low attenuation and subtle punctate calcification. Note that the lesion forms acute angles with the adja-
cent pleura. (c) Selective angiogram demonstrates exuberant tumor vascularity. (d) Photograph of a cut
section of the resected gross specimen demonstrates a heterogeneous spherical mass with extensive ne-
crosis, hemorrhage,and cystic change.

Figure 13. Benign LFTP in

an asymptomatic 71-year-old
woman. Targeted unenhanced
chest CT scan (mediastinal
window) demonstrates a ho-
mogeneous ovoid lobular soft-
tissue mass abutting the de-
scending aorta. Although the
lesion forms acute angles with
the pleura, a smoothly taper-
ing margin (arrow) is also
seen.
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Figure 14. Benign LFTP in a 54-year-old woman
with dyspnea. Contrast-enhanced chest CT scan (me-
diastinal window) demonstrates a heterogeneously en-
hancing soft-tissue mass of the left inferior hemithorax
with internal focal and linear areas of low attenuation.
rounded (n ⫽ 4), or linear (n ⫽ 2) areas of low
attenuation within the tumor (Figs 10, 11). All
five malignant neoplasms (100%) exhibited geo-
graphic (n ⫽ 4) or rounded (n ⫽ 2) areas of low
attenuation (Fig 12). Eight benign LFTP (40%)
exhibited homogeneous attenuation (Fig 13).
Sixty-six LFTP (53 benign, 13 malignant)
were imaged after the administration of intrave-
nous contrast material. Lesion enhancement was
defined as an increase in attenuation compared
with the attenuation of the lesion on the unen-
hanced CT scans or as attenuation greater than
that of the adjacent chest wall musculature. En-
hancement was seen in 41 (62%) lesions (37 be-
nign, four malignant) (Fig 14). Fifty-eight (88%)
lesions exhibited heterogeneous attenuation.
Thin linear foci of increased attenuation consis-
Rosado-de-Christenson et al 769
Figure 15. Benign LFTP in a 54-year-old woman
with chest pain and dyspnea. Contrast-enhanced chest
CT scan (mediastinal window) demonstrates an enor-
mous heterogeneously enhancing soft-tissue mass in
the right hemithorax that produces mass effect on the
heart. Note the serpiginous branching linear areas of
enhancement consistent with intralesional vessels and
the geographic and linear areas of low attenuation
within the lesion. Enhancing portions of the lesion have
a nodular pattern of attenuation.
tent with intratumoral vessels were seen in 10
(15%) cases (six benign, four malignant) (Fig
15). Fifty-four (82%) lesions exhibited heteroge-
neous areas of low attenuation. In 40 benign
LFTP (75%) low-attenuation areas were geo-
graphic (n ⫽ 33), focal (n ⫽ 27), or linear (n ⫽ 8)
(Figs 10, 14, 16, 17). All 13 malignant LFTP
(100%) exhibited areas of low attenuation charac-
terized as geographic (n ⫽ 11) or focal (n ⫽ 6).
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Figure 16. Benign LFTP in an asymptomatic 62-year-old man. (a) Contrast-enhanced chest
CT scan (mediastinal window) demonstrates a lobular heterogeneous soft-tissue mass with geo-
graphic and focal areas of low attenuation. Note that the lesion forms acute angles with the adja-
cent pleura. (b) Photograph of a cut section of the gross specimen demonstrates a well-circum-
scribed lobular mass with a focal area of necrosis that corresponds to the low-attenuation area seen
at CT.

Figure 17. Benign LFTP in a 77-year-old man with dyspnea, cough,

and weight loss. (a) Contrast-enhanced chest CT scan (mediastinal
window) demonstrates a large heterogeneous soft-tissue mass of lobu-
lar borders with a large ovoid area of focal low attenuation as well as
smaller foci of low attenuation. (b) Sagittal US scan through the left
upper quadrant allows visualization of the spleen and diaphragm as
well as the supradiaphragmatic hypoechoic LFTP. (c) Photograph of a
cut section of the gross specimen demonstrates a large lobular hetero-
geneous soft-tissue mass with a nodular cut surface as well as areas of
necrosis (arrow) and hemorrhage (arrowhead).
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Figure 18. Benign LFTP in an asymptomatic 68-year-old man. (a) Coronal T1-weighted MR
image demonstrates a large lobular heterogeneous mass of intermediate signal intensity with linear
areas of high signal intensity. Note mass effect on the ipsilateral hemidiaphragm and mediastinum.
(b) Coronal T1-weighted MR image obtained after intravenous administration of gadolinium
demonstrates heterogeneous patchy multifocal enhancement within the lesion.

Figure 19. Benign LFTP in a 48-year-old man with chest pain. (a) Sagittal T1-weighted MR
image demonstrates a lobular ovoid mass of intermediate signal intensity located in the right infe-
rior hemithorax. Note mass effect on the liver and focal chest wall invasion. (b) Sagittal T2-
weighted MR image at the same level demonstrates heterogeneous high signal intensity with flow
void areas within the lesion that represent vessels and intrinsic low-signal-intensity septa (arrow).

MR Imaging.—Eighteen LFTP (13 benign, five
malignant) were evaluated with MR imaging.
Coronal or sagittal images were obtained in all
cases. Eleven (85%) benign and four (80%) ma-
lignant LFTP manifested as lobular masses (Fig
18). Chest wall involvement was exhibited by one
benign (8%) and one malignant LFTP (20%)
(Fig 19), and diaphragm involvement was evident
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Figure 20. Malignant LFTP in a 53-year-old woman with abdominal pain. (a) Axial T1-weighted MR
image shows a mass of intermediate to low signal intensity in the right diaphragmatic region, which pro-
duces mass effect on the diaphragm and indents the liver. (b) Sagittal T1-weighted MR image obtained
after intravenous administration of gadolinium shows heterogeneous contrast enhancement and exten-
sion through the diaphragm. Note that a large portion of the mass is intrahepatic and thus a tumor of
liver origin would have to be considered. Diaphragm invasion was documented at surgery.

Table 3
MR Imaging Signal Intensity Characteristics of 18 LFTP

Signal Intensity Characteristic Benign (n ⫽ 13) Malignant (n ⫽ 5) Total (n ⫽ 18)

Heterogeneous on T1-weighted images 10 (77) 4 (80) 14 (78)
Heterogeneous on T2-weighted images 10 (77) 5 (100) 15 (83)
Areas of low to intermediate signal intensity on
T1-weighted images 6 (46) 4 (80) 10 (56)
Areas of low to intermediate signal intensity on
T2-weighted images 3 (23) 1 (20) 4 (22)
Areas of high signal intensity on T1-weighted images 6 (46) 2 (40) 8 (44)
Areas of high signal intensity on T2-weighted images 8 (62) 5 (100) 13 (72)
Increase in signal intensity seen on T2-weighted im-
ages, compared with that seen on T1-weighted
images 7 (54) 4 (80) 11 (61)
Vascular structures 0 (0) 2 (40) 2 (11)
Low-signal-intensity septa on T2-weighted images 10 (77) 4 (80) 14 (78)
Low-signal-intensity capsule on T2-weighted images 0 (0) 1 (20) 1 (6)
Note.—Numbers in parentheses are percentages.

in two malignant LFTP (40%) (Fig 20). Mass
effect on the diaphragm was documented in 12
(67%) cases (eight benign, four malignant) on
coronal or sagittal images (Figs 18, 19). Patterns
of signal intensity are summarized in Table 3. Six
lesions (five benign, one malignant) were imaged
after the administration of intravenous gadolin-
ium, and all exhibited heterogeneous contrast
enhancement (Figs 18, 20).
 RG f Volume 23 ● Number 3
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Angiography.—Ten LFTP were studied with
angiography (seven benign, three malignant). All
lesions (100%) exhibited exuberant tumor vascu-
larity (Fig 12c).
Ultrasonography.—Nine benign LFTP were
imaged with US. Six masses (67%) were hetero-
geneous and exhibited hypoechoic and hypere-
choic areas but no cysts or calcification. US visu-
alization of the diaphragm was documented in
five cases, established the intrathoracic location of
the lesion in all, and showed mass effect without
invasion in three (Fig 17b).
Radiologic Reports.—A review of the radiologic
reports generated at the time the lesions were
originally imaged (n ⫽ 80) showed that the diag-
nosis of LFTP was not mentioned in the preop-
erative differential diagnosis for 43 (54%) cases
(33 benign, 10 malignant). The specific diagnosis
of LFTP was included in the differential diagno-
sis for 21 (26%) lesions (15 benign, six malig-
nant), and 16 (20%) lesions (14 benign, two ma-
lignant) were characterized as pleural in location.
Discussion
Primary pleural neoplasms are rare and represent
less than 5% of all pleural neoplasia. The most
common primary pleural neoplasm is malignant
mesothelioma, a highly aggressive malignancy
that is associated with asbestos exposure. Malig-
nant mesothelioma is characterized by diffuse
circumferential pleural involvement and a poor
prognosis (1). The first report of a primary pleu-
ral neoplasm dates back to 1767 and is attributed
to Lieutaud (2). Wagner published the first mi-
croscopic description of a primary diffuse pleural
neoplasm in 1870 and proposed that the tumor
was derived from the endothelium of the pleural
lymphatics (3,4). In 1931, Klemperer and Rabin
classified primary pleural neoplasms as localized
and diffuse types and proposed that a subme-
sothelial mesenchymal cell was the cell of origin
for the localized type (5). LFTP are rare neo-
plasms; only over 600 cases have been reported in
the scientific literature (6). In a 1978 review of 60
cases of LFTP, Okike and colleagues (7) reported
a prevalence of only 2.8 cases per 100,000 regis-
trations at their institution, the Mayo Clinic.
Rosado-de-Christenson et al 773
Pathogenesis
Different theories about the cell of origin for
LFTP have been proposed and probably account
for the varied nomenclature used to designate this
rare neoplasm. Localized mesothelioma, fibrous
mesothelioma, benign fibrous mesothelioma, lo-
calized fibrous mesothelioma, benign mesothe-
lioma, benign localized mesothelioma, fibrosing
mesothelioma, and mesothelial fibroma represent
nomenclature that alludes to early theories sup-
porting a mesothelial cell of origin (3,8 –12).
Terms such as subpleural fibroma, submesothe-
lial fibroma, and submesothelioma suggest a sub-
mesothelial precursor cell (3,10). Pleural fibroma,
benign pleural fibroma, pleural fibromyxoma,
solitary fibrous tumor of the pleura, and localized
fibrous tumor of the serosal cavities are additional
terms that have been used to designate these le-
sions (10,11,13). The currently accepted nomen-
clature is localized fibrous tumor of the pleura,
and a derivation from submesothelial mesenchy-
mal cells with a fibroblastic differentiation is gen-
erally acknowledged (11).
Lesions of similar histologic characteristics
have been reported in extrapleural thoracic loca-
tions, including the mediastinum, lung, pericar-
dium, and heart (14 –16). Yousem and Flynn
(17) described three intrapulmonary localized
fibrous tumors and suggested a common origin
for this subset of lesions from tissues in the inter-
lobular septa. Localized fibrous tumors have also
been reported in the abdomen (liver, peritoneum,
and retroperitoneum) and in the meninges, orbit,
thyroid, salivary gland, and the soft tissues includ-
ing the breast (15,16,18). It has been suggested
that the upper respiratory tract may be a preferred
extrathoracic location, with reports of localized
fibrous tumors arising in the nose, paranasal si-
nuses, parapharyngeal tissues, nasopharynx and
epiglottis (15,19).
Clinical Characteristics
LFTP affect male and female patients, with a
slight female predominance reported in some
studies (3). Women represented 55% of patients
with benign LFTP and 50% of patients with ma-
lignant LFTP in our series. Affected patients
display a wide age range (5– 87 years), with the
 774 May-June 2003
RadioGraphics majority of cases reported in the 6th and 7th de-
cades of life and a reported mean age of 50 –57
years (3,20,21). Although 83% of the patients in
our series were older than 40 years, only 41%
were in the 6th and 7th decades of life. LFTP are
not thought to be related to exposure to cigarette
smoke, asbestos, or other environmental pollut-
ants (22,23). However, there are at least two case
reports of patients with LFTP who were exposed
to asbestos and one of a patient who developed a
LFTP following thoracic irradiation for the treat-
ment of a chest wall keloid (24,25).
Up to 50% of patients with LFTP, particular-
ly those with small neoplasms, may be entirely
asymptomatic, and the tumor is discovered inci-
dentally because of radiographs obtained for
other reasons (3,21,26). When signs and symp-
toms (particularly digital clubbing and hypertro-
phic osteoarthropathy) are present, they are usu-
ally associated with larger tumors and may occur
more frequently in association with malignant
subtypes of LFTP (7,27). Interestingly, the aver-
age size of LFTP that affected asymptomatic indi-
viduals in our series was 10.5 cm, whereas the
average size of those that occurred in symptom-
atic patients was 16.6 cm. Studies of patients with
LFTP published from 1942 to 1972 reported that
72% of patients were symptomatic at presenta-
tion, whereas studies published between 1973
and 1980 reported symptoms in only 54% of pa-
tients. This decrease in the prevalence of symp-
toms in patients with LFTP may relate to more
widespread imaging of asymptomatic populations
and the resultant detection of a larger number of
incidental tumors (3).
Reported symptoms are similar to those exhib-
ited by our patients and include respiratory and
thoracic complaints such as cough, dyspnea, he-
moptysis, chest pain, chest heaviness, and the
sensation of a mass moving within the chest
(3,21) (Table 1). Abdominal pain has been re-
ported in patients with supradiaphragmatic tu-
mors (28). Systemic complaints may occur and
include chills, sweats, weakness, and weight loss
(3). Paraneoplastic syndromes such as hypoglyce-
mia, digital clubbing, and hypertrophic osteoar-
thropathy are uncommon, but when they are as-
sociated with an intrathoracic mass they may sug-
gest the diagnosis of LFTP (4,29,30). These
RG f Volume 23 ● Number 3
systemic effects typically occur with large tumors
and generally resolve after excision of the neo-
plasms but may recur with subsequent tumor re-
currences (12,30 –32). Non–islet cell tumor hypo-
glycemia has been described in association with
both epithelial and mesenchymal neoplasms, in-
cluding LFTP (30,32–34). Many theories have
been proposed to explain this phenomenon, in-
cluding increased glucose consumption by the
tumor; proliferation of insulin receptors; impaired
growth hormone counter-regulatory responses to
hypoglycemia; and tumor secretion of insulin-like
substances, such as insulin-like growth factor 2
(IGF-2) and a high molecular weight (“big”)
IGF-2 (4,30,32,35–38). In some patients, hypo-
glycemic coma may be the presenting manifesta-
tion of a LFTP (34). Hypertrophic osteoar-
thropathy may relate to hyaluronic acid produc-
tion and its osteolytic effects and is reported in
17%–35% of cases (4,8,37,39). Although hyper-
trophic osteoarthropathy has been described in
cases of lung carcinoma, malignant pleural me-
sothelioma, and even empyema and other tho-
racic infections, LFTP may produce it more com-
monly (4). Interestingly, none of our patients pre-
sented with hypertrophic osteoarthropathy.
Gross Features
LFTP are typically solitary lesions with rare oc-
currences of conglomerate or multifocal masses.
Sixty-six percent to 70% of localized fibrous tu-
mors arise from the visceral pleura, and nearly
half are pedunculated, with the vascular supply to
the tumor contained within the pedicle (21,40)
(Figs 2, 3). Although a pedicle was present in
49% of our cases, 55% of the lesions arose from
the parietal pleural surface (Fig 1). This discrep-
ancy may relate to selection bias, because our
cases were collected through consultation for sec-
ond-opinion diagnosis or through case contribu-
tions by residents attending AFIP courses, and
only cases with imaging studies were included.
The AFIP series published by England and col-
leagues (21), based on a larger number of cases
(n ⫽ 223) submitted to the Institute before 1988,
documented origin of LFTP from the visceral
pleura in 66% of cases. Adhesions to the adjacent
pleural surfaces and pericardium are common
(3,21). LFTP are usually well-circumscribed
masses with lobular or smooth external surfaces
and a thin, glistening translucent serosa through
 RG f Volume 23 ● Number 3 Rosado-de-Christenson et al 775
RadioGraphics

Figure 21. Benign LFTP. (a, b) High-power (original magnification, ⫻400) (a) and intermediate-power
(original magnification, ⫻200) (b) photomicrographs (hematoxylin-eosin [H-E] stain) demonstrate the bland,
low-grade appearance of spindle-shaped tumor cells (a) that are arranged in a haphazard or so-called pattern-
less pattern (b), the classic appearance of localized fibrous tumors. (c, d) High-power photomicrographs
(original magnification, ⫻400; H-E stain) of the same neoplasm demonstrate the variable cellularity character-
istic of localized fibrous tumors. A hypercellular area (c) is composed of abundant spindle-shaped cells with a
paucity of collagen fibers, whereas a hypocellular area (d) demonstrates abundant collagen (*) between tumor
cells.

which a network of prominent blood vessels may
be seen (Fig 1) (40). The cut surface is firm,
pink-white or yellow-tan and often exhibits a
whorled or nodular pattern (Figs 3, 8c). Areas of
necrosis, hemorrhage, or cystic degeneration may
be evident, particularly in large or malignant le-
sions (Figs 5, 12d, 16b, 17c).
Histologic Characteristics
England and colleagues (21) proposed that LFTP
originated from the submesothelial connective
tissues and suggested a primitive multipotential
cell of mesenchymal differentiation as the cell of
origin. At histologic analysis, localized fibrous
tumors appear as low-grade neoplasms of variable
cellularity. The tumor cells are ovoid to spindle-
shaped with round to oval nuclei, an evenly dis-
tributed fine chromatin, inconspicuous nucleoli,
and bipolar faintly eosinophilic cytoplasm with
indistinct cell borders (Fig 21). Nuclear pleomor-
phism is minimal and mitoses are usually rare or
 776 May-June 2003 RG f Volume 23 ● Number 3

RadioGraphics

Figure 22. Benign LFTP. High-power photomicro-
graph (original magnification, ⫻400; H-E stain) of a
benign LFTP demonstrates the characteristic ropy col-
lagen that occurs in the hypocellular areas of these
lesions.
Figure 23. Benign LFTP. Low-power photomicro-
graph (original magnification, ⫻100; H-E stain) dem-
onstrates the second most commonly encountered his-
tologic pattern (hemangiopericytoma-like) character-
ized by staghorn-like vessels.

absent. Cellularity is variable and is inversely re-

lated to collagen content (21). Collagen ranges
from wispy fibrils surrounding tumor cells in hy-
percellular areas to thick, dense, wirelike or
“ropy” collagen forming sclerotic zones in hypo-
cellular areas (Figs 21, 22). Tumors are usually
well vascularized with vessels of varying sizes
(23). Degenerative features including myxoid
change and degeneration of collagen may occur
(41). Microscopic examination reveals a variety of
architectural patterns, with the most frequent be-
ing an intermingling of tumor cells and collagen
in a random fashion, the so-called patternless pat-
tern (Fig 21a) (41). The second most common
pattern is characterized by hypercellular zones
that contain a network of open anastomosing or Figure 24. Malignant LFTP. High-power photomi-
staghorn-shaped vessels that result in a heman- crograph (original magnification, ⫻400; H-E stain)
giopericytoma-like appearance (Fig 23). Less fre- demonstrates five mitotic figures within this one high-
power field. Note the nuclear pleomorphism that may
quently, localized fibrous tumors may adopt an-
also characterize malignant LFTP.
giofibroma-like (numerous small and medium-
sized vessels), fibrosarcoma-like (herringbone),
monophasic variant of synovial sarcoma–like S-100 protein. However, distinction from other
(densely cellular fascicles), and neural (wavy nu- soft-tissue neoplasms can be difficult, particularly
clei, palisading) patterns (41). in cases of hemangiopericytoma, the monophasic
Because of the highly variable light micro- variant of synovial sarcoma, and malignant fi-
scopic appearances of LFTP, the histologic differ- brous histiocytoma (41). Of these considerations,
ential diagnosis is broad and includes primary and hemangiopericytoma is perhaps the most difficult
metastatic spindle cell carcinoma, spindle cell entity to distinguish, and differentiation may not
melanoma, sarcomatoid mesothelioma, and a be possible with light microscopy in all instances
wide spectrum of primary and metastatic soft- (21). Commonly used criteria for malignancy are
tissue neoplasms. The exclusion of other tumors those described by England and colleagues (21),
is relatively straightforward with the aid of immu- which include (a) high cellularity with crowding
nohistochemical studies. Localized fibrous tu- and overlapping of nuclei, (b) high mitotic activ-
mors are immunoreactive with CD34 and bcl-2 ity of more than four mitotic figures per 10 high-
but typically lack expression for cytokeratin and power fields, and (c) pleomorphism (Fig 24). Be-
cause of the diversity of histologic patterns dis-
played by LFTP, even large biopsy specimens
 RG f Volume 23 ● Number 3
RadioGraphics
Figure 25. Benign LFTP in a 55-year-old man un-
dergoing evaluation for HIV disease. Unenhanced
chest CT scan (bone window) demonstrates a small
well-defined mass of the left superior hemithorax that
forms obtuse angles with the adjacent pleural surfaces.
may pose a significant diagnostic problem, and
biopsy samples obtained with percutaneous tech-
niques may be insufficient for diagnosis. In fact,
only 32% of the benign and 20% of the malignant
LFTP in our series were accurately diagnosed
with needle biopsy. That these lesions remain
problematic even for the experienced general sur-
gical pathologist is attested to by the fact that over
one-half of the benign tumors and three-fourths
of the malignant lesions in the England et al study
(21) were initially misclassified.
Imaging Appearance
Radiography.—Chest radiographs of patients
with small LFTP typically demonstrate a well-
defined, lobular, solitary nodule or mass, which
may appear to be in the lung periphery and typi-
cally abuts a pleural surface or is located within a
fissure (7,26) (Fig 9a, 9b). In 1977, Ellis (42)
described the incomplete border sign of ex-
trapleural lesions to differentiate them from pa-
renchymal masses. Because extrapleural lesions
may exhibit tapered borders, en face radiography
results in an ill-defined margin. Focal pleural
masses may also exhibit this “incomplete border”
in addition to sharply defined margins when im-
aged tangentially (42,43). This discrepancy in
margin visualization may allow the suggestion of a
pleural location (20,42,43), but it was seen in
only 33% of the LFTP in our series (Fig 9). Pe-
dunculated tumors may show mobility within the
pleural space or changes in shape and orientation
on fluoroscopy or with changes in the patient’s
position (20,44). Although this finding is a reli-
able indicator of pleural location, it is not fre-
quently demonstrated (Fig 9) (44).
LFTP are reported to affect predominantly the
middle and inferior hemithorax (11,45) (Figs
Rosado-de-Christenson et al 777
6 – 8, 12), and when in contiguity with the dia-
phragm, an LFTP may mimic diaphragmatic el-
evation (Fig 7). LFTP may exhibit slow growth
over time (Fig 9) and may reach enormous sizes
(46). Large lesions or those that arise from para-
mediastinal pleural surfaces may manifest with
typical radiographic features of pulmonary or me-
diastinal masses, respectively (14,47). Pleural ef-
fusion is reported in 6%–17% of cases and may
obscure a lesion in the inferior hemithorax (Fig
10) (11,18,21,48). To our knowledge, radio-
graphic evidence of chest wall involvement by
LFTP has been previously documented in only
one case report (45).
Computed Tomography.—CT of small LFTP
typically demonstrates homogeneous, well-de-
fined, noninvasive, lobular, soft-tissue masses,
which typically abut a pleural surface, may form
obtuse angles against the adjacent pleura, or may
be located within a fissure (Figs 9, 25) (11,26,
49). Large lesions are typically heterogeneous and
may not exhibit CT features suggestive of focal
pleural tumors (50). In fact, LFTP usually form
acute angles against adjacent pleural surfaces
(11,26,46,50) (Figs 9, 11–13, 16) (Table 2).
Only 33% of the LFTP in our series exhibited at
least one obtuse or right angle, and only 3% ex-
hibited only obtuse angles (Figs 9, 25). Interest-
ingly, the average sizes of the latter subsets of le-
sions (10.2 and 4.0 cm, respectively) were smaller
than the average size of all the LFTP in the series.
Dedrick and colleagues (26) stated that a smoothly
tapering margin adjacent to the tumor (seen in
five of their six cases) was a more characteristic
finding that could help establish the pleural loca-
tion of these tumors. However, this finding was
demonstrated in only one-third of our cases (Fig
13). Dedrick and colleagues (26) also reported
diaphragmatic crural thickening in one of their
cases and a fissural location in another. Although
fissural extension of the mass may be helpful in
establishing lesion location, it is an uncommon
finding (Fig 9). In addition, CT findings may not
allow differentiation of a fissural LFTP from a
peripheral lung lesion or exclusion of a tumor of
abdominal origin when the inferior hemithorax is
affected (49,51).
CT visualization of a pedicle is rarely reported
(Fig 9). However, a pedicle was indirectly dem-
onstrated in four of 16 patients with LFTP stud-
ied by Mendelson and colleagues through CT
documentation of mobility within the thorax (46)
(Fig 9). Masses originating in the mediastinal
pleura may mimic mediastinal neoplasms. In fact,
 778 May-June 2003 RG f Volume 23 ● Number 3

RadioGraphics

Figure 26. Malignant LFTP in an asymptomatic 37-year-old man. (a) PA chest radiograph demonstrates a lobular
mass in the right cardiophrenic angle. Note poor visualization of the superolateral border of the lesion. (b) Unen-
hanced chest CT scan (mediastinal window) demonstrates a spherical heterogeneous right paracardiac mass that pro-
duces mass effect on the heart and perilesional atelectasis. Note the small ipsilateral right pleural effusion.

Figure 27. Benign LFTP in a 27-year-old woman with chest pain. Contrast-enhanced chest CT scans (medi-
astinal window) demonstrate a heterogeneously enhancing mass of the left middle hemithorax that produces
pressure erosion on an adjacent rib. The lesion forms obtuse and acute angles with the adjacent pleura and ex-
hibits a smoothly tapering margin (b). Note enhancing serpiginous linear structures within the lesion (a),
which likely represent vascular structures.

Mendelson and colleagues (46) state that the di-
agnosis of LFTP should be considered when le-
sions abut the mediastinum or the paraspinal ar-
eas. However, it should be noted that lesions of
identical histologic characteristics (ie, localized
fibrous tumors) may arise in the mediastinum
without any relationship to the pleura (14). Mass
effect on the adjacent lung and mediastinum is
described as a typical finding (9,26) (Fig 26). In-
terestingly, although there was little difference in
size between the malignant and benign LFTP in
our series, atelectasis, mass effect on the mediasti-
num, and pleural effusion were more common in
malignant LFTP. Calcification is described in 7%
of cases and is usually reported in large lesions in
association with necrosis (11,18). Intralesional
calcification was documented in 26% of our cases
and was characterized as punctate, linear, or
coarse (Table 2) (Figs 11b, 12b). Local invasion
is rarely reported, and lymphadenopathy is not a
feature of LFTP (52). Chest wall involvement
was seen at CT in 8% of our cases, manifesting as
sclerosis or pressure erosion on adjacent ribs, a
characteristic feature of chest wall and mediasti-
nal neoplasms of neurogenic origin that is rarely
reported in association with LFTP (Fig 27) (45).
 RG f Volume 23 ● Number 3 Rosado-de-Christenson et al 779

RadioGraphics

Figure 28. Malignant LFTP in an asymptomatic HIV-positive 28-year-old woman. (a) Con-
trast-enhanced chest CT scan (mediastinal window) demonstrates an irregular soft-tissue mass in
the left middle hemithorax that exhibits heterogeneous enhancement and internal geographic areas
of low attenuation. (b) Photograph of the resected specimen demonstrates a centrally necrotic
mass. Note that complete excision of the LFTP required a pneumonectomy.

LFTP have been reported to exhibit intermedi-
ate to high attenuation on unenhanced CT scans.
This appearance has been attributed to the high
physical density of collagen and the abundant
capillary network within these lesions. However,
Francis and colleagues (53) studied nine cases of
fibromatosis (a tumor that contains a dense col-
lagenized matrix) and found no relationship be-
tween CT attenuation and histologic content of
the lesions. Enhancement may correlate with the
vascular nature of these lesions and may result in
higher attenuation than that of other soft tissues
in the thorax (46). Lee and colleagues (50) stud-
ied nine cases of LFTP with CT and demon-
strated significant enhancement in all. In addi-
tion, they documented high attenuation (equal to
that of the surrounding muscles) on unenhanced
CT scans in three of their cases (50). Enhance-
ment is typically heterogeneous (particularly in
large lesions) with central areas of low attenua-
tion, which have been shown to correlate with
myxoid change, hemorrhage, necrosis, or cystic
degeneration (11,37,46,50). Heterogeneity may
become more conspicuous after intravenous ad-
ministration of contrast material because areas of
viable tumor exhibit contrast material enhance-
ment and those with necrosis do not. Although
contrast material enhancement was common in
our series (62%) (Figs 10, 14), it was not demon-
strated in all cases.
The majority of the lesions in our series exhib-
ited heterogeneous attenuation on CT scans be-
fore and after the administration of intravenous
contrast material. Only benign LFTP exhibited
homogeneous attenuation, which was more com-
monly seen on unenhanced scans (Fig 13). Ho-
mogeneity of attenuation may indirectly relate to
size (as small lesions may exhibit necrosis less fre-
quently) and was seen in lesions with average
sizes of 8.1 cm on unenhanced CT scans and 4.6
cm on enhanced scans. In addition, hemorrhage,
necrosis, or cystic change were absent in seven of
eight lesions (88%) with homogeneous attenua-
tion on unenhanced scans and in all eight LFTP
with homogeneous attenuation on contrast-en-
hanced CT scans. Heterogeneous areas of low
attenuation on unenhanced CT scans correlated
with gross presence of necrosis, hemorrhage, or
cystic change in 86% of benign LFTP.
Heterogeneous attenuation of LFTP after con-
trast material administration was typical (Figs
15–17) and correlated with gross descriptions of
hemorrhage, necrosis, or cystic change in 22 of 40
(55%) cases of benign LFTP with intrinsic areas
of low attenuation (Figs 16, 17). Little has been
written about the CT appearance of malignant
LFTP. They are described as indistinguishable
from benign lesions: large masses of heteroge-
neous attenuation and patchy enhancement (52).
Although there was no difference in lesion size
when the benign and malignant LFTP in our se-
ries were compared, low-attenuation areas were
seen in all malignant LFTP whether the scans
were obtained before or after the administration
of intravenous contrast material and correlated
with gross descriptions of hemorrhage, necrosis,
or cystic change in 60% of unenhanced (Figs 12,
26) and 54% of enhanced CT studies (Fig 28).
 780 May-June 2003 RG f Volume 23 ● Number 3

RadioGraphics

Figure 29. Benign LFTP in a 64-year-old woman who presented with confusion and hypoglyce-
mia. (a) Sagittal T1-weighted MR image demonstrates a large soft-tissue mass of heterogeneous
intermediate signal intensity in the right hemithorax that produces mass effect on the hemidia-
phragm. Note flow void foci consistent with tumor vessels. (b) Sagittal T2-weighted MR image at
the same level demonstrates an overall heterogeneous increase in signal intensity in the lesion with
multiple hyperintense areas.

MR Imaging.—There are few reports of the MR
imaging features of LFTP (20,28,54,55). The
existing reports describe masses of predominant
low or intermediate signal intensity on both T1-
and T2-weighted images and on proton density–
weighted images, which is thought to relate to
high content of fibrous collagenous tissue, hypo-
cellularity, and relatively small numbers of mobile
protons (8,11,54,55). However, high signal inten-
sity on T2-weighted images has also been re-
ported and may relate to necrosis, cystic or myx-
oid degeneration, prominent vascular structures,
and hypercellular areas (20,55). In our study,
predominantly heterogeneous lesions were seen
on both T1- and T2-weighted images (Fig 29).
Areas of low to intermediate signal intensity were
more commonly seen on T1-weighted images,
and areas of high signal intensity were demon-
strated more frequently on T2-weighted images
(Figs 18, 29). Low-signal-intensity septa were a
common finding on T2-weighted MR images
(Fig 19). Most lesions increased in signal inten-
sity on T2-weighted images, compared with T1-
weighted images (Fig 29). MR imaging is supe-
rior to CT in assessment of tumor extent and is
more sensitive in excluding local invasion of the
diaphragm and chest wall (18). Sagittal and coro-
nal images permit tumor localization within the
thorax and allow diaphragmatic evaluation. Flow
voids reflecting the vascular nature of some LFTP
have previously been reported (55) and were seen
in 11% of our cases (Figs 19, 29). Intense hetero-
geneous enhancement after administration of in-
travenous gadolinium is typical (Figs 18, 20).
Angiography.—Angiography is useful in deter-
mining the vascular supply to the lesion, which
typically enters through the pedicle (26). Demon-
stration of blood supply from the inferior phrenic,
intercostal, or internal mammary arteries may be
a helpful clue to the extrapulmonary origin of
large LFTP (8).
Ultrasonography.—US is helpful in the evalua-
tion of large masses in the inferior hemithorax
through visualization of the diaphragm and estab-
lishment of their intrathoracic location, particu-
larly those lesions located in the inferior hemitho-
rax (4,26) (Fig 17b).
Patient Management
Diagnosis.—The diagnosis of LFTP may not be
suggested prospectively after imaging affected
patients. This is not surprising given the fact that
 RG f Volume 23 ● Number 3
RadioGraphics classically described radiographic features (in-
complete visualization of the lesion borders, fis-
sural location) and cross-sectional imaging fea-
tures (obtuse angles, mobility, pedicle visualiza-
tion) of focal pleural masses are usually not
present in these cases. The diagnosis of LFTP is
typically made after surgical excision of the mass.
Confident preoperative diagnosis can be made
with large-bore cutting needle biopsies, and be-
cause the needle can usually be introduced into
the mass while avoiding aerated lung, the risk of
pneumothorax is minimal (56). Although fine-
needle aspiration may yield characteristic and di-
agnostic morphologic features, cutting needle
biopsy is probably preferable because of wider
tissue sampling (10,56).
Treatment.—The therapy for patients with
LFTP is complete surgical excision. Aggressive
surgery is recommended given the possibility of
recurrent disease (57). Vascular adhesions to ad-
jacent tissues may result in massive intraoperative
hemorrhage, which is adequately handled with
good exposure, prompt removal of the mass, and
meticulous hemostasis (58). Cardillo and col-
leagues (6) described 55 patients with LFTP who
underwent resection via video-assisted thoraco-
scopic surgery (VATS) and thoracotomy with no
operative deaths. Although VATS may be ad-
equate for the resection of small tumors, large
tumors usually require a thoracotomy (39). In
many cases, resection of the lesion with its pedicle
and a small patch of adjacent lung may be suffi-
cient (4). In some cases, complete excision may
require lung resection (segmentectomy, lobec-
tomy, bilobectomy, pneumonectomy), partial
pleurectomy, or en bloc chest wall resection (Fig
28b) (4,6,7,12). Long-term imaging follow-up is
recommended in all cases to exclude tumor recur-
rence or metastatic disease. Recurrent disease
typically affects the ipsilateral pleura and may also
affect the lung. Repeat resection of recurrent le-
sions is recommended (4,49). Adjuvant therapy is
probably not helpful because of the low cellular
content and low mitotic rates characteristic of
LFTP (27,59).
Prognosis.—The prognosis for patients with
LFTP is generally favorable. The majority of le-
sions behave in a benign fashion (88%), but ap-
proximately 12% of patients die of extensive in-
trathoracic tumor growth or an unresectable re-
currence (3). Although tumor size does not
directly correlate with prognosis, pedunculated
noninvasive tumors are less likely to recur when
complete excision of all neoplastic tissue is ac-
complished. In fact, complete excision is the best
prognostic indicator (3,7,8,21,25,60). Malignant
Rosado-de-Christenson et al 781
lesions can be successfully resected particularly if
they are pedunculated (49,60). Recurrent tumors
may exhibit malignant histologic features and a
more aggressive biologic and clinical behavior
despite prior resection of histologically benign
lesions (14). Malignant tumors may metastasize,
and local recurrences are more common in cases
of malignant lesions than in benign lesions (22).
Conclusions
Localized fibrous tumors are rare primary pleural
neoplasms that may grow to large sizes and typi-
cally affect symptomatic men and women over
the age of 40 years. Small LFTP may be discov-
ered incidentally on chest radiographs of asymp-
tomatic individuals. Although small lesions may
exhibit the characteristic imaging features of pleu-
ral masses, classic findings of focal pleural dis-
ease, such as the “incomplete border” sign, ob-
tuse or right angles against the adjacent pleura,
fissural location, or mobility within the pleural
space, are usually absent. The majority of LFTP
occupy the inferior hemithorax, and those that
abut the ipsilateral hemidiaphragm may mimic
diaphragmatic elevation or eventration. The diag-
nosis should be considered in symptomatic adults
who present with solitary, large, lobular, hetero-
geneous intrathoracic masses in the absence of
local invasion, lymphadenopathy, or metastatic
disease. Small LFTP without gross necrosis,
hemorrhage, or cystic change may exhibit homo-
geneous attenuation on unenhanced and less fre-
quently on contrast-enhanced chest CT scans.
However, the majority of LFTP exhibit heteroge-
neous attenuation on CT scans, characterized as
intralesional geographic, focal or linear areas of
low attenuation that often correlate with hemor-
rhage, necrosis, or cystic changes. Calcification
may occur in one-fourth of cases. Atelectasis of
the adjacent lung and mass effect on the mediasti-
num are common associated findings. MR imag-
ing typically demonstrates intrathoracic lobular
masses of heterogeneous signal intensity with
both T1- and T2-weighted sequences. Internal
low-signal-intensity septa on T2-weighted images
are common. Studies that allow direct visualiza-
tion of the diaphragm such as MR imaging and
US are useful in establishing the intrathoracic
location of large lesions occupying the inferior
hemithorax. Although there are no imaging fea-
tures that definitively distinguish benign from
malignant subtypes of LFTP, heterogeneity on
cross-sectional images, mass effect, and pleural
effusion may be slightly more common in malig-
nant lesions.
 782 May-June 2003
RadioGraphics Acknowledgments: The authors wish to acknowl-
edge the immense assistance of Linda C. Wilkins, BS,
who constructed the database used to collect and ana-
lyze the data for the manuscript. We also acknowledge
the input and contribution of Philip A. Templeton,
MD, during the early planning stages of the project.
Finally, we thank the countless residents who through-
out the years have participated in the Radiologic Pa-
thology Courses of the Department of Radiologic Pa-
thology at the Armed Forces Institute of Pathology in
Washington, DC. Their case contributions enriched
the content of the Institute’s archives and enhanced our
understanding of radiologic-pathologic correlation of
thoracic diseases.
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