Pleural and Chest Wall Tumors
Ritu Gill, MD, MPH
CLASSIFICATION OF CHEST WALL TUMORS
• Primaryy neoplasm
p
of chest wall: malignant,
g
benign
• Metastatic neoplasms to chest wall: sarcoma,
carcinoma
i
• Adjacent neoplasms with local invasion:
lung, breast, pleura
Dr. Ritu R. Gill
D
Department
t
t off Radiology
R di l
Brigham and Women´
Women´s Hospital
Harvard Medical School
2
^dZϮϬϭϱ
INCIDENCE
PRIMARY CHEST WALL TUMORS
•
Primary chest wall tumors are uncommon.
•
50-80 % of these tumors are malignant.
•
Soft tissues are major sources of chest wall tumors.
•
The most common primary malignant chest wall tumors are
malignant fibrous histiocytoma, rhabdomyosarcoma and
chondrsarcoma.
•
The most common primary benign chest wall tumors
cartilaginous tumors, desmoids and fibrous dysplasia.
• Malignant
myeloma, malignant fibrous histiocytoma,
chondrosarcoma, rhbdomyosarcoma, Ewing’s
sarcoma liposarcoma,
sarcoma,
liposarcoma lymphoma
lymphoma, leiomyosarcoma,
leiomyosarcoma
hemangiosarcoma
• Benign
osteochondroma, chondroma, desmoids, fibrous
dysplasia,
lipoma fibroma,
lipoma,
fibroma neurilemoma
3
4
PRIMARY BONE TUMORS
Primary bone
uncommon.
neoplasms
BENIGN RIB TUMORS
involving
chest
wall
are
g bone tumors are cartilaginous
g
• The most common benign
in origin-osteochondroma and chondroma.
• The most common malignant
g
bone tumors are myeloma,
y
chondrosarcoma, malignant lymphoma and Ewing’s
sarcoma.
5
Osteochondroma
• It is the most common benign bone tumor( 50% of benign
rib tumors )
• It arises from the metaphyseal region of the rib and
present a bonyy p
p
protuberance with a cartilaginous
g
cap.
p
• Rarely, it can involves the diaphragm and induce
hemothorax.
6
MONDAY
3OHXUDODQG&KHVW:DOO7XPRUV
HISTIOCYTOSIS X
BENIGN RIB TUMORS
)LE
)LEURXVG\VSODVLD
G O L
MONDAY
• Cystic, non-neoplastic lesion with fibrous replacement of
med llar cavity
medullary
ca it of the rib.
rib
• Albright’s syndrome( multiple bone cysts, skin pigmentation
and
d precocious
i
sex maturity
t it in
i girls
i l ) should
h ld be
b suspected
t d if
multiple lesions occurs.
• Treatment
T t
t should
h ld be
b conservative.
ti
•
Majority stop growing at puberty.
•
Surgical resection is indicated if pain and enlarging lesions
7
occurs.
•
Comprises of a spectrum of disease involving the reticuloendothelial
system , including eosinophilic granuloma, Letterer-Siwe disease and
Hand Schuller
HandSchuller-Christian
Christian disease.
Histiocytosis X occurs in patients younger than 50 years.
Eosinophilic granuloma is limited only bone involvement.
Bone lesions may occurs in all types of histiocytosis X and skull is the
most common.
Surgical resection in solitary disease is curative.
Radiation therapy can be helpful in multi-site involvement.
•
•
•
•
•
8
MALIGNANT RIB TUMORS
MALIGNANT RIB TUMORS
0\HORPD
• It is the most common malignant rib tumor.
• Most myelomas involving chest wall are systemic myeloma
myeloma.
• Most myeloma occurs in people of 40-60 years and rare in
people< 30 years.
• Punched-out
P
h d
t lesion
l i with
ith cortical
ti l thi
thinning
i presents
t
radiographically.
• Pathologic fracture is common.
• Local excision is for diagnosis.
• Radiation is for a solitary lesion and both radiation and
chemotherapy for multiple lesion.
• 5-year survival is 20 %.
Chondrosarcoma
wall
• It is almost a tumor of the anterior chest wall.
• Most chondrosarcoma occurs in people of 20-30 years
and male.
• All tumors
t
from
f
costal
t l cartilages
til
should
h ld b
be considered
id d
malignancy.
• Pathological fracture is uncommon.
• Chest wall chondrosarcoma grows slowly and
metastases occur late
9
10
MALIGNANT RIB TUMORS
MALIGNANT RIB TUMORS
Osteogenic sarcoma
Ewing’s
Ewing
s sarcoma
•
2/3 patients is younger than 20 years.
•
An onion-skin
onion skin appearance of the surface of the bone may be
seen.
•
g
fracture is rare.
Pathological
•
Radiation is the treatment of choice and adjuvant
chemotherapy is also used.
11
•
It is more malignant and less common than chondrosarcoma.
•
More common in teenagers and young adults. Men more than women.
•
Serum ALP is frequently elevated.
•
P th l i fracture
Pathologic
f t
is
i rare.
•
The treatment is wide excision.
•
Radiation is not valuable and
•
chemotherapy is controversial.
•
5-year survival is 20%.
12
TUMORS OF THE MANUBRIUM , STERNUM ,
SCAPULA AND CLAVICLE
MALIGNANT RIB TUMORS
Those tumors are uncommon.
•
O t
Osteosarcoma
and
d soft
ft tissue
ti
sarcoma are the
th
most common.
•
The initial radiation is most common for breast
cancer and lymphoma.
•
The treatment is similar to tumors arising de novo.
novo
13
14
BENIGN SOFT TISSUE TUMOR
MALIGNANT SOFT TISSUE TUMORS
Desmoid
•
•
•
•
•
•
40 % of all demoids occur in the chest wall and the shoulder.
Encapsulation of vessels and brachial plexus in arms and
neck is common.
The tumor may extend into the pleural cavity and displace
mediastinal structure.
Desmoid is most common in people of between puberty and
40 years of age
age. Men and women was affected equally
equally.
The tumor originates in muscle and fascia.
The tumor must be treated with wide excision. Recurrence
may occur if inadequate excision.
excision
Malignant Fibrous Histiocytoma
•
It is the most common chest wall tumor that the
thoracic surgeon was asked.
• It is most common in people of 50-70 years of age.
2/3 of patients are male.
• The tumor was unresponsive to radiation and
chemotherapy, so wide resection is the choice.
• 5-year
5 year survival is 38 %
%.
15
16
MALIGNANT SOFT TISSUE TUMORS
MALIGNANT SOFT TISSUE TUMORS
Rhabdomyosarcoma
• It is the most second common malignant soft tissue
tumor.
• It is most common in children and young adults.
• The tumor is neither pain nor tender, despite rapid
growth.
growth
• Wide excision with postoperative chemotherapy
and
a
d radiotherapy
ad ot e apy results
esu ts in 5
5-year
yea su
survival
a o
of 70%.
0%
17
Liposarcoma
•
It is most common in people of 40-60 years of age.
•
Most patients are men.
•
Treatment is wide excision.5-year
excision 5-year survival is 60 %.
%
• Chemotherapy and radiotherapy have little to offer.
18
MONDAY
• Primary tumors of the manubrium and the sternum constitute
15 % of the chest wall tumors, but nearly all are malignant.
• The sternum is a frequent site metastasis from the
breast, thyroid and kidney.
• The scapula is not a frequent site of metastasis.
• Primary
Pi
t
tumors
off the
th clavicle
l i l are uncommon, b
butt 90 % are
malignant.
• The clavicle is more a site of a metastasis than a p
primary
y
tumor.
Radiation-Associated Malignant Tumors
•
PLEURAL MASS CHARACTERISTICS
MALIGNANT SOFT TISSUE TUMORS
• Obtuse angles with pleural surfaces
Neurofibrosarcoma
• Sharp margins on tangential views
MONDAY
•
Chest wall neurofibrosarcoma occurs along the
intercostal nerve.
• It is most common in people of 20-50 years of age.
Most patients are male.
• Half of patients are associated with von
Recklinghausen’s disease.
• Treatment is wide excision.
• Discrepant margins on different views
• Incomplete border
• May cross fissures
• Look for osseous and soft tissue--chest
wall
19
WHO HISTOLOGICAL CLASSIFICATION OF
PLEURAL TUMORS
Mesothelial Tumours
Diffuse malignant mesothelioma
p
mesothelioma
• Epithelioid
• Sarcomatoid mesothelioma
• Desmoplastic mesothelioma
p
mesothelioma
• Biphasic
• Localized malignant mesothelioma
Other Tumours of Mesothelial Origin
papillary
p
y
• Well differentiated p
mesothelioma
th li
•
Adenomatoid tumour
PLEURAL METASTASES
• 40% Bronchogenic adenocarcinoma
• 20% Breast cancer
• 10% Lymphoma
• 30% Other primaries
y
• Thymoma
• Carcinoid
• Sarcoma
S
• Splenosis
Lymphoproliferative Disorders
• Primary effusion lymphoma 9678/3
y
– associated lymphoma
y p
• Pyothorax
Mesenchymal Tumours
• Epithelioid hemangioendothelioma
• Angiosarcoma
• Synovial sarcoma
• Monophasic
• Biphasic solitary fibrous tumour
• Calcifying tumour of the pleura
• Desmoplastic round cell tumour
MALIGNANT PLEURAL MESOTHELIOMA
• 2,000-3,000
2,000 3,000 cases annually in the U.S.
• Incidence increasing worldwide
• Up to 10% lifetime risk of developing
mesothelioma among asbestos workers
• Asbestos + cigarette smoking Æ synergistic
• 60X more likely to develop lung cancer when
compared to non
non-smoking
smoking, non-asbestos
non asbestos exposed
cohort
Sterman, Daniel, ĞƚĂů. ಯEpidemiology of malignant mesothelioma.ರ hƉƚŽĂƚĞ. May 1, 2009
MALIGNANT PLEURAL MESOTHELIOMA
D
B
E
C
F
E ith li id 50%
Epithelioid
Stage
g
I
Malignant Mesothelioma
Percent survival
Epithelioid
Mixed
Sarcomatoid
WDPM
80
60
24
48
72
96
120
Time (months)
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RADIOGRAPHIC PRESENTATION
• Wide range
• Pleural effusion, small to large
• Encasement of lung
pleural masses
• Lobulated p
Mi d 34%
Mixed
S
Sarcomatoid
t id 16%
Definition
Disease confined to within the capsule of the parietal pleura; ipsilateral
pleura, lung, pericardium, diaphragm, or chest wall disease limited to
previous biopsy site
All of stage I with positive intrathoracic (N1 or N2) lymph nodes and
positive resection margins
III
Local extension of disease into chest wall or mediastinum; heart, or
through the diaphragm and peritoneum; with or without extrathoracic or
contralateral lymph node involvement (N3)
IV
Distant metastatic disease
20
0
I
II
40
0
H
BWH STAGING
Prognosis of Malignant Mesothelioma
is Dependent on the Histologic Type
100
G
Sugarbaker, DJ, Norberto, JJ, Swanson, SJ. ಯSurgical staging and work up of patients with diffuse malignant pleural
mesothelioma.ರ Mesotheliomas. ^ĞŵŝŶĂƌƐŝŶdŚŽƌĂĐŝĐĂŶĚĂƌĚŝŽǀĂƐĐƵůĂƌ^ƵƌŐĞƌLJ. Ed. Sugarbaker DJ. Volume 9,
issue 4, 1997
NODULAR PLEURAL THICKENING
MONDAY
• 3-6:1(men:women)
(
) follows exposure
p
• Latency of 30-40 years (2000-3000/yr)
• Present with
ith SOB
SOB, chest pain
pain, co
cough,
gh
and weight loss
• Right side with SVC, Horner syndrome
• HPO, clubbing
PATHOLOGIC SPECTRUM
A
MONDAY
CHEST WALL AND MEDIASTINUM
INVOLVEMENT
DIAPHRAGMATIC EXTENSION
diaphragm
PULMONARY, LYMPH NODE, AND LIVER
INVOLVEMENT
Sagittal
g
and coronal MDCT images
g reveal marked encasement of the
right lung with extension of the pleural mass into the mediastinum
(white arrows), and extension into the abdomen (arrowhead).
CHEST WALL INVASION
IN SOME CASES, COMPLEMENTARY
ANATOMICAL INFORMATION CAN BE
DERIVED FROM MRI
Accuracy of MPM staging by CT and MRI
p
T2
T2
Di h
Diaphragm
i l
involvement
.46
.69
.01
.05
.63
.59
.64
MONDAY
Endothoracic fascia involvement
or solitary resectable focus tumor
MRI IS SUPERIOR TO CT
DIFFUSION WEIGHTED IMAGING
3orthogonal
directions
4b-values
(0,250,500,750)
Time 6.5
6 5 mts
ADC = 0.883x
0 883x
10-3mm2/s
HASTE
CE
DW
I
ADC
FOLLOWING NEOADJUVANT CHEMOTHERAPY
Pre-chemotherapy
Post-chemotherapy
Rotating maximum intensity
projection and fusion PET-PT
images of the same patient
from pre and post chemotherapy studies show reduced
post treatment FDG avidity of
the right pleural mass. The
patient subsequently under-
Pre-chemotherapy
Prechemotherapy
Post-chemotherapy
Post-chemotherapy
MONDAY
ಯCourseರ of MPM
SOLITARY FIBROUS TUMOR
• Pleural effusion dominant early
• Benign mesothelioma
• Progressive encasement of lung
• Benign fibrous tumor of pleura
• Lobulated pleural masses late
• Localized fibrous tumor of pleura
• Solitary
S lit
fib
fibrous ttumor
• Invasion of lung
• Lung nodules and distant spread
SOLITARY FIBROUS TUMOR
• Rare
• Symptoms relate to size : cough,
chest pain,
pain Cough,
Cough dyspnea,
dyspnea chest
pain
• P
Paraneoplastic
l ti syndrome:
d
clubbing,
l bbi
hypertrophic osteo-arthropathy,
hypoglycemia
SOLITARY FIBROUS TUMOR
•
•
•
•
•
•
Visceral p
pleura 80%
Parietal pleura 20%
Encapsulated,
pedunculated
Nodular, whorled
appearance
CD34 positive; calretinin
and WT-1 negative
Hemorrhage, necrosis and
cysts
LIPOSARCOMA
SOLITARY FIBROUS TUMOR
•
•
•
•
Typically large, infiltrative, and
asymptomatic
N evidence
No
id
tto suggestt th
they
arise from pre-existing lipomas
Heterogeneous masses on CT
with soft tissue and fat
component. They tend to
measure less than 50 HU (mean
CT values) on pre and post
intravenous contrast
enhancement images
On MRI, they tend to have high
signal intensity on T2WI, because
of myxoid degeneration,
degeneration while
low signal is common and have
variable enhancement post
gadolinium administration
SYNOVIAL SARCOMA
•
•
•
•
CONCLUSION
• Metastases are 95% of pleural
lesions
• Focal pleural tumor can be favorable
DX
• Malignant
g
p
pleural mesothelioma and
Adenocarcinomatosis may look alike
LYMPHOMA
• Both primary and
secondary forms can
involve the pleura.
Follicular and B cell
lymphoma tend to be more
common
• There
Th
can be
b associated
i t d
lymphadenopathy, as well
as chest wall and marrow
involvement Tend to be
involvement.
low on T1WI and iso to
high signal on T2WI
• Diffuse homogenous
enhancement
RESOURCES
• Evans AL
AL, Gleeson FV
FV. Radiology in pleural disease:
State of the art. Respirology 2004; 9:300-312
• Sung
g SH,, Chang
g JW,, Jhingook
g
K,, et al. Solitaryy fibrous
tumors of the pleura: surgical outcome and clinical
course. Ann Thorac Surg 2005; 79:303-307
• Wang ZJ, Reddy GP, Gotway MB, et al. Malignant
pleural mesothelioma: evaluation with CT, MR imaging,
and PET
PET. Radiographics 2004; 24:105
24:105-119
119
MONDAY
•
Typically occurs in adolescents and
young adults between the ages of
15 and 40 years
It is belie
believed
ed to originate from
primitive pleuripotent mesenchyme
capable of synovial differentiation
Because of its rarity, it is often
mistaken on imaging and histology
for sarcomatoid mesothelioma
Molecular studies for the X:18
translocation are useful for
differentiation
Focally positive: EMA, AE1/AE3,
Cam5.2
• Negative: CD34
CD34, S-100
S 100, GFAP
• FISH: positive for SYT gene
rearrangement