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OCTOBER,
1975
MEDIASTINAL NEUROBLASTOMA AND GANGLIONEUROMA* THE
DIFFERENTIATION
BETWEEN
INVOLVEMENT
ON J. BAR-ZIV,
By
THE
PRIMARY CHEST
M.D.,
and
M.
MONTREAL,
AND
SECONDARY
ROENTGENOGRAM B. NOGRADY,
M.D.
QUEBEC
ABSTRACT:
The
roentgenologic
diagnosis
and
differentiation
of mediastinal
neurogenic
tumors
are possible on the chest roentgenogram as a rule. The soft tissue mass may be illdefined and the tumor “ghost-like” in the case of primary neuroblastoma, but it is usually obvious in ganglioneuroma and metastatic disease. The presence of calcifications differentiates neurogenic tumors from other posterior mediastinal tumors of childhood. They are common in primary and rare in secondary disease. Rib erosions and displacement are striking in neuroblastoma (after a few months of age), more subtle in ganglioneuroma, and absent with secondary involvement. In 3 out of 7 posterior mediastinal neuroblastomas the diagnosis and treatment were delayed, as the adjacent rib changes were not appreciated for some time. “Dumbbell” shaped tumors are usually associated with vertebral changes and myelography is indicated even in the absence of neurologic deficit. Thoracic deformity and disability subsequent to laminectomy, radiation therapy, or both, are present in all survivors.
T
countered case of a posterior mediastinal mass in a child with left lower lobe pneumonia in whom the tumor simulated right upper lobe collapse. In 3 out of 7 posterior mediastinal neuroblastomas the diagnosis
HE roentgenologic recognition of the tumorous nature of a primary posterior mediastinal neuroblastoma may be difficult
since
the
ill-defined
neoplasm
may
“ghost”
mass
roentgen appearance nary process.”3
of Institution
present
as
simulating a pleural of
known
to
be
common
importance
benign
in
of early
nature
of
the
the
and
pulmo-
adequate delay-
treatment is therefore occasionally ed. Intrathoracic neurogenic tumors The
an
the
From
the
at the Department
i7th
mediastinal
Annual
Meeting
of Diagnostic
time
some
The
toma,
The
and
study
from
1963
tumor
II,
III,
AND
since
the
not appreciated iv).
METHOD
roentgenologic
and
clinical
diag-
ganglioneuroma
were
comprises to 1973
material seen in the hospital
registry
patient included
and
our
reviewed.
departmental
cod-
ing.
A total of 44 patients documentation in the last indicates
i
the
histologic
Radiology,
Children’s
380
Hospital
diagnosis,
San
Francisco, and
McGill
had 10
distribution age
the number of deceased ing the location of the
for Pediatric Montreal
(Cases
available
delayed,
were
documentations in patients with the noses of neuroblastoma, ganglioneuroblas-
neuro-
of the Society Radiology,
changes
MATERIAL
The
the
blastomas versus the primary abdominal ones have also been stressed.2”#{176}”4” We reviewed the clinical and roentgenologic documentation of patients diagnosed with neuroblastoma, ganglioneuroblastoma and ganglioneuroma seen at our institution in the last JO years. Our aim was to detect the salient roentgenologic features which permit the improvement of the diagnostic accuracy on the chest roentgenogram. The study was prompted by a recently en*Presented
for
are
and
were
rib
young.6’8”#{176}”2
diagnosis
treatment
adjacent
of of the
acceptable years. Table tumors
by
patient,
children, neoplasm.
California,
September
University,
Montreal,Quebec,
and
disregard-
23,
1974. Canada.
VOL.
125,
No.
Mediastinal
a
Neuroblastoma
and
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OF
AND
Age
(yr.)
NEUROBLASTOMAS,
GANGLIONEUROBLASTOMAS
GANGLIONEUROMAS
Total
Neuroblastoma
TO 1973
Ganglioneuroblastoma
Ganglioneuroma
()
13
13
1-2
II
io(6)
i
3(3)
I
4
3-5
8(8)
io
6
5-10
44
(25
I
2
deceased)
REPORT
posteriorly
OF
indented the posterior (Fig. 2M. There were pedicles and widening of the intervertebral foramina at T-3 to T-7 on the right (not illustrated). In spite of the absence of neurologic signs and symptoms, a myelogram demonstrated a complete block at the level of T-7, caused by a right-sided extradural mass (Fig. 2B).
CASES
a 10 year old Caticlinical and roentgenologic evidence of left lower lobe pneumonia. In addition, a right upper lobe density with a well-defined inferior border was considered right upper lobe collapse secondary to pneu-
monia (not illustrated). Follow-up examination of the
persistence
left
of the right
erosions
upper
broadening ribs were recognized adjacent
rib erosions
and
(Fig. IB).
roentgenogram, were more
Total
No.
omy
were
the obvious
CAsE
only
survivor
subsequent
performed
and
was
removed.
tumor
of this group refer
was 9 days
to death.
2
INVOLVEMENT
12* old
when
diagnosed.
occa-
thoracot-
the
“dumbbell”
The
intraspinal
II.
P.S. No.
421432,
a
9
month
BY
THE
TUMORS
Ganglio-
neuroma
Secondary
6(2)
in brackets
and
Vanillyl-
on
II
MEDIASTINAL
Primary
normal.
negative
Ganglioneuroblastoma
23 The
Laminectomy
was
were
Neuroblastoma
of
Patients
Numbers
tests
measured 3X2X0.5 cm. The intrathoracic extrapleural mass, which was infiltrating the posterior chest wall, measured 13X8X 3.5 cm. after removal. The histologic diagnosis was matured ganglioneurofibroma from a preexistent neuroblastoma. Deforming kyphoscoliosis developed 6 months following surgery. This deformity was due to the surgical intervention alone (Fig. 2C).
TABLE OF
survey
acid
sions.
The ill-defineddensity was localized
DISTRIBUTION
and
iC),
portion
of the 4th to 7th to the soft tissue
lordotic separations
skeletal
shaped
lobe abnormality.
and
density (Fig. iA). On a somewhat
The mandelic
weeks later showed lobe pneumonia and
2
lower
(Fig.
of the esophagus of the vertebral
surface erosions
CASE I. C.A. No. 363226, casian boy, presented with
resolution
I
4(4)
Twenty-three of patients had mediastinal involvement. The distribution of these tumors is shown in Table II. There were 18 mediastinal neuroblastomas (6 primary, 12 secondary), I ganglioneuroblastoma, and ganglioneuromas.
*
1963
FROM
0-I
2-3
Rib
38!
I
TABLE DISTRIBUTION
Ganglioneuroma
(II)
I
4
old
J.
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382
Bar-Ziv
Case I. (A) Soft tissue density simulating On different projection the rib erosions is superimposed on the thoracic spine.
FIG.
I.
(B)
Caucasian hospital
boy, had for
tion.
A chest
tient
showed
and
M.
right
upper
and
of
roentgenogram
I month’s
sent
with
durathe pa-
an ill-defined perihilar density on the right. Rib erosion and separation involved the 5th and 6th ribs posteriorly (Fig. 3A). On initial physical examination the child was thought to be normal, but subsequently it became obvious that within the last few days he had lost the ability to stand up. Bone marrow examination and 2 vanillylmandelic acid assays were normal. A myelogram showed an almost complete block at T-6, and, after myelography, the child developed paraplegia and urinary retention. An emergency laminectomy was done. The intraspinal component of the tumor was partially blastoma.
removed
and
proved
to
be
a neuro-
lobe
separations
been treated at another
“pneumonia”
B. Nogrady
collapse.
are more
The
intrathoracic
treated 4,150
with r in
OCTOBER,
There
obvious.
1975
are posterior rib deformities. (C) The ill-defined density
of the mass was therapy (tumor dose the mediastinal tumor
component
radiation 36 days), and
subsequently disappeared. Recurrence was suspected and within i year of the initial radiation therapy, high-voltage radiation was delivered to the right lung on 2 separate occasions (3,7oo r, time not stated, and I,5oo r in 26 days). Chemotherapy was also given. Radiation pneumonitis, cor pulmonale, and congestive heart failure developed and the patient was digitalized. There was transient cortical blindness which was thought to be the result of respiratory and cardiac arrests. Bone marrow examination and vanillylmandelic acid assay were negative in the postoperative episode.
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VOL.
125, No.
FIG.
In ment, had
the the severe
considered
years
there
Mediastinal
a
2.
years
child
Case I. (A) Mild posterior (B) There is a complete
subsequent
had
14
to the hospital
complications,
to
Neuroblastoma
be iatrogenic. is no evidence
indentation block
initial
of At
of metastasis
age
of the esophagus (C) Postoperative
tinued,
were of
Ganglioneuroma
currence,
and
which the
at T-7.
treat-
admissions
most
and
but and
or re-
(Fig.
3B).
child
disease.
al
mediastin
by the ill-defined changes.
digoxin
the
respiratory 4
383
therapy suffers
There
displacement,
is
from are
mass.
right
and
being
severe
conchronic
fibrothorax,
kvphoscoliosis
J.
384
Bar-Ziv
and
M.
B.
Nogrady
OCTOBER,
1975
Downloaded from www.ajronline.org by West Virginia University on 06/20/16 from IP address 157.182.150.22. Copyright ARRS. For personal use only; all rights reserved
ration of the
and erosion of the ribs. The proximal ends humeri showed bony metastases (Fig. 4). The rib changes were not appreciated in the other hospital. Skeletal survey demonstrated disseminated bone involvement. Urography was normal. Both the bone marrow and lymph node
biopsy
showed
neuroblastom
a.
The patient was treated with chemotherapy and radiation therapy. The mediastinal tumor decreased
in
size
but
the
patient
D.A.
No.
404188,
expired
4
later.
months
CASE
I.
a
year
old
Caucasian girl, presented with left-sided chest pain, shortness of breath, and pallor, having been bumped by a cow 3 days prior to admission. Three months earlier she had fallen off a diving board and had suffered a brain concussion.
There was clinical and roentgenologic dence of left-sided tension hemothorax.
Li
eviIn addi-
tion, there were rib erosions and separations involving the posterior portions of the 4th to 6th ribs on the same side (Fig. 4). Thoracentesis yielded 175 ml. of blood. Subsequent examination showed the rib changes more clearly, as well as spotty soft tissue calcifi-
r
Fic. 3. Case
II.
A soft tissue mass and the thoracic deformity at 4 years of age.
(A)
changes. chronic
(B) Note lung changes
CASE
III.
K.M.
No.
387961,
a 4
year
rib and
old c
Eskimo girl, was referred from another hospital for bone marrow biopsy. She had a history of vomiting and vague abdominal pain of days’ duration. On physical examination, non-tender cervical and left axillary lymphadenopathy was
found. globin cytes
The had
There was normochromic 7 grams per cent), and were found tuberculin a BCG
in the test
vaccination
peripheral
was
positive, I
year
anemia
(hemo-
4.
atypical leukoblood smear. but she had
earlier.The chest
‘a
roentgenogram was Suggestive of primary pulmonary tuberculosis since a large left hilar mass was demonstrated. On admission she was pale with hemoglobin of
grams
revealed containing
per an
cent.
obvious calcifications,
The left
chest
roentgenogram
paravertebral and there were
mass sepa-
FIG.
4. Case
adjacent
to
in. the
There are paravertebral
obvious rib changes neuroblastoma.
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VoL.
FIG.
125,
No.
2
5. Case to 6th ribs.
IV.
(B)
Mediastinal
(ii)
Tension There are
Neuroblastoma
hemothorax rib changes
displacement of the descending months subsequent to therapy.
aorta
and
Ganglioneuroma
385
of the posterior portion of 4th in the same area. (C) Note the is regression of rib changes 6
with marked erosions and the multiple spotty
and
the
cations without an obvious mass (Fig. cB). Vertebral erosions were also demonstrated. Bronchography demonstrated external compression of the left main bronchus. The thoracic aorta was displaced by a calcified soft tiss’ie mass which was fed by tumor vessels (Fig. 5C).
and separation calcifications vessels. (D) ‘I’here
tumor
Spinal
cord
lylmandelic on
compression
demonstrated
was
2
acid
assa
No
occasions.
corded during lam inectomy
by on
the
was
an
myelographv. was found neurologic
child’s
followed
extradural
mass The
vanil-
to be elevated signs were re-
hospital stay. I)y thoracotomy
Elective and
J.
386
Bar-Ziv
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