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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

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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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and M.

B. Nogrady



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OCTOBER,

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Mediastinal neuroblastoma and ganglioneuroma. The differentiation between primary and secondary involvement on the chest roentgenogram.

The roentgenologic diagnosis and differentiation of mediastinal neurogenic tumors are possible on the chest roentgenogram as a rule. The soft tissue m...
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