Bronchial Carcinoid Tumors in Children and Adolescents By Richard
histologically
l Thirteen bronchial eight
carcinoid
cases from
Registry, rented. were
all
all
the
under
the
most
cough,
frequent
Bronchoscopy
16 cases
and
so studied.
Gross
and
William
scopic pathology treatment
Tumor
are
long-term
pre-
cept in
pneumonia
presenting
defined
Feldtman,
and
Forces
yr of age,
W.
cases of
the literature
Armed
20
Robert
proven
from
Hemoptysis,
plaints.
J. Andrassy,
is briefly
in these
described,
cases outlined.
prognosis those
Stanford
can be expected,
cases
with
a long
and
A good delay
exin
diagnosis.
com-
the lesion and
in
micro-
INDEX
WORDS:
Carcinoid;
cinoid;
bronchial
tumors.
bronchial
car-
I
N 1968, VERSHA AND CONNOLLY reviewed the literature on bronchial adenomas in children, and added an additional case for a total of 21 cases.’ Nine of the 21 were histologically proven bronchial carcinoids. Our review of over 5000 primary lung tumors in the Armed Forces Tumor Registry disclosed eight bronchial carcinoids in the under-20 age group. An additional four documented cases have been found in the recent literature.2m4 The report thus accumulated of 21 primary bronchial carcinoids in children and adolescents is, to the best of our knowledge, the largest review to date and comprises the basis for this report. CASE Thirteen
histologically
not previously
reported
proven from
STUDIES
cases of bronchial
the Armed
Forces
carcinoid Tumor
from
Registry
the literature comprise
series. There were I3 males and eight females. The age range was from 9-19
and eight cases
the 21 cases in this
yr with an average age
of 13.9 yr. Duration Table
of symptoms.
presenting
symptoms,
and treatment
with
follow-up
are
detailed
in
I.
Sixteen patients underwent
preoperative
diagnostic
bronchoscopy
and the tumor
was visualized
in all sixteen cases. There were no documented one patient
cases of carcinoid
was found to have an elevated
subsequently
syndrome
serum
associated with these lesions, although
SHydroxyindolacetic
acid level. This
patient
expired. DISCUSSION
Bronchial designated carcinoma As a group age groups; “bronchial
carcinoids have previously been categorized in a class of tumors “bronchial adenomas.” This classification included adenoid cystic and mucoepidermoid carcinoma in addition to the carcinoid tumors. they comprise about 5”, of primary pulmonary neoplasms from all however, the carcinoid tumors embody the largest proportion of adenomas.“5
From the Departments of General and Thoracic Surgerv. WilJord Hall USAF Medical Center. Lackland Air Force Base, Texas. Address reprint requests lo: Major Richard J. Andrassy. USAF, M.D.. Department of Surgerv (SGHSG, Wirford Hall USAF Medical Cenrer, Lackland Air Force Base, Texas 78236. @ 1977 bv Grune & Slratton, Inc.
Journal of Pediatric Surgery, Vol. 12, No. 4 (August), 1977
513
(1973)
Burcharth
(1966)
Versko
(1965)
Nunez
(1965)
Berkmon
(1961)
Weisel
(1955)
Hallmon
(1955)
(1955)
Sherman
(1951)
Smaller
(2)
(1)
(1)
(1)
(‘1
(1)
(1)
(1)
(1)
(1)
13
12
13
9
10
10
15
9
9
13
Souders
(1948)
Aw
Author
F
M
M
F
F
F
M
F
F
M
Sex
?*
24
12
6
8
24
8
7
4
?*
(MO)
Duration
Table
1.
0
+
-
Cough
Bronchial
0
+
+
+
_
_
+
_
+
_
Hemoptyris
Carcinoid
Reported
0
+
+
+
+
+
+
Pneumonia
Tumors
Lt Main
Findings
Rt Main
?*
Stem
Tumor
NDt
Bronchus
Lt Main
Rt Main
Rt Lower
Bronchus
Rt Moin-
Lt Upper
Bronchus
Tumor
Lobe
Tumor
NDP
stem
Tumor
Lobe
Tumor
Bronchus
Tumor
Bronchus
Tumor
Bronchoscopic
in the Literature
Treatmenf
Bronchus
Lt Lower
Stem
Resection
Lobectomy
Main
Sleeve
Rt Pneumonectomy
RemOVol
Lt
Lobectomy
Lobectomy
Bronchoscopic
Rt Lower
Rt Upper
Rt Pneumonectomy
Resection
Lobectomy
+ Sleeve
Lt Upper
Rt Pneumonectomy
Lt Pneumonectomy
Follow-Up
1 Yr Postop
Recurrence
1 Yr Postop
Recurrence
9 yr Postop
1 Yr postop Alive
No
Pneumonia 12 MO Postop
Died
cauterization
bronchoscopic
repeated
requiring
ReCUW3lCe
NRf
1 Yr Postop
No Recurrence
NRf
No
No Recurrence
NRI
$
5
E
2
;
%
s
i;
ITi
Not
Reported.
Not done.
=
t
1 =
= Unknown.
(1976)
Andrassy
(1976)
Androssy
(1976)
Androssy
(1976)
Androssy
(1976)
Andrassy
( 1976)
Andrassy
(1976)
Andrassy
(1976)
Androssy
(1975)
Salyer
(1974)
Kyriokos
(1973)
Burcharth
*?
-
21
20
19
18
17
16
15
14
13
12
11
(3)
(4)
(2)
18
17
19
18
17
17
10
18
18
13
15
M
M
F
M
M
M
M
M
M
M
F
1
9
0
36
1
6
48
24
?’
144
?*
+
-
t
+
_
+
+
-
_
+
0
-
+
_
-
+
_
-
_
+
+
0
+
_
+
+
_
-
_
+
+
+
0
Rt Bronchus
Bronchus
Rt Upper
Rt Bronchus
Bronchus
Lt tower
Bronchus
Rt lower
Bronchus
Lt Upper
Bronchus
Rt Upper
Rt Bronchus
Bronchus
Rt Middle
lntermedius
Tumor
Lobe
Tumor
lntermedius
Tumor
Lobe
Tumor
Lobe
Tumor
Lobe
Tumor
Lobe
Tumor
lntermedius
Tumor
Lobe
Tumor
?*
?’
Excision
Rods
Nodes
Biopsy
8 Lower
8 Lower
8 Lower Lobectomies
Rt Middle
Lobectomies
Rt Middle
Lobectomies
Rt Middle
Lobectomy
tt Lower
local
4000
(+)
Open
Loco1 Excision
Rt Thoracotomy
Lobectomies
8 Rt Middle
Resection
Rt Lower
Local
?*
Lobectomy
7 MO Postop
3 Yr Postop
3 Yr Postop
9 Yr Postop
2 MO Postop
10 Yr Postop
No Recurrence
10 Yr Postop
No Recurrence
2 Yr Postop
No Recurrence
2 Yr Postop
No Recurrence
2 Yr Postop
No Recurrence
Elev 5HIAA
Died
8 Y r Postop
No Recurrence
Alive
Alive
of Pneumonia
Dead
Alive % n
516
ANDRASSY,
FELDTMAN,
AND STANFORD
Generally these tumors involve major bronchi, and therefore the symptoms and physical signs are those of bronchial obstruction. Cough, hemoptysis. and pneumonia were the most frequent presenting findings. These findings in the young child are frequently associated with a foreign body and since an adequate history may not be available, diagnostic bronchoscopy is frequently employed. These same symptoms are unfortunately not vigorously pursued in the adolescent where a negative history for aspiration and nondiagnostic roentgenogram are obtained. In this series, all I6 patients subjected to diagnostic bronchoscopy were found to have a central lesion easily visualized via bronchoscopic evaluation. The carcinoid tumors generally present in one of two growth patterns: endobronchial polypoid masses that produce segmental bronchial obstruction followed by atelectasis and infection, or as “iceberg” lesions with predominantly extrabronchial growth, the small intrabronchial extent of which gives rise to mucosal ulceration and hemoptysis.6 bronchial and intestinal carcinoids are In routine histologic preparations, usually indistinguishable. Frequently, carcinoids are confused with undifferentiated small cell (oat cell) carcinomas on biopsy in adults. This should be somewhat less of a problem in children since statistically the oat cell tumor is extremely rare in adolescence. The carcinoid tumor consists histologically of festoons and ribbons of small polyhedral cells with central nuclei and eosinophilic cytoplasm arranged in a plexiform or an organoid pattern which resembles that of carcinoid tumors (ar-
Fig. 1.
Carcinoid tumor of the lung (hematoxylin-eorin,
x 250)
BRONCHIAL CARCINOID
517
TUMORS
gentaffinomas) of the gastrointestinal tract (Fig. I). A lengthy discussion of the histologic findings with clinical correlation has recently been presented by Salyer3 and co-workers. Treatment consisted of resection in the majority of our cases. In only one case’ was bronchoscopic removal employed, and this necessitated repeated bronchoscopic cauterization for recurrent tumor. Two patients (13, 15) underwent thoractomy and local excision and were alive without disease 9 and 2 yr, respectively. The survival rate following resection of these tumors is generally very good. It is of significant interest, however, that the two early postoperative deaths were seen in patients with symptoms for 144 and 48 mo, respectively. Perhaps earlier diagnosis would have led to curative resection. Bronchoscopic evaluation is indicated early in all children with symptoms of recurrent bronchial obstruction or hemoptysis. Prompt thoractomy and lobectomy offers a good long-term prognosis. REFERENCES I.
Versha
nomas 55:4I
I
JJ, Connolly
in children.
JE:
J Thor
Bronchial
Cardiovasc
adeSurg
67:634
417. 1968
2. Burchorth cinoids.
Stand
F.
Axelsson
J Thor
C:
Cardiovasc
Lung Surg
car7:72
7x. 1973 3. Salyer Bronchial 1537. 1975
4. Kyriakos
5. Holman vol
DC,
Salyer
WR,
tumors.
Eggleston Cancer
JC:
36: I522
2,
Webber
men.
B: Cancer
J Thor
CW.
Muschenbaum
Diseases
Hagerstown,
and
Surg
C: Broncho-
Related
Maryland.
Disorders. Harper
and
1972, pp 813-815
6. Nissane John M (ed) Pathology and
of the
Cardiovasc
64X. 1974
pulmonary Row,
carcinoid
M.
lung in young
Childhood.
1975, pp 520-523
2nd ed..
St.
of Infancy
Louis,
Mosby.
histologically
l Thirteen bronchial eight
carcinoid
cases from
Registry, rented. were
all
all
the
under
the
most
cough,
frequent
Bronchoscopy
16 cases
and
so studied.
Gross
and
William
scopic pathology treatment
Tumor
are
long-term
pre-
cept in
pneumonia
presenting
defined
Feldtman,
and
Forces
yr of age,
W.
cases of
the literature
Armed
20
Robert
proven
from
Hemoptysis,
plaints.
J. Andrassy,
is briefly
in these
described,
cases outlined.
prognosis those
Stanford
can be expected,
cases
with
a long
and
A good delay
exin
diagnosis.
com-
the lesion and
in
micro-
INDEX
WORDS:
Carcinoid;
cinoid;
bronchial
tumors.
bronchial
car-
I
N 1968, VERSHA AND CONNOLLY reviewed the literature on bronchial adenomas in children, and added an additional case for a total of 21 cases.’ Nine of the 21 were histologically proven bronchial carcinoids. Our review of over 5000 primary lung tumors in the Armed Forces Tumor Registry disclosed eight bronchial carcinoids in the under-20 age group. An additional four documented cases have been found in the recent literature.2m4 The report thus accumulated of 21 primary bronchial carcinoids in children and adolescents is, to the best of our knowledge, the largest review to date and comprises the basis for this report. CASE Thirteen
histologically
not previously
reported
proven from
STUDIES
cases of bronchial
the Armed
Forces
carcinoid Tumor
from
Registry
the literature comprise
series. There were I3 males and eight females. The age range was from 9-19
and eight cases
the 21 cases in this
yr with an average age
of 13.9 yr. Duration Table
of symptoms.
presenting
symptoms,
and treatment
with
follow-up
are
detailed
in
I.
Sixteen patients underwent
preoperative
diagnostic
bronchoscopy
and the tumor
was visualized
in all sixteen cases. There were no documented one patient
cases of carcinoid
was found to have an elevated
subsequently
syndrome
serum
associated with these lesions, although
SHydroxyindolacetic
acid level. This
patient
expired. DISCUSSION
Bronchial designated carcinoma As a group age groups; “bronchial
carcinoids have previously been categorized in a class of tumors “bronchial adenomas.” This classification included adenoid cystic and mucoepidermoid carcinoma in addition to the carcinoid tumors. they comprise about 5”, of primary pulmonary neoplasms from all however, the carcinoid tumors embody the largest proportion of adenomas.“5
From the Departments of General and Thoracic Surgerv. WilJord Hall USAF Medical Center. Lackland Air Force Base, Texas. Address reprint requests lo: Major Richard J. Andrassy. USAF, M.D.. Department of Surgerv (SGHSG, Wirford Hall USAF Medical Cenrer, Lackland Air Force Base, Texas 78236. @ 1977 bv Grune & Slratton, Inc.
Journal of Pediatric Surgery, Vol. 12, No. 4 (August), 1977
513
(1973)
Burcharth
(1966)
Versko
(1965)
Nunez
(1965)
Berkmon
(1961)
Weisel
(1955)
Hallmon
(1955)
(1955)
Sherman
(1951)
Smaller
(2)
(1)
(1)
(1)
(‘1
(1)
(1)
(1)
(1)
(1)
13
12
13
9
10
10
15
9
9
13
Souders
(1948)
Aw
Author
F
M
M
F
F
F
M
F
F
M
Sex
?*
24
12
6
8
24
8
7
4
?*
(MO)
Duration
Table
1.
0
+
-
Cough
Bronchial
0
+
+
+
_
_
+
_
+
_
Hemoptyris
Carcinoid
Reported
0
+
+
+
+
+
+
Pneumonia
Tumors
Lt Main
Findings
Rt Main
?*
Stem
Tumor
NDt
Bronchus
Lt Main
Rt Main
Rt Lower
Bronchus
Rt Moin-
Lt Upper
Bronchus
Tumor
Lobe
Tumor
NDP
stem
Tumor
Lobe
Tumor
Bronchus
Tumor
Bronchus
Tumor
Bronchoscopic
in the Literature
Treatmenf
Bronchus
Lt Lower
Stem
Resection
Lobectomy
Main
Sleeve
Rt Pneumonectomy
RemOVol
Lt
Lobectomy
Lobectomy
Bronchoscopic
Rt Lower
Rt Upper
Rt Pneumonectomy
Resection
Lobectomy
+ Sleeve
Lt Upper
Rt Pneumonectomy
Lt Pneumonectomy
Follow-Up
1 Yr Postop
Recurrence
1 Yr Postop
Recurrence
9 yr Postop
1 Yr postop Alive
No
Pneumonia 12 MO Postop
Died
cauterization
bronchoscopic
repeated
requiring
ReCUW3lCe
NRf
1 Yr Postop
No Recurrence
NRf
No
No Recurrence
NRI
$
5
E
2
;
%
s
i;
ITi
Not
Reported.
Not done.
=
t
1 =
= Unknown.
(1976)
Andrassy
(1976)
Androssy
(1976)
Androssy
(1976)
Androssy
(1976)
Andrassy
( 1976)
Andrassy
(1976)
Andrassy
(1976)
Androssy
(1975)
Salyer
(1974)
Kyriokos
(1973)
Burcharth
*?
-
21
20
19
18
17
16
15
14
13
12
11
(3)
(4)
(2)
18
17
19
18
17
17
10
18
18
13
15
M
M
F
M
M
M
M
M
M
M
F
1
9
0
36
1
6
48
24
?’
144
?*
+
-
t
+
_
+
+
-
_
+
0
-
+
_
-
+
_
-
_
+
+
0
+
_
+
+
_
-
_
+
+
+
0
Rt Bronchus
Bronchus
Rt Upper
Rt Bronchus
Bronchus
Lt tower
Bronchus
Rt lower
Bronchus
Lt Upper
Bronchus
Rt Upper
Rt Bronchus
Bronchus
Rt Middle
lntermedius
Tumor
Lobe
Tumor
lntermedius
Tumor
Lobe
Tumor
Lobe
Tumor
Lobe
Tumor
Lobe
Tumor
lntermedius
Tumor
Lobe
Tumor
?*
?’
Excision
Rods
Nodes
Biopsy
8 Lower
8 Lower
8 Lower Lobectomies
Rt Middle
Lobectomies
Rt Middle
Lobectomies
Rt Middle
Lobectomy
tt Lower
local
4000
(+)
Open
Loco1 Excision
Rt Thoracotomy
Lobectomies
8 Rt Middle
Resection
Rt Lower
Local
?*
Lobectomy
7 MO Postop
3 Yr Postop
3 Yr Postop
9 Yr Postop
2 MO Postop
10 Yr Postop
No Recurrence
10 Yr Postop
No Recurrence
2 Yr Postop
No Recurrence
2 Yr Postop
No Recurrence
2 Yr Postop
No Recurrence
Elev 5HIAA
Died
8 Y r Postop
No Recurrence
Alive
Alive
of Pneumonia
Dead
Alive % n
516
ANDRASSY,
FELDTMAN,
AND STANFORD
Generally these tumors involve major bronchi, and therefore the symptoms and physical signs are those of bronchial obstruction. Cough, hemoptysis. and pneumonia were the most frequent presenting findings. These findings in the young child are frequently associated with a foreign body and since an adequate history may not be available, diagnostic bronchoscopy is frequently employed. These same symptoms are unfortunately not vigorously pursued in the adolescent where a negative history for aspiration and nondiagnostic roentgenogram are obtained. In this series, all I6 patients subjected to diagnostic bronchoscopy were found to have a central lesion easily visualized via bronchoscopic evaluation. The carcinoid tumors generally present in one of two growth patterns: endobronchial polypoid masses that produce segmental bronchial obstruction followed by atelectasis and infection, or as “iceberg” lesions with predominantly extrabronchial growth, the small intrabronchial extent of which gives rise to mucosal ulceration and hemoptysis.6 bronchial and intestinal carcinoids are In routine histologic preparations, usually indistinguishable. Frequently, carcinoids are confused with undifferentiated small cell (oat cell) carcinomas on biopsy in adults. This should be somewhat less of a problem in children since statistically the oat cell tumor is extremely rare in adolescence. The carcinoid tumor consists histologically of festoons and ribbons of small polyhedral cells with central nuclei and eosinophilic cytoplasm arranged in a plexiform or an organoid pattern which resembles that of carcinoid tumors (ar-
Fig. 1.
Carcinoid tumor of the lung (hematoxylin-eorin,
x 250)
BRONCHIAL CARCINOID
517
TUMORS
gentaffinomas) of the gastrointestinal tract (Fig. I). A lengthy discussion of the histologic findings with clinical correlation has recently been presented by Salyer3 and co-workers. Treatment consisted of resection in the majority of our cases. In only one case’ was bronchoscopic removal employed, and this necessitated repeated bronchoscopic cauterization for recurrent tumor. Two patients (13, 15) underwent thoractomy and local excision and were alive without disease 9 and 2 yr, respectively. The survival rate following resection of these tumors is generally very good. It is of significant interest, however, that the two early postoperative deaths were seen in patients with symptoms for 144 and 48 mo, respectively. Perhaps earlier diagnosis would have led to curative resection. Bronchoscopic evaluation is indicated early in all children with symptoms of recurrent bronchial obstruction or hemoptysis. Prompt thoractomy and lobectomy offers a good long-term prognosis. REFERENCES I.
Versha
nomas 55:4I
I
JJ, Connolly
in children.
JE:
J Thor
Bronchial
Cardiovasc
adeSurg
67:634
417. 1968
2. Burchorth cinoids.
Stand
F.
Axelsson
J Thor
C:
Cardiovasc
Lung Surg
car7:72
7x. 1973 3. Salyer Bronchial 1537. 1975
4. Kyriakos
5. Holman vol
DC,
Salyer
WR,
tumors.
Eggleston Cancer
JC:
36: I522
2,
Webber
men.
B: Cancer
J Thor
CW.
Muschenbaum
Diseases
Hagerstown,
and
Surg
C: Broncho-
Related
Maryland.
Disorders. Harper
and
1972, pp 813-815
6. Nissane John M (ed) Pathology and
of the
Cardiovasc
64X. 1974
pulmonary Row,
carcinoid
M.
lung in young
Childhood.
1975, pp 520-523
2nd ed..
St.
of Infancy
Louis,
Mosby.
