Medical and Pediatric Oncology 17510-513 (1989)
Lung Cancer in Childhood
David M. Epstein, MD, and judith M. Aronchick, MD
A 16-year-old boy had unresolving right
lower lobe consolidation due to primary
adenocarcinoma of the lung. Lung cancer is
rare in children, is usually adenocarcinoma
or undifferentiated histology, and fre-
quently presents with advanced disease. lt
may be confused with atelectasis attribut-
I Key words: children, neoplasms, pulmonary
INTRODUCTION
Bronchogenic carcinoma is the leading cause of can-
cer mortality in adults today. In general, its incidence
increases with advancing age, becoming most frequent in
the 55-75 age group. It is unusual to encounter lung
cancer in pediatric patients, although multiple reports
have described its occurrence. In a review of 4,000 cases
of bronchogenic carcinoma, Ochsner reported an inci-
dence of 0.16% in the first decade and 0.7% incidence in
the second decade of life [ 11. We present a 16-year-old
boy with primary adenocarcinoma of the lung to describe
the clinical features of childhood lung cancer, problems
in differential diagnosis, and differences in lung cancer
between children and adults.
CASE REPORT
The patient was a 16-year-old white boy admitted to
another hospital with a 1-week history of increasing
right-sided chest discomfort and several episodes of
streaking hemoptysis. He had been followed as an out-
patient for 3 months for an asymptomatic right lower
lobe infiltrate with intermittent wheezing, receiving sev-
eral courses of antibiotics and bronchodilators.
Physical examination revealed a temperature of
99.2"F with decreased breath sounds at the right base and
dullness to percussion. There was no audible wheeze.
Laboratory data indicated hemoglobin 13.2 gidl and
WBC count 11.9-14.3 THO/UL. A repeat chest radio-
graph showed progression of a right lower lobe infiltrate
first seen 1 month before; there was also a probable right
pleural effusion. A chest computed tomographic (CT)
0 1989 Alan R. Liss, Inc.
able to a foreign body or bronchial ade-
noma, plasma cell granuloma, pulmonary
sequestration, or chronic infection. We re-
view the clinical features of pediatric lung
cancer and differences in lung cancer be-
tween children and adults.
examination showed extensive right lower lobe consoli-
dation with pleural reaction. There was no hilar or me-
diastinal lymphadenopathy. He underwent a nondiagnos-
tic fiberoptic bronchoscopy with no endobronchial
lesions seen.
The patient was transferred to our institution for fur-
ther evaluation. The chest radiograph showed right lower
lobe consolidation (Fig. 1). A repeat fiberoptic broncho-
scopy again demonstrated no endobronchial lesion; how-
ever, transbronchial biopsy in the right lower lobe indi-
cated carcinoma of unknown cell type. Bronchial lavage
cytology showed poorly differentiated carcinoma. The
patient underwent extensive evaluation to search for an
extrathoracic primary carcinoma or other signs of meta-
static disease. A CT examination of the chest revealed
right lower lobe consolidation with a right pleural effu-
sion (Fig. 2). No hilar or mediastinal adenopathy was
demonstrated. CT examination of the abdomen and re-
troperitoneum was normal, as were a radionuclide bone
scan and testicular ultrasound. The patient underwent a
thoracentesis, which showed no evidence of malignant
cytology.
In the absence of metastatic tumor or a demonstrable
extrathoracic primary carcinoma, the patient underwent a
right thoracotomy, which showed the right lower lobe to
From the Department of Radiology, Hospital of the University of
Pennsylvania, Philadelphia, Pennsylvania.
Address reprint requests to David M. Epstein, M . D . , Department of
Radiology, Hospital of the University of Pennsylvania, 3400 Spruce
St., Philadelphia, PA 19104.
 Lung Cancer in Childhood 511

Fig. 1. Radiographs of the chest demonstrate right lower lobe con-

solidation. A: Posteroanterior. B: Lateral. No hilar or mediastinal
lymphadenopathy is evident.

be largely replaced by tumor. It was removed. There

were adhesions to the pleura and diaphragm, which were
free of tumor in the excised specimen. However, all the
resected mediastinal lymph nodes were positive for tu-
mor, including the nodes surrounding the pulmonary
artery, azygos, and superior vena cava. In addition, the
bronchial margin was positive for adenocarcinoma. The
final diagnosis was adenocarcinoma of the right lower
lobe with metastatic tumor in all the sampled mediastinal
lymph nodes. The patient only recently completed a
course of postoperative radiation therapy.

DlSCUSSI0N
In their review of 230 pulmonary neoplasms in chil-
dren, Hartman and Shochat found that more than two
thirds of the tumors fell into three histologic categories:
Bronchial adenoma, inflammatory pseudotumor, and
bronchogenic carcinoma [2]. Bronchial adenoma that
includes bronchial carcinoid, mucoepidermoid carci-
noma, and adenocystic carcinoma is a low-grade malig-
nancy that has the potential for both local and lymphatic
or distant dissemination in 10-15% of patients.
Most children are symptomatic with cough (80%),
pneumonitis (60%), or hemoptysis (33%). Most lesions
are endobronchial and may produce atelectasis due to
bronchial obstruction [2]. A cherry red endoscopic ap-
pearance is characteristic, and biopsy may be hazardous
because of its tendency to hemorrhage. As in adults,
Fig. 2. Computed tomography image reveals right lower lobe con-
solidation with air bronchograms. Pleural effusion is identified poste-
riorly which is contiguous with the consolidation. No adenopathy is
identified on the other images.
carcinoid syndrome is unusual with bronchial carcinoid
tumors. Indeed, to our knowledge, no case of carcinoid
syndrome has been reported in children with bronchial
carcinoid. Treatment is usually lobectomy or pneumo-
nectomy with removal of involved lymphatics. Pulmo-
nary sleeve resection has also been advocated as a means
of conserving lung tissue in young patients with bron-
chial adenoma.
512 Epstein and Aronchick
Inflammatory pseudotumor or plasma cell granuloma
is a localized benign proliferation of mature plasma cells
and reticuloendothelial elements. Approximately two
thirds of patients are below the age of 30, and it is said
to be the most common tumorlike lesion of the lung in
children under the age of 16 [3]. Most patients are
asymptomatic, with more than 50% of lesions detected
on routine chest radiographs. It usually appears as a
well-circumscribed solitary round or ovoid mass that can
grow quite large and may occupy an entire lung. In
approximately 25% of patients, it may appear as a “coin
lesion.” This is an entirely benign entity with no evi-
dence of malignant transformation. The treatment of
choice is surgical removal by limited local resection.
Bronchogenic carcinoma is second in frequency only
to bronchial adenoma and comprises one third of malig-
nant primary pulmonary neoplasms in children. In those
patients reported in the pediatric age group, 20% are
under 3 years of age [2]. There is an equal sex distribu-
tion in childhood lung cancer, as contrasted to adults.
Cigarette consumption, the primary risk factor for lung
cancer in adults does not play a role in the pediatric age
group. It is difficult to identify other risk factors, al-
though Cayley and coworkers report, in their review of
15 cases of childhood lung cancer collected from the
literature, that 3 were associated with congenital cystic
malformations of the lung [4].
In contrast to adults with lung cancer, the incidence
of adenocarcinoma and undifferentiated or small cell
carcinoma (80%) is much higher than the squamous cell
type (12%) [2]. The most frequent radiographic
presentation is a peripheral mass. This is consistent with
the predominance of adenocarcinoma or an undifferen-
tiated histology. Squamous cell carcinomas may also
present as a peripheral mass, although they are some-
what more likely to be central or to have an
endobronchial lesion with resultant atelectasis. Most
children with lung cancer are symptomatic with cough,
recurrent pneumonia, or hemoptysis . They frequently
have widespread disease at presentation with mediastinal
and distant metastases [2,4]. These children may present
with systemic symptoms of bone pain or weight loss
indicative of distant metastatic disease. In areas where
screening has been done for tuberculosis, asymptomatic
masses due to lung cancer have been detected in children
[ 5 ] . This is also not surprising, since penpheral
pulmonary neoplasms may not cause symptoms until
dissemination occurs. However, in a child who is truly
asymptomatic, a pulmonary mass is twice as likely to be
benign as malignant.
Our patient typified many of the features characteristic
of pediatric lung cancer [ 1,2,4-61. He was symptomatic
with cough, chest pain, hemoptysis, and an unresolving
right lower lobe consolidation radiographically. His his-
tology was adenocarcinorna and mediastinal metastases
were present at diagnosis.
His clinical picture presented a variety of consider-
ations in terms of differential diagnosis. Foreign bodies
are frequently a cause of unresolving consolidation in
very young children, particularly in the right lower lobe.
Other fixed endobronchial lesions, such as bronchial
adenoma, should also be considered in this patient. How-
ever, a negative fiberoptic bronchoscopy should exclude
both possibilities. Pulmonary sequestration, both intra-
lobar and extralobar, may produce chronic consolidation
usually in either the right or left lower lobe. Sequestra-
tion may appear cavitary if there is communication with
the bronchial tree. This diagnosis is established by the
demonstration of systemic blood supply from the aorta to
the sequestered segments. Infectious etiologies, includ-
ing primary tuberculosis or fungal infection not effec-
tively treated with conventional antibiotics, should also
be considered. These etiologies were excluded through
negative skin tests, complement-fixation titers, and mi-
crobiological studies from bronchial washings and tho-
racentesis specimens.
The best hope for cure of lung cancer is surgery, so
that one is tempted to be particularly aggressive with
young patients. DeCaro and Benfield investigated 35
patients under the age of 40 [7]. The young patients
undergoing surgery fared better than the nonoperated
patients, but their 5-year survival rate was not signifi-
cantly different from that of their older operated coun-
terparts. This study concluded that there was no justifi-
cation for doing anything different for younger patients
with lung cancer, except that aggressive local resection
in the absence of lymph node metastases was indicated.
Moreover, evolving surgical opinion in adults suggests
that patients with both squamous and adenocarcinoma of
the lung and ipsilateral mediastinal metastases detected
at thoracotomy may benefit from excision of the tumor
and all accessible mediastinal lymph nodes when treated
with postoperative irradiation [8,9]. Encouraging results
in adults suggests that this approach might also be ap-
plicable and benefit selected pediatric patients with lung
cancer.
Our patient underwent an extensive preoperative eval-
uation for metastatic tumor that proved negative. Al-
though the presence of a pleural effusion is often indic-
ative of metastatic tumor, its presence does not exclude
attempts at curative surgery in the presence of a negative
pleural fluid cytology at thoracentesis [ 101. CT exami-
nation of the chest did not disclose any enlarged me-
diastinal lymph nodes. In the absence of identifiable
mediastinal lymphadenopathy with CT, many surgeons
will eliminate preoperative surgical staging and proceed
directly to thoracotomy [ 1 11. Unfortunately, at pulmo-
nary resection, mediastinal metastases were present.

While there are no series in the literature addressing
specifically pediatric lung cancer, it appears that lung
cancer in children has a variable prognosis and behaves
in a fashion similar to adult bronchogenic carcinoma.
The outlook is probably worse because of the higher
proportion of adenocarcinomas and undifferentiated car-
cinomas and the many cases of distant disease at diag-
nosis. While there are pediatric patients with prolonged
survival, the biologic behavior of the particular tumor
and the extent of disease at diagnosis are the major
factors influencing survival. The patient discussed here,
for example, had a poorly differentiated adenocarcinoma
with mediastinal involvement at surgery, both of which
portend a poor prognosis.
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Lung Cancer in Childhood 513
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