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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 able to a foreign body or bronchial ade-
lower lobe consolidation due to primary noma, plasma cell granuloma, pulmonary
adenocarcinoma of the lung. Lung cancer is sequestration, or chronic infection. We re-
rare in children, is usually adenocarcinoma view the clinical features of pediatric lung
or undifferentiated histology, and fre- cancer and differences in lung cancer be-
quently presents with advanced disease. lt tween children and adults.
may be confused with atelectasis attribut-

I Key words: children, neoplasms, pulmonary

INTRODUCTION examination showed extensive right lower lobe consoli-


dation with pleural reaction. There was no hilar or me-
Bronchogenic carcinoma is the leading cause of can- diastinal lymphadenopathy. He underwent a nondiagnos-
cer mortality in adults today. In general, its incidence tic fiberoptic bronchoscopy with no endobronchial
increases with advancing age, becoming most frequent in lesions seen.
the 55-75 age group. It is unusual to encounter lung The patient was transferred to our institution for fur-
cancer in pediatric patients, although multiple reports ther evaluation. The chest radiograph showed right lower
have described its occurrence. In a review of 4,000 cases lobe consolidation (Fig. 1). A repeat fiberoptic broncho-
of bronchogenic carcinoma, Ochsner reported an inci- scopy again demonstrated no endobronchial lesion; how-
dence of 0.16% in the first decade and 0.7% incidence in ever, transbronchial biopsy in the right lower lobe indi-
the second decade of life [ 11. We present a 16-year-old cated carcinoma of unknown cell type. Bronchial lavage
boy with primary adenocarcinoma of the lung to describe cytology showed poorly differentiated carcinoma. The
the clinical features of childhood lung cancer, problems patient underwent extensive evaluation to search for an
in differential diagnosis, and differences in lung cancer extrathoracic primary carcinoma or other signs of meta-
between children and adults. 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
CASE REPORT
demonstrated. CT examination of the abdomen and re-
The patient was a 16-year-old white boy admitted to troperitoneum was normal, as were a radionuclide bone
another hospital with a 1-week history of increasing scan and testicular ultrasound. The patient underwent a
right-sided chest discomfort and several episodes of thoracentesis, which showed no evidence of malignant
streaking hemoptysis. He had been followed as an out- cytology.
patient for 3 months for an asymptomatic right lower In the absence of metastatic tumor or a demonstrable
lobe infiltrate with intermittent wheezing, receiving sev- extrathoracic primary carcinoma, the patient underwent a
eral courses of antibiotics and bronchodilators. right thoracotomy, which showed the right lower lobe to
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 From the Department of Radiology, Hospital of the University of
WBC count 11.9-14.3 THO/UL. A repeat chest radio- Pennsylvania, Philadelphia, Pennsylvania.
graph showed progression of a right lower lobe infiltrate Address reprint requests to David M. Epstein, M . D . , Department of
first seen 1 month before; there was also a probable right Radiology, Hospital of the University of Pennsylvania, 3400 Spruce
pleural effusion. A chest computed tomographic (CT) St., Philadelphia, PA 19104.

0 1989 Alan R. Liss, Inc.


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 Fig. 2. Computed tomography image reveals right lower lobe con-
thirds of the tumors fell into three histologic categories: solidation with air bronchograms. Pleural effusion is identified poste-
riorly which is contiguous with the consolidation. No adenopathy is
Bronchial adenoma, inflammatory pseudotumor, and
identified on the other images.
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 carcinoid syndrome is unusual with bronchial carcinoid
or distant dissemination in 10-15% of patients. tumors. Indeed, to our knowledge, no case of carcinoid
Most children are symptomatic with cough (80%), syndrome has been reported in children with bronchial
pneumonitis (60%), or hemoptysis (33%). Most lesions carcinoid. Treatment is usually lobectomy or pneumo-
are endobronchial and may produce atelectasis due to nectomy with removal of involved lymphatics. Pulmo-
bronchial obstruction [2]. A cherry red endoscopic ap- nary sleeve resection has also been advocated as a means
pearance is characteristic, and biopsy may be hazardous of conserving lung tissue in young patients with bron-
because of its tendency to hemorrhage. As in adults, chial adenoma.
512 Epstein and Aronchick

Inflammatory pseudotumor or plasma cell granuloma tology was adenocarcinorna and mediastinal metastases
is a localized benign proliferation of mature plasma cells were present at diagnosis.
and reticuloendothelial elements. Approximately two His clinical picture presented a variety of consider-
thirds of patients are below the age of 30, and it is said ations in terms of differential diagnosis. Foreign bodies
to be the most common tumorlike lesion of the lung in are frequently a cause of unresolving consolidation in
children under the age of 16 [3]. Most patients are very young children, particularly in the right lower lobe.
asymptomatic, with more than 50% of lesions detected Other fixed endobronchial lesions, such as bronchial
on routine chest radiographs. It usually appears as a adenoma, should also be considered in this patient. How-
well-circumscribed solitary round or ovoid mass that can ever, a negative fiberoptic bronchoscopy should exclude
grow quite large and may occupy an entire lung. In both possibilities. Pulmonary sequestration, both intra-
approximately 25% of patients, it may appear as a “coin lobar and extralobar, may produce chronic consolidation
lesion.” This is an entirely benign entity with no evi- usually in either the right or left lower lobe. Sequestra-
dence of malignant transformation. The treatment of tion may appear cavitary if there is communication with
choice is surgical removal by limited local resection. the bronchial tree. This diagnosis is established by the
Bronchogenic carcinoma is second in frequency only demonstration of systemic blood supply from the aorta to
to bronchial adenoma and comprises one third of malig- the sequestered segments. Infectious etiologies, includ-
nant primary pulmonary neoplasms in children. In those ing primary tuberculosis or fungal infection not effec-
patients reported in the pediatric age group, 20% are tively treated with conventional antibiotics, should also
under 3 years of age [2]. There is an equal sex distribu- be considered. These etiologies were excluded through
tion in childhood lung cancer, as contrasted to adults. negative skin tests, complement-fixation titers, and mi-
Cigarette consumption, the primary risk factor for lung crobiological studies from bronchial washings and tho-
cancer in adults does not play a role in the pediatric age racentesis specimens.
group. It is difficult to identify other risk factors, al- The best hope for cure of lung cancer is surgery, so
though Cayley and coworkers report, in their review of that one is tempted to be particularly aggressive with
15 cases of childhood lung cancer collected from the young patients. DeCaro and Benfield investigated 35
literature, that 3 were associated with congenital cystic patients under the age of 40 [7]. The young patients
malformations of the lung [4]. undergoing surgery fared better than the nonoperated
In contrast to adults with lung cancer, the incidence patients, but their 5-year survival rate was not signifi-
of adenocarcinoma and undifferentiated or small cell cantly different from that of their older operated coun-
carcinoma (80%) is much higher than the squamous cell terparts. This study concluded that there was no justifi-
type (12%) [2]. The most frequent radiographic cation for doing anything different for younger patients
presentation is a peripheral mass. This is consistent with with lung cancer, except that aggressive local resection
the predominance of adenocarcinoma or an undifferen- in the absence of lymph node metastases was indicated.
tiated histology. Squamous cell carcinomas may also Moreover, evolving surgical opinion in adults suggests
present as a peripheral mass, although they are some- that patients with both squamous and adenocarcinoma of
what more likely to be central or to have an the lung and ipsilateral mediastinal metastases detected
endobronchial lesion with resultant atelectasis. Most at thoracotomy may benefit from excision of the tumor
children with lung cancer are symptomatic with cough, and all accessible mediastinal lymph nodes when treated
recurrent pneumonia, or hemoptysis . They frequently with postoperative irradiation [8,9]. Encouraging results
have widespread disease at presentation with mediastinal in adults suggests that this approach might also be ap-
and distant metastases [2,4]. These children may present plicable and benefit selected pediatric patients with lung
with systemic symptoms of bone pain or weight loss cancer.
indicative of distant metastatic disease. In areas where Our patient underwent an extensive preoperative eval-
screening has been done for tuberculosis, asymptomatic uation for metastatic tumor that proved negative. Al-
masses due to lung cancer have been detected in children though the presence of a pleural effusion is often indic-
[ 5 ] . This is also not surprising, since penpheral ative of metastatic tumor, its presence does not exclude
pulmonary neoplasms may not cause symptoms until attempts at curative surgery in the presence of a negative
dissemination occurs. However, in a child who is truly pleural fluid cytology at thoracentesis [ 101. CT exami-
asymptomatic, a pulmonary mass is twice as likely to be nation of the chest did not disclose any enlarged me-
benign as malignant. diastinal lymph nodes. In the absence of identifiable
Our patient typified many of the features characteristic mediastinal lymphadenopathy with CT, many surgeons
of pediatric lung cancer [ 1,2,4-61. He was symptomatic will eliminate preoperative surgical staging and proceed
with cough, chest pain, hemoptysis, and an unresolving directly to thoracotomy [ 1 11. Unfortunately, at pulmo-
right lower lobe consolidation radiographically. His his- nary resection, mediastinal metastases were present.
Lung Cancer in Childhood 513

While there are no series in the literature addressing 4. Cayley CK, Caez HJ, Mersheimer W: Primary bronchogenic
specifically pediatric lung cancer, it appears that lung carcinoma of the lung in children. Am J Dis Child 8249-60,
1951.
cancer in children has a variable prognosis and behaves 5 . Niitu Y, Kubota H , Hasegawa S , et al.: Lung cancer (squamous
in a fashion similar to adult bronchogenic carcinoma. cell carcinoma) in adolescence. Am J Dis Child 127:108-1 10,
The outlook is probably worse because of the higher 1974.
proportion of adenocarcinomas and undifferentiated car- 6. LaSalle AJ, Andrassy RJ, Stanford W: Bronchogenic squamous
cinomas and the many cases of distant disease at diag- cell carcinoma in childhood: A case report. J Pediatr Surg 1 2 5 19-
521, 1977.
nosis. While there are pediatric patients with prolonged 7. DeCaro L, Benfield JR: Lung cancer in young persons. J Thorac
survival, the biologic behavior of the particular tumor Cardiovasc Surg 83:372-376, 1982.
and the extent of disease at diagnosis are the major 8. Martini N, Flehinger BJ, Zaman MB, Beattie EJ: Prospective
factors influencing survival. The patient discussed here, study of 445 lung carcinomas with mediastinal lymph node me-
for example, had a poorly differentiated adenocarcinoma tastases. J . Thorac Cardiovasc Surg 80:390-399, 1980.
9. Pearson FG, Delarue NC, Ilves R, et al.: Significance of positive
with mediastinal involvement at surgery, both of which superior mediastinal nodes identified at mediastinoscopy in pa-
portend a poor prognosis. tient resectable cancer of the lung. J Thorac Cardiovasc Surg
83: 1-1 I , 1982.
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cytologically negative pleural effusion in bronchogenic carci-
1. Fontenelle LJ: Primary adenocarcinoma of the lung in a child: noma. Chest 74540-642, 1978.
Review of the literature. Am Surg 42:296-299, 1976. 11. Epstein DM, Stephenson LW, Gefter WB, et al.: The valve of
2. Hartman GE, Shochat SJ: Primary pulmonary neoplasms of child- computed tomography in the preoperative assessment of lung
hood: A review. Ann Thorac Surg 36: 108-1 19, 1983. cancer-A survey of thoracic surgeons. Radiology 16I :423-427,
3. Bahadari M, Liebow A: Plasma cell granuloma of the lung. 1987.
Cancer 31:191-208, 1973