A 40-year-old male smoker presented to his primary care physician for persistent chest-wall pain following a mild sports injury. He also complained of worsening exertional dyspnea and night sweats. There was no report of significant surgical or medical history.
Douglas W. White, MD
Justin Q. Ly, MD
from the Department of Radiology, Wilford Hall Medical Center,
Lackland AFB, TX;
Douglas P. Beall, MD
from the Department of Radiology and Nuclear Medicine, Uniformed
Services University of Health Sciences, Bethesda, MD;
Joseph H. McDermott, MD
from the Department of General Surgery, Wright-Patterson Medical
Center, Wright Patterson, OH; and
James A. Graham, MD
, from the Department of Pulmonology, Wilford Hall Medical
Center, Lackland AFB, TX.
A 40-year-old male smoker presented to his primary care
physician for persistent chest-wall pain following a mild sports
injury. He also complained of worsening exertional dyspnea and
night sweats. There was no report of significant surgical or
medical history. The physical examination was unremarkable except
for some minor chest-wall tenderness. Anteroposterior and lateral
chest films were obtained (Figure 1). Based on these findings, a
contrast-enhanced computed tomography (CT) study was performed
Typical bronchial carcinoid tumor
Anteroposterior (Figure 1) and lateral chest radiographs
revealed a large (3- to 4-cm) mass at the posterior aspect of the
left hilum. Contrast-enhanced CT scan of the chest revealed a large
mass in the superior segment of the left lower lobe, with
associated left hilar and subcarinal adenopathy (Figure 2).
18F-flourodeoxyglucose positron emission tomography (PET) revealed
a nonavid mass, excluding the possibility of a bronchogenic
carcinoma (not shown). Bronchoscopy revealed a mass within the
superior segmental bronchus of the left lower lobe (Figure 3), from
which a biopsy was obtained for pathologic identification (Figure
4). After the diagnosis was established, the patient underwent a
successful left pneumonectomy. Histologic examination of the tumor
was consistent with typical carcinoid tumor (Figure 5).
Carcinoid tumor is a serotonin-producing malignant tumor
belonging to a class of neoplasms known as the neuroendocrine
tumors. They are unique in that they can present with tumor-like
symptoms or with a combination of symptoms related to endocrine
dysregulation. Most cases of carcinoid are found incidentally in
the appendix following an appendectomy. The prevalence of carcinoid
in the general population is actually quite high, but >95% of
these tumors occur in one of three sites: the appendix, rectum, or
Carcinoid tumors of the lung are much less common and represent
1% to 2% of all lung cancers.
They arise from the neurosecretory cells of the bronchial mucosa.
Two different types have been identified.
The first and most common is referred to as typical carcinoid.
Typical carcinoid tumors are low-grade tumors, with 10-year
survival rates approaching 90%.
They are capable of local invasion, including invasion of local
lymph nodes, but rarely metastasize. The second type of carcinoid
is referred to as atypical carcinoid. It is much more aggressive
and carries a 5-year survival rate of 25% to 69%.
Both subtypes tend to arise from the bronchial tree and spread by
local invasion. Typical carcinoid tumors are more commonly found
centrally within the major bronchi, whereas the atypical carcinoids
tend to arise from the peripheral and central bronchi with equal
The most frequently presenting symptoms of carcinoid are lobar
obstruction, hemoptysis, dyspnea, cough, and lobar pneumonia
secondary to obstruction. Some are found serendipitously on chest
radiographs in asymptomatic patients; rarely, patients may present
with endocrine abnormalities, such as Cushing's syndrome.
Both types of carcinoid tumors of the lung can occur in patients of
any age, without gender preference. The role that smoking plays in
the development of carcinoid is still under debate.
Definitive diagnosis and classification is made by biopsy and
pathologic examination of the tissue. Due to its predilection for
arising from the luminal surface of the major bronchi, adequate
tissue samples can often be obtained through endobronchial biopsy.
Alternatively, tissue may be obtained through CT-guided fine-needle
biopsy or thoracotomy.
Helical chest CT is helpful in determining the size and position
of the tumor but is not as accurate in assessing tumor extension as
high-resolution CT (HRCT).
Helical chest CT may also help in narrowing the differential
diagnosis, by identifying characteristic findings of carcinoid.
These findings include dense ossification, scattered
calcifications, and intraluminal location.
Since carcinoid tumors are metabolically sluggish relative to most
other malignancies, PET imaging is most often negative. These
results can be misleading, because it may falsely suggest that the
lesion is benign.
Carcinoid is treated surgically, with the approach dependent on
the size, location, and tissue type. Treatment for intraluminal
typical bronchial carcinoid may be attempted with bronchoscopic
removal of the tumor. This technique combines direct bronchoscopic
visualization with HRCT. Bronchoscopic removal of these tumors
leads to complete eradication in the majority of patients.
Tumors not meeting the criteria for endobronchial resection may be
treated surgically with bronchial sleeve resection, segmentectomy,
lobectomy, or pneumonectomy.
Chemotherapy has not been demonstrated to be successful as a
treatment option but, rather, is limited to a palliative role in
the management of diffuse metastatic carcinoid; however,
investigations involving chemotherapy are still ongoing.
Bronchial carcinoid tumor is a low-grade malignant tumor with
slow progression and is associated with a favorable prognosis.
There are two subtypes, with the less aggressive type comprising
approximately 90% of all bronchial carcinoids. The definitive
diagnosis is made by histologic examination. Carcinoid tumors
typically arise from the luminal surface of major bronchi, and
definitive treatment involves surgical resection.