ACTA OTORHINOLARYNGOLOGICA ITALICA 2010;30:209-212
A primary squamous cell carcinoma of the trachea:
Case report and review of the literature
Carcinoma squamocellulare primitivo della trachea: Caso clinico e revisione della
G. ABBATE, A. LANCELLA, R. CONTINI, A. SCOTTI
Division of Otorhinolaryngology and Head and Neck Diseases, “S. Biagio” Hospital, Domodossola, Italy
Primary windpipe tumour is extremely rare. Squamous cell carcinoma is the most common malignant form, especially in smokers. The
radiological appearance of these neoplasms can be classifed as intra-luminal, wall-thickening, exophytic form; the majority of the lesions
are obstructive in nature and then tend to extra-luminal invasion. This tumour is usually diagnosed late on account of delayed specifc
symptoms: haemoptysis, dyspnoea, coughing, hoarseness, stridor. Surgery, followed by adjuvant radiotherapy, is the treatment of choice;
primary radiotherapy, in inoperable cases, can represent a curative management option. A case of primary tracheal cancer is described and
a review of the literature is presented.
KEY WORDS: ?????????????????????????????????????????????????????????????????
Il tumore primitivo della trachea è molto raro e il carcinoma squamocellulare è la forma più frequente, soprattutto nei fumatori. Radiolo-
gicamente si possono distinguere forme a sviluppo intraluminale, parietale, esoftico; nella maggior parte dei casi presentano un accresci-
mento in senso ostruttivo e poi tendono alla estensione extraluminale. Questa neoplasia di solito è diagnosticata in fase avanzata a causa
della comparsa tardiva di sintomi specifci quali emottisi, dispnea, tosse, raucedine, stridore. La chirurgia e la radioterapia postoperatoria
rappresentano il trattamento di scelta, nei casi inoperabili la radioterapia da sola può essere una valida opzione terapeutica. Viene pre-
sentato un caso di carcinoma primitivo della trachea e revisionata la letteratura sull’argomento.
PAROLE CHIAVE: ???????????????????????????????????????????????????????????????
Acta Otorhinolaryngol Ital 2010;30:209-212
Primary carcinoma of the trachea is not common 1-3.
This rarity makes research into the natural history and
treatment very diffcult 4. Furthermore, data related to
these tumours are limited 5. These tumours although ex-
tremely rare present extremely variable clinical and his-
tological features 6.
Most primary cervical tracheal tumours are malignant: ad-
enoid cystic carcinoma (ACC), squamous cell carcinoma
(SCC), adenocarcinoma 5-12, mucoepidermoid carcinoma,
carcinoid tumour 3, oat cell carcinoma 13.
SCC is the most common pathology in smokers, ACC is
more prevalent among non-smokers 5.
Benign tumours are xanthogranuloma and pleomorphic
A carcinoma arising in the thyroid or oesophagus can
spread to the trachea; moreover, the trachea can be the
site of a metastasis from recurrent carcinoid tumour in the
left main bronchus 9, larynx, lung 1 7, colon 2.
The radiological appearance of the tumours can be classi-
fed as: intra-luminal, wall-thickening, exophytic form 7.
Endoscopic evaluation reveals that the majority of the le-
sions are bulky and obstructive in nature 6.
Malignant tumours tend to spread to extra-luminal inva-
sion, for example, into the thyroid gland 7.
These tumours also tend to be diagnosed late on account
of delayed specifc symptoms 2: haemoptysis, dyspnoea,
cough, hoarseness, stridor being the most common 1 5-7;
when patients do not present blood in the sputum, the ini-
tial diagnosis is thought to be bronchial asthma; in many
cases, therefore, suffocation almost occurs before surgical
Computed tomography (CT), in coronal projection, is
the most useful radiological examination for tracheal tu-
mours. Bronchoscopy and radiological examination are
G. Abbate et al.
complementary procedures. The main advantage of imag-
ing is the demonstration of tracheal wall thickening and
extra-luminal changes 7.
Persistent or progressive local disease can cause compli-
cations: fatal haemorrhage, oesophago-tracheal fstula,
tracheal necrosis 15, tracheal stenosis 8.
Management of tracheal tumours includes interventional
endoscopy, surgery, radiotherapy, endoluminal brachy-
Extensive segmental resection of the trachea is the treat-
ment of choice for primary malignant, and, occasionally,
for benign tracheal tumours. Interventional endoscopy is
part of modern tracheal surgery 3. The resulting tracheal
clearance has both palliative and curative purposes 3.
Post-operative complications are mediastinitis, bilateral
pneumonia, wound-healing disorders 3 15.
Radiation therapy is effective treatment for primary tra-
cheal neoplasm. Surgery, followed by adjuvant radiother-
apy and primary radiotherapy in inoperable cases repre-
sent potentially curative treatment options 5 9 15.
Positive lymph nodes or invasive disease at resection mar-
gins appear to have an adverse effect on the management
of SCC; such an effect is not demonstrable with adenoid
cystic carcinoma 16.
Surgery is the treatment of choice and up to 50% of the
trachea can be resected with modern techniques 4 8.
With tracheostomy a curative excision or a palliative exci-
sion is possible 18.
The sleeve trachea resection is one of the optimal sur-
gical modalities, the other options are: partial tracheal
wall resection, immediate tracheal reconstruction, total
laryngectomy + partial resection of trachea and thyroid
lobectomy 8 12, resection and primary reconstruction,
laryngotracheal resection, cervico-mediastinal exentera-
tion, carinal resection and reconstruction 16.
Trachea anastomosis is suitable for small defects. The
platysma myocutaneous fap combined with the facial fap
of the sternohyoid muscle, sternocleidomastoid myoperio-
steal fap and the pectoralis major musculocutaneous fap
are applied to reconstruct the defects of cervical trachea 11.
Resection and post-operative radiation therapy are the
treatment of choice, complete resection is the desired goal
and demands knowledge of the principles of tracheal sur-
Debulking surgery, followed by radiotherapy, may pro-
vide effective and permanent control in ACC6 and can be
effective in obviating local recurrence 14.
Endo-bronchial high dose-rate brachytherapy may be used
for tracheal tumours, even as a boost for external beam ir-
radiation or in recurrences. Long-term survival may also
be expected, particularly for tumours with adenoid cystic
Palliation has improved with the introduction of laser re-
section, brachytherapy and stents. SCC may have a better
prognosis, in the trachea, than in the lung 4.
According to some Authors, patients who undergo pri-
mary surgery with adjuvant radiotherapy appear to have
better disease-specifc and overall survival rates compared
with patients undergoing primary radiotherapy with or
without chemotherapy 5.
After surgical management, the 3- and 5-year surviv-
al rates are 79.80%, 48.36% for ACC, 80% and 20%
for SCC 8; in 1, 3, 5, 10 years are 82.3%, 75%, 75%,
Carvalho Hde, et al. presented their experience with high
dose-rate endobronchial brachytherapy; they reported a
good local control, at the time of the frst bronchoscopic
control. They treated 4 patients with non-resectable tra-
cheal tumour: two patients with SCC died at the 6th and
33rd month, respectively, after treatment, only the second
presented local recurrence. The other two patients were
alive after 64 and 110 months of follow-up17.
Some Authors discussed the role of radiation therapy
alone: the median survival for SCC was 33 months, for
ACC 94.2. The 1-, 3-, 5-year survival rates were 64.7%,
64.7%, 26% for SCC, 85.7%, 85.7%, 85.7% for ACC. Pa-
tients with ACC and patients with complete remission fol-
lowing treatment had a signifcantly better survival prob-
Other Authors reported that of 14 resected patients suf-
fering from primary tracheal tumours, at the last follow
up (10 years), 9 are still alive. These Authors observed 5
long-term survivors > 6 years with adenoid cystic carci-
noma or muco-epidermoid carcinoma.
The 5-year survival rate for low grade malignant tumours
arising in the trachea (carcinoid, muco-epidermoid carci-
noma, adenoid cystic carcinoma) is 78.8% 3.
R.V. a 54-year-old male, heavy drinker and smoker, had
been suffering from dyspnoea, sometimes haemoptysis,
cough, weight loss ~10 Kg for approximately 2 months.
The thorax studied by standard radiology showed a left
pneumonia, routine blood tests revealed leukocytosis,
neutrophilia, high levels of transaminases. Nevertheless,
the patient refused admission to hospital and, therefore,
underwent treatment with ceftriaxone, moxifoxacina,
acetylcysteine, prednisone at home.
After 9 days, X-rays and blood tests had improved.
After 15 days dyspnoea had become more severe and was
treated as asthma with beclomethasone aerosol and oral
The rhino-pharyngo-laryngeal region, explored by fex-
ible fberoptic examination, showed evidence of right la-
Radiographic examination of the oesophagus was normal
A tracheobronchoscopic examination demonstrated evi-
dence of a voluminous tracheal mass; during performance
Primary squamous cell carcinoma of the trachea
of this test, heart arrest with respiratory failure occurred
and the patient was reanimated with heart massage and
Three hours later the patient underwent tracheostomy (IV
– V tracheal ring).
CT showed a voluminous wall-thickening solid tracheal
mass, extending from the subglottic space to the upper
mediastinic space, about 6-7 cm in length (Figs. 2, 3, 4).
Due to the size of the lesion, the patient was submitted
to palliative radiotherapy on T and N (trachea and neck),
total dose 50.4 Gy, fractionated 1.8 Gy/die, with boosts on
the trachea reaching 61.2 Gy. During follow-up, a relapse
was observed on the posterolateral wall of the trachea,
about 4 months after radiotherapy.
R.V. died one year after diagnosis.
Discussion and conclusions
Primary cancer of the trachea is extremely rare and, there-
fore, pertinent data are limited.
Most primary cervical tracheal tumours are malignant, for
example, SCC in smokers, ACC in non-smokers, carcinoid
tumour, mucoepidermoid carcinoma, oat cell carcinoma;
benign neoplasms can also be observed, for example, xan-
thogranuloma and pleomorphic adenoma.
We can diagnose tumours localized in the trachea as
metastasis from tumours of thyroid gland, bronchus,
oesophagus, larynx, lung, colon.
Tracheal neoplasms, benign and malignant, are usually
bulky and obstructive; they increase in size and tend even-
tually to extra-luminal invasion into the thyroid or upper
Unfortunately, these neoplasms are often diagnosed
late on account of delayed specifc symptoms (blood
sputum, coughing, dyspnoea). Very often, the patient
undergoes treatment for bronchial asthma, this mistake
thus delays diagnosis and the tracheal tumour becomes
Tracheo-bronchoscopy and CT, in coronal projection, are
basic examinations for these problems.
Surgery (segmental resection, sleeve resection, immediate
or secondary reconstruction using faps) followed by ra-
diotherapy is the treatment of choice. Radiotherapy alone
is a possible treatment option in inoperable cases.
Endo-bronchial brachytherapy may be used for tracheal
tumours, especially if the tumour is small in size.
Palliation has improved with the introduction of laser re-
section and stents.
Fig. 1. Barium swallow – oesophagus appears normal.
Fig. 2. CT scan shows a volumi-
nous wall-thickening solid tracheal
Fig. 3. CT scan shows postero-
lateral tracheal wall infiltration in
Fig. 4. Sagittal CT shows trache-
al tube in its usual site with tumour
G. Abbate et al.
The Authors chose to publish this report, given the rare
occurrence of this form of cancer.
A smoker presenting dyspnoea could be carrier of a tra-
cheal neoplasm and early diagnosis is very important for
We pay particular attention in all cases of dyspnoea, especially
if antibiotics and steroids have not led to an improvement in
symptoms. A rhino-pharyngo-laryngeal examination is always
necessary, a CT of the thorax, in a coronal projection, can clar-
ify diagnosis, thus making correct management possible.
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Received: December 9, 2008 - Accepted: May 20, 2009
Address for correspondence: Dr. G. Abbate, Via Alcide De Gasperi
14, 28845 Domodossola (VB), Italy. Fax +39 0324 491367. E-mail: