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Thoracic Surgery: Answers
219
Jones JW, Reynolds M, Hewitt RL, Drapanas T. Tracheo-innominate artery erosion:
Successful surgical management of a devastating complication. Ann Surg. 1976;184:194-
204.
3.17 Answer: E
The management of a patient with a recent history of a tracheostomy bleed should follow
a stepwise approach. The differential diagnosis of hemoptysis should be quickly considered,
and other more common etiologies such as tracheitis, wound bleeding, pneumonia, or
traumatic suctioning should be ruled out. Tracheitis rarely involves massive bleeding, but
when it does, the evaluation process should be much the same as for TIF. Postoperative
wound bleeding usually occurs less than 48 hours after placement of the tracheostomy, and
pneumonia is usually associated with increased secretions and fever. Diagnostic modalities
such as chest radiograph or flexible bronchoscopy can be used to confirm these other
conditions but cannot rule out TIF. If TIF is considered, flexible bronchoscopy is not
recommended because the area of concern is often not visualized, and the bronchoscope can
destabilize a clot which is preventing massive hemorrhage.
Once other aetiologies for tracheal bleeding have been excluded, then the possibility of
vascular erosion must be investigated. For bleeding in excess of 10 mL, the tracheal cuff
should first be hyperinflated. A presumptive diagnosis of vascular erosion is likely if the
bleeding is stopped by this maneuver. If massive hemorrhage occurs, the goal is temporary
stability to transfer the patient to the operating room. Overinflating the tracheostomy tube cuff
can be successful in temporarily stopping the bleeding in 85% of cases. If bleeding continues,
an oral endotracheal tube should then be placed. With the tracheostomy tube cuff still
hyperinflated and slightly withdrawn, the endotracheal tube cuff is passed distal to the
tracheostomy tube, and the cuff inflated. If bleeding still continues, the tracheostomy tube
should be removed, and an index finger should be inserted through the tracheostomy stoma
into either the trachea or the pretracheal fascial plane using blunt dissection if the
tracheostomy wound has not yet fully healed. Then digital pressure is applied with the
innominate artery between the finger and the posterior aspect of the upper sternum. This is
known as the Utley maneuver, first described in 1972. This maneuver is successful 90% of
the time, and the pressure should be continued during transfer to the operating room.
Hypotension should be aggressively managed with fluids and blood if needed. Even older
children may benefit from placement of an intraosseous catheter if prompt venous access
cannot be secured. The patient needs to be taken to the operating room for rigid
bronchoscopy. Rigid bronchoscopy allows for tamponade of the artery against the sternum by
the scope. The stoma and anterior wall can be inspected, and blood clots can be removed. If
needed, surgical repair can be done immediately. Arch angiography, computed tomography
angiography of the chest, and electroencephalographic monitoring have been used before
surgical repair in some cases, given the high rates of strokes and neurological sequelae after
surgery. All of these modalities are time consuming, and the patient may not remain
hemodynamically stable throughout the procedures. Furthermore, if the bleeding is
temporarily controlled with the hyperinflated cuff, the angiogram may not show
abnormalities.
Other methods of achieving tamponade have been reported, but it is recommended that
these be performed by those who are skilled in the procedures. Takano and colleagues used a
Fogarty balloon catheter inserted via the brachial artery into the innominate artery under
Shahzad G. Raja
220
radiograph guidance to achieve tamponade after balloon dilation. Myers and Pilch extended
the tracheostomy up the midline to the cricothyroid membrane while leaving the
tracheostomy tube in place. The trachea was opened widely, and packing was placed around
the tracheostomy tube to tamponade the artery between the sternum and spine. Once the
patient reaches the operating room, a variety of corrective surgical techniques can be
attempted, depending on the individual patient's condition. The 2 basic strategies include
those that maintain flow across the innominate artery and those that interrupt flow.
Maintenance of flow can be accomplished by direct repair of the defect or by interposition
grafting. Interruption of flow involves ligation or resection of the innominate artery with
preservation of the right carotid-right subclavian junction. It is best to have an intact circle of
Willis if the latter technique is used. Novel approaches involving various bypass grafts have
been reported as well.
References
Sessa C, Costache V, Porcu P, et al. Tracheoinnominate artery fistula: combined endovascular
and surgical management by emergency stent-graft placement followed by cryopreserved
arterial allograft repair. Ann Vasc Surg. 2006;20:731-5.
Ridley RW, Zwischenberger JB. Tracheoinnominate fistula: surgical management of an
iatrogenic disaster. J Laryngol Otol. 2006;120:676-80.
Thorp A et al. Tracheoinnominate artery fistula: a rare and often fatal complication of
indwelling tracheostomy tubes. Pediatr Emerg Care. 2005;21:763-6.
Gasparri MG, Nicolosi AC, Almassi GH. A novel approach to the management of tracheo-
innominate artery fistula. Ann Thorac Surg. 2004;77:1424-1426.
Allen JS, Wright CD. Tracheoinnominate fistula: diagnosis and management. Chest Surg Clin
N Am. 2003;13:331-41.
Deguchi J, Furuya T, Tanaka N, et al. Successful management of tracheo-innominate artery
fistula with endovascular stent graft repair. J Vasc Surg. 2001;33:1280-1282.
Takano H, Ihara K, Sato S, et al. Tracheo-innominate artery fistula following tracheostomy.
Successful surgical management of a case. J Cardiovasc Surg (Torino). 1989;30:860-3.
Myers RS, Pilch YH. Temporary control of tracheal-innominate artery tistula. Ann Surg.
1969;170:149-51.
Wright CD. Management of tracheoinnominate artery fistula. Chest Surg Clin N Am.
1996;6:865-873.
3.18 Answer: A
Primary tracheal tumors are rare, with an incidence of only 0.2 per 100,000 persons per
year. Tracheal malignancy accounts for less than 0.1% of the cancer deaths per year. When all
series are combined, adenoid cystic carcinoma (ACC) is the second most common type of
tracheal malignancy, preceded by squamous cell carcinoma. The first report of tracheal ACC
was described by Morgagni in 1762, but tracheal resection with primary anastomosis was not
performed until 1957. This procedure was modified in 1964 and popularized by Grillo and his
colleagues.
There have been three large series of primary tracheal malignancies reported in the
English Lature. The clinical behavior, natural history, and response to various treatment
modalities of ACC of the trachea are different from those of other malignant tracheal tumors.
Thoracic Surgery: Answers
221
References
Maziak DE, Todd TR, Keshavjee SH, et al. Adenoid cystic carcinoma of the airway: thirty-
two-year experience. J Thorac Cardiovasc Surg. 1996;112:1522-32.
Allen MS. Malignant tracheal tumors. Mayo Clin Proc. 1993;68:680-4.
Regnard JF, Fourquier P, Levasseur P. Results and prognostic factors in resections of primary
tracheal tumors: a multicenter retrospective study. J Thorac Cardiovasc Surg.
1996;111:808-13.
Pearson FG, Todd TRJ, Cooper JD. Experience with primary neoplasms of the trachea:
clinicopathologic study of 41 cases. Cancer. 1970;25:1448-56.
Grillo HC, Mathisen DJ. Primary tracheal tumors: treatment and results. Ann Thorac Surg.
1990;49:69-77.
3.19 Answer: D
Primary tracheal ACC is found in men and women with almost equal frequency. This
contrasts to tracheal squamous cell carcinoma (SCC) which occurs in males approximately
90% of the time. Extraction from the Lature of 174 patients with tracheal ACC showed a
female-to-male ratio of 1.1:1.0(90 women and 84 men). Average reported ages range from 45
to 60 years. Both sexes present with tracheal ACC at similar ages.
Patients with ACC present with vague symptoms and are often treated for conditions
such as asthma for months to years before being diagnosed. Most patients present with
multiple symptoms, generally caused by tumor involvement of local tissues. Review of three
studies of tracheal malignancies showed that the most frequent complaints are wheezing or
stridor, dyspnea, hemoptysis, and cough. Few patients present with hoarseness and weight
loss. Two studies that specifically considered people with ACC of the trachea found similar
complaints, mainly dyspnea, hoarseness, and wheezing or stridor. Few patients had
hemoptysis, cough, or weight loss.
Adenoid cystic carcinoma appears to have a much more indolent course than SCC. In a
review of 208 patients with tracheal tumors, patients with ACC had symptoms three times as
long as patients with SCC (12 mo versus 4 mo). ACC is three times more likely to extend
beyond the trachea to sites such as the larynx or trachea than is SCC.
Of the 44 patients previously reported, in which the location of the ACC was designated,
45.5% (n = 20) had tumors in the upper trachea, 20.5% (n = 9) in the middle third, and 34%
(n = 15) in the lower third. Tumor location has been correlated with prognosis; the prognosis
which is worse for more distal tumors. This finding is probably related more to difficulty in
resecting tumors that involve one or both of the mainstem bronchi and increasing operative
complications from carinal resection.
Adenoid cystic carcinoma spreads most commonly by direct extension, submucous or
perineural invasion, or hematogenous metastasis. Lymphatic spread is uncommon. Only one
author reports lymphatic spread in tracheal ACC: five of 38 patients (13%) had positive
regional nodes. Positive nodes in head and neck ACC correlate with decreased 10-year
survival. However, the effect of positive nodes on survival length has not been examined in
tracheal ACC. More than 50% of patients with tracheal ACC have hematogenous metastases.
Pulmonary metastases are the most common and can remain asymptomatic for many years.
Metastases to the brain, bone, liver, skin, kidney, abdomen, and heart have been also reported.
Local recurrence of tracheal ACC is common and occurs an average of 51 months after the
primary treatment. Other tracheal tumors recur at an average of 18 months after treatment.
Shahzad G. Raja
222
Treatment options include surgery alone, radiation therapy alone, or a combination. The
surgical options are primary tracheal resection and reconstruction, primary tumor resection,
and endoscopic resection, either by coring or using laser. Grillo and Mathisen have detailed
the various surgical techniques and have recommended tracheal resection and primary
reconstruction.
References
Maziak DE, Todd TR, Keshavjee SH, et al. Adenoid cystic carcinoma of the airway: thirty-
two-year experience. J Thorac Cardiovasc Surg. 1996;112:1522-32.
Allen MS. Malignant tracheal tumors. Mayo Clin Proc. 1993;68:680-4.
Regnard JF, Fourquier P, Levasseur P. Results and prognostic factors in resections of primary
tracheal tumors: a multicenter retrospective study. J Thorac Cardiovasc Surg.
1996;111:808-13.
Pearson FG, Todd TRJ, Cooper JD. Experience with primary neoplasms of the trachea:
clinicopathologic study of 41 cases. Cancer. 1970;25:1448-56.
Grillo HC, Mathisen DJ. Primary tracheal tumors: treatment and results. Ann Thorac Surg.
1990;49:69-77.
Hajdu SI, Huvos AG, Goodner JT, et al. Carcinoma of the trachea: clinicopathologic study of
41 cases. Cancer. 1970;25:1448-56.
Howard DJ, Haribhakti VV. Primary tumours of the trachea: analysis of clinical features and
treatment results. J Laryngol Otol. 1994;108:230-2.
Gelder CM, Hetzel MR. Primary tracheal tumors: a national survey. Thorax. 1993;48:688-92.
3.20 Answer: D
The fact that the healing potential of tissues is impaired when
large doses of radiation are
administered has long been recognized
by surgeons. Major factors responsible for this are the
dose
of radiation received and the time interval between irradiation
and a subsequent surgical
procedure. Traditionally, surgeons
are most concerned about those patients whose dose has
exceeded
4,500 cGy and who have undergone radiation more than a year
before their
operation. Although an excisional procedure,
such as neck dissection, may entail only a
moderate increase
in the complications of wound healing, anastomoses may dehisce
with
disastrous consequences. The ability of irradiated tissues
to initiate capillary budding and
fibroblastic proliferation
in response to injury is minimized or lost. Because
of hyalinizing
changes in small vessels, necrosis may occur
once the integrity of the organ is disturbed
during operation.
The omentum is preferred for augmenting anastomotic healing.
It appears to elaborate an
angiogenic factor that stimulates
the development of a new blood supply in avascular areas, as
well as provides new fibroblasts to enhance healing. As observed in experiments studying
devascularized canine
bronchi and in the setting of human single-lung transplantations,
a
network of fine vessels originating from omentum wrapped around
anastomoses forms within
4 days of the operation. The
same phenomenon probably occurs in irradiated tissues, thus
initiating the healing process. The use of omentum has a long
surgical history. It has been
used to cover intrathoracic esophageal
anastomoses to lessen the risk of anastomotic leakage.
The omentum will reach any part of the airway with ease. It
has been used in the treatment of
difficult thoracic problems,
such as in patients undergoing complex chest wall reconstruction,
and in those with mediastinal infection after cardiac procedures,
vascular infection,
Thoracic Surgery: Answers
223
esophageal perforation, and a bronchopleural
fistula. The omentum also appears to function
well in the
presence of infection.
Previous irradiation of trachea was believed to be a contraindication to resection, but
recent evidence suggests that in selected cases resection is still possible as long as the length
of remaining trachea following resection is enough to perform end-to-end- anastomosis.
Under these circumstances, the repair should be buttressed with omentum.
References
Wright CD, Grillo HC, Wain JC, et al. Anastomotic complications after tracheal resection:
prognostic factors and management. J Thorac Cardiovasc Surg. 2004;128:731-9.
Muehrcke DD, Grillo HC, Mathisen DJ. Reconstructive airway operation after irradiation.
Ann Thorac Surg. 1995;59:14-8.
Müller LC, Abendstein B, Salzer GM. Use of the greater omentum for treatment and
prophylaxis of anastomotic and stump dehiscence in major airway surgery. Thorac
Cardiovasc Surg. 1992;40:323-5.
3.21 Answer: B
The detailed arterial supply of the trachea was described as
a necessary corollary to
tracheal surgery. Grillo emphasized
the entry of small segmental arteries through "lateral
pedicles"
of tissue attached to either side of the trachea. Miura and
Grillo precisely defined the
blood supply to the upper
trachea in 1966, usually from three principal branches of the
inferior
thyroid artery, with the first—or lowest—branch
most often predominant. Salassa and
coworkers completed
a definitive study of tracheal blood supply in 1977, confirming
Miura
and Grillo‘s description of the cervical tracheal supply, and mapping the arterial supply of the
thoracic trachea
from bronchial, supreme intercostal, subclavian, right internal
thoracic, and
innominate arteries. Segmental tracheoesophageal
arteries connected often to lateral
longitudinal arteries and
then to transverse intercartilaginous arteries. Three to seven
tracheal
arteries were found in the "lateral pedicles." Unlike the intramural collateral of tracheal blood
supply in the dog, which maintains tracheal viability despite complete circumferential
dissection and subsequent division and anastomosis, the same
procedure in humans has led to
necrosis.
References
Grillo HC. Development of tracheal surgery: a historical review. Part I: Techniques of
tracheal surgery. Ann Thorac Surg. 2003;75:610-9.
Salassa JR, Pearson BW, Payne WS. Gross and microscopic blood supply of the trachea. Ann
Thorac Surg. 1977;24:100-107.
Grillo HC. The management of tracheal stenosis following assisted respiration. J Thorac
Cardiovasc Surg. 1969;57:52-71.
Miura T, Grillo HC. The contribution of the inferior thyroid artery to the blood supply of the
human trachea. Surg Gynecol Obstet. 1966;123:99-102.
3.22 Answer: E
Anastomotic complications after tracheal resection and reconstruction
are uncommon but
lead to severe morbidity. Anastomotic complications
include granulations at the anastomotic
line (presumably some
degree of separation allows ingrowth of granulation tissue between
the
Shahzad G. Raja
224
two cut edges of the divided trachea), stenosis (distraction
of the tracheal anastomosis allows
scar to develop at the suture
line), and separation (disruption of the suture line causes
a
catastrophic failure of the anastomosis). Previous human cadaver
studies have demonstrated a
progressive rise in tension at the
anastomosis with increasing length of tracheal resection,
suggesting
a safe limit of 4.5 cm (corresponding to about 1000 g tension)
to avoid
anastomotic failure.
Failure of primary repair and dehiscence is one of the most feared complications of
tracheal surgery. In a single-institution, retrospective review of 901 patients
who underwent
tracheal resection, anastomotic
complications occurred in 81 patients (9%). Eleven patients
(1%) died after operation, 6 of anastomotic complications and
5 of other causes (odds ratio
13.0, P= .0001 for risk of death
after anastomotic complication). When dehiscence occurs
acutely, primary repair may still be possible since it is most likely secondary to a technical
problem or to inadvertent hyperextension of the neck. When dehiscence occurs at about 10
days, primary repair is not likely to be successful. After such an interval the condition of the
trachea and local sepsis are both suboptimal conditions, and the predisposing factor of
excessive tension which led to the complication in the first place has not been remedied.
Definitive repair is best delayed until local risk factors are resolved and additional
mobilization techniques can be employed to reduce tension. Oral intubation is only a
temporary maneuver. Local sepsis is likely to continue and another airway is ultimately
needed for long-term care. A tracheal stent and muscle flap buttress is a possible solution, but
this choice provides no access to the airway. Ventilation may be complicated and the airway
remains prone to occlusion from stent migration or granulations. A T-tube is always preferred
because
it allows speech and normal airway humidification, and the resulting
stomal area is
invariably less damaged than with tracheostomy. Furthermore, it offers the ability to ventilate
the patient through the side arm if necessay, and ready access to suctioning to clear the
airway.
References
Wright CD, Grillo HC, Wain JC, et al. Anastomotic complications after tracheal resection:
prognostic factors and management. J Thorac Cardiovasc Surg. 2004;128:731-9.
Donahue DM, Grillo HC, Wain JC, et al. Reoperative tracheal resection and reconstruction
for unsuccessful repair of postintubation stenosis. J Thorac Cardiovasc
Surg.1997;114:934-939.
Grillo HC, Zannini P, Michelassi F. Complications of tracheal reconstruction. Incidence,
treatment and prevention. J Thorac Cardiovasc Surg.1986;91:322-328.
3.23 Answer: A
In a single-institution, retrospective review of 901 patients
who underwent tracheal
resection, stepwise
multivariable analysis revealed the following predictors of
anastomotic
complications: reoperation (odds ratio 3.03, 95%
confidence interval 1.69-5.43, P= .002),
diabetes (odds ratio
3.32, 95% confidence interval 1.76-6.26, P= .002), lengthy (≥4
cm)
resections (odds ratio 2.01, 95% confidence interval 1.21-3.35,
P= .007), laryngotracheal
resection (odds ratio 1.80, 95% confidence
interval 1.07-3.01, P= .03), age 17 years or
younger (odds ratio
2.26, 95% confidence interval 1.09-4.68, P= .03), and need for
tracheostomy before operation (odds ratio 1.79, 95% confidence
interval 1.03-3.14, P= .04).
Thoracic Surgery: Answers
225
Diabetes is a surprisingly important risk factor for anastomotic
complication, with an
odds ratio of 3. This may be a consequence
of impairment of an already compromised
collateral watershed
circulation at the end of the divided trachea. Tension might
be expected
to put this area at further risk. Diabetes is known
to impair the microcirculation, with resultant
deleterious effects
on wound healing. Unfortunately, this risk factor cannot be
modified, but it
should be taken into account in stratifying
risk.
References
Wright CD, Grillo HC, Wain JC, et al. Anastomotic complications after tracheal resection:
prognostic factors and management. J Thorac Cardiovasc Surg. 2004;128:731-9.
Grillo HC, Dignan EF, Miura T. Extensive resection and reconstruction of the mediastinal
trachea without prosthesis or graft: an anatomical study in man. J Thorac Cardiovasc
Surg.1964;48:741-749.
3.24 Answer: B
Acquired tracheoesophageal fistula (TEF) is a rare complication that can occur from a
variety of causes. The most common etiology of nonmalignant TEF is as a complication of
intubation with cuff-related tracheal injury. Most patients present with increased secretions,
pneumonia, and evidence of aspiration of gastric contents while the patient is on mechanical
ventilation. When diagnosed after extubation, the most frequent sign of TEF is coughing after
swallowing. A high index of suspicion is required in patients at risk for developing a TEF.
The diagnostic evaluation is by bronchoscopy and esophagoscopy. When the diagnosis has
been made, the immediate goal should be to minimize tracheobronchial soilage by placing the
cuff of a tracheostomy tube distal to the fistula. Reflux of gastric contents is diminished by
placement of a gastrostomy tube, and adequate nutrition is facilitated by inserting a
jejunostomy tube. Surgical correction is required because spontaneous closure is rare, but
surgery should be postponed until the patient is weaned from mechanical ventilation because
positive pressure ventilation after tracheal repair carries an increased risk of anastomotic
dehiscence and restenosis. An anterior cervical collar incision can be used for most cases of
post-intubation TEFs. The esophagus should be closed in two layers over a nasogastric tube
and buttressed with a pedicled strap muscle flap. If the tracheal defect is small, primary repair
can be employed. In most cases, however, the best results can be achieved with tracheal
resection and reconstruction. The patient should be extubated at the completion of the case, if
possible. With this strategy, as first described by Grillo and colleagues, single-stage repair can
be performed safely and with a high success rate.
References
Reed MF, Mathisen DJ. Tracheoesophageal fistula. Chest Surg Clin N Am. 2003;13:271-89.
Mathisen DJ, Grillo HC, Wain JC, Hilgenberg AD. Management of acquired non-malignant
tracheoesophageal fistula. Ann Thorac Surg. 1991;52:759-65.
Hilgenberg AD, Grillo HC. Acquired non-malignant tracheoesophageal fistula. J Thorac
Cardiovasc Surg. 1983;85:492-8.
3.25 Answer: E
Mucoepidermoid carcinoma is defined by the World Health Organization as a tumor
characterized by a combination of mucus-secreting, squamous, and intermediate cell types
Shahzad G. Raja
226
and is not an uncommon tumor in general. It usually arises in the parotid and submandibular
salivary glands and in the minor salivary glands of the oral cavity and perimaxillary region.
Mucoepidermoid carcinoma of the lung, however, is rare with a reported frequency of 0.1%
to 0.2% of primary lung tumors. Mucoepidermoid carcinoma of the bronchus occurs in
patients with a wide age range from 3 to 78 years. In the recent Lature, it equally affects
males and females. The majority of cases documented are in the form of case reports or small
series. It also appears that most of the cases are generated from the pediatric population. One
report has suggested that mucoepidermoid carcinoma accounts for approximately 10% of
primary lung tumors in this age group.
Mucoepidermoid carcinoma of the bronchus mainly arises from the large airways,
including the trachea and the main or lobar bronchi, but occasionally may involve segmental
bronchi or, rarely, the peripheral lung. It usually presents as an exophytic luminal mass,
which can be sessile, polypoid with a broad base connected to the bronchial wall, or
pedunculated with a well-formed stalk. The cut surface is gray-white-tan with a glistening
mucoid texture. Cystic degeneration can be appreciated in some mucoepidermoid carcinomas.
The size of the tumor varies considerably within a range from several millimeters to 6 cm in
diameter in some studies. The bronchus usually is dilated with abundant mucoid luminal
material in the distal portion. The adjacent lung parenchyma generally demonstrates
atelectasis or pneumonia. Because of the typical pattern of involvement of large airways, the
clinical symptoms and signs of bronchial mucoepidermoid carcinoma include cough,
hemoptysis, bronchitis, wheezing, fever, chest pain, and rarely, clubbing of the fingers. The
clinical and radiographic differential diagnosis usually includes asthma, pneumonia,
atelectasis, middle lobe syndrome, and pleural effusion. Recurrent pneumonia in the same
region of the lung should raise clinical suspicion of an endobronchial lesion or mass, such as
mucoepidermoid carcinoma.
Although imaging studies generally provide significant information in the evaluation of
pulmonary lesions, conventional chest radiography and computed tomography sometimes are
not helpful in establishing the diagnosis of mucoepidermoid carcinoma of the bronchus. This
may be because of the small size of the neoplasm, especially during the early phase, or
because of the endobronchial location combined with interference of associated pneumonia or
atelectasis. To circumvent the problems with imaging studies, flexible fiberoptic
bronchoscopy is an excellent diagnostic modality with the capability of visualizing the lesion
directly and obtaining tissue for definitive diagnosis.
References
Liu X, Adams AL. Mucoepidermoid carcinoma of the bronchus: a review. Arch Pathol Lab
Med. 2007;131:1400-4.
Kim TS, Lee KS, Han J, et al. Mucoepidermoid carcinoma of the tracheobronchial tree:
radiographic and CT findings in 12 patients. Radiology. 1999;212:643-648.
Granata C, Battistini E, Toma P, et al. Mucoepidermoid carcinoma of the bronchus: a case
report and review of the literature. Pediatr Pulmonol. 1997;23:226-232.
Heitmiller RF, Mathisen DJ, Ferry JA, et al. Mucoepidermoid lung tumors. Ann Thorac Surg.
1989;47:394-399.
Yousem SA, Hochholzer L. Mucoepidermoid tumors of the lung. Cancer. 1987;60:1346-
1352.
Thoracic Surgery: Answers
227
3.26 Answer: B
Recent cytogenetic analysis using comparative genomic hybridization and spectral
karyotyping of cell lines derived from parotid and bronchial mucoepidermoid carcinomas
showed multiple reciprocal translocations. These reciprocal translocations involve essentially
every chromosome but more frequently involve chromosomes 1, 5, 7, and 11. Several
reciprocal translocations were identified: t(11;19), t(1;16), t(6;8), t(1;7), t(3;15), and t(7;15),
with the reciprocal t(11;19)(q21;p13) being the major chromosomal abnormality observed in
2 cell lines. Although cell lines derived from malignant tumors are notorious for genetic
instability, as characterized by the presence of multiple genetic abnormalities of varied nature,
including chromosomal translocations, other reports have independently demonstrated
t(11;19) in 38.4% of mucoepidermoid carcinomas, and the reported translocations involve the
q14-21 region of chromosome 11 and the p12-13 region of chromosome 19. In 1
mucoepidermoid carcinoma of the lung, t(11;19)(q14-21;p12) was the only karyotypic
abnormality identified. These results hint that the reciprocal t(11;19)(q14-21;p12) may play a
significant role in the tumorigenesis of mucoepidermoid carcinoma. Indeed, recently Tonon
and colleagues elegantly demonstrated that the t(11;19)(q21;p13) observed in
mucoepidermoid carcinoma encodes a novel fusion product, mucoepidermoid carcinoma
translocated 1–mastermind-like 2 (MECT1-MAML2), capable of disrupting the Notch
signaling pathway.
Other primary lung neoplasms, including adenocarcinoma and adenosquamous
carcinoma, comprise the major differential diagnoses for mucoepidermoid carcinoma of the
bronchus. Immunophenotyping of the tumor lesion may be helpful, particularly in the case of
a small biopsy sample in which the whole spectrum of the typical histomorphology may not
be present in the submitted material. Immunoperoxidase staining with antibodies to
cytokeratin 7 and thyroid transcription factor 1 may be useful, for example, in differentiating
mucoepidermoid carcinoma of the bronchus from primary lung adenocarcinoma.
Mucoepidermoid carcinoma of the lung may be surgically treated by lobectomy, sleeve
resection, local resection, segmental resection, or even endoscopic removal. In 1 review
report involving 45 patients with disease-free follow-up ranging from 8 months to 21 years, 1
patient had lymph node metastasis and died, 1 patient developed lymph node metastasis at 5
years of follow-up, and 1 patient had questionable lymph node metastasis. These data suggest
that patients with mucoepidermoid carcinoma of the lung should be provided with long-term
clinical follow-up.
References
Liu X, Adams AL. Mucoepidermoid carcinoma of the bronchus: a review. Arch Pathol Lab
Med. 2007;131:1400-4.
Tonon G, Gehlhaus KS, Yonescu R, et al. Multiple reciprocal translocations in salivary gland
mucoepidermoid carcinomas. Cancer Genet Cytogenet. 2004;152:15-22.
Dahlenfors R, Gertzen H, Wedell B, Mark J. Cytogenetical observations in a cultured
polymorphous low-grade adenocarcinoma originating from the minor salivary glands.
Anticancer Res. 1997;17:105-106.
Dinopoulos A, Lagona E, Stinios I, et al. Mucoepidermoid carcinoma of the bronchus.
Pediatr Hematol Oncol 2000;17:401-408.
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228
3.27 Answer: D
Adenoid cystic carcinoma (ACC), known as cylindroma in the past,
is a variant of
adenocarcinoma with distinct histopathologic
and clinical features. ACC occurs most
commonly in the salivary
glands and, less commonly, at other sites such as the breast,
skin,
uterine cervix, upper aerodigestive tract, and lung. Bronchial adenoid cystic carcinoma is
characterized clinically by an indolent course, a relatively high rate of local recurrence, and
late onset of distant metastases. Histologically, adenoid cystic carcinomas may have a mixture
of 3 distinctive growth patterns: cribriform, tubular, and solid. The most common histologic
type is the cribriform pattern, in which monomorphic tumor cells form islands with punched-
out spaces, producing a so-called Swiss-cheese appearance. The predominant growth pattern
is predictive to some extent of clinical outcome. Most studies have suggested that adenoid
cystic carcinomas with both tubular and cribriform patterns generally have a better prognosis
than those with the solid pattern.
References
Lin CM, Li AF, Wu LH, et al. Adenoid cystic carcinoma of the trachea and bronchus-a
clinicopathologic study with DNA flow cytometric analysis and oncogene expression.
Eur J Cardiothorac Surg. 2002;22:621-5.
Albers E, Lawrie T, Harrell JH, Yi ES. Tracheobronchial adenoid cystic carcinoma: a
clinicopathologic study of 14 cases. Chest. 2004;125:1160-5.
3.28 Answer: A
Prognosis of carcinoid tumors of lung is highly dependent on histologic features. Typical
carcinoids have an excellent outcome, even with hilar or ipsilateral
mediastinal lymph node
metastasis. The overall survival
rate at 5, 10, and 15 years is reported to be 92%, 88%, and
76%, respectively. Patients with atypical carcinoids have
a worse prognosis, with 5- and 10-
year survival rates of 69%
and 24%–52%, respectively. In a study by Gould
et al., tumor size,
histologic subtype, and nodal involvement
at initial diagnosis were the factors that most
influenced recurrence
and outcome.
Carcinoid tumors that are >3 cm have a 10-year survival of approximately 60% which is
significantly less than tumors less than or equal to 3 cm which have a 10-year survival of
approximately 95%. Patients without lymph node metastases have a 10-year survival of
approximately 85% versus 55% for those who have lymph node metastases. This group
includes those with N1 and N2 disease. There is a trend that 10-year survival for patients with
N1 disease is greater than N2 disease, however, it does not reach significance. Stage for stage
the cell type is the most important prognostic factor. This includes those patients with and
without nodal metstatses. For patients with a typical carcinoid the 10-year survival is about
90% and for those with atypical carcinoid the 10-year survival is approximately 50%.
References
Jeung MY, Gasser B, Gangi A, et al. Bronchial carcinoid tumors of the thorax: spectrum of
radiologic findings. Radiographics. 2002;22:351-65.
Gould PM, Bonner JA, Sawyer TE, et al. Bronchial carcinoid tumors: importance of
prognostic factors that influence patterns of recurrence and overall survival. Radiology.
1998;208:181-185.