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Thoracic Surgery: Answers
259
sublobar and lobar resection in tumors <2 cm in diameter.
In contrast, median survival was
significantly better for patients
with larger tumors (2 to 3 cm) undergoing lobar resection
group
(70 months) than for similar patients treated with sublobar
resection (44.7 months).
Birdas et al. retrospectively reviewed the outcomes of 167
patients with stage IB lung
cancer treated with lobectomy (n
= 126) or sublobar resection (n = 41) with
125
I brachytherapy
over the resection staple line. The local recurrence rate was
similar between the sublobar with
brachytherapy group (4.8%)
and the lobectomy group (3.4%). At 4 years, the disease-free
survival was equivalent for sublobar (43.0%) and lobectomy (42.8%)
patients. Overall
survival did not differ for sublobar patients
(54.1%; median, 50.2 months) and lobectomy
patients (51.8%;
median, 56.9 months; p = 0.38).
References
Ginsberg RJ, Rubinstein LV. Randomized trial of lobectomy versus limited resection for
T1N0 non-small cell cancer by the Lung Cancer Study Group. Ann Thorac Surg.
1995;60,615-623.
Martin-Ucar AE, Nakas A, Pilling JE, et al. A case-matched study of anatomical
segmentectomy versus lobectomy for stage I lung cancer in high-risk patients. Eur J
Cardiothorac Surg. 2005;27,675-695.
Watanabe S, Oda M, Tsunezuka Y, et al. Recent results of postoperative mortality for surgical
resections in lung cancer. Ann Thorac Surg. 2004;78,999-1025.
Jackevicius A, Cicenas S, Naujokaitis P. Limited resection of lung cancer. Acta Chir Hung.
1999;38,49-51.
Tsubota N, Ayabe K, Doi O, et al. Ongoing prospective study of segmentectomy for
small lung tumors. Study Group of Extended Segmentectomy for Small Lung Tumor.
Ann Thorac Surg. 1998;66:1787-90.
Landrenau RJ, Sugarbaker DJ, Mack MJ, et al. Wedge resection versus lobectomy for stage I
(T1N0) non-small cell lung cancer. J Thorac Cardiovasc Surg. 1997;113,691-698.
Fernando HC, Santos RS, Benfield JR, et al. Lobar and sublobar resection with and without
brachytherapy for small stage IA non-small cell lung cancer. J Thorac Cardiovasc Surg.
2005;129,261-267.
Birdas TJ, Koehler RP, Colonias A, et al. Sublobar resection with brachytherapy versus
lobectomy for stage IB non-small cell lung cancer. Ann Thorac Surg. 2006;81,434-438.
3.58 Answer: A
Although surgery offers the best chance of cure for patients with non-small cell lung
cancer (NSCLC), the overall 5-year survival rate is modest, and improvements are urgently
needed. In the 1990s, much interest was generated from two small trials that reported striking
results with neo-adjuvant chemotherapy, and therefore Medical Research Council LU22
intergroup randomised trial was designed to investigate whether, in patients with operable
non-small cell lung cancer of any stage, outcomes could be improved by giving platinum-
based chemotherapy before surgery. Patients were randomised to receive either surgery alone
(S), or three cycles of platinum-based chemotherapy followed by surgery (CT-S). Before
randomisation, clinicians chose the chemotherapy that would be given from a list of six
standard regimens. The primary outcome measure was overall survival, which was analysed
on an intention-to-treat basis.
Shahzad G. Raja
260
519 patients were randomised (S: 261, CT-S: 258) from 70 centres in the UK,
Netherlands, Germany, and Belgium. Most (61%) were clinical stage I, with 31% stage II,
and 7% stage III. Neo-adjuvant chemotherapy was feasible (75% of patients received all three
cycles of chemotherapy), resulted in a good response rate (49% [95% CI 43%-55%]) and
down-staging in 31% (25%-37%) of patients, and did not alter the type or completeness of the
surgery (lobectomy: S: 56%, CT-S: 60%, complete resection: S: 80%, CT-S: 82%). Post-
operative complications were not increased in the CT-S group, and no impairment of quality
of life was observed. However, there was no evidence of a benefit in terms of overall survival
(hazard ratio [HR] 1.02, 95% CI 0.80-1.31, p=0.86).
Reference
Gilligan D, Nicolson M, Smith I, et al. Preoperative chemotherapy in patients with resectable
non-small cell lung cancer: results of the MRC LU22/NVALT 2/EORTC 08012
multicentre randomised trial and update of systematic review. Lancet. 2007;369:1929-37.
3.59 Answer: A
Despite the conduct of a number of randomised controlled trials (RCTs) which have
recruited a total of over 2000 patients, the role of postoperative radiotherapy (PORT) in the
treatment of non small cell lung cancer (NSCLC) remains unclear. Individually, trials have
shown inconclusive and conflicting results. However, because of their size (74 to 539
patients), individual trials have not had sufficient statistical power to detect the moderate
survival differences that might be expected of PORT. The Meta-analysis Group of the British
Medical Research Council (MRC) Clinical Trials Unit (CTU) therefore initiated an individual
patient data meta-analysis to assess this question. This approach to meta-analysis and
systematic review involves the central collection, validation and analysis of the original trial
data. It does not rely on data extracted from publications. At the outset, the secretariat
contacted the investigators responsible for each trial and established the PORT Meta-analysis
Trialists Group, under whose auspices the meta-analysis was conducted and published. This
review was first published in The Lancet on 25 July 1998 (PORT 1998). In 2004, data from
one new trial (Trodella et al.) were added and the meta-analysis was updated. This update was
published in Lung Cancer in 2005 (PORT 2005). In 2008, this meta-analysis was updated
again to include data from another new trial reported by Park in the Journal of Thoracic
Oncology.
The most recent meta-analysis was based on the results of 11 RCTs and 2343 individuals.
In these trials PORT doses ranged from 30 to 60 Gy, given in between 10 and 30 fractions,
and there was considerable diversity in other aspects of radiotherapy planning. All trials
included patients with completely resected tumours where the disease stage was no greater
than IIIA. Follow up was updated in most trials giving a median of 4.4 years for surviving
patients (2.3 to 11.4 years for individual trials). The patient characteristics showed that
patients were mostly male with stage II/III squamous cell carcinoma (though histology was
unknown for a relatively large number of patients) and with good performance status.
The combined results showed a significant adverse effect of PORT on survival (P =
0.001), with a hazard ratio (HR) of 1.18 (95% CI 1.07 to 1.31), or an 18% relative increase in
the risk of death. This was equivalent to an absolute detriment of 5% at two years (95% CI
2% to 9%) reducing overall survival from 58% to 53%. Analysis of local recurrence-free
survival, based on 1556 events (498 local recurrences (200 on PORT, 298 on surgery alone)
Thoracic Surgery: Answers
261
and 1058 deaths (593 on PORT, 465 surgery alone)), gave a HR of 1.12 (95% CI 1.01 to
1.23), significantly in favour of surgery alone (P = 0.03). Analysis of distant recurrence-free
survival based on 1570 events (892 distant recurrences (438 on PORT, 454 on surgery alone)
and 678 deaths (361 on PORT, 317 on surgery alone)) gave a HR of 1.13 (95% CI 1.02 to
1.24) in favour of surgery alone (P = 0.02).
Analyses were performed to determine whether there was evidence of a differential effect
of PORT in predefined subgroups of patients. For survival there was no evidence to suggest
that PORT was differentially effective in any group of patients defined by age (trend P =
0.32), sex (interaction P = 0.84) or histology (interaction P = 0.42). There was some evidence
that the effects of PORT were more detrimental in those patients with stage I than with stage
II disease; considering the results for stage III patients alone there was no clear evidence of a
detriment (trend across all stages P = 0.004). Similarly, there was a trend that PORT was
increasingly detrimental with lower nodal status (trend P = 0.03). Results were similar for the
endpoints of local, distant and overall recurrence-free survival.
References
PORT Meta-analysis Trialists Group. Postoperative radiotherapy in non-small-cell lung
cancer: systematic review and meta-analysis of individual patient data from nine
randomised controlled trials. Lancet 1998;352:257-63.
Trodella L, Granone P, Valente S, et al.Adjuvant radiotherapy in non-small cell lung cancer
with pathological stage I: definitive results of a phase III randomised trial. Radiother
Oncol 2002;62:11-9.
Burdett S, Stewart L on behalf of the PORT Meta-analysis Trialist Group. Postoperative
radiotherapy in non-small cell lung cancer: update of an individual patient data meta-
analysis. Lung Cancer 2005;47:81-3.
Park JH. Postoperative adjuvant therapy for stage IIIa non-small cell lung cancer. J Thora
Oncol 2007;2 (8 Suppl 4):S651.
PORT Meta-analysis Trialists Group. Postoperative radiotherapy for non-small cell lung
cancer. Cochrane Database Syst Rev 2005;(2):CD002142.
3.60 Answer: C
Soft tissue sarcoma is a rare neoplasm: there are approximately 6600 cases annually in
the United States. Sarcoma may arise virtually anywhere, but the extremity is the most
common primary site. The lungs are the most common sites of metastatic disease. Of patients
with extremity sarcoma, approximately 20% will have isolated pulmonary metastatic disease
at some point in the course of their disease. Although pulmonary metastases most commonly
arise from primary tumors in the extremities, they may arise from almost any histologic
variant or primary site.
There is evidence that surgical resection is the treatment of choice for pulmonary
metastases from soft tissue sarcoma. Three-year survival rates after complete resection range
from 30% to 42%. Chemotherapy has not been proven to increase survival after the resection
of pulmonary metastasis.
Several prognostic variables have been identified that are associated with favorable
survival after pulmonary metastasectomy. Favorable factors include an extended disease-free
interval, three or fewer pulmonary nodules, and a longer tumor doubling time. The most
consistent favorable prognostic factor is metastatic disease that is amenable to resection.
Shahzad G. Raja
262
The survival benefit associated with resection of pulmonary metastatic disease appears to
be significant. In a large series from Memorial Sloan-Kettering Cancer Center, patients who
underwent at least one complete pulmonary resection had a median survival of 33 months and
a 3-year actuarial survival rate of 46%. The actuarial 5-year survival rate was 37%. Patients
who did not undergo resection had a median survival of 11 months and a 3-year actuarial
survival rate of 17%.
References
Chao C, Goldberg M. Surgical treatment of metastatic pulmonary soft-tissue sarcoma.
Oncology (Williston Park). 2000;14:835-41
Billingsley KG, Burt ME, Jara E, et al. Pulmonary metastases from soft tissue sarcoma:
analysis of patterns of diseases and postmetastasis survival. Ann Surg. 1999;229:602-10.
Billingsley KG, Lewis JJ, Leung DH, et al. Multifactorial analysis of the survival of patients
with distant metastasis arising from primary extremity sarcoma. Cancer. 1999;85:389-95.
3.61 Answer: D
A total of 5206 cases of lung metastasectomy were included in
the published analysis of
The International Registry of Lung Metastases, of which 4572 (88%) involved complete
surgical resection. Metastasectomy was considered incomplete
in 634 patients (12%) because
of microscopic (n = 127) or macroscopic
(n = 507) residual disease.
In 43% of patients lung metastases were from an epithelial tumor,
in 42% from sarcomas,
in 7% from germ cell tumors, in 6% from
melanomas, and in 2% from other types, including
30 cases of
Wilms' tumors. In the whole series, 31% of patients had a disease-free interval
(DFI)
of 0 to 11 months, including 11% who had synchronous metastases;
36% had a DFI of
12 to 35 months and 31% of 36 months or more.
Median DFI was 19 months. In most patients
(64%) with germ cell
tumors the DFI was less than 12 months; the corresponding value
was
39% for sarcomas, 21% for epithelial tumors, and 17% for
melanomas. In 126 (2%) patients
the DFI was not specified.
On the basis of pathologic assessment, single metastases accounted
for 46% and multiple
metastases 52%. Overall, 26% had four or
more metastases, 9% (n = 457) ten or more, and
3% (n = 165)
twenty or more; the maximum number of lesions resected was 154.
Multiple
metastases were resected in 64% of sarcomas, 57% of
germ cell tumors, 43% of epithelial
tumors, and 39% of melanomas.
The total number of perioperative deaths was 51, corresponding
to an overall operative
mortality of 1.0%. This figure was 2.4%
(n = 15) after incomplete resections and 0.8% (n =
36) after
complete metastasectomy. In the group of patients with resectable
lesions, the
mortality varied according to the maximum resection
volume, being 0.6% (n = 20) for
sublobar resections, 1.2% (n
= 12) for lobectomies and bilobectomies, and 3.6% (n = 4) for
pneumonectomies. By adding to the reported surgical deaths 18
patients who died within 30
days of metastasectomy, the overall
mortality was 1.3% and the corresponding figure for
complete
resections, 1.0%.
The
survival after complete metastasectomy was 36% at 5 years, 26%
at 10 years, and
22% at 15 years, with a median survival of
35 months; the number of patients alive at these
intervals was
809, 254, and 78, respectively. The corresponding survivals
for incomplete
resections were 13% at 5 years and 7% at 10 and
15 years, with a median of 15 months. In
Thoracic Surgery: Answers
263
this group 35 patients
were alive at 5 years, five at 10 years, and only one at 15
years. The
difference was highly significant with a log-rank
χ
2
of 245.8 (1 df).
For patients with a DFI of 0 to 11 months,
the survival was 33% at 5 years and 27% at 10
years, with a
median of 29 months. For a DFI of 12 to 35 months, the corresponding
values
were 31%, 22%, and 30 months; for a DFI of 36 months
or longer, survivals were 45%, 29%,
and 49 months, respectively.
Patients
with single metastases had a survival of 43% at 5 years and
31% at 10 years, with
a median of 43 months. In the group of
patients with two or three metastases, the survival was
34%
at 5 years and 24% at 10 years, with a median of 31 months.
Patients with four or more
metastases had a lower survival:
27% at 5 years and 19% at 10 years, with a median of 27
months.
However, even in the group of patients who had 10 or more metastases
resected (n =
342), the survival reached 26% at 5 years and
17% at 10 years, with a median of 26 months.
Patients with
germ cell tumors had by far the best survival (68% at 5 years
and 63% at 10
years) and melanoma the worst (21% at 5 years
and 14% at 10 years, median 19 months). The
survivals of patients
with epithelial tumors (37% at 5 years and 21% at 10 years,
median 40
months) and sarcomas (31% at 5 years and 26% at 10
years, median 29 months) did not differ
significantly when these
two large groups were compared. However, there were significant
differences among the specific histologic types of sarcoma and
the various sites of epithelial
cancer. The
survival of all tumor types combined (other than germ cell and
Wilms' tumors)
was 34% at 5 years and 23% at 10 years, with
a median of 33 months.
The long-term outcome of patients who were treated by a second
metastasectomy was
remarkably good: a 44% survival at 5 years
and 29% at 10 years, compared with 34% and
25%, respectively,
for patients having had one single operation. This is not surprising
in the
short term, inasmuch as redo surgery is generally offered
to patients with limited pulmonary
relapse and good general
condition. However, the favorable long-term results suggest
a real
curative benefit of repeated salvage operations, rather
than a simple selection effect.
Relative risks of death and multivariate analysis were calculated
on patients who had a
complete metastasectomy. When considered
separately, DFI, number of metastases, and
tumor type were highly
significant prognostic variables; age was only marginally significant,
and sex was not significant.
References
The International Registry of Lung Metastases, Writing Committee: Pastorino U, et al. Long-
term results of lung metastasectomy: prognostic analyses based on 5206 cases. J Thorac
Cardiovasc Surg. 1997;113:37-49.
3.62 Answer: D
Chest radiograph shows perihilar consolidation and an air bronchogram in the left lung.
The radiologic appearance is suggestive of postpneumonectomy pulmonary edema. It is a
life-threatening complication of pneumonectomy, with a reported prevalence of 2.5%–5% and
an associated mortality rate of 80%–100%. The causal mechanism of this complication
remains largely undetermined. Increased hydrostatic pressure and altered permeability of
capillaries are probably contributing factors. Predisposing factors include an excessive
perioperative fluid load, transfusion of fresh frozen plasma, arrhythmia, marked postsurgical
diuresis, and low serum colloidal osmotic pressure.
Shahzad G. Raja
264
Postpneumonectomy pulmonary edema occurs more commonly after right
pneumonectomy, probably because a larger amount of fluid in the post–right pneumonectomy
space results in an increased pulmonary blood flow through the left lung, which normally
receives only about 45% of the total pulmonary blood flow and contains approximately 45%
of the total lymphatic capacity of the lungs.
Postpneumonectomy pulmonary edema is diagnosed primarily through a process of
exclusion; the diagnosis is valid only if there is no clinical or radiologic evidence of
aspiration pneumonia, bacterial pneumonia, heart failure, thromboembolism, bronchopleural
fistula, or other possible causes of ARDS. On serial chest radiographs, severe
postpneumonectomy pulmonary edema appears as increased opacity identical to that seen in
ARDS. In less severe cases, the imaging findings resemble those in hydrostatic pulmonary
edema: The most frequently observed features include Kerley lines, peribronchial cuffing
(i.e., thickening of the bronchial wall), and ill-defined vessels. These features have a tendency
to disappear within a few days, a tendency that strongly indicates that lesions of the capillary
endothelial cells, if present, are mild in this form of the disorder.
References
Chae EJ, Seo JB, Kim SY, et al. Radiographic and CT findings of thoracic complications
after pneumonectomy. Radiographics 2006;26:1449-68.
Jordan S, Mitchell JA, Quinlan GJ, et al. The pathogenesis of lung injury following
pulmonary resection. Eur Respir J 2000;15:790-9.
Deslauriers J, Aucoin A, Grégoire J. Postpneumonectomy pulmonary edema. Chest Surg Clin
N Am 1998;8:611-31.
Parquin F, Marchal M, Mehiri S, et al. Post-pneumonectomy pulmonary edema: analysis and
risk factors. Eur J Cardiothorac Surg 1996;10:929-32.
3.63 Answer: C
The radiographs are suggestive of postpneumonectomy bronchopleural fistula (BPF).
BPF is a potentially fatal complication of pneumonectomy, although its incidence has
decreased over the years: An incidence of 0%-9% and an associated mortality rate of 16%-
23% have been reported. A BPF can occur any time during the postoperative period but more
often occurs within 8 to 12 days after surgery. If seen within the first 4 postoperative days, the
BPF is probably secondary to a mechanical failure of closure of the stump and requires re-
exploration and reclosure.
A BPF is more likely to occur after right pneumonectomy than after left pneumonectomy.
The increased frequency of BPF on the right side is likely multifactorial.
It should be noted
that the right bronchial stump is shorter, more exposed
in the pleural space and less likely to
be naturally buttressed
by mediastinal tissues as compared with the left. This anatomic
difference alone is likely a significant factor in the increased
risk of developing a BPF on the
right side.
In addition, the right bronchus is more vulnerable to ischemia with blood supplied
via a single bronchial artery.Devascularization of the bronchial stump is also a recognized
risk
factor for BPF. This may occur because of extensive
dissection required for a proximal tumor
or because of extensive
lymphadenectomy rather than lymph node sampling. De Perrot and
colleagues noted an increase
in postpneumonectomy BPF from 3% to 9% coincident with an
increase
in mediastinal lymphadenectomy and the use of bronchial staplers
in their center.
Thoracic Surgery: Answers
265
Treatment options of BPF include surgical procedures as well as medical therapy, and in
particular the use of bronchoscopy and different glues, coils, and sealants. Success has been
variable, and the lack of consensus suggests that no optimal therapy is available; rather, the
current interventions seem to be complementary and that treatment should be individualized.
Bronchoscopy is indicated to exclude injury to the proximal airways. Initial nonoperative
management focuses on decreasing the gradient between airway pressures and the pleural
space. Mean airway pressure should be minimized, and if the lung remains fully inflated,
suction on pleural tubes should be minimized.
Cooper and Miller recommended a logical stepwise evaluation and management of these
patients. The initial treatment is aimed to control any life-threatening condition. If there is a
tension pneumothorax, emergent drainage is required. In the case of pulmonary flooding,
airway control and postural drainage positioning the affected lung down are required. If a
major bronchial stump dehiscence is suspected, immediate resuture and reinforcement are
necessary. Furthermore, these patients are often debilitated due to the underlying process, and
therefore aggressive management of the underlying comorbidities and conditions that led to
the BPF is mandatory. When infectious etiologies are present or empyema is suspected,
adequate drainage of the pleural space is mandatory along with proper antimicrobial
coverage. Proper nutrition is required, frequently requiring enteral and/or parenteral feeding.
Successful treatment of chronic BPF requires aggressive control of infection, adequate
drainage of the chest cavity, closure of the fistula with vascularized tissue, and obLation of
the chest cavity.
The most common cause of death associated with this condition is aspiration pneumonia
with subsequent ARDS.
References
Chae EJ, Seo JB, Kim SY, et al. Radiographic and CT findings of thoracic complications
after pneumonectomy. Radiographics 2006;26:1449-68.
Cerfolio R. The incidence, etiology and prevention of postresectional bronchopleural fistula.
Semin Thorac Cardiovasc Surg 2001;13:3-7.
Cooper, WA, Miller, JI Management of bronchopleural fistula after lobectomy. Semin Thorac
Cardiovasc Surg 2001;13,8-12.
de Perrot M, Licker M, Robert J, Spiliopoulos A. Incidence, risk factors and management of
bronchopleural fistulae after pneumonectomy. Scand J Thorac Cardiovasc Surg
1999;33:171-174.
3.64 Answer: E
Chest radiograph shows overexpansion and anterior herniation of the left lung and
rightward deviation of the trachea and heart. The axial CT image depicts stretching of the left
main bronchus, which is visible between the left pulmonary artery and the vertebral body.
These radiographic findings are suggestive of postpneumonectomy sysndrome.
Postpneumonectomy syndrome is a delayed complication seen primarily in children and
young adults within a year after surgery. The syndrome is manifested in exertional dyspnea,
inspiratory stridor, and recurrent pulmonary infections. Most cases of postpneumonectomy
syndrome have been described as occurring after right pneumonectomy, when the powerful
negative pressure of the involved hemithorax and over-expansion of the remaining lung move
the mediastinum rightward. As the overexpanded lung further displaces the mediastinum
Shahzad G. Raja
266
toward the right side, the heart descends in the hemithorax and rotates counterclockwise along
its main axis. The trachea also is displaced toward the right side, with resultant stretching of
the left main bronchus, which is compressed downward by the aortic arch and the left main
pulmonary artery. When tracheobronchomalacia occurs in combination with the classic
symptoms of postpneumonectomy syndrome, the deleterious effects of bronchial compression
are compounded. The frequency with which this syndrome occurs in infants, young children,
and women is believed to be related to the increased elasticity and compliance of their lungs
and mediastinum, compared with those of older patients and men.
Various surgical procedures have been used to reposition the mediastinum. Silicone
breast implants have been used in the postpneumonectomy space to prevent rotational shifting
after pneumonectomy in children, a method that has brought good results. In patients with a
history of right pneumonectomy and subsequent dyspnea and recurrent pulmonary infection, a
proper radiologic evaluation is crucial to establish a diagnosis of postpneumonectomy
syndrome. CT is the best method for obtaining information about the status of the
postpneumonectomy space; the position of the mediastinal blood vessels, bronchi, and
thoracic spine; and the specific site of bronchial narrowing.
References
Soll C, Hahnloser D, Frauenfelder T, et al. The postpneumonectomy sysndrome: clinical
presentation and treatment. Eur J Cardiothorac Surg 2009 Feb;35:319-24.
Shen KR, Wain JC, Wright CD, et al. Postpneumonectomy syndrome: surgical management
and long-term results. J Thorac Cardiovasc Surg 2008;135:1210-6.
Chae EJ, Seo JB, Kim SY, et al. Radiographic and CT findings of thoracic complications
after pneumonectomy. Radiographics 2006;26:1449-68.
Podevin G, Larroquet M, Camby C, et al. Poastpneumonectomy syndrome in children:
advantages and long-term follow-up of expandable prostheis. J Pediatr Surg
2001;36:1425-7.
3.65 Answer: C
The term ―broncholithiasis” is used to denote the presence of calcified or ossified
material within the lumen of the bronchus. A broncholith is usually formed by erosion by and
extrusion of a calcified adjacent lymph node into the bronchial lumen and is usually
associated with long-standing foci of necrotizing granulomatous lymphadenitis. Most
broncholiths are a result of fungal infections,
often Histoplasmosis capsulatum or
mycobacterial granulomatous
lymphadenitis, silicosis, or both.Other causes of
broncholithiasis include (a) aspiration of bone tissue or in situ calcification of aspirated
foreign material; (b) erosion by and extrusion of calcified or ossified bronchial cartilage
plates; and (c) migration to a bronchus of calcified material from a distant site, such as a
pleural plaque or the kidney (via a nephrobronchial fistula). Some authors expanded the
definition to include those cases in which peribronchial calcified lymph nodes distort the
bronchial tree without extrusion of the lymph node into the bronchus.
The most common symptoms of broncholithiasis are nonproductive cough frequently
associated with hemoptysis and, less common, the presence of secondary infection after
obstruction of the distal portion of the lung that causes pain, chills, and fever. In some cases, a
history of expectoration of calcified material (lithoptysis) may prompt the diagnosis.
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267
Even though broncholithiasis is endobronchial disease, bronchoscopy often fails to aid in
a correct diagnosis, because the calcified endobronchial material is often obscured by
overlying bronchial wall inflammation or is distal to inflamed and narrowed airways rendered
inaccessible to bronchoscopy. Furthermore, many endoscopists
have reservations about
removal because of the fear of massive
hemorrhage, bronchial injury, or both with attempted
extraction
of broncholiths bronchoscopically. Thus many currently recommend
avoiding
bronchoscopic broncholithectomy and prefer thoracotomy
with broncholithotomy.
Cerfolio et al. based upon their experience recently presented an algorithm for
the current
management of patients with broncholiths. They conclude that despite previous reports,
broncholiths that are
not fixed in the airway can be safely removed with rigid and
flexible
bronchoscopic equipment. Thoracotomy with broncholithectomy
appears safe and effective
and is reserved for symptomatic lesions
that cannot be removed bronchoscopically or for
lesions that
cause airway esophageal fistula. The removal of other ipsilateral
calcified lymph
nodes during that operation is safe. Calcified
nodes in asymptomatic patients are not an
indication for intervention.
References
Cerfolio RJ, Bryant AS, Maniscalco L. Rigid bronchoscopy and surgical resection for
broncholithiasis and calcified mediastinal lymph nodes. J Thorac Cardiovasc Surg
2008;136:186-90.
Seo JB, Song KS, Lee JS, et al. Broncholithiasis: review of the causes with radiologic-
pathologic correlation. Radiographics 2002;22 Spec No:S199-213.
3.66 Answer: E
Localized fibrous tumors of the pleura (LFTP) previously known as ―localized
mesothelioma‖ as well as a variety of other terms (Table), are actually different from diffuse
mesothelioma. In contrast with mesothelioma, these tumors are thought to arise from
mesenchymal cells beneath the mesothelial lining of the pleura and, interestingly, have no
association with prior asbestos exposure. This latter fact may be important for the patient
seeking financial compensation for previous occupational exposure to asbestos. Benign and
malignant variants of the tumor may occur. Solitary fibrous tumors have also been identified
in a number of extrapleural sites, including the mediastinum, pericardium, peritoneum, orbit
and lacrimal gland fossae, nasopharynx and sinus cavities, thyroid and salivary glands, and
others.
Table. Synonyms of LFTP
Localized mesothelioma
Localized fibrous mesothelioma
Fibrous mesothelioma
Solitary fibrous mesothelioma
Pleural fibroma
Subserosal fibroma
Submesothelial fibroma
Shahzad G. Raja
268
LFTP are seen equally in men and women. The peak incidence occurs in the fifth to
eighth decades but may be found in any age group. The majority of patients are asymptomatic
at presentation. Symptoms are typically vague when present and consist of cough, chest pain,
and dyspnea. Hemoptysis, fever, and pneumonitis occur rarely. Malignant LFTP are more
commonly associated with symptoms than the benign variant.
References
Mitchell JD. Solitary fibrous tumor of the pleura. Semin Thorac Cardiovasc Surg.
2003;15:305-309.
de Perrot M, Fischer S, Bründler MA, et al. Solitary fibrous tumors of the pleura. Ann Thorac
Surg. 2002;74:285-293.
Chan JK. Solitary fibrous tumour—everywhere, and a diagnosis in vogue. Histopathology.
1997;31:568-576.
3.67 Answer: E
The most likely diagnosis in this case is localized fibrous tumor of the pleura (LFTP). A
number of interesting paraneoplastic syndromes may be present with LFTP. Hypertrophic
pulmonary osteoarthropathy may be seen in up to 20% of patients. This symptom complex
consists of bilateral pain and stiffness and swelling of joints and the surfaces of long bones,
such as the tibia. At times, these symptoms can be severe but usually remit following
complete resection of the tumor. Clubbing of the distal phalanges of the hands and feet may
also be seen. The etiology of hypertrophic pulmonary osteoarthropathy and clubbing remains
unknown. Significant hypoglycemia is also associated these neoplasms in approximately 4%
of cases, which is thought to be due to the production of insulin-like growth factors by the
tumor. Resection leads to normoglycemia within days.
References
Mitchell JD. Solitary fibrous tumor of the pleura. Semin Thorac Cardiovasc Surg.
2003;15:305-309.
Chamberlain MH, Taggart DP. Solitary fibrous tumor associated with hypoglycemia: An
example of the Doege-Potter syndrome. J Thorac Cardiovasc Surg. 2000;119:185-187.
Chaugle H, et al. Hypoglycaemia associated with a solitary fibrous tumour of the pleura. Eur
J Cardiothorac Surg. 1999;5:84-86.
3.68 Answer: D
The table below gives the immunohistochemical profile of localized fibrous tumors of the
pleura (LFTP) and other similar neoplasms. The lack of cytokeratin reactivity combined with
strong CD34 and bcl-2 immunoreactivity differentiates LFTP from malignant mesothelioma.
References
Mitchell JD. Solitary fibrous tumor of the pleura. Semin Thorac Cardiovasc Surg.
2003;15:305-309.
van de Rijn M, Lombard CM, Rouse RV. Expression of CD34 by solitary fibrous tumors of
the pleura, mediastinum, and lung. Am J Surg Pathol. 1994;18:814-820.
Chilosi M, Facchettti F, Dei Tos AP, et al. bcl-2 expression in pleural and extrapleural
solitary fibrous tumours. J Pathol. 1997;181:362-367.