elastofibroma dorsi: clinicopathological analysis of 71 cases

11
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Instructions to Contributors Dear Contributor: Enclosed in this document please find the page proofs and copyright transfer agreement (CTA) for your article in The Thoracic and Cardiovascular Surgeon. Please print this document and complete and return the CTA, along with corrected proofs, within 72 hours. 1) Please read proofs carefully for typographical and factual errors only; mark corrections in the

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Elastofibroma Dorsi: ClinicopathologicalAnalysis of 71 CasesFilippo Lococo 1 Alfredo Cesario 2 Francesca Mattei 2 Gianluigi Petrone 3 Letizia M. Vita 4

Leonardo Petracca Ciavarella 4 Stefano Margaritora 4 Pierluigi Granone 4

1Department of Thoracic Surgery, Catholic University of Sacred Heart,Rome, Italy

2 IRCCSQ1Q1Q1 San Raffaele Pisana, Rome, Italy

3Department of Pathology, Catholic University of Rome, Rome, Italy4Department of General Thoracic Surgery, Catholic University, Rome,Italy

Thorac Cardiovasc Surg 2012;00:1–8.

Address for correspondence and reprint requests Dr. Filippo Lococo,

Q2Q2Q2Department of Thoracic Surgery, Catholic University of Rome, LargoF.Vito 1, 00168 Rome, Italy (e-mail: [email protected]).

Keywords

► elastofibroma dorsi► soft tissue tumors► chest wall tumors► elastin

Abstract Introduction Elastofibroma dorsi (ELD) is a rare soft tissue benign tumor of the chestwall. So far, only a few large series have been reported in the English literature and, tothe best of our knowledge, radiological assessment and clinical management remainwithout consensus. The aim of this study is to provide, on the basis of a single-institutional, homogeneous and large experience, ample evidences to support etiologi-cal and “clinical-usefulness-grade” classification hypotheses.Materials and Methods We report observational information on 71 ELD cases and, onthe basis of these, we discuss the clinical onset features, radiological and surgicalcharacteristics, as well as pathological and immunohistochemical evidences.Results In the period between January 1994 and September 2009, 71 consecutivepatients (23 male and 48 female; mean age: 60.2 years; standard deviation [SD] � 8.3years) with ELD diagnosis were surgically treated at our institution. ELD was right sidedin 34 patients (47.9%), left in 25 (35.2%), and bilateral in 12 (16.9%). In nine patients,ELD were diagnosed synchronously and three metachronously. Thirty-eight patients(53.5%) had no significant symptoms; 33 (46.5%) reported a clunking sensation or alocalized scapular swelling during the shoulder movements. Sixty-six (93%) patientsunderwent surgical excision with radical intent while in five patients, a biopsy-onlyprocedure was undertaken. Mean hospital stay was 3.0 days (SD � 1.2 days) with amorbidity of 10.6% (one case of major postoperative bleeding requested a surgicalrevision of the hemostasis). At the univariate analysis, the probability of occurrence ofmorbidity increases with tumor size. All operated patients are alive and well at follow-upwith no sign of recurrence and complete resolution of the symptomatology.Conclusions ELD is relatively uncommon, benign, and well controlled by radicalsurgery.

receivedMay 29, 2012accepted after revisionJuly 11, 2012

Copyright © 2012 by Thieme MedicalPublishers, Inc., 333 Seventh Avenue,New York, NY 10001, USA.Tel: +1(212) 584-4662.

DOI http://dx.doi.org/10.1055/s-0032-1328932.ISSN 0171-6425.

Original Thoracic 1

Background

Elastofibroma dorsi (ELD) is a relatively uncommon, slowlygrowing benign tumor of the soft tissue characterized by theproliferation of elastin fibers in a stroma of collagenous andfatty connective tissue. Since the original report by Jarvi andSaxen,1 this neoplasm has received little attention in themodern literature and, to date and to the best of our knowl-edge, only small to very small series (up to single case report)have formed the basis for the community discussion onetiology and clinical behavior. We report herein our single-institutional experience in the surgical management of ELDand, on the basis of our data, we discuss the main features ofthis entity.

Materials and Methods

Following communication to the IRBQ3Q3Q3, the 71 ELD cases

described, critically evaluated, and discussed in this observa-tional analysis have been identified through a data-miningexercise into the clinical records, the imaging documentation,the histopathological centralized database, and the follow-uprecords of the outpatient clinic of the “Agostino Gemelli”Hospital (School of Medicine of the Catholic University ofRome) and refer to the period January 1994 toSeptember 2009.

Clinical Work-UpThe preoperative diagnostic work-up did not differ from ourroutine approach to thoracic neoplasms and included: clinicalhistory, physical examination with special attention to clini-cal signs of the tumor, basic laboratory tests (routine serumbiochemical, hematological, and coagulation investigations),and chest X-ray. Computed tomography (CT) of the thorax,magnetic resonance imaging (MRI) and/or ultrasound (US)scan, or bilateral tomographic examinations were performedalone or in combination during preoperative diagnosticwork-up evaluation (see below).

SurgeryIn all cases with significant symptomatology or when thelesion appeared suspicious for malignancy (due to the sizebigger than 5 cm or to radiological findings), a radical surgicalexcision was done. In detail, the resection was labeled “radi-cal” when the lesion was completely removed without signsof neoplastic residues, especially at the level of the intercostalspaces just under the scapula. On the contrary, in case ofasymptomatic patients considered as clinically “unfit” forsurgery, a bioptic procedure was performed if a doubt re-garding the nature of the neoplasm occurred during work-upbaseline evaluation. This could, in fact, radically change theclinical work-up and the therapeutic strategy.

PathologyA systematic review of the histological slides has beenperformed before compiling this report with the doubleaim to verify the initial diagnosis with a homogeneousprocedure and then to analyze, in depth, the tumor morphol-

ogy to obtain those information which we perceived arerelevant to the present analysis. Original pathological blocksas stored in the histopathology archive had been typicallyfixed in 10% neutral buffered formalin and embedded inparaffin and the histological sections stained with hematox-ylin-eosin, Masson trichrome, and elastic Van-Gieson Q4Q4

Q4

stains. Immunohistochemical stain with CD34 Q5Q5Q5, transform-

ing growth factor-β (TGF-β), α-smooth muscle actin (ASMA),and h-caldesmon was performed on selected cases.

Follow-UpFollow-up procedures consisted of 6-month clinical evalua-tion (substantially based on manual palpation of subscapulararea) amongwithUS bilateral examination for thefirst 2 yearsand thereafter annual US scan for other 3 years. In case ofclinical and/or radiological suspect of relapse of disease, asecond-step radiological examination (CT scan or MRI) wasperformed only when considered as pivotal tool for thesurgical planning.

Statistical AnalysisThe description of patients’ characteristics was made byfrequency distribution of the different variables and bydescriptive statistics. The χ2 test was calculated to investigatethe usefulness of the drainage in terms of postoperativeoutcome. The relationship between complication and tumorsize was explored by the t test for independent samples.Finally, a logistic regression model was applied to individuatethe predictive variables for morbidity.

Results

PatientsA total of 71 patients (23 male and 48 female; mean age atdiagnosis: 60.2 � 8.3 years) with ELD have been observed andtreated at our institution in the period between January 1994and September 2009. Population characteristics are describedin ►Table 1.

OccupationsData were available in 68 out of the 71 patients. Of the 68, 42(61.8%) patients were manual laborers (either currently orformerly), and the remaining 26 did not report any significantinformation on heavymanual labor or agonistic sport activity.

LocationELD appeared in the subscapular region in 67 patients(94.4%), parascapular in 3 (4.2%), and dorsal subcutaneousin 1 (1.4%). The tumor was right sided in 34 patients (47.9%),left sided in 25 (35.2%), and bilateral in 12 (16.9%) (►Table 1).Nine patients experienced ELD synchronously whereas onlythree metachronously. Mean time to diagnosis was 29.4months (standard deviation [SD] � 28.6 months; range 1 to141 months).

Clinical FindingsThirty-eight patients (53.5%) had no significant symptoms;33 (46.5%) were symptomatic and reported a clunking

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Elastofibroma Dorsi Lococo et al.2

sensation during the shoulder movements during abductionand adduction2 or a localized scapular swelling with low tomoderate pain.3 The mean duration of symptoms was 29.4months (SD � 28.6 months). In general, the preoperative in-

patient clinical evaluation demonstrated a firm, deep, swell-ing lesion in the infrascapular region, usually almost fixed tothe rib cage. The swelling was more prominent on forwardflexion of the shoulder due to the inferior angle of the scapulamoving forward (►Fig. 1A, B).

Laboratory and Radiological FindingsNo specific findings have been recognized so far in biochemi-cal examinations and in peripheral blood analysis. Details ofthe preoperative radiological findings are reportedin►Table 2. First line radiology comprised US scan and thoraxCT and of the scapular region (►Fig. 2A, B). MRI was per-formed only in second instance to add information relevant todiagnostic clarification (►Fig. 2C, D) Q6Q6

Q6. In particular, MRIwith the use of the technique of contrast uptake was consid-ered very useful when a malignant chest wall tumor wassuspected at first-step radiological evaluation. At real-timeUS, the lesions were characterized by an almost homoge-neous morphology: an oval mass with ill-defined margins onboth the superficial and deep planes, almost often fixed to thedeep costal plane and mobile with respect to the superficialsoft tissues. In particular, a typical alternating pattern ofhyper- and hypoechogenic lines, roughly parallel to the chestwall, was the commonest of findings. When color Dopplerwas used, seldom was intralesional vascularization observedand the transducer compression did not detect any significantor useful morphologic variation.4 Regarding the featuresrelated to the CT scan, ELDs typically appear as a homoge-neous mass with a density inferior to that of muscles. Themethodology has a far lesser discrimination power, if com-pared with that of MRI, to detect thin fatty layers around themass: MRI was in fact used in all those cases where themargins of the tumor could not be clearly and unmistakablydiscriminated against the surrounding fat (and other struc-tures). This interface may be show up indistinct at first-levelradiological work-up examination since the mass, as a space-occupying process, does push against solid boundaries: thechest wall and the scapula typically, therefore minimizingthe thickness of the tissue (usually fat) interposed in betweenthe mass and the said bony structures. At MRI, T1 and T2sequences show a fibrous tissue producing a low-intensitysignal identical to that of muscular tissue, whereas the fattytissue is seen as a high-intensity signal on T1 sequences andintermediate on T2 ones. At STIR Q7Q7

Q7 sequences, the mass isseen as amosaic of low- and high-intensity areas. Overall, theELD appears as a large mass with ill-defined contours andmarked gadolinium enhancement.

Surgical FindingsIn accordance with the above reported surgical, a total of 66patients underwent surgical excision through a subscapularamyotomical incision in between the latissimus dorsi and theserratum muscles (►Fig. 3A). Although the level on theincision site varied, slightly depending on size of the tumor,it was usually located at the level of the sixth up to eighthintercostal space. The lesion was very often fixed to thescapular periosteum or to the external thoracic fascia and areal capsule was found in two cases only. In the entire cohort

Table 1 Clinical, radiological, and surgical characteristics of thepopulation

n %

Sex

Male 23 32.4

Female 48 67.6

Side

Bilateral 12 16.9

Left 25 35.2

Right 34 47.9

Comorbidity

No 57 80.3

Diabetes 7 9.9

Cardiovascular diseases 7 9.9

Location

Dorsal subcutaneous 1 1.4

Parascapular 3 4.2

Subscapular 67 94.4

Incidental found

No 31 43.7

Yes 40 56.3

Symptoms

No 38 53.5

Yes 33 46.5

Surgery

No 5 7.0

Yes 66 93.0

Drainage

No 11 16.7

Yes 55 83.3

Capsulated

No 64 97.0

Yes 2 3.0

Drainage at home

No 39 71.0

Yes 16 29.0

Complication

No 55 83.3

Yes 11 16.7

Local recurrence

No 63 95.5

Yes 3 4.5

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Elastofibroma Dorsi Lococo et al. 3

of patients, the neoplasm was easily and completely (R0resection) removed preserving the suprajacent Q9Q9

Q9 musclelayers. The remaining five patients (with no symptoms andconsidered “unfit” for surgical resection) had their diagnosisvia through-cut biopsy to rule out a malignancy.

Postoperative Management and MorbidityMean hospitalization was 3.0 days (SD � 1.2 days, range 1 to8 days). At the beginning of our experience, we did not use apostoperative drain in the first consecutive 11 patients(►Table 1). Despite the attention put in the hemostasisprocedure at the end of the surgical maneuvers, complica-tions often occurred, as detailed in►Tables 3 and 4. Leaving adrain in situ therefore became our standard behavior and atotal of 55 patients had had a drain inserted at the end of the

Fig. 1 Physical examination (A): the presence of a subscapular lesion (elastic in consistent and free-moving at palpation) could be easily detectableafter abduction of ipsilateral shoulder (B).

Table 2 Diagnostic radiological work-up examination of thepatients

Radiological investigations nQ8Q8 (%)

MRI scan 3 (4.2)

CT scan 14 (19.7)

US scan 6 (8.5)

CT scan þ MRI scan 17 (23.9)

MRI scan þ US scan 15 (21.1)

CT scan þ US scan 7 (9.9)

MRI scan þ CT scan þ US scan 9 (12.7)

Abbreviations: CT, computed tomograpghy; MRI, magnetic resonanceimaging; US, ultrasound.

Fig. 2 Radiological imaging of elastofibroma dorsi: ultrasound scan (A), computed tomography scan (B), and magnetic resonance imagingfindings (C and D).

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operation and removed during the hospital stay (in 39) orafter discharge (in 16) following the diminution of dischargeas monitored in the outpatient clinic.

Mean time for drain removal was 2.7 days (SD � 2.2 days;range 0 to 8). Major complications occurred in one patient

only, where a significant bleeding indicated a redo surgicalhemostasis. The patient had been originally diagnosed with avery large (14 � 10 � 7 cm) ELD. Overall postoperative mor-bidity was 10.6%. The presence of the drain did not weigh insignificantly in the morbidity rate (9.1% with and 18.2%without; p: 0.330). Instead, the probability of complicationsoccurrence significantly (OR Q12Q12

Q12: 2.25; confidence interval95%, 1.28 to 3.97; p ¼ 0.005) increased with the increase ofthe tumor size (►Table 4). One patient under anticoagulantmedications experience a vast hematoma after the biopticprocedure, controlled conservatively (repeated needle evac-uations associated with antibiotic prophylaxis).

HistopathologyIn general, the lesions macroscopically appeared as irregularmasses with indistinct borders and hard-elastic consistence(►Fig. 3B). The cut surface typically shows whitish strandsalternated with sparse yellowish streaks. All the lesions buttwo did not have any capsule. Microscopically the tumorswere composed of dense collagen fiber bundles, elongated or

Fig. 3 Surgical findings: operative results (A and B) and macroscopic view of elastofibroma dorsi (C Q10Q10Q10 and D).

Table 3 Postoperative outcomes: frequency distribution ofcomplications

Complications n %

Hematoma 4 5.6

Seroma 5 7.0

Fever 3 4.2

Other 2 1.4

Totala 14 19.7

aIn three cases, more than one complication occurred in the samepatient.

Table 4 Output Q11Q11Q11 of the logistic regression analysis for the complications’ occurrences of patients undergoing surgery (odds ratio,

p value, and confidence interval 95%)

Variables Odds ratio Confidence interval 95% p value

Dimension 2.25 1.28–3.97 0.005

Sex (female) 0.75 0.09–6.01 0.783

Age 1.23 1.00–1.51 0.046

Drainage 0.06 0.00–1.15 0.062

Comorbidity 1.23 0.10–15.20 0.873

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Elastofibroma Dorsi Lococo et al. 5

round-shaped elastic fibers, and fibroblastic cells. Entrappedislets of mature fat tissue without atypia were detected inbetween the collagenous strands (►Fig. 4A, B). Massontrichrome stain made collagenous bundles detectable asdensely distributed in the tumors. The abnormal elastic fiberswere rendered visible with a highly contrasted black appear-ance by the elastic van-Gieson stain (►Fig. 4C). The elasticfibers are densely packed and form discoid or globularstructures. Fibroblasts present in the context of the neoplasticmass are positive for CD34 (►Fig. 4D) and focally for TGF-βbut negative for ASMA and h-caldesmon. In our series, wefound a mean tumor size of 7.2 cm (SD � 2.1 cm; range 3 to14 cm).

Follow-UpMean duration of the follow-up was 59.32 � 36.82 months.At the end of the follow-up analysis, all patients were aliveand well with no sign of recurrent disease. Symptoms werecompletely controlled and significant discomfort from thesurgical procedure was completely resolved within a fewmonths interval from the operation. Interestingly, threepatients underwent surgery for a local recurrence of thetumor that was surgically treated in other institution (surgi-cal borders was not available at our attention at moment ofthe surgical procedure). In that case, the median relapse timewas 28 months.

Discussion

ELD is a relatively uncommon benign lesion of the connectivetissue of the chest wall, first described in 1961.1 Althoughoriginally considered a very rare occurrence, recent autopsy

studies reported an incidence of 13 to 17% and, in subjectsover 55 years of age up to 24%.4–6 Pathophysiological deter-minants are still not clear and several hypotheses have beenput forward. Following the lines of extreme simplification, wecould summarize the prevalent ones as follows:

• Mechanical theory: According to this hypothesis, the tumorwould represent a reaction to the persistent microtrau-matic action and consequent inflammation due to thefriction of the inferior end of scapula to the thoracic wallthat, in turn, induces the hyperproliferation of the fibroe-lastic tissue. This theory is especially suitable to explainthe occurrence of the disease in patients with history ofintensive and often repetitive manual labor. Within thissituation, a different mechanisms would consider therepeated trauma as the cause of a form of degenerationof the elastic fibers.7

• Genetic predisposition: This theory is based on two differ-ent evidences in the literature: (1) as reported in a 170cases strong series,8 approximately 32% of the reportedcases have some familiar predisposition (no specific inves-tigation on the genetic status were performed); (2) thecause would lie in an increased instability and gene hyper-or hypoexpression at the level of different chromo-somes.9,10 Insufficiently investigated, these observationwould point the compass needle toward a process typicalof malignant neoplasms in which a given genetic predis-position is matched with an environmental exposure.

Vascular InsufficiencySome authors have advocated a causal role for a status ofvascular insufficiency and relative ischemia at the level of the

Fig. 4 Pathological and immunohistochemical features of elastofibroma dorsi: high-definition hematoxylin and eosin stain (A and B), elastic Van-Gieson stain (C), and positive immunostaining for CD34 (D).

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Elastofibroma Dorsi Lococo et al.6

scapular area. This pathogenetic theory has not been widelyuptaken and, so far, is reported as a hypothesis in reference11

only.Regarding the clinical characteristics, these are substan-

tially nonspecific; in fact ELDs occur with a relatively higherprevalence in the population with age < 50 years old people,with a peculiar characteristics of a marginal, still present,incidence in children, a fact that would advocate for a geneticpredisposition rather than a consequence of mechanicalrepetitive microinjuries.12,13 Information available so fardoes not justify the hypothesis of a dual origin, therefore, ofdifferent pathological ontologies. In the vastmajority of cases,as in our experience (83.1%, 59 cases), the lesion is unilateraland shows a tendency to arise in a very specific anatomicallocation, namely the region beneath the rhomboid major andlatissimus dorsi muscles, which is the sitewherewe observedthe occurrence of the disease in the 94.4% of cases (67). Theside distribution is almost consistently even (right sided in 34cases [47.9%] and left-sided in 25 cases [35.2%] in our experi-ence). Bilateral tumors can occur bilaterally14–16: synchro-nously or metachronously (respectively, nine and three casesin our series).

ApproximatelyQ13Q13Q13, half of the patients do experience

typical symptoms at diagnosis and these consist of localscapular swelling and a clunking sensation during abduc-tion and adduction of the shoulder, with pain of moderateor, rarely, severe entity. In the present study, overall 33patients (46.5%) reported some symptoms because theyhave had a clunking sensation during the abduction andadduction of the shoulder (17 patients) or a local scapularswelling with low to moderate pain (16 patients). The meanduration of symptoms before surgery or biopsy was29.4 � 28.6 months. The remaining (53.5%) did not experi-ence any symptom at all.

In general, the preoperative physical examination shows afirm, deep, swelling lesion in the infrascapular region thatalso appears as fixed to the rib cage.

Next discussion topic, still a forum of open discussion, iswhat should be the preoperative diagnostic work-up once anELD is diagnosed. We consider the execution of US and CTscan (or MRI) as mandatory for a comprehensive assessmentof themass, including a careful evaluation of the vascularity ofthe tumor and its relationship with the surrounding struc-tures. Given the nonmarginal occurrence of bilateral lesions,CT scan is useful, as well, to eventually exclude this situation.Seldom MRI has been used in the evaluation of ELDs and itdoes not add any additional information with respect to USand CT. Curiously enough in three of our cases, the ELD wasdetected as an incidental finding during MRI scan performedfor other reasons. Controversial, as well, is the adoption ofpositron emission tomography (PET) scan. Advocates3,17

recognize that PET could help in discriminating, in thepreoperative setting, amongbenign and eventuallymalignanttumor. Given the very nature of ELD and the substantialcapability of standard imaging techniques to provide enoughand reliable information for the diagnosis,3,17 we deem thatthe use of PET is not yet fully supported by strong enoughevidences to include it routinely.

Evenmore debated and controversial are the data reportedon the utility of the preoperative cytohistological assessment.Some authors2,5 concluded their clinical analyses with thestatement that biopsy is not mandatory in selected cases(advanced age, female gender, typical, or bilateral location ofthe lesion) whereas they recommend open biopsy (or, as aminimum, a core needle biopsy) in all the remaining cases.Others argue that the biopsy is never required because thediagnosis can always be done by means of radiologicalinvestigations only.18,19 We Q14Q14

Q14 would advocate for a preop-erative biopsy only in those suspicious cases for a malignancysuch as: (1) the lesion has increased in size very rapidly in ashort period; (2) the location is unusual; (3) there areradiological signs of suspect, or (4) when surgical resectionwas improper for the poor general conditions of the patients.Fine needle aspiration biopsy is not recommended due to thehypocellularity of the tumor.

A final point of discussion is the indication for surgicaltreatment.We do not have, so far, any guideline regarding theappropriate behavior in this setting, and this is due to thepaucity of observed cases, even in the most numerous series.We could suggest that the surgical indication (if the patient isclinically considered as “fit” for surgery) should follow adecision-making process pivoted on the following threecriteria.

ClinicalELD with clinical symptoms, regardless of size. Surgery isreserved only when the patient is symptomatic and sufferspain or has a functional impairment. This condition normallyoccurs in lesions with a major axis greater than 5 cm but it isnot rare in cases with smaller tumors.

DimensionalELD bigger than 5 cm must undergo excisional surgery.20

RadiologicalAny inhomogeneity in the tumoral radiological appearanceshould be treated as a sign of potential malignancy.

In our study, 93% (n ¼ 66) of patients satisfied one or morethan one of these criteria and were operated with intent ofradicality.

Correlated with surgery is the occurrence of postoperativeseromas/hematomas, the most frequent morbidity observedin patients with ELD. So far, there is no guideline driving thesurgical drainage policy. On the basis of our experience,wherewehave observed a high incidence of this complicationin the early phases of our experience whenwe were not usedto put any drain (first consecutive 11 patients), we havechanged our behavior and, instead, consistently put a drainin the following 55 patients with far better results (nooccurrence of seroma/hematomas). Drains have been re-moved during the hospital stay in all but 16 patients thathave been sent home with the drain in situ and monitoredthrough outpatients’ clinic visit where drains have beenremoved (mean 6 days after the operation, range 2 to 8days). We have never imposed any shoulder movementrestriction in the postoperative period as this is not standard

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Elastofibroma Dorsi Lococo et al. 7

recommended precaution. Bleeding and infection can occur,especially in large ELD where our suggestion to put a surgicaldrain is particularly enforced. In our series, only one patientwas reoperated due to postoperative hemorrhage. His ELDwas 14 � 10 � 7 cm.

In our experience only the sizewas significantly correlatedwith the occurrence of postoperative complications. Underthis respect, the procedure of positioning a drain is neutral(►Table 4).

Regarding the follow-up, our last item for discussion, weadvocate for US to be performed bilaterally once a year afterthe operation for 5 years. In case of US detection of acontralateral lesion after ELD resection, a CT scan or MRI ofthe thorax seem to be indicated only as useful tool for surgicalplanning.

Limitations and Strengths

This article has the usual limitations of retrospective mono-centric studies (the long duration of patient’s inclusion).

On the other hand, this represents the largest clinicallydetailed series of ELD treated in a single institution, this beinga crucial issue when analyzing surgical outcome of suchuncommon disease.

Moreover, despite all the limitations mentioned above,this study has the great merit to report a detail description ofthe surgical strategy and its management, suggesting analgorithm of treatment and surveillance after surgery.

Conclusion

ELD is relatively rare and benign disease, usually well con-trolled by radical surgery. Bilateral disease is not uncommon(17% in our series) and a local relapse could be occurred afterincomplete resection. The etiology is still controversial andenigmatic; thus we strongly advocate further investigationson the pathophysiological determinants.

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