Pediatric Pulmonology 46:610–613 (2011)

Case Report

Endobronchial Fibrosarcoma Presenting as RecurrentLeft-Sided Pneumonia

Atul Gupta,1* Rosemary Marsh,1 Simon Jordan,2 Simon Padley,3 Kareem Aboualfa,4

Ola Smith,5 Cyril Fisher,6 Andrew G. Nicholson,4 and Andrew Bush1

Summary. We report a 6-year old girl with recurrent and non-resolving left-sided ‘‘pneumonia’’

over a 3-year period who was diagnosed with an endobronchial low-grade fibrosarcoma. This

highlights the importance of considering underlying localized airway obstruction in any case of

clinically or radiologically atypical pneumonia in a child and therefore the need for careful follow-up

of all but the most typical cases. Pediatr Pulmonol. 2011; 46:610–613. � 2010 Wiley-Liss, Inc.

Key words: endobronchial; fibrosarcoma; polyp; pneumonia; tumor.

Funding source: none reported.

INTRODUCTION

Community-acquired pneumonia is relatively commonin children, with an incidence of 10–40 cases per1,000 per year.1 It is usually due to viral or bacterialinfection in an otherwise healthy child and resolvescompletely.1 Typically, such infections are clinicallyresolved before radiological resolution is complete.2

When infections have an atypical course (such as recurrentepisodes, incomplete resolution or atypical radiologicalappearances) or atypical features (e.g., failure to thrive,positive family history, and gastrointestinal symptoms)further investigations are warranted to exclude diseaseslike cystic fibrosis, immunodeficiency, primary ciliarydyskinesia and aspiration. We describe an unusual case ofrecurrent pneumonia, which highlights two importantlessons.

Case

A fully immunized 6-year old girl was referred to RoyalBrompton Hospital for investigation of multiple episodesof a pneumonia-like illness over the previous 3 years.At three and a half years of age shewas presented to the

local hospital with a short history of fever, cough, andvomiting. Chest radiography showed left-sided opacifica-tion. She was treated for community-acquired pneumoniawith intravenous antibiotics and was discharged home onoral antibiotics after clinical improvement. Ten days later

she was readmitted with respiratory distress and fever.Chest radiograph showed persistent left sided opacifica-tion and was treated with 2 weeks of broad-spectrumintravenous antibiotics. Within 3 weeks of her recoveryfrom this episode, shewas again admitted with respiratoryexacerbation for intravenous antibiotics.

1Department of Paediatric Respiratory Medicine, Royal Brompton and

Harefield NHS Foundation Trust, London, UK.

2Department of Thoracic Surgery and Respiratory Medicine, Royal

Brompton and Harefield NHS Foundation Trust, London, UK.

3Department of Radiology, Royal Brompton and Harefield NHS Founda-

tion Trust, London, UK.

4Department of Histopathology, Royal Brompton and Harefield NHS

Foundation Trust, London, UK.

5Department of Paediatrics, William Harvey Hospital, Ashford, Kent, UK.

6Department of Histopathology, Royal Marsden Hospital NHS Trust,

London, UK.

*Correspondence to: Atul Gupta, Royal Brompton Hospital, Sydney Street,

London SW3 6NP, UK. E-mail: atulgupta@doctors.org.uk

Received 8 September 2010; Revised 28 October 2010; Accepted 28

October 2010.

DOI 10.1002/ppul.21407

Published online 30 December 2010 in Wiley Online Library

(wileyonlinelibrary.com).

� 2010 Wiley-Liss, Inc.

Follow-up chest radiograph (2months after last episodeof illness) showed some but not complete resolution of leftsided opacification. Further investigations were done toexclude tuberculosis, cystic fibrosis, and immune defi-ciency. Six months later she was discharged from follow-up after clinical resolution of her illness.

Three years later, at six and a half years of age, shepresented again with intermittent dry cough forseveral weeks and short history of fever. She was treatedwith intravenous and oral antibiotics. Chest radiographshowed extensive left lung consolidation. After thisepisode she was referred to Royal Brompton Hospitalfor further management.

Clinical assessment showed dramatically reducedbreath sounds throughout the left hemithorax with theapex beat displaced to the left axilla. There was a mildscoliosis concave to the left. Spirometry revealed an FEV1of 0.82 L, 67% predicted, FVC 1.05 L, 79% predicted.Chest radiograph (Fig. 1) showed a collapse of the left lungwith a soft tissue opacity protruding into the left mainbronchus. Contrast enhanced chest CT scan (Fig. 2)confirmed obstruction of the leftmain bronchus by amass.Three small soft tissue nodules measuring 2–3mm werealso noted in the right lung. Flexible bronchoscopy (Fig. 3)revealed an endobronchial polypoid mass, completelyoccluding the left main bronchus. Following biopsyconfirmation of neoplasia, classified at that time as low-grade spindle cell neoplasm not otherwise specified, thepatient underwent left pneumonectomy. At thoracotomy,the tumorwas found to be arising from a broad basewithinthe left main bronchus and so a sleeve resection was notpossible. A left pneumonectomy was therefore performedwith resection of a firm broad-based mass measuring30� 25� 20mm obstructing the left main bronchus andinvolving the bronchial wall. The proximal bronchialresection margin was examined by frozen section micro-scopy during the operation, and showed no evidence ofdisease.

Microscopy showed a submucosal proliferation of mildlypleomorphic spindle cells arranged in sheets, stroriformwhorls, and fascicles with scattered multinucleate giantcells and some peripheral inflammatory cells (Fig. 4).There were occasional mitoses present but no necrosis.Staining for CD34 was very focally positive, althoughpositivity for this antibodywas not viewed as specific for atumor type in this context. The tumor was negative forSMA and ALK-1, arguing strongly against an inflamma-tory myofibroblastic tumor. Staining for MNF116 andEMA was negative, thus excluding carcinoma from thediagnosis. Neural and perineural tumors were excludedbased on the lack of S-100 and claudin-1 positivity. Therewas no histiocytic component morphologically and thiswas corroborated by the lack of CD68 positivitywithin the

Pediatric Pulmonology

Fig. 1. Chest X-ray showing a collapse of the left lungwith a soft

tissue opacity, protruding into the left main bronchus.

Fig. 2. CTscanof chest confirmedacollapsed left lower lobeand

asoft tissuemass in the leftmainbronchusextending into the left

lung.

Fig. 3. Flexible bronchoscopy showed a large endobronchial

mass obstructing the left main bronchus.

Recurrent Left-Sided Pneumonia 611

neoplastic cells. PCR did not detect the FUS-CREB3L2 orETV6-NTRK3 gene fusions, thus ruling out a low-gradefibromyxoid sarcoma and congential fibrosarcoma. Over-all, the features were regarded as those of a low-gradefibrosarcoma.Serial follow-up chest CT scans (over last 2 years) have

not shown any change in the number, size, or appearancesof the nodules in the right lung.

DISCUSSION

Community-acquired pneumonia is common in youngchildren and usually resolves completely with antibiotictreatment and supportive care.1 In most cases it is usuallynot essential to investigate for an underlying cause. Inadults chest radiography is routinely repeated afterconvalescence from community acquired pneumonia toscreen for underlying neoplastic disease.3 Current guide-lines on management of community acquired pneumoniain children state that, ‘‘follow-up chest radiography shouldonly be performed after lobar collapse, an apparent roundpneumonia, or for continuing symptoms’’.1 This recom-mendation is supported by prospective studies whichdemonstrate no need for routine follow-up chest radiog-raphy.2,4–6

However, if there is a history suggesting progressive orpersistent collapse or consolidation it is important toconsider the possibility, albeit rare, of a structuralabnormality including a neoplastic endobronchial lesion.Case series of endobronchial neoplasms in childrensuggest that the majority of these present as persistentcollapse or consolidation7–10 and that delay in diagnosiscan have implications for future surgical management.Primary lung tumors in children are rare. They have a

tendency to present endobronchially, in particular inflam-

matory myofibroblastic tumor, carcinoids, salivary-glandtumors, hamartomas, papillomas, and sarcomas. Inflam-matory myofibroblastic tumors are the most commonprimary pulmonary tumors in children under the age of16.11 This entity was excluded in our patient based on thelack of characteristicmorphology, in particular an absenceof inflammatory cells within the tumor, and the absence ofboth SMA and ALK-1 staining. Guccion and Rosen notedthat themajority of endobronchial fibrosarcomas occurredin children, whereas intrapulmonary fibrosarcomas pre-sented mostly in adults and elderly populations. Theyclassified fibrosarcomas of the lung into endobronchialand intrapulmonary types and this classification schemewas said to have prognostic and clinical importance.12

In another series, metastases were more common withpleuropulmonary blastoma and carcinoid being morefrequently seen than mesenchymal neoplasms.13

CONCLUSION

This child had recurrent consolidation in the left lungwith several relapses despite aggressive antibiotic treat-ment. She also never had a completely normal chest X-rayand perhaps this should have prompted earlier referral andinvestigation. We have reported this case because itillustrates two important lessons: firstly, the importance offollowing-up all but the most typical cases of communityacquired pneumonia to ensure complete recovery (clinicaland radiological). Secondly, although rare, primarypulmonary tumors can occur in children and should beconsidered in the differential diagnoses of non-resolvingradiological shadowing.

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Fig. 4. Microscopic image showing spindle cells arranged in

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coma. (H&E, 100�magnification).

612 Gupta et al.

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