Critical Care: Global Case Report Poster - Miscellaneous |

A 2-Year-Old Male With Pleuropulmonary Blastoma Type II: A Case Report FREE TO VIEW

Lorraine Sharon Salvana, MD
Author and Funding Information

Philippine Heart Center, Quezon City, Philippines

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):282A. doi:10.1016/j.chest.2016.08.295
Text Size: A A A
Published online

SESSION TITLE: Global Case Report Poster - Miscellaneous

SESSION TYPE: Global Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Pleuropulmonary Blastoma [PPB] is a very rare,highly aggressive and malignant tumor that originates from either the lungs or pleura [1].It has poor prognosis and it mainly occurs in children less than five or six years with three different subtypes:cystic [type I],combined cystic and solid [type II] and solid [type III].We report a case of a 2 year old boy presenting with cough, fever and difficulty of breathing, whose management was carried out successfully with surgical resection followed by neoadjuvant chemotherapy.

CASE PRESENTATION: This is a case of a 2 year-old-male who presented with productive cough two months prior associated with fever and dyspnea who was initially managed as a case of pneumonia. However, condition persisted. Work up for TB was negative.Chest X-ray showed an opacity filling the right hemithorax and leading to mediastinal shift to the opposite side.Computed Tomography revealed a large mass in the right hemithorax containing solid and cystic components. He was then transferred in this institution intubated,chest examination showed asymmetry on chest expansion,subcostal retractions and vesicular breath sounds over the left lung. The right hemithorax showed dullness on percussion,decreased vocal and tactile fremitus,and absent breath sounds.Alpha Feto protein,Urine VMA were within normal limits,Serum lactate dehydrogenase was elevated. No significant change was seen on repeat chest X-ray. Repeat CT scan with contrast showed a large hypodense mass lesion on the right hemithorax.On 2D echocardiogram,the mass was compressing the right atrium,prompting emergency surgical procedure.Excision of the entire mass with frozen section was done which showed myoplasty with heterologous component possibly chondroid fat,vascular component with atypical cellular areas.Final tissue biopsy aided by immunohistochemistry staining revealed an Pleuropulmonary Blastoma,Type II because it contained both solid and cystic components.Post procedure inflammatory changes were demonstrated with serial chest X-rays which was resolved spontaneously.He was successfully extubated and was discharged improved. He recently completed the the first course of chemotherapy after discharged.

DISCUSSION: Pleuropulmonary blastoma is an aggressive tumor accounting for less than 1% of all primary malignant lung tumors in the pediatric population [1].The age of presentation is usually less than 4 years. In patients over 10 very few cases have been reported,only one case has been reported at 36 years (2). There was no gender predilection noted and the tumor occurs more commonly on the right side. In our case, we are presented with a 2 years old male and the tumor is located on the right hemithorax.The tumor has no characteristic findings on imaging studies;however,it should be considered in the differential diagnosis of other benign cystic lung lesions on imaging studies.Our patient in this case like most reported cases presented with pulmonary infection and respiratory distress.Immunohistochemistry plays a vital role in the diagnosis of PBB wherein each component can be clearly distinguished with each other[4].In our case, complete excision biopsy was done,and a panel of immunohistochemical markers confirmed the diagnosis consistent with PBB type II.This condition carries a grave prognosis.Types II and III PPBs are clearly aggressive malignancies with projected overall survival of 62% at 2 yrs and 42% at 5 yrs, even after multimodality therapy.[4]

CONCLUSIONS: We report a case of a 2 year old male with PPB type II which is a rare aggressive tumor that usually occurs in young children.Clinicians should have a high index of suspicion for lung masses that present in pediatric age and should be managed with multimodal therapy despite the presence of poor prognostic factors, it may provide remission and a long-term disease-free period.

Reference #1: Calabria R, Srikanth MS, Chamberlin K, Bloch J, Atkinson JB. Management of pulmonary blastoma in children. Am Surg. 1993;59(3):192-6.

Reference #2: R. I. Walker, K. Suvarna, and S. Matthews, “Pulmonary blastoma: presentation of two atypical cases and review of the literature,” British Journal of Radiology, vol. 78, no. 929, pp. 437-440, 2005

Reference #3: Priest JR, McDermott MB, Bhatia S, Watterson J, Manivel JC, Dehner LP, et al. Pleuropulmonary blastoma: A clinicopathol study of 50 cases. Cancer. 1997;80(1):147-61.5.

DISCLOSURE: The following authors have nothing to disclose: Lorraine Sharon Salvana

No Product/Research Disclosure Information




Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

Find Similar Articles
PubMed Articles
  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543