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A pleuropulmonary blastoma type III in a 4-month-old infant: First case report in Syria

INTRODUCTION: Pleuropulmonary blastoma is a rare, aggressive intrathoracic neoplasm of early childhood. CASE PRESENTATION: We report a case of a 4-month-old male baby who has presented with recurrent respiratory infections since birth. A surgical team was consulted due to abnormal opacification obse...

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Autores principales: AlKhamisy, Ayman, Abbas, Walaa, Barakat, Aya, Ghabreau, Lina, Khanji, Khaled, Morjan, Mohamad
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10382812/
https://www.ncbi.nlm.nih.gov/pubmed/37423144
http://dx.doi.org/10.1016/j.ijscr.2023.108461
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author AlKhamisy, Ayman
Abbas, Walaa
Barakat, Aya
Ghabreau, Lina
Khanji, Khaled
Morjan, Mohamad
author_facet AlKhamisy, Ayman
Abbas, Walaa
Barakat, Aya
Ghabreau, Lina
Khanji, Khaled
Morjan, Mohamad
author_sort AlKhamisy, Ayman
collection PubMed
description INTRODUCTION: Pleuropulmonary blastoma is a rare, aggressive intrathoracic neoplasm of early childhood. CASE PRESENTATION: We report a case of a 4-month-old male baby who has presented with recurrent respiratory infections since birth. A surgical team was consulted due to abnormal opacification observed on a chest X-ray. An enhanced-contrast CT scan of the chest revealed a heterogenous, well-delineated mass measuring about 3,8 × 6 cm in the posterior mediastinum. A left posterolateral thoracotomy was performed. The mass was separated from the lung parenchyma, located behind the parietal pleura, and adherent to the chest wall and superior ribs. The lesion was totally removed. Histologically, the lesion was a pleuropulmonary blastoma type III. Currently, the patient is on a 6-month course of chemotherapy. CLINICAL DISCUSSION: The aggressive, insidious behavior of PPB requires a high index of suspicion for diagnosis. The clinical manifestations and imaging modalities are atypical and nonspecific. However, PPB should be kept in mind when a huge solid or cystic mass is observed in the lung field on imaging. CONCLUSION: Extrapulmonary pleuropulmonary blastoma is a very rare entity characterized by highly aggressive behavior and a poor prognosis. Early excision of thoracic cystic lesions in children is warranted regardless of the symptoms to avoid future mishaps.
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spelling pubmed-103828122023-07-30 A pleuropulmonary blastoma type III in a 4-month-old infant: First case report in Syria AlKhamisy, Ayman Abbas, Walaa Barakat, Aya Ghabreau, Lina Khanji, Khaled Morjan, Mohamad Int J Surg Case Rep Case Report INTRODUCTION: Pleuropulmonary blastoma is a rare, aggressive intrathoracic neoplasm of early childhood. CASE PRESENTATION: We report a case of a 4-month-old male baby who has presented with recurrent respiratory infections since birth. A surgical team was consulted due to abnormal opacification observed on a chest X-ray. An enhanced-contrast CT scan of the chest revealed a heterogenous, well-delineated mass measuring about 3,8 × 6 cm in the posterior mediastinum. A left posterolateral thoracotomy was performed. The mass was separated from the lung parenchyma, located behind the parietal pleura, and adherent to the chest wall and superior ribs. The lesion was totally removed. Histologically, the lesion was a pleuropulmonary blastoma type III. Currently, the patient is on a 6-month course of chemotherapy. CLINICAL DISCUSSION: The aggressive, insidious behavior of PPB requires a high index of suspicion for diagnosis. The clinical manifestations and imaging modalities are atypical and nonspecific. However, PPB should be kept in mind when a huge solid or cystic mass is observed in the lung field on imaging. CONCLUSION: Extrapulmonary pleuropulmonary blastoma is a very rare entity characterized by highly aggressive behavior and a poor prognosis. Early excision of thoracic cystic lesions in children is warranted regardless of the symptoms to avoid future mishaps. Elsevier 2023-07-04 /pmc/articles/PMC10382812/ /pubmed/37423144 http://dx.doi.org/10.1016/j.ijscr.2023.108461 Text en © 2023 The Author(s) https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
AlKhamisy, Ayman
Abbas, Walaa
Barakat, Aya
Ghabreau, Lina
Khanji, Khaled
Morjan, Mohamad
A pleuropulmonary blastoma type III in a 4-month-old infant: First case report in Syria
title A pleuropulmonary blastoma type III in a 4-month-old infant: First case report in Syria
title_full A pleuropulmonary blastoma type III in a 4-month-old infant: First case report in Syria
title_fullStr A pleuropulmonary blastoma type III in a 4-month-old infant: First case report in Syria
title_full_unstemmed A pleuropulmonary blastoma type III in a 4-month-old infant: First case report in Syria
title_short A pleuropulmonary blastoma type III in a 4-month-old infant: First case report in Syria
title_sort pleuropulmonary blastoma type iii in a 4-month-old infant: first case report in syria
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10382812/
https://www.ncbi.nlm.nih.gov/pubmed/37423144
http://dx.doi.org/10.1016/j.ijscr.2023.108461
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