Type II pleuropulmonary blastoma mistaken for rhabdomyosarcoma: A case report

INTRODUCTION: Pleuropulmonary blastoma (PPB) is rare, representing 0.3 % of all pediatric cancers. PPB is classified into three subtypes and may progress from type I to types II and III, with a worse prognosis. Given its rarity, the diagnosis is frequently challenging. CASE PRESENTATION: We report a...

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Autores principales: Karray, Amina, Boussetta, Abir, Sassi, Farah, Cherifi, Walid, Haouet, Slim, Gargah, Tahar
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10163632/
https://www.ncbi.nlm.nih.gov/pubmed/37119756
http://dx.doi.org/10.1016/j.ijscr.2023.108261
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author Karray, Amina
Boussetta, Abir
Sassi, Farah
Cherifi, Walid
Haouet, Slim
Gargah, Tahar
author_facet Karray, Amina
Boussetta, Abir
Sassi, Farah
Cherifi, Walid
Haouet, Slim
Gargah, Tahar
author_sort Karray, Amina
collection PubMed
description INTRODUCTION: Pleuropulmonary blastoma (PPB) is rare, representing 0.3 % of all pediatric cancers. PPB is classified into three subtypes and may progress from type I to types II and III, with a worse prognosis. Given its rarity, the diagnosis is frequently challenging. CASE PRESENTATION: We report an occurrence of PPB in a 3-year-old girl, who presented recurrent pneumopathy. Imaging investigations revealed a large solid lesion in the left hemithorax. Biopsy followed by histological analysis suggested rhabdomyosarcoma. The patient received neoadjuvant chemotherapy before proceeding to complete tumor excision. Surgical exploration revealed that the tumor was primitively related to parietal pleura and lower lobe of left lung. Histopathology of the tumor retained a definitive diagnosis of PPB type II. Postoperative course was uneventful, and a cerebral MRI ruled out brain metastasis. Adjuvant chemotherapy was administered. DISCUSSION: Clinical expression of PPB is nonspecific and variable. It ranges from a dry cough to respiratory distress. Standard radiography is the first examination to perform and CT is the gold standard for characterization thoracic masses. Surgery and chemotherapy are the pillars of treatment. Indications depend on the tumor type, its extent and its resectability. CONCLUSION: PPB is an aggressive tumor that occurs only in children. Due to the rarity of PPB, evidence on optimal treatment is still insufficient. Careful follow-up is necessary searching for local recurrence or metastasis.
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spelling pubmed-101636322023-05-07 Type II pleuropulmonary blastoma mistaken for rhabdomyosarcoma: A case report Karray, Amina Boussetta, Abir Sassi, Farah Cherifi, Walid Haouet, Slim Gargah, Tahar Int J Surg Case Rep Case Report INTRODUCTION: Pleuropulmonary blastoma (PPB) is rare, representing 0.3 % of all pediatric cancers. PPB is classified into three subtypes and may progress from type I to types II and III, with a worse prognosis. Given its rarity, the diagnosis is frequently challenging. CASE PRESENTATION: We report an occurrence of PPB in a 3-year-old girl, who presented recurrent pneumopathy. Imaging investigations revealed a large solid lesion in the left hemithorax. Biopsy followed by histological analysis suggested rhabdomyosarcoma. The patient received neoadjuvant chemotherapy before proceeding to complete tumor excision. Surgical exploration revealed that the tumor was primitively related to parietal pleura and lower lobe of left lung. Histopathology of the tumor retained a definitive diagnosis of PPB type II. Postoperative course was uneventful, and a cerebral MRI ruled out brain metastasis. Adjuvant chemotherapy was administered. DISCUSSION: Clinical expression of PPB is nonspecific and variable. It ranges from a dry cough to respiratory distress. Standard radiography is the first examination to perform and CT is the gold standard for characterization thoracic masses. Surgery and chemotherapy are the pillars of treatment. Indications depend on the tumor type, its extent and its resectability. CONCLUSION: PPB is an aggressive tumor that occurs only in children. Due to the rarity of PPB, evidence on optimal treatment is still insufficient. Careful follow-up is necessary searching for local recurrence or metastasis. Elsevier 2023-04-26 /pmc/articles/PMC10163632/ /pubmed/37119756 http://dx.doi.org/10.1016/j.ijscr.2023.108261 Text en © 2023 Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. 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
Karray, Amina
Boussetta, Abir
Sassi, Farah
Cherifi, Walid
Haouet, Slim
Gargah, Tahar
Type II pleuropulmonary blastoma mistaken for rhabdomyosarcoma: A case report
title Type II pleuropulmonary blastoma mistaken for rhabdomyosarcoma: A case report
title_full Type II pleuropulmonary blastoma mistaken for rhabdomyosarcoma: A case report
title_fullStr Type II pleuropulmonary blastoma mistaken for rhabdomyosarcoma: A case report
title_full_unstemmed Type II pleuropulmonary blastoma mistaken for rhabdomyosarcoma: A case report
title_short Type II pleuropulmonary blastoma mistaken for rhabdomyosarcoma: A case report
title_sort type ii pleuropulmonary blastoma mistaken for rhabdomyosarcoma: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10163632/
https://www.ncbi.nlm.nih.gov/pubmed/37119756
http://dx.doi.org/10.1016/j.ijscr.2023.108261
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