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Pleuropulmonary blastoma in a young adult presenting as a ruptured cystic teratoma in radiology.

Pleuropulmonary blastoma (PPB) is a rare malignant dysontogenetic neoplasm primarily affecting children and is characterized histologically by a variably mixed blastematous and sarcomatous patterns. We herein report a very exceptional adult case of PPB. A 21-yr-old male patient presented with a left...

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Autores principales: Lee, Chang Hun, Kim, Keun Il, Kim, Young Dae, Lee, Min Ki, Kim, Jee Yeon, Park, Do Youn, Sol, Mee Young, Suh, Kang Suek
Formato: Texto
Lenguaje:English
Publicado: Korean Academy of Medical Sciences 2003
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3055094/
https://www.ncbi.nlm.nih.gov/pubmed/12923341
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author Lee, Chang Hun
Kim, Keun Il
Kim, Young Dae
Lee, Min Ki
Kim, Jee Yeon
Park, Do Youn
Sol, Mee Young
Suh, Kang Suek
author_facet Lee, Chang Hun
Kim, Keun Il
Kim, Young Dae
Lee, Min Ki
Kim, Jee Yeon
Park, Do Youn
Sol, Mee Young
Suh, Kang Suek
author_sort Lee, Chang Hun
collection PubMed
description Pleuropulmonary blastoma (PPB) is a rare malignant dysontogenetic neoplasm primarily affecting children and is characterized histologically by a variably mixed blastematous and sarcomatous patterns. We herein report a very exceptional adult case of PPB. A 21-yr-old male patient presented with a left chest pain of two weeks' duration. A computed tomography scan revealed a large, multicystic tumor occupying the left lower hemithorax, leading to the impression of a ruptured mediastinal cystic teratoma. A thoracotomy for resection of the tumor was performed. On histologic examination, the tumor consisted of cystic walls and associated solid lesions which showed undifferentiated blastemal tissues with focal fibrosarcomatous and rhabdoid features. Immunohistochemically the tumor cells only showed diffuse strong positivity for vimentin. The histologic findings corresponded to a type II PPB. The authors suggest that PPB, especially of type I or II, should be included in the radiologic differential diagnosis of mediastinal cystic neoplasms in a young adult.
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spelling pubmed-30550942011-03-15 Pleuropulmonary blastoma in a young adult presenting as a ruptured cystic teratoma in radiology. Lee, Chang Hun Kim, Keun Il Kim, Young Dae Lee, Min Ki Kim, Jee Yeon Park, Do Youn Sol, Mee Young Suh, Kang Suek J Korean Med Sci Research Article Pleuropulmonary blastoma (PPB) is a rare malignant dysontogenetic neoplasm primarily affecting children and is characterized histologically by a variably mixed blastematous and sarcomatous patterns. We herein report a very exceptional adult case of PPB. A 21-yr-old male patient presented with a left chest pain of two weeks' duration. A computed tomography scan revealed a large, multicystic tumor occupying the left lower hemithorax, leading to the impression of a ruptured mediastinal cystic teratoma. A thoracotomy for resection of the tumor was performed. On histologic examination, the tumor consisted of cystic walls and associated solid lesions which showed undifferentiated blastemal tissues with focal fibrosarcomatous and rhabdoid features. Immunohistochemically the tumor cells only showed diffuse strong positivity for vimentin. The histologic findings corresponded to a type II PPB. The authors suggest that PPB, especially of type I or II, should be included in the radiologic differential diagnosis of mediastinal cystic neoplasms in a young adult. Korean Academy of Medical Sciences 2003-08 /pmc/articles/PMC3055094/ /pubmed/12923341 Text en
spellingShingle Research Article
Lee, Chang Hun
Kim, Keun Il
Kim, Young Dae
Lee, Min Ki
Kim, Jee Yeon
Park, Do Youn
Sol, Mee Young
Suh, Kang Suek
Pleuropulmonary blastoma in a young adult presenting as a ruptured cystic teratoma in radiology.
title Pleuropulmonary blastoma in a young adult presenting as a ruptured cystic teratoma in radiology.
title_full Pleuropulmonary blastoma in a young adult presenting as a ruptured cystic teratoma in radiology.
title_fullStr Pleuropulmonary blastoma in a young adult presenting as a ruptured cystic teratoma in radiology.
title_full_unstemmed Pleuropulmonary blastoma in a young adult presenting as a ruptured cystic teratoma in radiology.
title_short Pleuropulmonary blastoma in a young adult presenting as a ruptured cystic teratoma in radiology.
title_sort pleuropulmonary blastoma in a young adult presenting as a ruptured cystic teratoma in radiology.
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3055094/
https://www.ncbi.nlm.nih.gov/pubmed/12923341
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