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Pneumothorax caused by cystic and nodular lung metastases from a malignant uterine perivascular epithelioid cell tumor (PEComa)
Perivascular epithelioid cell tumors (PEComas) are mesenchymal neoplasms with immunoreactivity for both melanocytic and smooth muscle markers. PEComas occur at multiple sites, and malignant PEComas can undergo metastasis, recurrence and aggressive clinical courses. Although the lung is a common meta...
Autores principales: | , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5496452/ https://www.ncbi.nlm.nih.gov/pubmed/28706850 http://dx.doi.org/10.1016/j.rmcr.2017.06.011 |
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author | Okamoto, Shouichi Komura, Moegi Terao, Yasuhisa Kurisaki-Arakawa, Aiko Hayashi, Takuo Saito, Tsuyoshi Togo, Shinsaku Shiokawa, Akira Mitani, Keiko Kobayashi, Etsuko Kumasaka, Toshio Takahashi, Kazuhisa Seyama, Kuniaki |
author_facet | Okamoto, Shouichi Komura, Moegi Terao, Yasuhisa Kurisaki-Arakawa, Aiko Hayashi, Takuo Saito, Tsuyoshi Togo, Shinsaku Shiokawa, Akira Mitani, Keiko Kobayashi, Etsuko Kumasaka, Toshio Takahashi, Kazuhisa Seyama, Kuniaki |
author_sort | Okamoto, Shouichi |
collection | PubMed |
description | Perivascular epithelioid cell tumors (PEComas) are mesenchymal neoplasms with immunoreactivity for both melanocytic and smooth muscle markers. PEComas occur at multiple sites, and malignant PEComas can undergo metastasis, recurrence and aggressive clinical courses. Although the lung is a common metastatic site of PEComas, they usually appear as multiple nodules but rarely become cystic or cavitary. Here, we describe a female patient whose lungs manifested multiple cystic, cavity-like and nodular metastases 3 years after the resection of uterine tumors tentatively diagnosed as epithelioid smooth muscle tumors with uncertain malignant potential. This patient's subsequent pneumothorax necessitated video-assisted thoracoscopic surgery, and examination of her resected lung specimens eventually led to correcting the diagnosis, i.e., to a PEComa harboring tuberous sclerosis complex 1 (TSC1) loss-of-heterozygosity that originated in the uterus and then metastasized to the lungs. The administration of a gonadotropin-releasing hormone analogue later stabilized her clinical course. To the best of our knowledge, the present case is the first in the literature that associates PEComas with a TSC1 abnormality. Additionally, the pulmonary manifestations, including imaging appearance and pneumothorax, somewhat resembled those of lymphangioleiomyomatosis, a representative disease belonging to the PEComa family. Although PEComas are rare, clinicians, radiologists and pathologists should become aware of this disease entity, especially in the combined clinical setting of multiple cystic, cavity-like, nodular lesions on computed tomography of the chest and a past history of the tumor in the female reproductive system. |
format | Online Article Text |
id | pubmed-5496452 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | Elsevier |
record_format | MEDLINE/PubMed |
spelling | pubmed-54964522017-07-13 Pneumothorax caused by cystic and nodular lung metastases from a malignant uterine perivascular epithelioid cell tumor (PEComa) Okamoto, Shouichi Komura, Moegi Terao, Yasuhisa Kurisaki-Arakawa, Aiko Hayashi, Takuo Saito, Tsuyoshi Togo, Shinsaku Shiokawa, Akira Mitani, Keiko Kobayashi, Etsuko Kumasaka, Toshio Takahashi, Kazuhisa Seyama, Kuniaki Respir Med Case Rep Case Report Perivascular epithelioid cell tumors (PEComas) are mesenchymal neoplasms with immunoreactivity for both melanocytic and smooth muscle markers. PEComas occur at multiple sites, and malignant PEComas can undergo metastasis, recurrence and aggressive clinical courses. Although the lung is a common metastatic site of PEComas, they usually appear as multiple nodules but rarely become cystic or cavitary. Here, we describe a female patient whose lungs manifested multiple cystic, cavity-like and nodular metastases 3 years after the resection of uterine tumors tentatively diagnosed as epithelioid smooth muscle tumors with uncertain malignant potential. This patient's subsequent pneumothorax necessitated video-assisted thoracoscopic surgery, and examination of her resected lung specimens eventually led to correcting the diagnosis, i.e., to a PEComa harboring tuberous sclerosis complex 1 (TSC1) loss-of-heterozygosity that originated in the uterus and then metastasized to the lungs. The administration of a gonadotropin-releasing hormone analogue later stabilized her clinical course. To the best of our knowledge, the present case is the first in the literature that associates PEComas with a TSC1 abnormality. Additionally, the pulmonary manifestations, including imaging appearance and pneumothorax, somewhat resembled those of lymphangioleiomyomatosis, a representative disease belonging to the PEComa family. Although PEComas are rare, clinicians, radiologists and pathologists should become aware of this disease entity, especially in the combined clinical setting of multiple cystic, cavity-like, nodular lesions on computed tomography of the chest and a past history of the tumor in the female reproductive system. Elsevier 2017-06-27 /pmc/articles/PMC5496452/ /pubmed/28706850 http://dx.doi.org/10.1016/j.rmcr.2017.06.011 Text en © 2017 The Authors http://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 Okamoto, Shouichi Komura, Moegi Terao, Yasuhisa Kurisaki-Arakawa, Aiko Hayashi, Takuo Saito, Tsuyoshi Togo, Shinsaku Shiokawa, Akira Mitani, Keiko Kobayashi, Etsuko Kumasaka, Toshio Takahashi, Kazuhisa Seyama, Kuniaki Pneumothorax caused by cystic and nodular lung metastases from a malignant uterine perivascular epithelioid cell tumor (PEComa) |
title | Pneumothorax caused by cystic and nodular lung metastases from a malignant uterine perivascular epithelioid cell tumor (PEComa) |
title_full | Pneumothorax caused by cystic and nodular lung metastases from a malignant uterine perivascular epithelioid cell tumor (PEComa) |
title_fullStr | Pneumothorax caused by cystic and nodular lung metastases from a malignant uterine perivascular epithelioid cell tumor (PEComa) |
title_full_unstemmed | Pneumothorax caused by cystic and nodular lung metastases from a malignant uterine perivascular epithelioid cell tumor (PEComa) |
title_short | Pneumothorax caused by cystic and nodular lung metastases from a malignant uterine perivascular epithelioid cell tumor (PEComa) |
title_sort | pneumothorax caused by cystic and nodular lung metastases from a malignant uterine perivascular epithelioid cell tumor (pecoma) |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5496452/ https://www.ncbi.nlm.nih.gov/pubmed/28706850 http://dx.doi.org/10.1016/j.rmcr.2017.06.011 |
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