Dermatofibrosarcoma protuberans with lung metastasis requiring pneumonectomy
We report the case of a young man diagnosed with dermatofibrosarcoma protuberans lung metastases seven years after primary tumor resection. Notably, no previous local recurrences had been observed. A multimodal approach was used for the management of this patient: surgery, radiotherapy and targeted...
| Main Authors: | , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
SAGE Publishing
2015-12-01
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| Series: | Rare Tumors |
| Subjects: | |
| Online Access: | http://www.pagepress.org/journals/index.php/rt/article/view/5981 |
| _version_ | 1829484083460177920 |
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| author | Ana Collazo Lorduy Berta Obispo Karen Villar David Buchser Jacobo Rogado Maria Dolores Fenor de la Maza Yolanda Lage Alfranca Ramon Colomer |
| author_facet | Ana Collazo Lorduy Berta Obispo Karen Villar David Buchser Jacobo Rogado Maria Dolores Fenor de la Maza Yolanda Lage Alfranca Ramon Colomer |
| author_sort | Ana Collazo Lorduy |
| collection | DOAJ |
| description | We report the case of a young man diagnosed with dermatofibrosarcoma protuberans lung metastases seven years after primary tumor resection. Notably, no previous local recurrences had been observed. A multimodal approach was used for the management of this patient: surgery, radiotherapy and targeted therapy with Imatinib. The patient is alive with stable disease after thirty months of the metastases diagnoses. Dermatofibrosarcoma protuberans metastasizes very rarely, and when it does, it is usually either after local recurrence or whenever fibrosarcomatous transformation is found in the histopathological analysis, which confers an increased risk of local recurrence and metastases. This is the second report of a metastatic dermatofibrosarcoma protuberans occurring in a patient with no previous local recurrence or histological fibrosarcomatous features, emphasizing the rarity of the disease presentation and the importance of targeted therapy in improving patient quality of life and survival. |
| first_indexed | 2024-12-14T22:19:15Z |
| format | Article |
| id | doaj.art-e911675f47d746c9aa640815e78202cc |
| institution | Directory Open Access Journal |
| issn | 2036-3605 2036-3613 |
| language | English |
| last_indexed | 2024-12-14T22:19:15Z |
| publishDate | 2015-12-01 |
| publisher | SAGE Publishing |
| record_format | Article |
| series | Rare Tumors |
| spelling | doaj.art-e911675f47d746c9aa640815e78202cc2022-12-21T22:45:32ZengSAGE PublishingRare Tumors2036-36052036-36132015-12-017410.4081/rt.2015.59813246Dermatofibrosarcoma protuberans with lung metastasis requiring pneumonectomyAna Collazo Lorduy0Berta Obispo1Karen Villar2David Buchser3Jacobo Rogado4Maria Dolores Fenor de la Maza5Yolanda Lage Alfranca6Ramon Colomer7Department of Medical Oncology, Hospital Universitario de la Princesa, Madrid, Spain; Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, NYDepartment of Medical Oncology, Hospital Universitario de la Princesa, MadridDepartment of Pathology, Hospital Universitario de la Princesa, MadridDepartment of Radiation Oncology, Hospital Universitario de la Princesa, MadridDepartment of Medical Oncology, Hospital Universitario de la Princesa, MadridDepartment of Medical Oncology, Hospital Universitario de la Princesa, MadridDepartment of Medical Oncology, Hospital Universitario de la Princesa, MadridDepartment of Medical Oncology, Hospital Universitario de la Princesa, MadridWe report the case of a young man diagnosed with dermatofibrosarcoma protuberans lung metastases seven years after primary tumor resection. Notably, no previous local recurrences had been observed. A multimodal approach was used for the management of this patient: surgery, radiotherapy and targeted therapy with Imatinib. The patient is alive with stable disease after thirty months of the metastases diagnoses. Dermatofibrosarcoma protuberans metastasizes very rarely, and when it does, it is usually either after local recurrence or whenever fibrosarcomatous transformation is found in the histopathological analysis, which confers an increased risk of local recurrence and metastases. This is the second report of a metastatic dermatofibrosarcoma protuberans occurring in a patient with no previous local recurrence or histological fibrosarcomatous features, emphasizing the rarity of the disease presentation and the importance of targeted therapy in improving patient quality of life and survival.http://www.pagepress.org/journals/index.php/rt/article/view/5981Dermatofibrosarcoma protuberanslung metastasesimatinibpneumonectomy |
| spellingShingle | Ana Collazo Lorduy Berta Obispo Karen Villar David Buchser Jacobo Rogado Maria Dolores Fenor de la Maza Yolanda Lage Alfranca Ramon Colomer Dermatofibrosarcoma protuberans with lung metastasis requiring pneumonectomy Rare Tumors Dermatofibrosarcoma protuberans lung metastases imatinib pneumonectomy |
| title | Dermatofibrosarcoma protuberans with lung metastasis requiring pneumonectomy |
| title_full | Dermatofibrosarcoma protuberans with lung metastasis requiring pneumonectomy |
| title_fullStr | Dermatofibrosarcoma protuberans with lung metastasis requiring pneumonectomy |
| title_full_unstemmed | Dermatofibrosarcoma protuberans with lung metastasis requiring pneumonectomy |
| title_short | Dermatofibrosarcoma protuberans with lung metastasis requiring pneumonectomy |
| title_sort | dermatofibrosarcoma protuberans with lung metastasis requiring pneumonectomy |
| topic | Dermatofibrosarcoma protuberans lung metastases imatinib pneumonectomy |
| url | http://www.pagepress.org/journals/index.php/rt/article/view/5981 |
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