Beyond the rare: a case of pseudomyogenic hemangioendothelioma treated sequentially with everolimus, denosumab, and pazopanib
ABSTRACT Pseudomyogenic hemangioendothelioma is an ultra-rare vascular sarcoma that most commonly affects young adults, with a male predominance. It is diagnosed using a combination of imaging studies, histopathological examinations, and immunohistochemical staining. Surgical excision is the mainsta...
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Instituto Israelita de Ensino e Pesquisa Albert Einstein
2024-12-01
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| author | Douglas Dias e Silva Camila Bobato Lara Gismondi Murilo Marques Almeida Silva Renee Zon Filipi Fernando Moura Reynaldo Jesus-Garcia Roberto Carmagnani Pestana |
| author_facet | Douglas Dias e Silva Camila Bobato Lara Gismondi Murilo Marques Almeida Silva Renee Zon Filipi Fernando Moura Reynaldo Jesus-Garcia Roberto Carmagnani Pestana |
| author_sort | Douglas Dias e Silva |
| collection | DOAJ |
| description | ABSTRACT Pseudomyogenic hemangioendothelioma is an ultra-rare vascular sarcoma that most commonly affects young adults, with a male predominance. It is diagnosed using a combination of imaging studies, histopathological examinations, and immunohistochemical staining. Surgical excision is the mainstay of treatment for pseudomyogenic hemangioendothelioma, with the goal of achieving a wide local excision and reducing the risk of recurrence. The role of systemic therapies is not well established because of the rarity of pseudomyogenic hemangioendothelioma, uncertainty regarding its response to currently approved medications, and lack of randomized controlled trials. We describe the case of an 18-year-old male patient diagnosed with multifocal pseudomyogenic hemangioendothelioma of the left lower limb who was treated with everolimus in addition to denosumab, achieving a partial response that was consolidated with resection, radiofrequency ablation, and radiotherapy of multiple local lesions, achieving a long-lasting response. Following subsequent disease progression, the patient responded favorably to pazopanib, with no significant toxicities. |
| format | Article |
| id | doaj-art-cf3d18b326394bd7b07be379511eb7dd |
| institution | Kabale University |
| issn | 2317-6385 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | Instituto Israelita de Ensino e Pesquisa Albert Einstein |
| record_format | Article |
| series | Einstein (São Paulo) |
| spelling | doaj-art-cf3d18b326394bd7b07be379511eb7dd2024-12-17T08:02:10ZengInstituto Israelita de Ensino e Pesquisa Albert EinsteinEinstein (São Paulo)2317-63852024-12-012210.31744/einstein_journal/2024rc1107Beyond the rare: a case of pseudomyogenic hemangioendothelioma treated sequentially with everolimus, denosumab, and pazopanibDouglas Dias e Silvahttps://orcid.org/0000-0001-9536-6504Camila Bobato Lara Gismondihttps://orcid.org/0009-0006-5217-6237Murilo Marques Almeida Silvahttps://orcid.org/0000-0003-3748-5649Renee Zon Filipihttps://orcid.org/0000-0002-2589-1183Fernando Mourahttps://orcid.org/0000-0003-1967-1544Reynaldo Jesus-Garciahttps://orcid.org/0000-0001-7895-5729Roberto Carmagnani Pestanahttps://orcid.org/0000-0002-1141-7608ABSTRACT Pseudomyogenic hemangioendothelioma is an ultra-rare vascular sarcoma that most commonly affects young adults, with a male predominance. It is diagnosed using a combination of imaging studies, histopathological examinations, and immunohistochemical staining. Surgical excision is the mainstay of treatment for pseudomyogenic hemangioendothelioma, with the goal of achieving a wide local excision and reducing the risk of recurrence. The role of systemic therapies is not well established because of the rarity of pseudomyogenic hemangioendothelioma, uncertainty regarding its response to currently approved medications, and lack of randomized controlled trials. We describe the case of an 18-year-old male patient diagnosed with multifocal pseudomyogenic hemangioendothelioma of the left lower limb who was treated with everolimus in addition to denosumab, achieving a partial response that was consolidated with resection, radiofrequency ablation, and radiotherapy of multiple local lesions, achieving a long-lasting response. Following subsequent disease progression, the patient responded favorably to pazopanib, with no significant toxicities.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1679-45082024000100516&lng=en&tlng=enHemangioendotheliomaHemangioendothelioma, epithelioidNeoplasms, vascular tissueEverolimusDenosumabPazopanib |
| spellingShingle | Douglas Dias e Silva Camila Bobato Lara Gismondi Murilo Marques Almeida Silva Renee Zon Filipi Fernando Moura Reynaldo Jesus-Garcia Roberto Carmagnani Pestana Beyond the rare: a case of pseudomyogenic hemangioendothelioma treated sequentially with everolimus, denosumab, and pazopanib Einstein (São Paulo) Hemangioendothelioma Hemangioendothelioma, epithelioid Neoplasms, vascular tissue Everolimus Denosumab Pazopanib |
| title | Beyond the rare: a case of pseudomyogenic hemangioendothelioma treated sequentially with everolimus, denosumab, and pazopanib |
| title_full | Beyond the rare: a case of pseudomyogenic hemangioendothelioma treated sequentially with everolimus, denosumab, and pazopanib |
| title_fullStr | Beyond the rare: a case of pseudomyogenic hemangioendothelioma treated sequentially with everolimus, denosumab, and pazopanib |
| title_full_unstemmed | Beyond the rare: a case of pseudomyogenic hemangioendothelioma treated sequentially with everolimus, denosumab, and pazopanib |
| title_short | Beyond the rare: a case of pseudomyogenic hemangioendothelioma treated sequentially with everolimus, denosumab, and pazopanib |
| title_sort | beyond the rare a case of pseudomyogenic hemangioendothelioma treated sequentially with everolimus denosumab and pazopanib |
| topic | Hemangioendothelioma Hemangioendothelioma, epithelioid Neoplasms, vascular tissue Everolimus Denosumab Pazopanib |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1679-45082024000100516&lng=en&tlng=en |
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