Successful management of Kaposiform Hemangioendothelioma with long-term sirolimus treatment: a case report and review of the literature
Background: Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumour of the infancy and of the first decade of life. It is locally aggressive and potentially life threatening when associated to consumptive coagulopathy, known as Kasabach-Merritt syndrome (KMS). No consensus or guideline for t...
Saved in:
| Main Authors: | , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
PAGEPress Publications
2018-07-01
|
| Series: | Mediterranean Journal of Hematology and Infectious Diseases |
| Subjects: | |
| Online Access: | https://www.mjhid.org/index.php/mjhid/article/view/3450 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1846096429920026624 |
|---|---|
| author | Matteo Chinello Daniela Di Carlo Francesca Olivieri Rita Balter Massimiliano De Bortoli Virginia Vitale Ada Zaccaron Elisa Bonetti Alice Parisi Simone Cesaro |
| author_facet | Matteo Chinello Daniela Di Carlo Francesca Olivieri Rita Balter Massimiliano De Bortoli Virginia Vitale Ada Zaccaron Elisa Bonetti Alice Parisi Simone Cesaro |
| author_sort | Matteo Chinello |
| collection | DOAJ |
| description | Background: Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumour of the infancy and of the first decade of life. It is locally aggressive and potentially life threatening when associated to consumptive coagulopathy, known as Kasabach-Merritt syndrome (KMS). No consensus or guideline for the therapy has been reached because of the lack of prospective trials and the different standard care suggestions are based on retrospective case series.
Case report: We report the case of a 9-month-old male with KHE and KMS in which the initial response, obtained with prednisone and vincristine, was subsequently consolidated and strengthened by long-term treatment with sirolimus, an mTOR inhibitor. A summary of the published data is presented as well.
Conclusions: The inhibition of mTOR pathway represents the most important therapeutic innovation introduced in the last few years for KHE. Our case shows the effectiveness and good tolerance of long-term therapy with sirolimus.
Keywords: Kaposiform Hemangioendothelioma, Kasabach-Merrit syndrome, sirolimus, prednisone, vincristine |
| format | Article |
| id | doaj-art-cc13d3b545d44e8d8ca250df330e3307 |
| institution | Kabale University |
| issn | 2035-3006 |
| language | English |
| publishDate | 2018-07-01 |
| publisher | PAGEPress Publications |
| record_format | Article |
| series | Mediterranean Journal of Hematology and Infectious Diseases |
| spelling | doaj-art-cc13d3b545d44e8d8ca250df330e33072025-01-02T06:46:49ZengPAGEPress PublicationsMediterranean Journal of Hematology and Infectious Diseases2035-30062018-07-01101e2018043e201804310.4084/mjhid.2018.0431766Successful management of Kaposiform Hemangioendothelioma with long-term sirolimus treatment: a case report and review of the literatureMatteo Chinello0Daniela Di Carlo1Francesca Olivieri2Rita Balter3Massimiliano De Bortoli4Virginia Vitale5Ada Zaccaron6Elisa Bonetti7Alice Parisi8Simone Cesaro9Pediatric Hematology Oncology, Azienda Ospedaliera Universitaria Integrata, Verona, ItalyMother and Child Department, University of VeronaMother and Child Department, University of VeronaPediatric Hematology Oncology, Azienda Ospedaliera Universitaria Integrata, Verona, ItalyPediatric Hematology Oncology, Azienda Ospedaliera Universitaria Integrata, Verona, ItalyPediatric Hematology Oncology, Azienda Ospedaliera Universitaria Integrata, Verona, ItalyPediatric Hematology Oncology, Azienda Ospedaliera Universitaria Integrata, Verona, ItalyPediatric Hematology Oncology, Azienda Ospedaliera Universitaria Integrata, Verona, ItalyDepartment of Pathological Anatomy, Azienda Ospedaliera Universitaria Integrata, Verona, Italy.Pediatric Hematology Oncology, Azienda Ospedaliera Universitaria Integrata, Verona, ItalyBackground: Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumour of the infancy and of the first decade of life. It is locally aggressive and potentially life threatening when associated to consumptive coagulopathy, known as Kasabach-Merritt syndrome (KMS). No consensus or guideline for the therapy has been reached because of the lack of prospective trials and the different standard care suggestions are based on retrospective case series. Case report: We report the case of a 9-month-old male with KHE and KMS in which the initial response, obtained with prednisone and vincristine, was subsequently consolidated and strengthened by long-term treatment with sirolimus, an mTOR inhibitor. A summary of the published data is presented as well. Conclusions: The inhibition of mTOR pathway represents the most important therapeutic innovation introduced in the last few years for KHE. Our case shows the effectiveness and good tolerance of long-term therapy with sirolimus. Keywords: Kaposiform Hemangioendothelioma, Kasabach-Merrit syndrome, sirolimus, prednisone, vincristinehttps://www.mjhid.org/index.php/mjhid/article/view/3450Kaposiform Hemangioendothelioma, Kasabach-Merrit syndrome, sirolimus, prednisone, vincristine |
| spellingShingle | Matteo Chinello Daniela Di Carlo Francesca Olivieri Rita Balter Massimiliano De Bortoli Virginia Vitale Ada Zaccaron Elisa Bonetti Alice Parisi Simone Cesaro Successful management of Kaposiform Hemangioendothelioma with long-term sirolimus treatment: a case report and review of the literature Mediterranean Journal of Hematology and Infectious Diseases Kaposiform Hemangioendothelioma, Kasabach-Merrit syndrome, sirolimus, prednisone, vincristine |
| title | Successful management of Kaposiform Hemangioendothelioma with long-term sirolimus treatment: a case report and review of the literature |
| title_full | Successful management of Kaposiform Hemangioendothelioma with long-term sirolimus treatment: a case report and review of the literature |
| title_fullStr | Successful management of Kaposiform Hemangioendothelioma with long-term sirolimus treatment: a case report and review of the literature |
| title_full_unstemmed | Successful management of Kaposiform Hemangioendothelioma with long-term sirolimus treatment: a case report and review of the literature |
| title_short | Successful management of Kaposiform Hemangioendothelioma with long-term sirolimus treatment: a case report and review of the literature |
| title_sort | successful management of kaposiform hemangioendothelioma with long term sirolimus treatment a case report and review of the literature |
| topic | Kaposiform Hemangioendothelioma, Kasabach-Merrit syndrome, sirolimus, prednisone, vincristine |
| url | https://www.mjhid.org/index.php/mjhid/article/view/3450 |
| work_keys_str_mv | AT matteochinello successfulmanagementofkaposiformhemangioendotheliomawithlongtermsirolimustreatmentacasereportandreviewoftheliterature AT danieladicarlo successfulmanagementofkaposiformhemangioendotheliomawithlongtermsirolimustreatmentacasereportandreviewoftheliterature AT francescaolivieri successfulmanagementofkaposiformhemangioendotheliomawithlongtermsirolimustreatmentacasereportandreviewoftheliterature AT ritabalter successfulmanagementofkaposiformhemangioendotheliomawithlongtermsirolimustreatmentacasereportandreviewoftheliterature AT massimilianodebortoli successfulmanagementofkaposiformhemangioendotheliomawithlongtermsirolimustreatmentacasereportandreviewoftheliterature AT virginiavitale successfulmanagementofkaposiformhemangioendotheliomawithlongtermsirolimustreatmentacasereportandreviewoftheliterature AT adazaccaron successfulmanagementofkaposiformhemangioendotheliomawithlongtermsirolimustreatmentacasereportandreviewoftheliterature AT elisabonetti successfulmanagementofkaposiformhemangioendotheliomawithlongtermsirolimustreatmentacasereportandreviewoftheliterature AT aliceparisi successfulmanagementofkaposiformhemangioendotheliomawithlongtermsirolimustreatmentacasereportandreviewoftheliterature AT simonecesaro successfulmanagementofkaposiformhemangioendotheliomawithlongtermsirolimustreatmentacasereportandreviewoftheliterature |