Epithelioid hemangioendothelioma prognosis and outcome: report of two challenging cases
Abstract Background Epithelioid hemangioendothelioma (EHE) is an uncommon vascular malignancy characterized by an unpredictable clinical course and a high potential for recurrence and metastasis. The lack of standard treatment guidelines, coupled with the tumor’s inconsistent response to available t...
Saved in:
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
BMC
2024-12-01
|
| Series: | Journal of Cardiothoracic Surgery |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s13019-024-03338-x |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1841559169141833728 |
|---|---|
| author | Parviz Mardani Zahra Hashempour Reza Shahriarirad Armin Amirian |
| author_facet | Parviz Mardani Zahra Hashempour Reza Shahriarirad Armin Amirian |
| author_sort | Parviz Mardani |
| collection | DOAJ |
| description | Abstract Background Epithelioid hemangioendothelioma (EHE) is an uncommon vascular malignancy characterized by an unpredictable clinical course and a high potential for recurrence and metastasis. The lack of standard treatment guidelines, coupled with the tumor’s inconsistent response to available treatments, complicates the management of EHE and leads to widely varying patient prognoses. Case presentation We report two cases of EHE with distinct presentations reflecting the site of involvement. The first case involved a patient with pulmonary EHE who initially benefited from complete surgical resection of the tumor after chemotherapy. However, the patient experienced two episodes of metastatic recurrence, resistant to further surgical and chemotherapeutic interventions, ultimately leading to the patient’s death forty-two months after the diagnosis. The second case presented with a mediastinal EHE involving the para-esophageal area. Partial tumor resection was achieved but postoperative imaging revealed extensive metastasis and pleural effusion. Despite additional treatments, the disease progressed rapidly, and the patient passed away fourteen months following the initial diagnosis. Conclusions The optimal therapeutic approach for EHE remains controversial due to its unpredictable treatment response, particularly in recurrent or metastatic cases. Therapeutic investigations are needed to establish evidence-based guidelines that can address treatment challenges and ensure the best possible outcome for each patient. |
| format | Article |
| id | doaj-art-bc57556e834f4a31805e8f50d29caca7 |
| institution | Kabale University |
| issn | 1749-8090 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | BMC |
| record_format | Article |
| series | Journal of Cardiothoracic Surgery |
| spelling | doaj-art-bc57556e834f4a31805e8f50d29caca72025-01-05T12:45:51ZengBMCJournal of Cardiothoracic Surgery1749-80902024-12-011911710.1186/s13019-024-03338-xEpithelioid hemangioendothelioma prognosis and outcome: report of two challenging casesParviz Mardani0Zahra Hashempour1Reza Shahriarirad2Armin Amirian3Thoracic and Vascular Surgery Research Center, Shiraz University of Medical SciencesSchool of Medicine, Shiraz University of Medical SciencesThoracic and Vascular Surgery Research Center, Shiraz University of Medical SciencesThoracic and Vascular Surgery Research Center, Shiraz University of Medical SciencesAbstract Background Epithelioid hemangioendothelioma (EHE) is an uncommon vascular malignancy characterized by an unpredictable clinical course and a high potential for recurrence and metastasis. The lack of standard treatment guidelines, coupled with the tumor’s inconsistent response to available treatments, complicates the management of EHE and leads to widely varying patient prognoses. Case presentation We report two cases of EHE with distinct presentations reflecting the site of involvement. The first case involved a patient with pulmonary EHE who initially benefited from complete surgical resection of the tumor after chemotherapy. However, the patient experienced two episodes of metastatic recurrence, resistant to further surgical and chemotherapeutic interventions, ultimately leading to the patient’s death forty-two months after the diagnosis. The second case presented with a mediastinal EHE involving the para-esophageal area. Partial tumor resection was achieved but postoperative imaging revealed extensive metastasis and pleural effusion. Despite additional treatments, the disease progressed rapidly, and the patient passed away fourteen months following the initial diagnosis. Conclusions The optimal therapeutic approach for EHE remains controversial due to its unpredictable treatment response, particularly in recurrent or metastatic cases. Therapeutic investigations are needed to establish evidence-based guidelines that can address treatment challenges and ensure the best possible outcome for each patient.https://doi.org/10.1186/s13019-024-03338-xCase reportEpithelioid hemangioendotheliomaMetastasesRecurrence |
| spellingShingle | Parviz Mardani Zahra Hashempour Reza Shahriarirad Armin Amirian Epithelioid hemangioendothelioma prognosis and outcome: report of two challenging cases Journal of Cardiothoracic Surgery Case report Epithelioid hemangioendothelioma Metastases Recurrence |
| title | Epithelioid hemangioendothelioma prognosis and outcome: report of two challenging cases |
| title_full | Epithelioid hemangioendothelioma prognosis and outcome: report of two challenging cases |
| title_fullStr | Epithelioid hemangioendothelioma prognosis and outcome: report of two challenging cases |
| title_full_unstemmed | Epithelioid hemangioendothelioma prognosis and outcome: report of two challenging cases |
| title_short | Epithelioid hemangioendothelioma prognosis and outcome: report of two challenging cases |
| title_sort | epithelioid hemangioendothelioma prognosis and outcome report of two challenging cases |
| topic | Case report Epithelioid hemangioendothelioma Metastases Recurrence |
| url | https://doi.org/10.1186/s13019-024-03338-x |
| work_keys_str_mv | AT parvizmardani epithelioidhemangioendotheliomaprognosisandoutcomereportoftwochallengingcases AT zahrahashempour epithelioidhemangioendotheliomaprognosisandoutcomereportoftwochallengingcases AT rezashahriarirad epithelioidhemangioendotheliomaprognosisandoutcomereportoftwochallengingcases AT arminamirian epithelioidhemangioendotheliomaprognosisandoutcomereportoftwochallengingcases |