Retiform hemangioendothelioma: a case series and review of the literature
Abstract Background Retiform Hemangioendothelioma (RH) is an extremely rare vascular tumor of intermediate biological behavior, which is prone to local recurrence but rarely shows metastasis to distant sites. It may harbor areas resembling Dabska tumor in some cases and angiosarcoma, which in its we...
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| Format: | Article |
| Language: | English |
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BMC
2021-02-01
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| Series: | Journal of Medical Case Reports |
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| Online Access: | https://doi.org/10.1186/s13256-021-02671-2 |
| _version_ | 1819283660170330112 |
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| author | Qurratulain Chundriger Muhammad Usman Tariq Shabina Rahim Jamshid Abdul-Ghafar Nasir Ud Din |
| author_facet | Qurratulain Chundriger Muhammad Usman Tariq Shabina Rahim Jamshid Abdul-Ghafar Nasir Ud Din |
| author_sort | Qurratulain Chundriger |
| collection | DOAJ |
| description | Abstract Background Retiform Hemangioendothelioma (RH) is an extremely rare vascular tumor of intermediate biological behavior, which is prone to local recurrence but rarely shows metastasis to distant sites. It may harbor areas resembling Dabska tumor in some cases and angiosarcoma, which in its well differentiated form may exhibit similar pathological appearance in some areas, making it problematic to rule out a possibility of a malignant diagnosis on a core biopsy. Therefore, complete surgical resection with negative margins is essential for accurate diagnosis and local control. Results In our series, two of the three Pakistani cases were in females, with an age range between 18 and 50 years. Our first patient presented with symptoms of cardiac compromise and pulmonary hypertension. Her computed tomography scan showed multiple tumor masses within the mediastinum. The second patient presented with an ulcerated lesion on his scalp, at right temple. The third patient presented with a hard growth on her left 4th toe which was amputated. Histologically, all cases exhibited retiform arborizing vascular spaces lined by bland endothelial cells with hobnail nuclei, characteristic of retiform hemangioendothelioma. Immunohistochemical markers CD31, CD34 and ERG confirmed the vascular nature of the tumor. The first and the second patient are alive and healthy at 4 and 7 months follow up respectively, while the third patient is lost to follow up. Conclusion Owing to the rate of local recurrence, RH should always be considered in the differential diagnosis of vascular tumors showing arborizing blood vessels, as it may have an atypical presentation and it should be carefully differentiated from Dabska tumor and an angiosarcoma. |
| first_indexed | 2024-12-24T01:35:00Z |
| format | Article |
| id | doaj.art-2751369756c94b5c93b95dc9c8f9b51f |
| institution | Directory Open Access Journal |
| issn | 1752-1947 |
| language | English |
| last_indexed | 2024-12-24T01:35:00Z |
| publishDate | 2021-02-01 |
| publisher | BMC |
| record_format | Article |
| series | Journal of Medical Case Reports |
| spelling | doaj.art-2751369756c94b5c93b95dc9c8f9b51f2022-12-21T17:22:13ZengBMCJournal of Medical Case Reports1752-19472021-02-011511710.1186/s13256-021-02671-2Retiform hemangioendothelioma: a case series and review of the literatureQurratulain Chundriger0Muhammad Usman Tariq1Shabina Rahim2Jamshid Abdul-Ghafar3Nasir Ud Din4Department of Pathology and Laboratory Medicine, Aga Khan University HospitalDepartment of Pathology and Laboratory Medicine, Aga Khan University HospitalDepartment of Pathology and Laboratory Medicine, Aga Khan University HospitalDepartment of Pathology and Clinical Laboratory, French Medical Institute for Mothers and Children (FMIC)Department of Pathology and Laboratory Medicine, Aga Khan University HospitalAbstract Background Retiform Hemangioendothelioma (RH) is an extremely rare vascular tumor of intermediate biological behavior, which is prone to local recurrence but rarely shows metastasis to distant sites. It may harbor areas resembling Dabska tumor in some cases and angiosarcoma, which in its well differentiated form may exhibit similar pathological appearance in some areas, making it problematic to rule out a possibility of a malignant diagnosis on a core biopsy. Therefore, complete surgical resection with negative margins is essential for accurate diagnosis and local control. Results In our series, two of the three Pakistani cases were in females, with an age range between 18 and 50 years. Our first patient presented with symptoms of cardiac compromise and pulmonary hypertension. Her computed tomography scan showed multiple tumor masses within the mediastinum. The second patient presented with an ulcerated lesion on his scalp, at right temple. The third patient presented with a hard growth on her left 4th toe which was amputated. Histologically, all cases exhibited retiform arborizing vascular spaces lined by bland endothelial cells with hobnail nuclei, characteristic of retiform hemangioendothelioma. Immunohistochemical markers CD31, CD34 and ERG confirmed the vascular nature of the tumor. The first and the second patient are alive and healthy at 4 and 7 months follow up respectively, while the third patient is lost to follow up. Conclusion Owing to the rate of local recurrence, RH should always be considered in the differential diagnosis of vascular tumors showing arborizing blood vessels, as it may have an atypical presentation and it should be carefully differentiated from Dabska tumor and an angiosarcoma.https://doi.org/10.1186/s13256-021-02671-2RetiformHemangioendotheliomaDabska tumorAngiosarcomaHobnail |
| spellingShingle | Qurratulain Chundriger Muhammad Usman Tariq Shabina Rahim Jamshid Abdul-Ghafar Nasir Ud Din Retiform hemangioendothelioma: a case series and review of the literature Journal of Medical Case Reports Retiform Hemangioendothelioma Dabska tumor Angiosarcoma Hobnail |
| title | Retiform hemangioendothelioma: a case series and review of the literature |
| title_full | Retiform hemangioendothelioma: a case series and review of the literature |
| title_fullStr | Retiform hemangioendothelioma: a case series and review of the literature |
| title_full_unstemmed | Retiform hemangioendothelioma: a case series and review of the literature |
| title_short | Retiform hemangioendothelioma: a case series and review of the literature |
| title_sort | retiform hemangioendothelioma a case series and review of the literature |
| topic | Retiform Hemangioendothelioma Dabska tumor Angiosarcoma Hobnail |
| url | https://doi.org/10.1186/s13256-021-02671-2 |
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