Patient with composite haemangioendothelioma containing angiosarcoma-like areas in the setting of congenital lymphoedema mimicking Stewart-Treves syndrome: a case report
Abstract Background Composite haemangioendothelioma is a rare vascular neoplasm with indolent to intermediate malignant potential. Diagnosis of this disease relays on histopathological identification of at least two different morphologically distinctive vascular components in proper clinical setting...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2023-06-01
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| Series: | Diagnostic Pathology |
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| Online Access: | https://doi.org/10.1186/s13000-023-01365-1 |
| _version_ | 1797795884320161792 |
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| author | Jan Balko Andrej Ozaniak Lenka Krskova Zuzana Strizova Robert Lischke Josef Zamecnik |
| author_facet | Jan Balko Andrej Ozaniak Lenka Krskova Zuzana Strizova Robert Lischke Josef Zamecnik |
| author_sort | Jan Balko |
| collection | DOAJ |
| description | Abstract Background Composite haemangioendothelioma is a rare vascular neoplasm with indolent to intermediate malignant potential. Diagnosis of this disease relays on histopathological identification of at least two different morphologically distinctive vascular components in proper clinical settings. Exceedingly rare cases of this neoplasm can exhibit areas resembling high-grade angiosarcoma, which does not change the biological behaviour. Such lesions tend to occur in the setting of chronic lymphoedema and thus, can mimic Stewart-Treves syndrome, which has a much worse clinical outcome and prognosis. Case presentation We present a case of 49 years old male suffering from chronic lymphoedema of the left lower extremity who had developed a composite haemangioendothelioma with high grade angiosarcoma-like areas mimicking the Stewart-Treves syndrome. Given the multifocality of the disease, the only potentially curable surgical treatment would be hemipelvectomy, which was refused by the patient. The patient has been followed-up, with no signs of local progression of the remaining disease, nor a distant spread outside the involved extremity for two years. Conclusions Composite haemangioendothelioma represents a rare malignant vascular tumour, with significantly more favourable biological behaviour than angiosarcoma, even in cases where angiosarcoma-like areas are present. For that reason, composite haemangioendothelioma can be easily misdiagnosed as true angiosarcoma. The rarity of this disease unfortunately hampers the development of clinical practice guidelines and the implementation of treatment recommendations. Most of the patients with localized tumour are treated by wide surgical resection, without neo- or adjuvant radiotherapy or chemotherapy. However, in the case of this diagnosis, the watch-and-wait approach is better than mutilating procedure, highlighting the necessity of establishing of the correct diagnosis. |
| first_indexed | 2024-03-13T03:24:46Z |
| format | Article |
| id | doaj.art-e899e15e0f564d289a11290dc33a7b3c |
| institution | Directory Open Access Journal |
| issn | 1746-1596 |
| language | English |
| last_indexed | 2024-03-13T03:24:46Z |
| publishDate | 2023-06-01 |
| publisher | BMC |
| record_format | Article |
| series | Diagnostic Pathology |
| spelling | doaj.art-e899e15e0f564d289a11290dc33a7b3c2023-06-25T11:07:33ZengBMCDiagnostic Pathology1746-15962023-06-011811910.1186/s13000-023-01365-1Patient with composite haemangioendothelioma containing angiosarcoma-like areas in the setting of congenital lymphoedema mimicking Stewart-Treves syndrome: a case reportJan Balko0Andrej Ozaniak1Lenka Krskova2Zuzana Strizova3Robert Lischke4Josef Zamecnik5Department of Pathology and Molecular Medicine, 2nd Faculty of Medicine, Charles University and Motol University Hospital3rd Department of Surgery, 1st Faculty of Medicine, Charles University and Motol University HospitalDepartment of Pathology and Molecular Medicine, 2nd Faculty of Medicine, Charles University and Motol University HospitalDepartment of Immunology, 2nd Faculty of Medicine, Charles University and Motol University Hospital3rd Department of Surgery, 1st Faculty of Medicine, Charles University and Motol University HospitalDepartment of Pathology and Molecular Medicine, 2nd Faculty of Medicine, Charles University and Motol University HospitalAbstract Background Composite haemangioendothelioma is a rare vascular neoplasm with indolent to intermediate malignant potential. Diagnosis of this disease relays on histopathological identification of at least two different morphologically distinctive vascular components in proper clinical settings. Exceedingly rare cases of this neoplasm can exhibit areas resembling high-grade angiosarcoma, which does not change the biological behaviour. Such lesions tend to occur in the setting of chronic lymphoedema and thus, can mimic Stewart-Treves syndrome, which has a much worse clinical outcome and prognosis. Case presentation We present a case of 49 years old male suffering from chronic lymphoedema of the left lower extremity who had developed a composite haemangioendothelioma with high grade angiosarcoma-like areas mimicking the Stewart-Treves syndrome. Given the multifocality of the disease, the only potentially curable surgical treatment would be hemipelvectomy, which was refused by the patient. The patient has been followed-up, with no signs of local progression of the remaining disease, nor a distant spread outside the involved extremity for two years. Conclusions Composite haemangioendothelioma represents a rare malignant vascular tumour, with significantly more favourable biological behaviour than angiosarcoma, even in cases where angiosarcoma-like areas are present. For that reason, composite haemangioendothelioma can be easily misdiagnosed as true angiosarcoma. The rarity of this disease unfortunately hampers the development of clinical practice guidelines and the implementation of treatment recommendations. Most of the patients with localized tumour are treated by wide surgical resection, without neo- or adjuvant radiotherapy or chemotherapy. However, in the case of this diagnosis, the watch-and-wait approach is better than mutilating procedure, highlighting the necessity of establishing of the correct diagnosis.https://doi.org/10.1186/s13000-023-01365-1Composite haemangioendotheliomaAngiosarcoma-like areasStewart-Treves syndromeCongenital lymphoedemaAngiosarcomaVascular neoplasm |
| spellingShingle | Jan Balko Andrej Ozaniak Lenka Krskova Zuzana Strizova Robert Lischke Josef Zamecnik Patient with composite haemangioendothelioma containing angiosarcoma-like areas in the setting of congenital lymphoedema mimicking Stewart-Treves syndrome: a case report Diagnostic Pathology Composite haemangioendothelioma Angiosarcoma-like areas Stewart-Treves syndrome Congenital lymphoedema Angiosarcoma Vascular neoplasm |
| title | Patient with composite haemangioendothelioma containing angiosarcoma-like areas in the setting of congenital lymphoedema mimicking Stewart-Treves syndrome: a case report |
| title_full | Patient with composite haemangioendothelioma containing angiosarcoma-like areas in the setting of congenital lymphoedema mimicking Stewart-Treves syndrome: a case report |
| title_fullStr | Patient with composite haemangioendothelioma containing angiosarcoma-like areas in the setting of congenital lymphoedema mimicking Stewart-Treves syndrome: a case report |
| title_full_unstemmed | Patient with composite haemangioendothelioma containing angiosarcoma-like areas in the setting of congenital lymphoedema mimicking Stewart-Treves syndrome: a case report |
| title_short | Patient with composite haemangioendothelioma containing angiosarcoma-like areas in the setting of congenital lymphoedema mimicking Stewart-Treves syndrome: a case report |
| title_sort | patient with composite haemangioendothelioma containing angiosarcoma like areas in the setting of congenital lymphoedema mimicking stewart treves syndrome a case report |
| topic | Composite haemangioendothelioma Angiosarcoma-like areas Stewart-Treves syndrome Congenital lymphoedema Angiosarcoma Vascular neoplasm |
| url | https://doi.org/10.1186/s13000-023-01365-1 |
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