Composite hemangioendothelioma
Updates to Article Attributes
Composite haemangioendotheliomas are locally aggressive and rarely metastasising vascular tumours consisting of different and histologically distinct elements.
Epidemiology
Composite haemangioendotheliomas are very rare and mostly seen in adults. Women are slightly more commonly affected 1,2.
Clinical presentation
The typical complaint is a lump with a reddish to purple appearance which has been there for a long time 1.
Associations
Composite haemangioendotheliomas are seen in patients with lymphoedema 1.
Pathology
Composite haemangioendotheliomas are formed by at least two histologically different vascular tumour components. These parts often resemble retiform or epithelioid haemangioendotheliomas 1.
Aetiology
Chronic lymphoedema and irradiation are thought to have a role in the aetiology 1.
Subtypes
Neuroendocrine composite haemangioendothelioma is the only subtype.
Location
Composite haemangioendotheliomas most frequently affect the skin and subcutaneous tissues of the distal extremities or the head and neck area but have been also found in other sites 1,2.
Macroscopic appearance
Macroscopically composite haemangioendotheliomas are nodular infiltrative ill-defined lesions 1,2.
Microscopic appearance
The microscopic spectrum of composite haemangioendotheliomas consists of a composition of different morphological vascular components and areas including the following 1,2:
- epithelioid haemangioendothelioma
- retiform haemangioendothelioma
- spindle-cell haemangioma
- angiosarcoma-like
- lymphangioma
- angiomatosis
- cavernous haemangioma
Neuroendocrine composite haemangioendotheliomas are typically composed of epithelioid and retiform haemangioendothelioma-like parts with a nest-like appearance 1.
ImmunohistochemistryImmunophenotype
Immunohistochemistry stains usually express CD31, ERG and FLI1 and can be positive CD34 and D2-40 1.
Genetics
Fusions of the PTBP1-MAML2 and EPC1-PCH2 genes have been observed in neuroendocrine composite haemangioendotheliomas 1.
Radiographic features
MRI
Composite haemangioendotheliomas are vascular lesions with a lobulated and heterogeneous appearance 2-4.
Signal characteristics
- T1: low to intermediate signal intensity
- T2: high signal intensity
- T2FS/STIR: high signal
- T1 C+ (Gd): avid enhancement
Radiology report
The radiological report should include a description of the following features:
- form, location and size
- tumour margins
- relation to the muscular fascia
- relationship to bones, tendons and joints
- relationship to local nerves and vessels
Treatment and prognosis
Due to the rarity of the tumours, there is no clear management strategy as yet. Treatment usually consists of surgical excision. Local recurrence is common and can happen multiple times. Lymph node metastases can occur. Distant metastases are seen in neuroendocrine composite haemangioendotheliomas, which are more aggressive 1.
History and etymology
Composite haemangioendotheliomas were first described in 2000 by SJ Nayler 5.
Differential diagnosis
The differential diagnosis of composite haemangioendotheliomas consists of the following:
- retiform haemangioendothelioma
- epithelioid haemangioendothelioma
- Dabska tumour
- Kaposiform haemangioendothelioma
- Kaposi sarcoma
- angiosarcoma
-</ul><p>Neuroendocrine composite haemangioendotheliomas are typically composed of epithelioid and retiform haemangioendothelioma-like parts with a nest-like appearance <sup>1</sup>.</p><h5>Immunohistochemistry</h5><p>Immunohistochemistry stains usually express CD31, ERG and FLI1 and can be positive CD34 and D2-40 <sup>1</sup>.</p><h5>Genetics</h5><p>Fusions of the <em>PTBP1-MAML2</em> and <em>EPC1-PCH2</em> genes have been observed in neuroendocrine composite haemangioendotheliomas <sup>1</sup>.</p><h4>Radiographic features</h4><h5>MRI</h5><p>Composite haemangioendotheliomas are vascular lesions with a lobulated and heterogeneous appearance <sup>2-4</sup>. </p><h6>Signal characteristics</h6><ul>- +</ul><p>Neuroendocrine composite haemangioendotheliomas are typically composed of epithelioid and retiform haemangioendothelioma-like parts with a nest-like appearance <sup>1</sup>.</p><h5>Immunophenotype</h5><p>Immunohistochemistry stains usually express CD31, ERG and FLI1 and can be positive <a title="CD34" href="/articles/cd34">CD34</a> and D2-40 <sup>1</sup>.</p><h5>Genetics</h5><p>Fusions of the <em>PTBP1-MAML2</em> and <em>EPC1-PCH2</em> genes have been observed in neuroendocrine composite haemangioendotheliomas <sup>1</sup>.</p><h4>Radiographic features</h4><h5>MRI</h5><p>Composite haemangioendotheliomas are vascular lesions with a lobulated and heterogeneous appearance <sup>2-4</sup>. </p><h6>Signal characteristics</h6><ul>
References changed:
- 1. Rubin BP, Folpe AL. Composite haemangioendothelioma. In: WHO Classification of Tumours Editorial Board. Soft tissue and bone tumours. Lyon (France): International Agency for Research on Cancer; 2020. (WHO classification of tumours series, 5th ed.; vol. 3). <a href="https://publications.iarc.fr/Book-And-Report-Series/Who-Classification-Of-Tumours/Soft-Tissue-And-Bone-Tumours-2020">https://publications.iarc.fr</a>
- 2. Chin S, Kim J, Jung M et al. Intramuscular Composite Hemangioendothelioma: Case Report of an Unusual Tumor in an Unusual Location. Int J Clin Exp Pathol. 2020;13(6):1421-5. <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7344001">PMC7344001</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/32661479">Pubmed</a>
- 3. Wildgruber M, Sadick M, Müller-Wille R, Wohlgemuth W. Vascular Tumors in Infants and Adolescents. Insights Imaging. 2019;10(1):30. <a href="https://doi.org/10.1186/s13244-019-0718-6">doi:10.1186/s13244-019-0718-6</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/30868300">Pubmed</a>
- 4. Gok S, Berkman M, Baykara E. Composite Hemangioendothelioma Settled in the Paraspinal Region: A Rare Case Report. Turk Neurosurg. 2018;30(2):299-302. <a href="https://doi.org/10.5137/1019-5149.jtn.22256-17.4">doi:10.5137/1019-5149.jtn.22256-17.4</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/29757449">Pubmed</a>
- 5. Nayler S, Rubin B, Calonje E, Chan J, Fletcher C. Composite Hemangioendothelioma. Am J Surg Pathol. 2000;24(3):352-61. <a href="https://doi.org/10.1097/00000478-200003000-00003">doi:10.1097/00000478-200003000-00003</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/10716148">Pubmed</a>
- 1. W.H.O. Classification WHO Classification of Tumours Editorial Board, Who Classification of Tumours Editorial. Soft Tissue and Bone Tumours. (2020) <a href="https://books.google.co.uk/books?vid=ISBN9789283245025">ISBN: 9789283245025</a><span class=“ref_v4”></span>
- 2. Chin S, Kim J, Jung MJ, Kim MJ, Moon A, Kim HK, Kim YH, Koh ES. Intramuscular composite hemangioendothelioma: case report of an unusual tumor in an unusual location. (2020) International journal of clinical and experimental pathology. 13 (6): 1421-1425. <a href="https://www.ncbi.nlm.nih.gov/pubmed/32661479">Pubmed</a> <span class=“ref_v4”></span>
- 3. Wildgruber M, Sadick M, Müller-Wille R, Wohlgemuth WA. Vascular tumors in infants and adolescents. (2019) Insights into imaging. 10 (1): 30. <a href="https://doi.org/10.1186/s13244-019-0718-6">doi:10.1186/s13244-019-0718-6</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/30868300">Pubmed</a> <span class=“ref_v4"></span>
- 4. Gok S, Berkman MZ, Baykara E. Composite Hemangioendothelioma Settled in the Paraspinal Region: A Rare Case Report. (2020) Turkish neurosurgery. 30 (2): 299-302. <a href="https://doi.org/10.5137/1019-5149.JTN.22256-17.4">doi:10.5137/1019-5149.JTN.22256-17.4</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/29757449">Pubmed</a> <span class="ref_v4"></span>
- 5. Nayler SJ, Rubin BP, Calonje E, Chan JK, Fletcher CD. Composite hemangioendothelioma: a complex, low-grade vascular lesion mimicking angiosarcoma. (2000) The American journal of surgical pathology. 24 (3): 352-61. <a href="https://doi.org/10.1097/00000478-200003000-00003">doi:10.1097/00000478-200003000-00003</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/10716148">Pubmed</a> <span class="ref_v4"></span>