Composite Hemangioendothelioma of the Submandibular Region
Identifieur interne : 000A16 ( Pmc/Curation ); précédent : 000A15; suivant : 000A17Composite Hemangioendothelioma of the Submandibular Region
Auteurs : Sarah Lam Shang Leen ; Peter M. Clarke [Royaume-Uni] ; John Chapman [Royaume-Uni] ; Cyril Fisher [Royaume-Uni] ; Khin Thway [Royaume-Uni]Source :
- Head and Neck Pathology [ 1936-055X ] ; 2015.
Abstract
Composite hemangioendothelioma (HE) is a rare vascular neoplasm of intermediate malignant potential that predominantly occurs within the dermis or subcutis of the extremities, and occurs in a wide age range. It is locally aggressive with a high rate of local recurrence, and more rarely regional lymph node or distant metastasis. Histologically, it is composed of a complex admixture of benign, intermediate and malignant vascular components. Although composite HE may contain angiosarcoma-like areas, its prognosis is better than that of pure angiosarcoma. We describe a case of composite HE presenting as a submandibular mass in a 43 year-old male, which included areas of prominent ‘high grade’ epithelioid angiosarcoma. This adds to the range of anatomic sites of these neoplasms, highlights the importance of recognition of the head and neck as a potential site, and emphasizes the importance of accurate diagnosis for correct management (including of long term follow up) and prognostication.
Url:
DOI: 10.1007/s12105-015-0612-6
PubMed: 25666464
PubMed Central: 4651927
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Sarah Lam Shang Leen<affiliation><nlm:aff id="Aff1">Sarcoma Unit, The Royal Marsden NHS Foundation Trust, 203 Fulham Road, London, SW3 6JJ UK</nlm:aff>
<wicri:noCountry code="subfield">SW3 6JJ UK</wicri:noCountry>
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<affiliation><nlm:aff id="Aff1">Sarcoma Unit, The Royal Marsden NHS Foundation Trust, 203 Fulham Road, London, SW3 6JJ UK</nlm:aff>
<wicri:noCountry code="subfield">SW3 6JJ UK</wicri:noCountry>
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<front><div type="abstract" xml:lang="en"><p>Composite hemangioendothelioma (HE) is a rare vascular neoplasm of intermediate malignant potential that predominantly occurs within the dermis or subcutis of the extremities, and occurs in a wide age range.
It is locally aggressive with a high rate of local recurrence, and more rarely regional lymph node or distant metastasis. Histologically, it is composed of a complex admixture of benign, intermediate and malignant vascular components. Although composite HE may contain angiosarcoma-like areas, its prognosis is better than that of pure angiosarcoma. We describe a case of composite HE presenting as a submandibular mass in a 43 year-old male, which included areas of prominent ‘high grade’ epithelioid angiosarcoma. This adds to the range of anatomic sites of these neoplasms, highlights the importance of recognition of the head and neck as a potential site, and emphasizes the importance of accurate diagnosis for correct management (including of long term follow up) and prognostication.</p>
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Sarcoma Unit, The Royal Marsden NHS Foundation Trust, 203 Fulham Road, London, SW3 6JJ UK</aff>
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Head and Neck Unit, The Royal Marsden NHS Foundation Trust, London, UK</aff>
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Department of Histopathology, Ipswich Hospital, Ipswich, UK</aff>
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<abstract id="Abs1"><p>Composite hemangioendothelioma (HE) is a rare vascular neoplasm of intermediate malignant potential that predominantly occurs within the dermis or subcutis of the extremities, and occurs in a wide age range.
It is locally aggressive with a high rate of local recurrence, and more rarely regional lymph node or distant metastasis. Histologically, it is composed of a complex admixture of benign, intermediate and malignant vascular components. Although composite HE may contain angiosarcoma-like areas, its prognosis is better than that of pure angiosarcoma. We describe a case of composite HE presenting as a submandibular mass in a 43 year-old male, which included areas of prominent ‘high grade’ epithelioid angiosarcoma. This adds to the range of anatomic sites of these neoplasms, highlights the importance of recognition of the head and neck as a potential site, and emphasizes the importance of accurate diagnosis for correct management (including of long term follow up) and prognostication.</p>
</abstract>
<kwd-group xml:lang="en"><title>Keywords</title>
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<kwd>Soft tissue tumor</kwd>
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