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Hemangioendothelioma.

Luis Requena1, Heinz Kutzner

  • 1Department of Dermatology, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain. lrequena@fjd.es

Seminars in Diagnostic Pathology
|January 19, 2013
PubMed
Summary
This summary is machine-generated.

Hemangioendothelioma encompasses vascular neoplasms with intermediate behavior between benign hemangiomas and malignant angiosarcomas. This review details various types, including papillary intralymphatic angioendothelioma and epithelioid hemangioendothelioma, highlighting their distinct histopathologic and immunohistochemical features.

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Area of Science:

  • Vascular Neoplasms
  • Pathology
  • Oncology

Background:

  • Hemangioendothelioma represents a spectrum of vascular tumors with behavior intermediate between benign hemangiomas and malignant angiosarcomas.
  • Spindle cell hemangioendothelioma is now recognized as a benign lesion, often termed spindle cell hemangioma.
  • Authentic hemangioendotheliomas include papillary intralymphatic angioendothelioma (Dabska tumor), retiform hemangioendothelioma, kaposiform hemangioendothelioma, epithelioid hemangioendothelioma, pseudomyogenic hemangioendothelioma, and composite hemangioendothelioma.

Purpose of the Study:

  • To provide a comprehensive overview of the distinct clinicopathologic and histopathologic features of various authentic hemangioendothelioma subtypes.
  • To discuss the immunohistochemical profile, emphasizing the lymphatic endothelial cell immunophenotype common to these neoplasms.
  • To outline the general biological behavior, including recurrence rates and metastatic potential, with specific attention to epithelioid hemangioendothelioma.

Main Methods:

  • Histopathologic examination of various hemangioendothelioma subtypes.
  • Review of characteristic cellular and architectural features for each type.
  • Immunohistochemical analysis to determine cell immunophenotype.
  • Correlation of histopathologic findings with clinical behavior and prognosis.

Main Results:

  • Papillary intralymphatic angioendothelioma exhibits papillary tufts with hobnail-like endothelial cells.
  • Retiform hemangioendothelioma presents as elongated, arborizing vessels resembling rete testis.
  • Kaposiform hemangioendothelioma shows solid nodules with glomeruloid vascular patterns and adjacent lymphangiomatosis.
  • Epithelioid hemangioendothelioma is characterized by cords and aggregates of epithelioid cells in a fibromyxoid stroma.
  • Pseudomyogenic hemangioendothelioma displays fascicular growth with rhabdomyoblastic-like cells.
  • Composite hemangioendothelioma demonstrates mixed benign and malignant vascular components.
  • All hemangioendotheliomas express a lymphatic endothelial cell immunophenotype.
  • Most are low-grade with local recurrence and low metastatic potential, though epithelioid type can be more aggressive.

Conclusions:

  • Hemangioendotheliomas are a diverse group of vascular neoplasms with distinct histopathologic features.
  • Immunohistochemistry confirms a shared lymphatic endothelial cell origin.
  • While generally low-grade, specific subtypes like epithelioid hemangioendothelioma warrant closer monitoring due to potentially aggressive behavior.