Kaposiform Hemangioendothelioma

A rare, locally aggressive vascular neoplasm that primarily occurs in childhood. It originates on the skin and is characterized by one or multiple masse; it is often comorbid with Kasabach-Merritt syndrome and lymphangiomatosis. The viscera is usually not affected, but invasion of bone, retroperitoneum, and MEDIASTINUM has occured in some cases. No metastases have been reported.

Also Known As:

Congenital cutaneous multifocal kaposiform hemangioendothelioma; Kaposiform hemangio-endothelioma

Neoplasms: 1361496
- Neoplasms by Histologic Type: 2
  - Vascular Tissue Neoplasms: 142
    - Hemangioma: 5236
      - Hemangioendothelioma: 309
        Kaposiform Hemangioendothelioma: 116
      - Kasabach-Merritt Syndrome: 262
        Kaposiform Hemangioendothelioma: 116
    - Kaposi Sarcoma: 4032
      - Kaposiform Hemangioendothelioma: 116
  - Connective and Soft Tissue Neoplasms: 1
    - Sarcoma: 23591
      - Kaposi Sarcoma: 4032
        Kaposiform Hemangioendothelioma: 116
Infections: 539155
- Virus Diseases: 42919
  - DNA Virus Infections: 145
    - Herpesviridae Infections: 836
      - Kaposi Sarcoma: 4032
        Kaposiform Hemangioendothelioma: 116
Hemic and Lymphatic Diseases: 7
- Hematologic Diseases: 3445
  - Cytopenia: 2659
    - Thrombocytopenia: 27291
      - Kasabach-Merritt Syndrome: 262
        Kaposiform Hemangioendothelioma: 116
  - Blood Platelet Disorders: 129
    - Thrombocytopenia: 27291
      - Kasabach-Merritt Syndrome: 262
        Kaposiform Hemangioendothelioma: 116

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