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: 1324753
- Neoplasms by Histologic Type: 2
  - Vascular Tissue Neoplasms: 141
    - Hemangioma: 5157
      - Hemangioendothelioma: 302
        Kaposiform Hemangioendothelioma: 113
      - Kasabach-Merritt Syndrome: 255
        Kaposiform Hemangioendothelioma: 113
    - Kaposi Sarcoma: 4000
      - Kaposiform Hemangioendothelioma: 113
  - Connective and Soft Tissue Neoplasms: 1
    - Sarcoma: 23140
      - Kaposi Sarcoma: 4000
        Kaposiform Hemangioendothelioma: 113
Infections: 526564
- Virus Diseases: 41777
  - DNA Virus Infections: 139
    - Herpesviridae Infections: 821
      - Kaposi Sarcoma: 4000
        Kaposiform Hemangioendothelioma: 113
Hemic and Lymphatic Diseases: 6
- Hematologic Diseases: 3366
  - Cytopenia: 2582
    - Thrombocytopenia: 26776
      - Kasabach-Merritt Syndrome: 255
        Kaposiform Hemangioendothelioma: 113
  - Blood Platelet Disorders: 126
    - Thrombocytopenia: 26776
      - Kasabach-Merritt Syndrome: 255
        Kaposiform Hemangioendothelioma: 113

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