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Multifocal kaposiform haemangioendothelioma.

Karen Deraedt1, Vincent Vander Poorten, Chris Van Geet

  • 1Department of Pathology, University Hospital St. Rafaël, Katholieke Universiteit Leuven, Minderbroedersstraat 12, 3000 Leuven, Belgium.

Virchows Archiv : an International Journal of Pathology
|April 6, 2006
PubMed
Summary

Kaposiform haemangioendothelioma (KHE), a rare vascular tumor, presented multifocally in a child, including a novel thyroid involvement. This case highlights KHE

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Area of Science:

  • Vascular Tumors
  • Pediatric Oncology
  • Pathology

Background:

  • Kaposiform haemangioendothelioma (KHE) is a rare vascular tumor typically affecting infants and children.
  • KHE often presents in the skin or retroperitoneum and can be associated with Kasabach-Merritt phenomenon (KMP).

Observation:

  • A 3-year-old boy exhibited multifocal KHE with submandibular lymphadenopathy, a lip lesion, and a previously undescribed thyroid lobe involvement.
  • Clinical signs suggestive of KMP were present.
  • Histopathological analysis confirmed KHE with characteristic spindle cell proliferation and vascular spaces.

Findings:

  • Immunohistochemistry revealed CD34, CD31, and alpha-smooth muscle actin positivity.
  • Herpes virus type 8 expression was notably absent.

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  • The case represents the first documented instance of KHE involving the thyroid gland.

    • Implications:

    • This report expands the known clinical spectrum of KHE, particularly its potential for multifocal presentation.
    • The thyroid involvement in KHE is a novel finding, suggesting a broader differential diagnosis for pediatric neck masses.
    • Further research may elucidate the pathogenesis and optimal management of KHE in unusual locations.