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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.
Abstract:
Kaposiform haemangioendothelioma (KHE) is a rare, locally aggressive vascular spindle cell proliferation, with resemblance to Kaposi's sarcoma. This tumour usually occurs in skin and retroperitoneum of infants and young children and is often complicated by the Kasabach-Merritt phenomenon (KMP). A 3-year-old boy presented with a right submandibular swelling due to lymphadenopathies, a violaceous skin lesion at the left commissure of the lips and an ill-defined lesion in the right thyroid lobe. There were some signs of KMP. Histological examination revealed a typical infiltrative multilobular spindle cell proliferation with slit-like vascular spaces in these three localisations. Immunohistochemical stains showed positivity for CD34 and CD31 and many alpha-smooth muscle actin-positive spindle cells around the vascular spaces. There was no Herpes virus type 8 expression. The presented case is unique in two ways. First, thyroid involvement of KHE has never been described in the literature until now. Secondly, and most remarkably, the multifocal presentation in three anatomically distinct and separated localisations is extremely unusual.
Insights
Kaposiform haemangioendothelioma (KHE), a rare vascular tumor, presented multifocally in a child, including a novel thyroid involvement. This case highlights KHE
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.
- 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.
