Kaposiform haemangioendothelioma of the head and neck

- Only 53 cases of KHE arising from the head and neck region have been reported in the literature to date.
- Prognoses in adults are better compared to children due to lower risks of KMP.
- Although regarded as a tumor of intermediate malignant potential, a case of distant metastasis had been reported.
- Complete surgical excision with clear margins remains the treatment of choice and offers best outcome.

BackgroundKaposiform haemangioendothelioma (KHE) is a tumor of intermediate malignant potential derived from vascular endothelial cells. Due to rarity of head neck KHE (HN-KHE) this comprehensive review aims to compile, analyze and present details to develop a consensus and augment available literature on HN-KHE.Materials and methodsA comprehensive literature search was performed on PUBMED/MEDLINE, EMBASE, CINAHL and Science Citation Index for HN-KHE using MeSH words. Statistical analysis was performed using a variety of tests.ResultsCommon sites of involvement were neck 41.5%, face and scalp 32.0% and tympanomastoid region in 13.2% patients. Kasabach-Merritt phenomenon was seen in 58.5% patients.Surgical excision was performed in 37.7% patients while 39.6% patients underwent medical management/chemotherapy (CT). Significantly better disease free survival (DFS) was seen in patients undergoing surgical excision vs. CT (p = 0.001), without recurrence vs. with recurrence (p = 0.001) and those presenting within 0-1 year of life vs. 1-5 years (p = 0.021).ConclusionRecurrence and metastasis were seen in 35.8% and 20.0% patients respectively. Complete surgical excision with clear margins remains the treatment of choice.