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.