Composite haemangioendothelioma: report of four cases with emphasis on atypical clinical presentation.

摘要

To date, there have been approximately 20 cases of composite haemangioendothelioma (HE) reported.Owing to its rarity and complex morphological variations, composite HE continues to pose diagnostic challenges in the distinction from other mimicking vascular tumours, particularly for those occurring in atypical clinical settings. To further broaden the clinicopathological and immunohistochemical spectrum of composite HE, we describe four new cases, including two occurring in the hitherto unreported hypopharynx and one in a patient with neurofibromatosis type 2 (NF2).Composite HE was first designated by Nayler et al. in 2000 as one of the rarest borderline vascular tumours with an admixture of concurrent benign (e.g., spindle cell haemangio-mas), intermediate (e.g., retiform HE), and malignant (e.g., angiosarcoma-like) vascular lesions of various types. Retiform and epithelioid HEs represent the two most common histologi-cal types of composite HE.However, the variations in composition of vascular tumour types appear not correlated with clinical outcomes. Local relapses are not uncommon in composite HE and tend to occur several years after tumour resection, while the potential of distal and regional nodal metastases is considered very low.