Table 2.

Tumours and tumour-like lesions with at least partial lymphatic differentiation

Name	Biological behavior according to ISSVAa 2015 [55]
Primary lymphoedema/lymphatic malformation	Congenital malformation. Isolated or in combination with other vascular or nonvascular malformations or genetic syndromes
Tufted angioma	Benign tumor of skin. Partial lymphatic differentiationb
Spindle cell angioma	Provisionally unclassified. Probably vascular malformation with partial lymphatic differentiation
Verrucous angioma	Provisionally unclassified; probably congenital malformation with partial lymphatic
Angiokeratoma	Provisionally unclassified vascular anomaly. Partial lymphatic differentiation
dMLT/CATc	Provisionally unclassified lesion.
Kaposiform hemangioendothelioma	Locally aggressive tumor. Extends into deep tissues; resembles tufted angioma
PILAd	Borderline malignant tumour
Kaposi sarcoma	Borderline malignant tumour
Lymphangiosarcoma	Malignant. Variable expression patterns with lymphatic markers
Kaposiform lymphangiomatosis	Provisionally unclassified vascular lesion

^aInternational Society for the Study of Vascular Anomalies

^bPartially lymphatic indicates: composed of blood vessels and lymph vessels

^cDiffuse multiple lymphangioendotheliomatosis

^dPapillary intralymphatic angioendothelioma (Dabska tumour)