Table 1.

PROS classification

A. Phenotypic spectrum
Localised or tissue-specific, MIM	Multisystemic and with possible brain involvement, MIM
Macrodactyly, #155500	Fibroadipose Overgrowth/Hemihyperplasia-Multiple Lipomatosis (FAO/HHML)
Epidermal Nevi, #162900	Congenital Lipomatous Overgrowth, Vascular Malformations, Epidermal Nevi, Scoliosis/Skeletal and Spinal Abnormalities syndrome (CLOVES), #612918
Seborrheic Keratosis, #182000	Capillary malformation of the lower lip, Lymphatic malformation of face and neck, Asymmetry of face and limbs, and Partial/generalized Overgrowth syndrome (CLAPO), #613089
PIK3CA-related muscular overgrowth with ectopic accessory muscles (Al-Qattan et al., 2018).	Megalencephaly-Capillary Malformation-Polymicrogyria syndrome (MCAP), #602501
Hemiorofacial asymmetry with peripheral nerve enlargement andperineuriomatous pseudo-onion bulb proliferations (Koutlas et al., 2021)
Facial infiltrating lipomatosis (FIL) (Couto et al., 2017)
Lymphatic malformations (LM) (Zenner et al., 2019)
Klippel-Trenaunay syndrome (KTS), %149000
B. Management*
MCAP	Non-MCAP PROS
Activating, mosaic PIK3CA variant	Activating, mosaic PIK3CAvariant
Meg and/or PMG	No significant Meg or PMG or Sx
Extra-CNS overgrowth and/or vasc findings	Extra-CNS overgrowth and vasc findings

^*rare PROS individuals have been reported with consitutional variants (De Graer et al., 2020)

Meg: Megalencephaly (see Appendix), PMG: Polymicrogyria, Sx: syrinx, CNS: Central Nervous System, vasc: vascular including cutaneous