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. 2021 Jul 8;16:306. doi: 10.1186/s13023-021-01929-8

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© The Author(s) 2021

Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

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Fig. 2 — PIK3CA-related disorders. The category of PIK3CA-related disorders can be divided into 3 subcategories: PROS, PIK3CA-related vascular malformations, and PIK3CA-related nonvascular lesions. CLAPO: capillary malformation of the lower lip, lymphatic malformation of the face and neck, asymmetry and partial/generalized overgrowth; CLOVES: congenital lipomatous overgrowth, vascular malformations, epidermal nevi, scoliosis/skeletal and spinal; CLVM: combined capillary-lymphatic-venous malformation; DCMO: diffuse capillary malformation with overgrowth; DMEG: dysplastic megalencephaly; FAO/HHML: fibroadipose hyperplasia or overgrowth/hemihyperplasia-multiple lipomatosis; FAVA: fibroadipose vascular anomaly; FIL: fibroadipose or facial infiltrating lipomatosis; GLA, generalized lymphatic anomaly; HH: hemihyperplasia; HMEG: hemimegalencephaly; LM, lymphatic malformation; LON: lipomatosis of nerve; LVM: combined lymphatic-venous malformation; MCAP: megalencephaly-capillary malformation; PIK3CA: phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha; VM, venous malformation. *Malformations that are composed of only one type of vessel are classified as “simple” (with the exception of arteriovenous malformation). If a lesion contains ≥ 2 types of vascular malformations, it is classified as “combined.” Vascular malformations are also classified according to major named vessels or by association with other anomalies (e.g., PROS) Wassef 2015 [33]. †Some common (cystic) LM are classified as PROS when they are associated with overgrowth ISSVA 2018 [2]. ‡Can occur as isolated lesions or with other clinical features. Supporting Sources: PIK3CA-related overgrowth spectrum: Keppler-Noreuil 2015 [3]; ISSVA 2018 [2]; Goss 2020 [28]; Hughes 2020 [29]; Mahan 2014 [30]; Rios 2013 [31]; Rodriguez-Laguna 2019 [32]; PIK3CA-related vascular malformations: ISSVA 2018 [2]; Rodriguez-Laguna 2019 [32]; PIK3CA-related nonvascular lesions: Keppler-Noreuil 2015 [3]; Combined vascular malformations: Wassef 2015 [33]; Brandigi 2018 [27]