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. 2022 Mar 2;17:95. doi: 10.1186/s13023-022-02234-8

Table 1.

Study characteristics

References Country Patient number Patient age, mean (range; median) Female: male ratio CMN location CMN size and inclusion criteria Follow-up time mean (range; median) Level of evidence
Ruiz-Maldonado et al. [17] Mexico 13 11 yr 7 mo (0.6–32 yr) 8:5 Whole body 'Giant' CMN involving the head and neck in reportedly asymptomatic patients Unreported 4
Bittencourt et al. [23] U.S.A 160 6 yr 6 mo (1–63 yr; 1.2 yr) 89:71 Whole body  ≥ 20 cm or ≥ 20 cm PAS 66.2 mo SD 64.1 (1–238 mo; 42 mo) 3
Foster et al. [9] U.S.A 46 5 mo (4 days–8 yr) 22:27 Head, neck, trunk  ≥ 9 cm (scalp) or ≥ 6 cm (body) CMN involving the head, neck and dorsal spine 5 yr (2–8 yr) (of 44pt) 3
Bett et al. [48] U.S.A 1072 6 yr 11 mo (1–23 yr) 22:23 Head, neck, trunk (information of extremities CMN available)  ≥ 9 cm (scalp), ≥ 6 cm (body) or nevus covering a substantial part of a small body area (face, hand, foot), multiple small/medium 5.6 yr 3
Chan et al. [25] Singapore 39 18 yr 11 mo (1.9–60 yr) 16:23 Whole body  > 10 cm 16.9 yr (12 mo–38 yr; 15.4 yr) 3
Lovett et al. [20] Canada 54 1 yr 4 mo (0–14 yr) 29:25 Whole body  ≥ 9 cm (scalp) or ≥ 6 cm (body) in infants, ≥ 20 cm PAS or at least 3 medium-sized (1.5–19.9 cm diameter) CMN of the head 89 mo (82.5 mo) 3
Ramaswamy et al. [19] U.S.A 14 (1–6 yr; 2.6 yr) (of 8 survivors) 3:11 Unreported High risk patients, according criteria of Kadonaga and Frieden, ≥ 20 cm PAS or multiple small/medium Unreported 4
Bekiesińska-Figatowska et al. [47] Poland 24 (12 days–7 yr) 4:3 Whole body  ≥ 9 cm (scalp) or ≥ 6 cm (body), ≥ PAS (15–62 mo) 4
Price et al. [55] U.S.A 45 Unreported 28:17 Whole body  > 10 cm PAS and multiple CMN > 1, 5 cm PAS, Krengel classifications No follow up 4
Waelchli et al. [7] U.K 636 1 yr 6 mo (0.6 yr) 1:2 Whole body Before 2008: > 2 cm/1% BSA (overlying spine or brain) after 2008: > 1 CMN (regardless the size) 11.0 yr (8.5 yr) 3
Viana et al. [56] Brazil 57 8 yr 4 mo (2 yr 5 mo) 28:29 Whole body  ≥ 20 cm or ≥ 20 cm PAS 5.5 yr ± 3.8 SD, (5.2 yr) 3
Wramp et al. [24] Germany 83 11 yr 4 mo 41:42 Whole body  > 15–20 cm PAS, Krengel classification 4.6 yr 3
Jakchairoongruang et al. [18] U.S.A 80 1 yr 10 mo (1 day–22 yr; 6 mo) Unreported Unreported 'Large' CMN (1 mo–11 yr) (of 9pt with brain melanocytosis) 3
Qian et al. [21] China 13 36 yr 6 mo (18–59 yr) 4:9 Whole body CMN > 20 cm and/or multiple (3 or more) small/medium CMN (neurocutaneous melanocytosis according Kadonaga and Frieden, included 3 patients without cutaneous CMN) 3 yr 4

CMN, congenital melanocytic naevi; Mo, months; PAS, projected adult size; yr, years

CMN patient included in the study

According to the Oxford Centre for Evidence-based Medicine