Supplemental Table 1. Comprehensive review of the literature.

Author / Year / Cases / Age / Gender / Anatomic site / Intraepidermal component / Blue nevus component / History / Follow-up
Allen and Spitz / 1953 / 6 / 37-68 / M / Chest, thigh, hand, neck, scalp / None / CBN / n/a / DOD 1 yr; DOD 2 yrs; AWMD 2yrs; AWMD 10 yrs;
Allen and Spitz / 1953 / 61 / F / Buttock / None / CBN / n/a / ANED 6 yrs
Dorsey / 1954 / 1 / 16 / M / Right cheek/ temporal / None / Nevus of Ota / Nevus of Ota since birth with 6 month history of firm mass / n/a
Fisher / 1955 / 1 / 27 / M / Occipital / None / n/a / Lesion present since age 17; enlarging since 21 / AWMD 72 mos
Kwittken / 1966 / 1 / 33 / F / Foot / None / Resembled CBN / 7 year history of painless mass with recent increase in size / DOD 90 mos
Merkow / 1969 / 1 / 34 / F / Scapula / None / Non-CBN/CBN / Present since birth; rapidly enlarging / AWMD, 22 mos
Mishima / 1970 / 2 / Scalp / None / Resembled CBN / n/a / AWMD 24 mos
Mishima / 1970 / Scalp / None / Resembled CBN / n/a / MD 36 mos; DOD 60 mos
Hourihane / 1971 / 1 / 35 / M / Foot / None / n/a / n/a / n/a
Silverberg / 1971 / 1 / 3 / M / Scalp / None / Non-CBN/CBN / Congenital BN with development of multiple CBN and then MM into brain / NED 7 mos
Gartmann / 1972 / 1 / 46 / F / Scalp / None / Resembled CBN / n/a / 2 yrs with MD
Hernandez / 1973 / 1 / 35 / M / Back T-8 / None / Resembled CBN / Painless lump on back for 8 months / Metastases to viscera and brain @ 8 months
Hendrickson / 1981 / 1 / 5 / M / Scalp / None / non-CBN / Present since birth; rapidly enlarging / DOD 8 yrs
Rubinstein / 1985 / 1 / 33 / M / Dorsum of hand / None / non-CBN/CBN / Present for many years with recent increase in size and ulceration / ANED 2 years
Wetherington / 1987 / 1 / 14 / M / Scalp / None / CBN / Present since birth; CBN at age 1; then recurrent multinodular disease / AWMD (ileum) 36 mos
Temple-Camp / 1988 / 1 / 70 / M / Back / None / CBN / 70 yr history / ANED 9 mos
Author / Year / Cases / Age / Gender / Anatomic site / Intraepidermal component / Blue nevus component / History / Follow-up
Goldenhersh / 1988 / 1 / 70 / F / Scalp / None / non-CBN/CBN / 60 yr history of dime sized blue nodule / DOD 9 mos
Kuhn / 1988 / 1 / 41 / F / Arm / None / CBN / 1 yr history of enlarging blue-black nodule / AWMD-lung 24 mos
Modly / 1989 / 1 / 66 / M / Buttock / None / BN / Lupus; asymptomatic nodule of unknown duration on buttock / 6 mos +SLN; ANED; length not designated
Boi / 1991 / 4 / 40 / F / Back / None / Congenital BN / Large lesion since childhood; developed nodules 12, 5 and 1 year prior / ANED 5 years
Boi / 1991 / 39 / F / L arm / None / Common BN; resembled CBN / Present since childhood began changing 7 years prior / 24 mos local recurrence; 26 mos axillary LNs; 60 mos ANED
Boi / 1991 / 41 / M / Pre-auricular / None / Resembled CBN / Present since infancy; recently enlarged / 6 mos cervical LNs; 62 mos DOD
Boi / 1991 / 53 / F / Right leg / None / Resembled CBN / None / Multiple LN metastasis; 3 yrs ANED
Connelly / 1991 / 40 / M / Scalp / None / CBN/non-CBN adjacent to MM / n/a / DOD 23 mos
Connelly / 1991 / 56 / F / Scalp / None / CBN/non-CBN adjacent to MM / n/a / AWMD 60 mos
Connelly / 1991 / 44 / F / Scalp / None / CBN/non-CBN adjacent to MM / n/a / DOD 8 mos
Connelly / 1991 / 12 / 75 / M / Scalp / None / CBN/non-CBN adjacent to MM / n/a / DOD 11 mos
Connelly / 1991 / 56 / M / Scalp / None / CBN adjacent to MM / n/a / DOD 21 mos
Connelly / 1991 / 57 / M / Scalp / None / CBN adjacent to MM / n/a / AWMD, 24 mos
Connelly / 1991 / 33 / F / Scalp / None / CBN adjacent to MM / n/a / DOD, 79 mos
Connelly / 1991 / 64 / F / Scalp / None / CBN adjacent to MM / n/a / DOD 16 mos
Connelly / 1991 / 35 / M / Chest / None / CBN adjacent to MM / n/a / DOD 114 mos
Author / Year / Cases / Age / Gender / Anatomic site / Intraepidermal component / Blue nevus component / History / Follow-up
Connelly / 1991 / 30 / F / Buttock / None / CBN adjacent to MM / n/a / ANED 24 mos
Connelly / 1991 / 36 / M / Eyelid / None / CBN adjacent to MM / n/a / DOD 60 mos
Connelly / 1991 / 60 / M / Ear / None / unknown / n/a / n/a
Mehregan / 1992 / 8 / 35 / M / Buttock / None / Resembled CBN / 1cm blue-black nodule present since birth / ANED 2 yrs
Mehregan / 1992 / 53 / F / Scalp / None / Resembled CBN / 1.5 cm blue nodule / ANED 2.5 yrs
Mehregan / 1992 / 16 / F / Gluteal cleft / None / Resembled CBN / 2.5 cm pigmented growth since birth / n/a
Mehregan / 1992 / 52 / M / Buttock / None / Resembled CBN / 1cm dome shaped / n/a
Mehregan / 1992 / 25 / F / Scalp / None / Resembled CBN / Recent 3 cm growth in blue nevus present since birth / DOD Brain metastasis
Mehregan / 1992 / 22 / F / Dorsum foot / None / Resembled CBN / 1.5 cm blue-black nodule present since birth with recent increase size and pain / DOD widespread visceral metastasis
Mehregan / 1992 / 59 / F / Dorsum foot / None / Resembled CBN / 2 cm nodular growth / n/a
Mehregan / 1992 / 35 / F / Dorsum foot / None / Resembled CBN / 1.5 cm nodular growth present since birth with recent erythema / n/a
Pathy / 1993 / 1 / 65 / M / Scalp / None / Common BN; neurocristic hamartoma / Lesion present since childhood; first biopsy common BN / 35 LN/satellite mets
Aloi / 1996 / 6 / 42 / F / Trunk / None / CBN / Birth / ANED 34 mos
Aloi / 1996 / 52 / F / Scalp / None / CBN / Birth / DOD, 12 mos
Aloi / 1996 / 73 / F / Neck / None / Resembled CBN / Infancy / ANED 70 mos
Aloi / 1996 / 53 / F / Scalp / None / Resembled CBN / Infancy / AWMD 18 mos
Aloi / 1996 / 64 / M / Scalp / None / Resembled CBN / Birth / DOD 8 mos
Aloi / 1996 / 57 / M / Trunk / None / Resembled CBN / Birth / AWMD 15 mos
Scott / 1993 / 1 / 12 / F / Ear / None / Resembled CBN / 1 yr history of blue-grey area / Metastasis 4 mos; DOD 8 mos
Boni / 1996 / 1 / 52 / F / Suprapatellar / None / Resembled CBN / 12 year history of three recurrent CBN / Satellite on thigh; length not specified
Author / Year / Cases / Age / Gender / Anatomic site / Intraepidermal component / Blue nevus component / History / Follow-up
English III / 1996 / 1 / 57 / M / Scalp / None / Resembled CBN / 2 year history of growing scalp nodule; bled with trauma / n/a
Ozgur / 1997 / 1 / 63 / F / left upper arm / None / CBN / 5-6 year history of stable blue-black nodule; increased in size 3 months / Metastasis in 5/30 axillary LNs @ 7 mos; liver metastasis @ 11 mos; DOD 11 mos
Calista / 1998 / 1 / 70 / F / Scalp / None / Resembled CBN / 40 year stable 1 cm plaque which became large ulcerated / Metastases to liver @ 3 years. Death @ 4 years
Duteille / 1998 / 3 / 73 / M / Abdomen / None / CBN / Congenital / ANED
Duteille / 1998 / 21 / F / Maxilla / None / CBN / Congenital with 3-4 yr history of increased size / ANED
Duteille / 1998 / 50 / M / Arm / None / Resembled CBN / n/a / ANED 1 yr
Spatz / 1998 / 1 / 28 / F / Vulva / None / Resembled CBN / n/a / Metastasis to ovary 15 yrs later
Granter / 2001 / 10 / 69 / F / Back / None / CBN / Rapid growth 1 yr / Local recurrence 14 mos
Granter / 2001 / 15 / M / Lower lumbar / None / Resembled CBN / n/a / Cutaneous metastasis 1 yr; Lung mets; DOD 2yrs
Granter / 2001 / 37 / F / Scalp / None / Resembled CBN / n/a / Recurred 1 yr; LFU
Granter / 2001 / 62 / M / Buttock / None / Resembled CBN / n/a / SLN positive; mets to spine 1 yr; AWD 18 mos
Granter / 2001 / 77 / F / Scalp / None / Resembled CBN / n/a / LFU
Granter / 2001 / 36 / M / Inferior orbit / None / Common BN / Present for many years / Recurred 2 yr; ANED 3 yrs
Granter / 2001 / 54 / F / Scalp / None / Resembled CBN / n/a / Liver and lung mets; DOD 4yrs
Granter / 2001 / 11 / F / Scalp / None / CBN / n/a / n/a
Granter / 2001 / 64 / M / Scalp / None / Common BN / n/a / Liver metastasis; DOD 1yr
Granter / 2001 / 56 / M / Back / None / Resembled CBN / n/a / n/a
Author / Year / Cases / Age / Gender / Anatomic site / Intraepidermal component / Blue nevus component / History / Follow-up
Ariyanayagam-Baksh / 2003 / 1 / 41 / M / Back / None / non-CBN; CBN / 9 mos history of enlargement of congenital blue nevus / Metastasis s to LN and lungs; AWD 2 yrs
Hu / 2004 / 1 / 30 / F / Scalp / n/a / Combined nevus: congenital and common BN / Prior removal 5 yrs ago as congenital nevus / Metastasis in 5/26LN; DOD 13 mos
Pozo / 2004 / 1 / 30 / F / Back / None / Sclerosing blue nevus / 5 yr history slowly enlarging lump in a "birthmark" / ANED; length not stated
Hagiwara / 2005 / 1 / 42 / F / Perineum/Labium minus / None / Associated common BN cervix / n/a / DOD 8 mos
Odashiro / 2005 / 1 / 43 / F / Right orbit / None / common BN / n/a / AWD 16 mos
Schneider / 2006 / 1 / 46 / F / Scalp / None / CBN / Nodule arising in a blue macule / Positive SLN; ANED 22 mos
Kato / 2007 / 1 / 55 / m / Scalp / None / Resembled CBN / n/a / DOD 13 mos
Mellone / 2008 / 1 / 35 / M / Trunk / None / common BN / 1 yr history of slowly progressing lesion / ANED 1 yr
Lobo / 2008 / 1 / 17 / M / Lower back / None / common BN / Giant congenital blue nevus, “bathing-trunk” since birth; also aromatase excess syndrome / ANED 3 yrs
Zyrek-Betts / 2008 / 1 / 65 / M / Scalp / None / CBN / Discovered after metastasis to cervical LNs / Metastasis to 2/15 cervical LN; ANED 3 yrs
Martin / 2009 / 23 / 22-72 / 15M; 8F / 8 Scalp/Face; 8 Thorax; 7 Arm/Leg / NA / Resembled CBN; associated BN / n/a / Same as matched controls
Lee / 2010 / 1 / 50 / M / Lip / None / Common BN / 3 year h/o blue-black macules in upper lip / Metastasis to cervical LN; ANED 1 yr
North / 2012 / 2 / 30 / M / Chest / None / Non-CBN/CBN: Plaque type BN / Nodules arose at site of "hemangioma" at age 9; plaque type BN / Capsular nevus; ANED 10m mos
North / 2012 / 72 / F / Breast / None / Non-CBN/CBN: Plaque type BN / Plaque type BN / ANED 10 mos
Sanada / 2012 / 1 / 65 / M / Abdomen / None / Non-CBN/CBN: Plaque type BN / Multiple pigmented lesions since childhood on skin / n/a
Author / Year / Cases / Age / Gender / Anatomic site / Intraepidermal
component / Blue nevus component / History / Follow-up
Held / 2012 / 1 / 20 / M / Right ear / None / Non-CBN/CBN: Plaque type CBN / Nodules and blue grey pigmentation since age 11 / DOD 3 mos; Neck and liver metastases
Parada / 2012 / 1 / 76 / F / Uterine Cervix / Pagetoid glandular extension / Common BN / n/a / 1/11 LNs; ANED
Yeh / 2012 / 1 / 38 / F / Breast / None / Plaque type BN / n/a / Metastasis to LN 9 yr; Mets to liver 22 yrs; DOD 22 yrs

Abbreviations: ANED: Alive no evidence of disease, AWD: alive with disease, AWMD: alive with metastatic disease, BN: blue nevus, CBN: cellular blue nevus, DOD: Dead of disease, LFU: lost to follow up, LN: lymph node, MD: metastatic disease, MM: malignant melanoma, n/a: not available, NED: no evidence of disease, SLN: sentinel lymph node

* Not every parameter was available for review for every case and therefore the numbers provided may not add up to the total cases reported in the literature up to date.