Clinical dermatology • Review article

Treatment options for giant congenital naevi

C. M. Lawrence
Dermatology, Royal Victoria Infirmary, Newcastle,UK

Summary Giant congenital naevi (GCN) are disfiguring, potentially malignant pigmented naevi
present at birth. The naevus cells in GCN are found throughout the dermis and
sometimes penetrate the subcutaneous septa. It is claimed that superficial, more heavily
pigmented and biologically different naevus cells reside in the upper dermis. Partial
removal of these superficial naevus cells by dermabrasion, laser therapy, curettage or
shave excision is less traumatic than excision surgery and produces an acceptable
cosmetic result. However, none of these techniques or excision of GCN to superficial fat
completely removes the risk of malignant transformation.

of a uniform definition of GCN. If size matters,9 then

Definition
including easily excised and potentially less malignant
Giant congenital naevi (GCN) have been variously small naevi will affect the conclusion. Unfortunately
defined by their proportionate size,1,2 actual size3,4 and we are unlikely to improve on these estimates. GCN are
ease of excision.5 Confusingly, the term large congenital rare: naevi >100 mm diameter occur once in every
naevus is used for naevi measuring or predicted to 20 000 healthy live births.10 Thus, it would be necessary
become >20 cm in diameter.6 An agreed definition is to monitor 20 million live births to obtain a cohort of
required to facilitate meaningful comparison between 1000 babies with GCN.
studies. Proportionate measurements are difficult to
make7 and observers frequently overestimate. Direct Histology
measurements are simpler but unless compared to
actual body size may correlate poorly with the relative Much has been written on the histology of GCN and
area involved. Definitions that permit the inclusion of this review will not discuss the various histological
anticipated sizes are likely to be too elastic. Defining GCN patterns described. What matters clinically is whether
by actual size at particular ages is likely to produce the naevus cells lie principally in the upper dermis, i.e. above
most clinically applicable definition. follicles, where they could be destroyed while leaving
intact follicles from which the wounded skin could
re-epithelialize, or throughout the skin thickness, such
Why bother to treat that only complete excision down to or even beyond deep
GCN need to be treated to minimize disfigurement. fascia could have any chance of preventing malignant
Furthermore, most studies conclude that there is a transformation.
3–5% life-time risk of developing melanoma in a GCN There is wide variation in the reported histological
(Table 1). appearances of early congenital naevi and there may
These estimates of risk are confounded by the also be histological differences between small and large
selective bias of retrospective studies8 and the absence naevi.3 In some GCN naevus cells are present through-
out the dermis, in adenexal structures11 and penetrate
to fascia or very occasionally into muscle.12 By contrast,
Correspondence: Dr C. M. Lawrence, Dermatology, Royal Victoria Infirm- small congenital naevi removed from new-born infants
ary, Newcastle, NE1 4LP, UK. E-mail: C.M.Lawrence@ncl.ac.uk showed a very superficial or predominantly junctional
Accepted for publication 23 July 1999 location of naevus cells without conspicuous deeper

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Treatment options for giant congenital naevi • C. M. Lawrence

Table 1 Studies of giant congenital naevi.

GCN Skin Nonskin

patients melanomas melanomas
Study Study type studied (n) described (n) recorded (n) Comments

Quaba2 Postal survey of cases looked after by 39 2 0 8% risk of melanoma during the
British plastic surgeons first 15 years of life
Lorentzen1 Subjects collected over 60-year period 151 3 1 Calculated 4.6% life-time risk.
from selected clinics Assumed that the risk remained
constant during a life-time
Margoob6 Prospective study of large CN (>20 cm 0 3 Average follow-up 5.4 years;
92 diameter or predicted to become 61% treated surgically
that size)
Arons5 Retrospective. Small, large and giant 46 0 0
naevi

Greeley4 Retrospective patients records, over 56 6

25-year period

Swerdlow9 Retrospective: all had GCN covering 33 2 0

>5% body surface area
Lanier8 Restrospective patients records, over 72 5 0 Overall risk not stated. Naevi of
30-year period various sizes described

Japanese studies21 Collection of personal communications 154 7 not recorded 4.5% risk of melanoma
and one published study

infiltration.13 Review of the same material some <16 weeks and from a 10–12-year-old revealed no
10 years later confirmed these findings14 but also demon- overall change in histological appearance.14
strated that S100 positive cells were present in hair
follicles and eccrine ducts at the mid-reticular dermal
level or deeper. Advocates of superficial or partial remo- Factors that influence the risk of melanoma
val have claimed that in the early phases the majority,15 (Table 2)
the active14 or the most heavily pigmented16,17 naevus
cells are localized to the upper dermis. Others have Naevus size
concluded that naevus cells are present throughout
the skin,3,14 and superficial removal is therefore not Swerdlow’s study9 supported the idea that melanoma
viable. risk increases with naevus size. Two of his 33 patients
The advocates of superficial excision claim that in with the largest naevi developed cutaneous melano-
the first few months of life naevus cells in GCN are chiefly mas whereas none occurred in the 232 smaller
present in the upper dermis but later on penetrate naevi. However, 67% of the smallest naevi had been
deeper into the skin. Removal of the upper dermis by excised.
dermabrasion, shave excision or curettage can produce
significant cosmetic improvement before the age of 5,18
1,15 or 4 months16 but do not work if delayed until the
child is older. Unfortunately, despite the clinical obser-
Table 2 Congenital naevi and risk of melanoma.
vation that naevi become darker, hairy and indurated
in the first few months of life,3,11 there is no histological Approximate 3–5% life-time risk
supportive evidence that naevus cells do migrate deeply Risk increases with size of the naevus
with increasing age. Paired biopsies taken from GCN3 Risk greatest at younger age
Melanoma can occur outside skin, i.e. central nervous system and
showed a pan-dermal, pigment cell infiltrate at both 2–6
retro-peritoneal
weeks and 5–15 months of age. Similarly comparison Melanoma can be present at birth and before puberty
of paired biopsies taken from small congenital naevi at

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 Treatment options for giant congenital naevi • C. M. Lawrence

Patient’s age Treatments

19
Melanomas in GCN can be present at birth and
Complete removal
frequently occur before puberty.2 In Kaplan’s review20
eight of the 53 (15%) melanomas described were present Excision
at birth, 25 (47%) presented before the age of 15 years Because naevus cells are found in deep fat and fascai3,
and 37% between 16 and 45 years of age. In an unpub- many surgeons, fearing malignant transformation,
lished Japanese study quoted in Hori’s article21 six of the consider that the entire naevus must be excised down
12 melanomas arising in GCN developed in children to fascia.8 Despite going to this depth benign recurrences
under 4 years of age. The risk of malignant transforma- occur.5 Melanoma has been described after ‘complete’
tion in GCN thus reduces with age but does not disappear excision of a GCN to superficial fat24 and after combined
(Fig. 1). There is no data on how great this risk is after complete and partial removal.25 Different ways have
45 years of age; it may be equivalent to that associated been devised to deal with the defects at different sites.
with common acquired naevi in this age group.
Tissue expanders, serial resection and excision using
Melanomas arising outside the skin
skin expansion
Tissue expanders12,26 particularly for head and neck
A group of children with GCN, especially those with lesions, even before closure of the skull sutures, are
naevi on the back, scalp and multiple satellite naevi,22 well described. Repeat excision has been used for naevi
also have neurocutaneous melanosis.23 These children on the back.27 On the abdomen and limbs French
have melanotic cells in the central nervous system and surgeons have described excising the naevus and then
are at risk from central nervous system and retro- preventing the wound from stretching by fixing the
peritoneal melanoma6 and hydrocephalus. There are limb, etc. in a position for 6 weeks where the scar is
two peak ages for presentation of this complication. In not placed under tension. Although obviously inconve-
their first 2–3 years children with GCN who have neuro- nient for the child this enforced postoperative position28
cutaneous melanosis may develop increased intracranial closure produces impressive results.
pressure, hydrocephalus or developmental delay. The
second peak age of presentation occurs in the second Split skin grafts
and third decades when patients present with signs of The cosmetic results of split skin grafting are poor.12
space-occupying lesions, spinal cord compression and Various methods to increase the amount of donor skin
increased intracranial pressure. Magnetic resonance for grafting have been devised including cultured epi-
imaging is usually diagnostic but the changes need to dermal autografts.29 Because cultured cells take poorly
be reviewed by an expert. on fat the GCN needs to be excised down to deep fascia30
or the recipient site prepared by first placing allogenic
grafts (i.e. split skin grafts taken usually from parents)
over the excision site.

Partial removal

Dermabrasion
Following the accidental removal of part of a scalp GCN
following a forceps delivery it was noted that the
wound healed leaving normal skin. Subsequent derm-
abrasion of the remaining naevus at 2 months produced
permanent pigment loss.18 Later advocates confirmed
that provided the dermabrasion was done before 5
months of age much of the pigmentation but not the
hair, could be removed.15 Others have claimed that
because naevus cells extend below the depth removed
Figure 1 Melanoma arising in a congenital naevus in a
70-year-old man. This 3.0 mm thick melanoma developed within
by dermabrasion the treatment is inherently flawed.3
a 50 × 20 mm flat, apparently innocuous, hairy congenital The report of melanoma occurring after dermabrasion21
naevus on the forearm. tends to support this viewpoint.

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Treatment options for giant congenital naevi • C. M. Lawrence

Shave excision 8 Lanier VC, Pickrell KL, Georgiade NG. Congenital giant
Others have tried shaving the upper dermis off in the nevi: clinical and pathological considerations. Plast
same way as a split skin graft is taken.17 In babies Reconstr Surg 1976; 58: 48.
younger than 9 weeks old this produced pale scars, 9 Swerdlow AJ, English JSC, Qiao Z. The risk of melanoma
in patients with congenital nevi. A cohort study. J Am
some with spotted pigmentation but older children
Acad Dermatol 1995; 32: 595–9.
fared less well. Partial removal of a GCN in this way
10 Castilla EE, Dutra MG, Oriolo-Parreiras IM. Epidemiology
does not prevent melanoma.9,25 of congenital pigmented naevi: Incidence rates relative
frequencies. Br J Dermatol 1981; 104: 307–15.
Curettage 11 Mark GJ, Mihm MC, Liteplo MG, Reed RJ, Clark WH.
Curettage of GCN in children less than 6 months of age Congenital melanocytic nevi of the small and giant type.
is possible because of a natural plane of cleavage Clinical histologic and ultrastructural studies. Hum
between the superficial bulk of the naevus and the Pathol 1973; 4: 395–418.
lower dermis.16 Cosmetically this results in soft, pale 12 Bauer BS, Vicari FA. An. approach to excision of
scars with occasional spotted recurrences and lush hair congenital giant pigmented nevi in infancy and early
growth. One group have suggested that the curetted childhood. J Paediatr Surg 1988; 23: 509–14.
13 Walton RG, Jacobs AH, Cox AJ. Pigmented lesions in
superficial naevus cells may be biologically different
new-born infants. Br J Dermatol 1976; 95: 389–96.
from the retained deeper cells31 because superficial
14 Nickoloff BJ, Walton R, Pregerson-Rodan K, Jacobs AH,
naevus cells stained positive for the melanocyte marker Cox AJ. Immunohistologic patterns of congenital
HMB-45 whereas deeper naevus cells did not. nevocellular nevi. Arch Dermatol 1986; 122: 1263–8.
15 Miller CJ, Becker DW. Removing pigmentation by
Laser therapy dermabrading naevi in infancy. Br J Plast Surg 1979; 32:
The potential of laser therapy has been justified on 124–6.
the basis of the superficial position of the majority of 16 Moss ALH. Congenital giant naevus: a preliminary report
the pigment in GCN. A high energy CO2 laser was used of a new surgical approach. Br J Plast Surg 1987; 40:
to vaporize the upper dermis in a 15-day-old child 410–9.
with a GCN resulting in impressive improvement at 4 17 Sandsmark M, Eskeland G, Ogaard AR, Abyholm F,
Clausen OPF. Treatment of large congenital naevi. Scand
months.32 Selective photo-thermolysis of pigment laden
J Plast Reconstr Hand Surg 1993; 27: 223–32.
cells produced poor results.33
18 Johnson HA. Permanent removal of pigmentation from
giant hairy nevi by dermabrasion in early life. Br J Plast
References Surg 1977; 30: 321–3.
19 Baader W, Kropp R, Tapper D. Congenital malignant
1 Lorentzen M, Pers M, Bretteville-Jensen G. The incidence melanoma. Plast Reconstr Surg 1992; 90: 53–6.
of malignant transformation in giant pigmented nevi. 20 Kaplan EK. The risk of malignancy in large congenital
Scand J Plast Reconstr Surg 1977; 11: 163–7. nevi. Plast Reconstr Surg 1974; 53: 421–8.
2 Quaba AA, Wallace AF. The incidence of malignant 21 Hori Y, Nakayama J, Okamoto M et al. Giant congenital
melanoma (0–15 years of age) arising in large congenital nevus and malignant melanoma. J Invest Dermatol 1989;
nevocellular nevi. Plast Reconstr Surg 1986; 78: 174–87. 92: 310S–4S.
3 Zitelli JA, Grant MG, Abell E, Boyd JB. Histologic patterns 22 DeDavid M, Orlow SJ, Provost N et al. Neurocutaneous
of congenital naevocytic nevi, implications for treatment. melanosis: clinical features of large melanocytic nevi in
J Am Acad Dermatol 1984; 11: 402–9. patients with manifest central nervous system melanosis.
4 Greeley PW, Midleton AG, Curtin JW. Incidence of J Am Acad Dermatol 1996; 35: 529–38.
malignancy in giant pigmented nevi. Plast Reconstr Surg 23 Kadonaga JN, Frieden IJ. Neurocutaneous melanosis:
1965; 36: 26–37. definition and review of the literature. J Am Acad
5 Arons MS, Hurwitz S. Congenital nevocellular nevus: a Dermatol 1991; 24: 747–55.
review of the treatment controversy and report of 46 24 Rhodes AD. Congenital nevomelanocytic nevi histologic
cases. Plast Reconstr Surg 1983; 72: 354–65. patterns in the first year of life and evolution during
6 Margoob AA, Schoenbach SP, Kopf AW, Orlow SJ, childhood. Arch Dermatol 1986; 1222: 1257–62.
Nossa R, Bart RS. Large congenital melanocytic nevi 25 Shaw MH. Malignant melanoma arising from a giant
and the risk for development of malignant melanoma. hairy naevus. Br J Plast Surg 1962; 15: 426.
Arch Dermatol 1996; 132: 170–5. 26 Vergnes P, Taieb A, Malville J, Larregue M, Bondonny JM.
7 Ramsay B, Lawrence CM. Measurement of involved Plast Reconstr Surg 1993; 91: 450–5.
surface area in patients with psoriasis. Br J Dermatol 27 Chretien-Marquet B, Bennaceur S, Fernandez R. Surgical
1991; 124: 565–70. treatment of large cutaneous lesions of the back in

10 q 2000 Blackwell Science Ltd • Clinical and Experimental Dermatology, 25, 7–11
 Treatment options for giant congenital naevi • C. M. Lawrence

children by concentric cutaneous mobilisation. Plast 31 De Raeve LE, de Connick AL, Diericke PR, Roseeuw DI.
Reconstr Surg 1997; 100: 926–36. Neonatal curettage of giant congenital melanocytic nevi.
28 Chretien-Marquet B, Benaceur S, Cerceau M, Arch Dematol 1996; 132: 20–2.
Fernandez R, Saouma S, Murthy J. Cutaneous expansion 32 Kay AR, Kenealy J, Mercer NSG. Successful treatment
using enforced position in the treatment of large skin of a giant congenital melanocytic naevus with the
defects. Plast Reconstr Surg 1994; 93: 337–44. high energy pulsed CO2 laser. Br J Plast Surg 1998; 51:
29 Coleman JJ, Siwy BK. Cultured epidermal autografts: a life 22–4.
saving and skin saving technique in children. J Paediatr 33 Scheepers JH, Quaba AA. Clinical experience with the
Surg; 27 (1029–32): 1992. PLDL-1 (pigment dye laser) in the treatment of pigmented
30 Gallico GG, O’Connor NE, Compton GC et al. Cultured birthmarks: a preliminary report. Br J Plast Surg 1993;
epithelia autografts for giant congenital nevi. Plast 46: 247–51.
Reconstr Surg 1989; 84: 1–9.

Key points
• Generally accepted definition of giant congenital naevi is • Melanoma can be present at birth and before puberty
required that is based on size at birth e.g. >100 mm broad- • Melanoma may arise in the CNS or in the retro-peritoneal
est diameter space
• Life time risk of melanoma in giant congenital naevi • Superficial removal or destruction of naevus cells eg by
estimated at 3–5% shave excision or laser therapy, can produce acceptable
• Risk greatest with younger ages and increases with naevus cosmetic results
size

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