Pediatric Dermatology Vol. 29 No.

1 1523, 2012

Nevus Sebaceous Revisited

Megan N. Moody, M.D., M.P.H.,* Jennifer M. Landau, B.S.,*
and Leonard H. Goldberg, M.D.*,
*Derm Surgery Associates, Houston, Texas, Department of Dermatology, Weill Cornell Medical College,
Methodist Hospital, Houston, Texas

Abstract: Nevus sebaceous of Jadassohn is a hamartoma with a combination of abnormalities of the epidermis, hair follicles, and sebaceous and
apocrine glands. Herein, we discuss the results of an extensive literature
review on the topic of nevus sebaceous with a particular focus on the debate
about the necessity for prophylactic excision. We also focus on the documentation of associated malignant tumors that were reported to develop
within NS. In addition to reporting the number and types of neoplasms, we
documented the recommendations of all authors for therapeutic handling of
these nevi.

Nevus sebaceous (NS) is a hamartoma with epithelial

and nonepithelial origins (1,2). It is often referred to as an
organoid nevus because it may contain any or all components of the skin (2). Although typically a clinical
diagnosis because of its characteristic clinical appearance
and location, NS may be conrmed with a biopsy. Histologically, NS displays an array of abnormalities within
sebaceous glands, sweat glands, and hair follicles, often
with a lack of terminally dierentiated hair follicles
(Fig. 1) (3). NS presents at birth and grows proportionally with the patient. The etiology is unknown, but recent
studies point to a possible link with mothers positive for
the human papilloma virus (4) or mutations in the patched gene (PTCH) (5). NS may also present as a feature
of certain genetic syndromes, including didymosis
aplasticosebacea and SCALP (sebaceous nevus, central
nervous system malformations, aplasia cutis congenital,
limbal dermoid, and pigmented nevus) syndrome (6).
It most commonly presents as a round, oval, or linear;
smooth; yellowish-orange plaque on the head, neck, and
scalp (8795% of cases) leading to alopecia (Fig. 2) (7).

The natural history of NS is traditionally divided into

three overlapping stages (8). The rst is the early infantile
stage, characterized by papillomatous hyperplasia and
immature hair follicles. The second stage involves
pubertal expansion, characterized by rapid growth of the
nevus due to hormonally driven development of sebaceous glands and maturation of apocrine glands. The
lesion transforms from a smooth into a verrucous
plaque (Fig. 3). The third stage is the development of
benign and malignant epithelial neoplasms (in 1020%
of cases), including, but not limited to, trichoblastoma,
syringocystadenoma papilliferum, sebaceous epithelioma,
basal cell carcinoma (BCC), trichilemmoma, adnexal
carcinoma, and eccrine poroma.
The denitive treatment of NS is full-thickness dermal
and epidermal excision, although there is an ongoing
debate regarding whether NS should be excised and, if so,
when. Excision may be justied for cosmetic reasons or
to avoid the formation of tumors. Because NS characteristically expand during puberty, some argue that
excision should be performed before this growth phase to

Address correspondence to Leonard H. Goldberg, M.D., Derm

Surgery Associates, 7515 Main Street, Suite 240, Houston, TX
77030, or e-mail: goldb1@dermsurgery.org.
DOI: 10.1111/j.1525-1470.2011.01562.x

 2011 Wiley Periodicals, Inc.

15

16 Pediatric Dermatology Vol. 29 No. 1 January February 2012

Figure 1. The specimen consists of an excision of epidermis, dermis, and subcutaneous fat. Epithelial hyperplasia is
associated with poorly formed hair follicles and prominent, but
mature, sebaceous glands. Apocrine glands are seen
focally. The features are those of an organoid nevus. 4
magnification.

Figure 3. Verrucous, flesh-colored plaque

4.0 2.5 cm on the right parietal scalp.

measuring

documentation of associated malignant tumors that were

reported to develop within NS. In addition to reporting
the number and types of neoplasms, we documented the
recommendations of all authors for therapeutic handling
of these nevi, with a particular focus on whether to excise
them prophylactically.
REVIEWS OF NS

Figure 2. Alopecic, waxy, yellowish plaque measuring

4.0 1.0 cm on the right frontal scalp. There is a central red
papule.

decrease the extent of the required surgery. Arguably, a

major factor when considering prophylactic excision of
NS is the risk of malignancies arising within the lesion,
but the true risk for malignancy is uncertain. BCC may
be the most common neoplasm arising in NS (2), but
some argue that the majority of tumors that were historically labeled as BCC were actually trichoblastomas,
which are benign basaloid proliferations (3,9). Accordingly, those who believe that the risk of malignancy is low
argue against prophylactic excision and instead opt for
close monitoring of the NS for any suspicious growths.
We performed an extensive literature search in
PubMed [19322010] of articles about NS. We included
case reports and a total of 21 reviews. We focused on

We evaluated 21 reviews of NS for the development of

secondary benign and malignant neoplasms (Table 1).
Four thousand nine hundred twenty-three cases of NS
were reviewed, and the average percentage of secondary
tumor development was then calculated. We found that
benign tumors developed in 16% of cases (range 051%)
and that malignant tumors developed in 8% of cases
(range 022%). The earlier studies report more malignant growths, a trend that shifted over time toward the
reporting of signicantly more benign growths developing within NS.
Four of the rst large studies with patient populations
of 150 (8), 160 (10), 140 (11), and 181 (12) were published
in the 1960s to 1980s and mostly had higher incidences of
benign neoplasms (1539%) and malignant neoplasms
(921%) compared to studies conducted from the 1990s
until the present. The vast majority of the malignant
lesions reported in these studies were BCC. The largest
study of 997 patients was published in 1979 (13) and had
a low incidence of benign (10%) and malignant (1%)
secondary tumors, although the authors excluded conventional BCC from their evaluation.
From the 1990s to the present, the largest studies
(N > 100) (3,9,1419) all had a lower percentage of
malignant tumors than our previously calculated average, and most had a lower incidence of benign tumors as

Moody et al: Nevus Sebaceous Revisited

17

TABLE 1. Reviews of Nevus Sebaceous (NS)

Author

Year of
publication

Patients, n

Mehregan (8)

1965

150

15

19

Pinol Aguade (47)

Michalowski (10)
Wilson-Jones (11)

1968
1969
1970

40
160
140

28
39

5
21
9

Domingo* (13)

1979

997

10

Serpas de Lopez (48)

1985

29

NA

10

Morioka (22)

1985

80

51

18

Smolin (12)

1986

181

12

Weng (43)

1990

62

32

19

Perez Oliva (49)

Chun (14)
Minami (15)

1991
1995
1998

5
7

13
0
5

Beer (26)

1999

18

22

Cribier (9)

2000

596

13

Jaqueti (3)
Kaddu (16)
Munoz-Perez (17)
Santibanez-Gallerani (18)
Taklif (50)

2000
2000
2002
2003
2004

155
316
226
658
42

36
7
18
<2
5

0
1
4
0
10

Simi (51)
Rosen (19)
Average

2008
2009

21
631 (651 NS)
234 NS

Benign, %

40
175 (225 NS)
136

Malignant, %

10
2
16 (Range 051)

5
1
8 (Range 022)

Type of malignant
neoplasm (n)
BCC (21)
KA (4)
Sebaceous ep (4)
BCC (2)
BCC (34)
BCE (9)
SCE (1)
Sebaceous epithelioma (ep) (1)
KA (2)
Adnexal carcinoma (3)
Apocrine carcinoma (4)
SCC (1) SCC adnexal (1)
BCC (2)
Leiomyoma (1)
BCE (10)
Apocrine ep (1)
Sebaceous ep (3)
BCC (21)
Prickle cell carcinoma (1)
BCC (6)
Sebaceous ep (6)
BCC (5)
BCE (4),
Sebaceous carcinoma (3)
BCC (3)
BCC KA (1)
BCC (5)
KA (4)
BCC (2)
BCC (8)
BCC (3)
Inverted KA (1)
SCC (1)
BCC (5)

*Excluded basal cell carcinoma (BCC) (not stated how many excluded).
KA, keratoacanthoma; BCE, basal cell epithelioma; SCC, squamous cell carcinoma.

well. Cribier et al (9) performed a retrospective study to

review all cases of NS surgically excised within their
institution between 1932 and 1998. They reviewed 596
cases and reported a low incidence of BCC (0.8%). More
than 90% of the tumors they found associated with NS
were benign trichoblastomas (9). They also describe a
common histologic change consistent with benign epidermal hyperplasia and suggested that many previously
reported cases of BCC arising in NS were benign trichoblastomas. Accordingly, they stated that the previous
recommendations for prophylactic excision might be
excessive and unnecessary. Instead, they recommended
close follow-up of NS to seek out any new growths or
changes indicative of malignancy. Jaqueti et al (3) came
to similar conclusions when examining a series of 155 NS.
They also report that the most common neoplasm arising
within NS is trichoblastoma. Moreover, these authors
examined all previous studies reporting higher incidences

of BCC and commented on the gures and images and

histologic descriptions. In doing so, they argued that
many of the claims of BCC within NS were trichoblastomas or benign basaloid proliferations. SantibanezGallerani et al (18), who studied 757 NS and did not nd
a single case of BCC, share this opinion. They argue that
BCC development is a function of age and cannot necessarily be attributed to NS. Most recently, in 2009
Rosen et al (19) found that, for 651 NS, only 1% of
patients had malignancies (all BCC), and 2% had benign
growths. The authors did not mention the development
of neoplasms in the remaining 97% of the NS.
MALIGNANT NEOPLASMS ARISING IN NS
Although locally aggressive or metastatic carcinomas
within NS are rare, they have been reported, and some
cases have been fatal (13,20,21). Historically, NS has

18 Pediatric Dermatology Vol. 29 No. 1 January February 2012

TABLE 2. Malignancies Developing in Nevus Sebaceous Reported in the Literature

Malignant tumor

Reported, n

In children, n (%)

References

BCC*
SCC
KA
Melanoma
Porocarcinoma
Adnexal carcinoma or adenocarcinoma
Apocrine carcinoma
Sebaceous carcinoma
Leiomyosarcoma or piloleiomyoma
Multiple malignancies
(SCC adnexal carcinoma, BCC sebaceous
carcinoma, BCC SCC, BCC KA)

119
15
14
1
1
5
7
33
3
6

14 (12)
7 (47)
3 (21)
0
0
0
0
0
0
0

(8,9,11,12,1517,19,2230,42,43,4850,5262)
(1,11,13,3133,51,6367)
(8,9,11,3436,50)
(68)
(69)
(13,21,70)
(13,7173)
(8,15,43,71,7479)
(48,80,81)
(2,13,20,26,82,83)

*Some authors have be questioned whether the BCC were trichoblastomas (3,9).
SCC, squamous cell carcinoma; KA, keratoacanthoma

been considered a premalignant lesion, and prophylactic

excision was recommended because of the risk of
malignant transformation (8). We have compiled a list of
the reported malignant tumors developing within NS
as determined from our extensive literature review
(Table 2). As expected, the most common malignancy
observed was BCC (119 reported), more than all of the
other kinds of malignant tumors combined. (This total
includes potentially misdiagnosed BCCs that may be
trichoblastomas (3,9). The next most common malignancy was sebaceous carcinoma (n = 33), followed by
squamous cell carcinoma (SCC) (n = 15) and keratoacanthoma (KA) (n = 14). Other tumors observed
included melanoma (n = 1), porocarcinoma (n = 1),
adnexal carcinoma or adenocarcinoma (n = 5), apocrine carcinoma (n = 7), and leiomyosarcoma or piloleiomyoma (n = 3). Occasionally, more than one
malignant lesion occurred in the same NS (n = 6).
Tumors observed together included BCC with SCC,
sebaceous carcinoma, or KA and SCC with adnexal
carcinoma.
Although most of these tumors develop in the third
stage of the evolution of NS (during adulthood), malignancies have been reported in children younger than
18 years old. We found that 12% of the BCC (19,2230),
47% of the SCC (1,3133), and 21% of the KA (3436)
were reported in children. This percentage may be higher
than the incidence of childhood malignancies in NS
because authors tend to write reports of unusual cases.
Nevertheless, it is important to recognize that malignancies have developed within NS in young children.
To Excise or Not to Excise?
The two main reasons to excise NS include concern
about malignancy and undesirable cosmesis. Once a
malignant lesion arises within NS, it is generally agreed

that the tumor and the entire nevus should be removed,

yet there have been changing recommendations with
regard to whether to excise NS prophylactically for the
sole purpose of decreasing the risk of malignant growths.
Table 3 illustrates the evolution of therapeutic management recommendations for NS in chronological order.
Whereas the majority of dermatologists used to advocate
early prophylactic excision to avoid the development of
malignant tumors, there is currently no consensus as to
whether NS should be excised prophylactically because
the true risk of malignancy within NS remains unknown.
Although earlier studies showed high rates of BCC with
strong recommendation for early prophylactic excision,
by the 1990s, larger studies showed lower rates of BCC
arising in NS, and authors questioned the high prevalence of BCC in the earlier studies, thinking instead that
these basaloid proliferations were misdiagnosed trichoblastomas. According to the nding that trichoblastomas, not BCC, predominate within NS, many
physicians now believe that close observation of the
lesion may be a better choice than prophylactic excision,
especially in cases in which the patient (and his or her
parents) not only have a thorough understanding of the
condition and associated risk, but are also willing to take
on the responsibility of recommended follow-up. Nevertheless, authors who report the development of invasive malignant tumors in NS often still recommend
prophylactic excision.
Additional considerations when determining whether
to remove NS surgically are cosmetic implications.
Sebaceous nevi most commonly develop on the face and
scalp and cause alopecia, with a signicant eect on
physical appearance. The natural history of the lesion
must also be taken into account, because these lesions
are often small and manageable during the infantile
stage yet tend to enlarge during puberty. The risks of
more extensive surgery, which can be performed in stages

Moody et al: Nevus Sebaceous Revisited

19

TABLE 3. Recommendations for and Against Prophylactic Treatment of Nevus Sebaceous

First author

Year
published

Mehregan (8)

1965

Lillis (42)

1979

Alper (84)

1983

Weng (43)

1990

Buescher (34)
Chun (14)

1991
1995

Hughes (25)
Stavrianeas (60)

1995
1997

Beer (26)
Jaqueti (3)

1999
2000

Cribier (9)

2000

Kaddu (16)

2000

Santibanez-Gallerani (18)
Taklif (50)

2003
2004

Rosen (19)

2009

Recommendation for prophylactic treatment?

In many cases, it may be advisable to do preventative excision or plastic surgery later in
childhood before the unsightly blossoming out in puberty may be anticipated.
This case suggests the importance of prophylactic removal and close follow-up of nevus
sebaceous.
It is the authors recommendation that these lesions be excised as soon after birth as is
practicable. The resulting scar will also be smaller than that inicted when the child is
fully grown.
Early excision of the lesion in childhood before it enlarges and matures is recommended.
to prevent malignant degeneration and further disguration of the patients appearance.
Careful clinical evaluation and consideration of earlier excision in certain cases.
If this same tendency prevails in other prospective studies, we strongly believe that
prophylactic excision of all nevus sebaceus are not warranted. Excision should be
recommended only when benign or malignant neoplasms are clinically suspected or for
cosmetic considerations.
should monitor nevus sebaceous in childhood and investigate changes.
The lesions should be closely monitored on a periodic basis, surgically completely excised,
especially before puberty, when sebaceous glands enlarge, and histologically examined
in detail.
to excise such tumors (NS) as early in childhood as possible.
On the basis of these ndings, the classically recommended treatment for this hamartoma,
which consists of early excision to prevent the development of malignancy, seems to be
inappropriate.
we believe that there is no reason to remove lesions of NSJ in early childhood.
Because most tumors occurred in adults older than 40 years, our study suggests that
prophylactic surgery in young children is of uncertain benet. Clinical follow-up is probably
sucient and even those cases with clinical changes often proved to be benign tumors or warts.
our large series together with recent observations may suggest a more conservative
approach. It is therefore prudent to advise treatment of these lesions (NS with TB or BCC)
with complete excision and appropriate clinical follow-up presently, while awaiting further
biological evidence to support the benign nature of TB.
question the need for prophylactic surgical removal of the nevus sebaceous.
On the basis of these ndings, prophylactic treatment which consists of early excision for
preventing the development of the malignancy is recommended for this hamartoma.
we believe all NS should be excised, primarily due to the risk of malignant transformation
and secondarily for cosmesis. Young age and uniform appearance of the lesion are not reliable
criteria to reassure that a lesion is truly benign. The timing of excision, while not an exact
science, is best determined when the surgeon, parent, and patient are actively involved in the
decision together.

to decrease complications, accompany excision of larger

lesions.
In addition to the low risk of malignancy, the risk of
surgery, especially in younger children, can be a deterrent
for the prophylactic excision of NS. Physicians and
parents may opt to monitor the NS closely and consider
excision only if changes occur within the lesion. For the
most part, dermatologic procedures are elective in nature
and are performed under local anesthesia, but in many
cases of lesions in children, the age of the patient may
make the use of local anesthesia dicult, because they
may not fully understand the procedure and cannot
cooperate. Multiple retrospective studies have evaluated
the risks of anesthesia use and surgery in pediatric populations; the nal result of the majority of these studies is
that anesthesia is safe in healthy children, with low
morbidity. The most commonly reported complications
are nausea and emesis (37,38). Reported complication

rates are generally low in dermatologic surgery (2%)

(37).
Therapeutic Recommendations
There are few literature reports outlining the surgical
management of NS (Table 4). Lesions should be excised
with 2- to 3-mm margins, which are the current recommendations for BCC (7,39). Full-thickness dermal excision is necessary, because NS extends at least as deep as
the subcutaneous tissue, with involvement of adnexal
structures (7,40).
Davison et al (7) performed a retrospective review of
13 people with NS with lesions located on the temporal
scalp region to evaluate cosmetic results after surgery.
The temporal scalp, anterior hairline, and forehead are
prominent, cosmetically sensitive facial landmarks that
surgical manipulation aects greatly (7). In this study,

20 Pediatric Dermatology Vol. 29 No. 1 January February 2012

TABLE 4. Excision and Reconstruction Options for Nevus Sebaceous (NS)

First author

Year
published

Patients, n
(Location)

Excision or reconstruction
recommended

Mehregan (8)

1965

150

Necessary to do a full-thickness
excision and can excise large nevi
in several stages without skin grafts

Lillis (42)

1979

Weng (43)

1990

62

Ashino (44)

1993

Hughes (25)

1999

18

Surgically remove and examine

histologically

Davison (7)

2005

13 (temporal
scalp)

Donelan (41)

2008

In (45)

2010

12 (face)

Excise NS with at least 2- to 3-mm

margins
Full-thickness excision (stop at level
of temporoparietal fascia to
preserve nerves)
Rotation ap based on the
supercial temporal artery
Expander and closure with a purse
string technique
CO2 laser MAL PDT
(light-emitting diode)

Kim (46)

2010

1 (face)

1 (scalp)

Prefers surgical excision (specically

Mohs surgery) over electrodesiccation
and curettage
Recommends full-thickness, complete
excision with primary reconstruction

Three treatments with CO2 laser,

power density of 191 W cm2

16 treatment sessions: ALA or

MAL PDT (intense pulsed light, red
light, CO2 laser, Er:YAG laser, or
fractionated Er:YAG laser)

Specics
Presence of apocrine glands requires
full-thickness excision
To remove in stages, a longitudinal
strip is removed, the skin is sutured,
and the scalp is given time to stretch
before another strip is removed
Incomplete removal could lead to
recurrence
Does not recommend electrocautery,
fulguration, or supercial destruction of
the lesion because they may provoke
cellular transformation or mask
potentially malignant changes in NS
Greatly improves cosmetic appearance
but does not remove the entire lesion
(only that part in the epidermis and
papillary dermis)
Do not treat using dermabrasion or
dermablation, because they may miss
epithelialization and leave malformed
parts of NS, because the treatments
remove only the part of the lesion in the
epidermis and upper dermis
Excellent blood supply of the supercial
temporal artery system
Preserves hair on scalp
Cosmetically good results
Minimizes extension into forehead skin
Two expansions
No lasting alopecia
25% had 2550% improvement
58% had 5075% improvement
17% had >75% improvement
Transient improvement

CO2, carbon dioxide; MAL, methylaminolevulinate; PDT, photodynamic therapy; Er:YAG, erbium-doped yttrium aluminum garnet.

they used a temporal rotation ap to close the surgical

defects, preserving scalp hair and maintaining the natural
hairline. Their conclusions outline surgical recommendations for complete excision and cosmetically elegant
repair: Primary excision of NS with a minimum of 2 to
3-mm margins; full-thickness excision through the epidermis, dermis, subcutaneous tissue, and underlying fat;
stopping the excision at the level of the temporoparietal
fascia to preserve branches of the facial nerve; because
the ultimate defect is often too large for primary closure,
use of a posterior inferiorly based rotation ap, taking
advantage of the rich supercial temporal artery blood
supply. To minimize alopecia, they suggest avoiding the
use of resorbable sutures around hair follicles (which
leads to inammation). They use staples, which results in
less irritation when removed within 7 days.

Tissue expansion might be necessary to aid in the

closure of large defects resulting from the excision of
large NS lesions. Tissue expansion procedures consist of
multiple steps and a substantial amount of time with
expansion materials in place; these intervals are stressful
for patients and their families, but the results tend to be
favorable. Donelan and Garcia (41) present the case of a
9-year-old boy with a large (11 13 cm) NS on his vertex
scalp. His rst procedure involved the use of an 18-cmdiameter round expander that remained in place for
11 weeks. The defect was closed using a purse string
technique, with minimal subsequent alopecia. A second
surgery with an 8-week expansion was performed 2 years
later. The end result was excellent, with no lasting
alopecia. For large NS lesions, Mehregan et al (8)
recommend removing the tumor in stages. During each

Moody et al: Nevus Sebaceous Revisited

stage, a longitudinal strip is removed, the skin is sutured

together, and the scalp is given time to stretch before
another strip is removed at a later date (8).
There is a balance between the risk of surgery and that
of potential malignancy. Those who fear the risk of
malignancy consider treatments other than surgical
excision to be ill advised. Lillis et al (42) argue that
electrodesiccation and curettage could lead to incomplete
removal with subsequent risk for recurrence. Weng et al
(43) warn that electrocautery, fulguration, or supercial
destruction of the lesion may provoke cellular transformation or mask potentially malignant changes in NS.
Hughes et al (25) state that NS should not be treated
using dermabrasion or dermablation, because these
modalities may miss epithelialization and leave parts of
NS, because the treatments remove only the part of the
lesion in the epidermis and upper dermis, leaving the deep
dermal portion fully intact. Ashino (44), who describe
their technique of cosmetically improving the appearance of a NS using a carbon dioxide (CO2) laser, state
that this treatment is not eective for removing the entire
lesion.
The use of photodynamic therapy (PDT) with
aminolevulinic acid (ALA) or methylaminolevulinate
(MAL) has shown contradictory results. In et al (45)
report good results with treatments with a CO2 laser
followed by MAL PDT with light-emitting diodes. Of 12
patients, 25% had 25% to 50% improvement, 58% had
50% to 75% improvement, and 17% had better than
75% improvement. On the other hand, Kim et al (46)
found only transient improvement in their one patient
after 16 treatments with ALA or MAL uisng a variety of
dierent light sources.
CONCLUSION
Prophylactic excision may be considered for NS in all
healthy children. Because these lesions tend to occur in
cosmetically apparent regions and may result in alopecic
areas of the scalp, they have the potential to warrant
excision solely for cosmetic concerns. Despite the fact
that risk of malignant transformation may not be as high
as previously believed, there is still the potential for
malignant tumors to develop within NS in children and
adults; metastases and death have also been rarely
reported.
It may be less complicated to excise a lesion early in
childhood, before the expansion phase of the NS, when
the lesion is small, facilitating a less complicated procedure and a less noticeable scar. The risks associated with
general anesthesia are low for healthy children, and the
need for general anesthesia does not pose a substantial
obstacle to the prophylactic excision of NS. An

21

additional consideration is advocacy for personal

healthcare decision-making and ethics; should excision
be put o until the aected child is old enough to be part
of the discussion and decision-making process? Patients
and their parents should weigh the cosmetic appearance of NS against a potential scar from the removal;
together, they should reach a collaborative decision
about whether to proceed with excision.
Lasers and photodynamic therapy are currently being
explored for treatment of NS, with varying degrees of
success. When treating NS with anything other than
excision, it is crucial to determine how much of the lesion
is being removed. If fragments of the NS remain, recurrence may occur, and secondary neoplasms can subsequently develop. If a tumor develops within NS, we
recommend removal of the tumor and the entire NS
using excision with frozen section examination of the
margins. Mohs micrographic surgery ensures complete
removal of the entire tumor with clear margins to
decrease the risk of recurrence (32,38).
Although prophylactic removal may be warranted in
many cases of NS, each case should be evaluated separately. Some patients with NS may have an accompanying syndrome (didymosis aplasticosebacea and
SCALP syndrome (6)), which may complicate the surgery and be a contraindication for general anesthesia.
In these cases, observation and close follow-up are the
ideal recommendation, although in healthy patients with
NS, patient adherence to a follow-up protocol must be
strongly considered when deciding whether to remove or
observe the lesion. Some patients and their parents may
have overwhelming stress about the cosmetic appearance
and malignant risks associated with NS. Ultimately, the
decision of whether to excise prophylactically should be
determined after a thorough review of the options and
personal evaluation of the patient and his or her medical
history.
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