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Submitted: 28.10.2017 DOI: 10.1111/ddg.13546


Articl
Accepted: 10.4.2018
Conflict of interest
None.

Scalptumors

Christine Maria Prodinger, Summary


Josef Koller, Martin Laimer
Tumors of the scalp are characteriz ed by an impressively broad and heterogeneous clinical spectrum. They frequently exhibit site - specific features distinguishing them from their counterparts els ewhere on the skin. Although mostly benign, dia gnosis and treatment of these le sions may pose a significant challenge due to impair ed vi - sibility (and thus delayed detection), anatomical circumstances, exposure to (exo - genous) noxious agents, dis tinct his tological features, as well as the often - advanced age of affected indiv iduals . This is even more true for malignant tumors of the scalp, which are uncommon but associated with a poor prognosis . Adequate patient care therefore requires interdis ciplinary management. Against this background,
the pre - sent article addresses general prin ciple s and dis tinct features of the most important tumors of the scalp.

Department of Dermatology, Salzburg


Regional Medical Center, Paracelsus
Medical University, Salzburg, Austria

Section Editor
Prof. Dr. D. Nashan, Dortmund

I
T
M

Although the in cidence of tumors ari - Although the incidence of tumors arising on the
is increased compared sing on the scalp is increased compared to those occurring elsewhere
the skin, these neoplasms are fortu nately predo - to those occurring elsewhere on the min
benign. Cysts constitute over 50 % of benign scalp tumors and primarily skin, these neopla
are fortunately include trichilemma l, epidermoid and dermoid cysts; the estima ted prevalence
predominantly benign. the Western population is roughly 20 % [2 ] . Not least be
of the hig h density of sebaceous glands, the scalp is the most common site
trichilemma l cysts, ac - counting for approximately 80 % of such lesions. There
numerous other benign scalp tumors such as lipoma, fi broma, pilomatricoma (c
ed cyst), sebor

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While only approximately 1–2 % of all While only approximately 1–2 % of all scalp tumors are malignant, they com-
scalp tumors are malignant, they com- prise up to 13 % of all malignant cutaneous neoplasms [4]. However, relevant
prise up to 13 % of all malignant cuta- epidemiologic data is sketchy. In one of the few more comprehensive studies, Chui
neous neoplasms. et al. examined 398 Taiwanese patients with malignant scalp tumors. They found
that, in terms of sheer frequency, basal cell carcinoma (41.2 %) and squamous cell
carcinoma (16.6 %) were the most common lesions (as in our part of the world),
with a peak incidence between 60 and 79 years, followed by metastases, adnexal
tumors, angiosarcoma as well as lymphoma in decreasing order of prevalence [5].
Against this complex background, the present article is intended to provide a
concise overview of general principles and distinct features of the most important
scalp tumors, without claiming to be exhaustive. The interested reader is referred
to relevant publications.

Special characteristics of scalp tumors


Not only are scalp tumors characterized Not only are scalp tumors characterized by an impressively broad and heteroge-
by an impressively broad and heteroge- neous clinical spectrum, they frequently exhibit site-specifi c features that distingu-
neous clinical spectrum, they frequent- ish them from their counterparts elsewhere on the skin (Table 1). Examples of these
ly exhibit site-specific features that dis- peculiarities will be discussed below.
tinguish them from their counterparts
elsewhere on the skin.
Nonmelanoma skin cancer
Nonmelanoma skin cancer, predo- Nonmelanoma skin cancer, predominantly basal cell carcinoma and squamous cell
minantly basal cell carcinoma and carcinoma, is the most common type of skin cancer among Caucasians, showing
squamous cell carcinoma, is the most a continuously increasing incidence. Overall, 2–18 % of basal cell carcinomas and
common type of skin cancer among 3–8 % of squamous cell carcinomas are located on the scalp [4, 6, 7]. In this
Caucasians, showing a continuously particular location, both tumors are clinically characterized by a greater tendency
increasing incidence. for ulceration. Thus, they more frequently present as chronic, non-healing ulcer,
compared to elsewhere on the skin. Partly because squamous cell carcinomas of
the scalp are often diagnosed at an advanced stage (which usually correlates with
tumor diameter and depth of invasion), this location may be considered an inde-
pendent risk factor (along with degree of differentiation and evidence of perineu-
ral infi ltration) for invasiveness, increased risk of recurrence and therefore a more
unfavorable prognosis. However, this is not yet refl ected in current classifi cations,
including the UICC (2009) and the AJCC (2011) classifi cation [8–13].
Basal cell carcinomas, too, recur more often on the scalp. In rare cases, they
In this particular location, both tumors may even infi ltrate the cranium and dura (estimated incidence 0.03 %), especially
are clinically characterized by a greater when neglected or in case of particularly advanced disease [14–17]. Compared to
tendency for ulceration. Thus, they elsewhere on the skin, basal cell carcinomas of the scalp are more often nodular
more frequently present as chronic, and also pigmented. Some cases therefore meet the clinical and dermoscopic cri-
non-healing ulcer, compared to else- teria for a melanocytic lesion, which may render it diffi cult to distinguish them
where on the skin. from melanoma. For example, dermoscopy of basal cell carcinomas in this loca-
tion more often shows blue-gray ovoid nests, brown-black dots/globules, radial
lines connecting to a common base, a blue-white veil and usually two or more
melanocytic patterns at the same time [18, 19].

Melanocytic scalp tumors


Similar to melanocytic nevi of special si- Clinical subtypes of melanocytic scalp lesions include common, papillomatous,
tes (acral, genital), there is an increased congenital, blue, atypical and eclipse nevi (with central hypopigmentation). Der-
prevalence of histological features of moscopy usually shows a globular and globular-reticular pattern and prominent
dysplasia in scalp nevi. perifollicular hypopigmentation. Similar to melanocytic nevi of special sites (acral,

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Table 1 Examples of site-specific characteristics of important scalp tumors. (↑ = increased occurrence).

Scalp tumors Subtype Epidemiology Site-specific characteristics


(percentage of be-
nign and malignant
scalp tumors)
Benign (98–99 %) Trichilemmal cyst 40 %
Lipoma 30 %  Note: sometimes difficult to distinguish from
well-differentiated liposarcoma by fine need-
le aspiration or biopsy
Melanocytic nevi 28 %  ↑ dysplastic features
Dermoscopy:↑globular, globular-reticular
pattern, perifollicular hypopigmentation
↑blue nevi withGNA11mutation (note: in-
creased risk of malignant transformation)
Malignant (1–2 %) Basal cell carcinoma 41 %  ↑ tendency for ulceration, recurrence
↑nodular, pigmented subtype
Dermoscopy:↑melanocytic pattern

Squamous cell carcinoma 16 %  ↑ tendency for ulceration, invasion, recurrence

Metastases 12 %  Alopecia neoplastica: hematogenous breast


cancer metastases
(histological) correlation with primary tumor
essential for diagnosis
Lymphoma 5%  Mycosis fungoides;, folliculotropic variant
more common
Cutaneous B-cell lymphomas show a predi-
lection for the scalp
Note: transcranial invasion of aggressive
subtypes
Angiosarcoma < 1 % of all scalp tumors  Highly aggressive (invasive, recurrent)
Note: clinically mimics hematoma, rosacea,
erysipeloid, radiodermatitis
Melanoma < 1 % of all scalp tumors  ↑ tendency for ulceration, lymphovascular
infiltration, local recurrence
 ↑ desmoplastic subtype
↑Breslow thickness (mean: 2.4 mm ± 1.66)

Adnexal carcinoma < 1 % of all scalp tumors  ↑ invasiveness


 ↑ lymphovascular infiltration
Merkel cell carcinoma < 1 % of all scalp tumors  ↑ invasiveness

genital), there is an increased prevalence of histological features of dysplasia in scalp


nevi [20].
Blue nevi, which usually present as fl at, blue-gray to blue-black pigmented lesions
with a diameter of less than one centimeter, can grow to a size of several centimeters,
especially the cellular variant. They are marked by an increased risk of malignant
transformation, especially when there are mutations in the GNAQ and GNA11 gene.
GNA11-mutated blue nevi – similar to blue nevus-associated

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Figure 1 70-year-old patient. Well-differentiated squamous cell carcinoma (ulcera-


ted nodule; black arrow) in association with lentigo maligna melanoma (pigmented
area; white arrow) on actinically damaged skin in the parietal region (a). Junctional
proliferation of atypical melanocytes with prominent dendrites and solar elastosis,
consistent with melanoma in situ (lentigo maligna type). In addition, there is scar
tissue with focal evidence of squamous cell carcinoma (bottom right). (Immunohis-
tochemical staining for S100, original magnification x 40) (b). Well-differentiated
squamous cell carcinoma (black arrow) (hematoxylin-eosin stain, original magnifi-
cation x 40) (c).

Blue nevi are marked by an increased melanoma in general and melanoma mimicking cellular blue nevus – show a mar-
risk of malignant transformation, espe- ked predilection for the scalp [21].
cially when there are mutations in the Three to six percent of primary melanomas are located on the scalp. Men are
GNAQ and GNA11 gene. GNA11-muta- more frequently affected. Common types include superfi cial spreading, nodular
ted blue nevi – similar to blue ne- and lentigo maligna melanoma with a high mitotic rate (> 3/mm). A more ag-
vus-associated melanoma in general gressive variant, desmoplastic melanoma, too, shows a predilection for the scalp
and melanoma mimicking cellular blue (about 20 % of all cases) [22] (Figure 1). On dermoscopy, pigmented lesions in
nevus – show a marked predilection for this location are characterized in particular by areas of regression and diffuse
the scalp. hypopigmentation. Histologically, they exhibit an average (Breslow) thickness of
2.4 mm (± 1.66) and show a tendency for ulceration, lymphovascular infi ltration
and local recurrence. At the same time, the rate of positive regional sentinel lymph
A more aggressive variant, desmo- nodes (SLNs) is lower; however, this may also be attributed to false-negative SLN
plastic melanoma, too, shows a fi ndings. Potential reasons for the latter may include site-specifi c methodical li-
predilection for the scalp (about 20 % mitations (more frequent extracervical location of SLNs), the presence of multiple
of all cases). nuchal/cervical SLNs or inability to detect them due to diffuse lymphatic drainage.
Patients with undetectable SLNs are known to have a poorer prognosis compared
with SLN-positive patients [23–25]. Scalp melanoma, which accounts for 10 %
of melanoma-related deaths, generally has a poorer prognosis than melanoma at
other sites. Studies suggest that it is not just the location itself that is a risk factor.
Rather, the clinical and pathological triad of tumor thickness, SLN involvement
and ulceration, which is less favorable overall on the scalp, has a major impact
on overall survival. Lymph node metastases (in the form of micrometastases wi-
thout lymphadenopathy occurring in 30 % of all patients [26]), distant and, in
particular, cerebral metastases are more common in patients with scalp melanoma
[23, 24, 27–32].

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Table 2 Classification of the most important adnexal tumors based on their predominant histomorphological pattern (seba-
ceous vs. follicular differentiation [92].

Classification/characteristics Examples
Benign tumors Sebaceous adenoma
-
Malignant tumors Extraocular sebaceous gland carcinoma
Tumors with predo
minant sebaceous

Mantleomas Fibrofolliculoma
differentiation

Cysts Steatocystoma
Nonneoplastic / hamartomatous tumors Sebaceous nevus
Ectopic sebaceous glands and related lesions
Follicular germ cell differentiation Basal cell carcinoma
Biphasic epithelial-mesenchymal tumors with differentiation Fibroepithelioma
toward follicular germ cells and specific follicular stroma
Predominant matrix differentiation Pilomatrixoma
Differentiation toward outer root sheath Proliferating trichilemmal tumor
Tumors with predominant

Infundibular differentiation Trichoadenoma


Panfollicular differentiation Fibrous papule

folliculardifferentia tion
Follicular cysts Trichilemmal cyst
Nonneoplastic/hamartomatous tumors Basaloid follicular hyperplasia

Adnexal tumors
Clinically, benign adnexal tumors Given that cutaneous adnexa are complex epithelial, myoepithelial and mesenchy-
usually present as firm, elastic, non-ul- mal structures, the neoplasms originating therefrom are highly varied. Not sur-
cerated, skin-colored or erythematous/ prisingly, about one in ten malignant scalp tumors is of adnexal origin [5]. They
bluish and frequently hairless lesions originate in hair follicles, sebaceous glands and more rarely in eccrine and apocrine
of varying sizes (note: hair loss is an sweat glands or their ducts. Thus, the histomorphological spectrum is broad. With
unfavorable sign). They typically remain the exception of eccrine glands, said structures originate from a common embryo-
asymptomatic for a long time and grow nic primordium, which not uncommonly leads to a single tumor exhibiting mixed
very slowly. On the other hand, rapid differentiation (Table 2).
growth and ulceration, especially in el- Adnexal tumors can occur both sporadically and as part of rare genetic disor-
derly patients, raise the suspicion of ma- ders such as Birt-Hogg-Dubé, Brooke-Spiegler, Cowden or Muir-Torre syndrome
lignancy or malignant transformation. (Table 3). Early occurrence – starting at puberty – of multiple monomorphic lesions
in particular corroborates the clinical suspicion of a predisposing syndrome. Spo-
radic cases usually manifest themselves in the 5th or 6 th decade [33–36].
Clinically, benign adnexal tumors usually present as fi rm, elastic, non-ulcera-
ted, skin-colored or erythematous/bluish and frequently hairless lesions of varying
sizes (note: hair loss is an unfavorable sign). They typically remain asymptomatic
for a long time and grow very slowly. On the other hand, rapid growth and ulcera-
tion, especially in elderly patients, raise the suspicion of malignancy or malignant
transformation. In familial tumor syndromes, such alarm signs are often observed
at a young age.
A (predominantly) benign adnexal tumor with a marked predilection for
the scalp, proliferating trichilemmal cyst (PTC) primarily affects older women.
It usually presents as a solitary nodulocystic lesion, measuring 1–10 cm, and is
thought to originate from a preexisting trichilemmal cyst. Histologically, it may

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Table 3 Examples of hereditary syndromes associated with a greater risk of developing scalp tumors [34, 93].

Syndrome Associated scalp tumors Other associated symp- Inheritance Mutations


toms (selection)
Gorlin-Goltz syndrome Basal cell carcinoma Skeletal anomalies, intracra- AR PTCH1 + 2,
nial calcifications, keratocy- SUFU
stic odontogenic tumors
Xeroderma pigmentosum Squamous and basal cell carci- Photosensitivity, freckles, AR DDB2, ERCC2
noma, melanoma (initial onset poikiloderma, CNS ano- (-5), XPA-G, XPV
during childhood) malies
Schimmelpenning Extensive sebaceous nevus CNS, ocular malformations, Mosaic HRAS, KRAS,
syndrome along Blaschko’s lines horseshoe kidney NRAS
Phacomatosis Sebaceous nevus + (papular) Vascular malformations, he- Mosaic HRAS
pigmentokeratotica nevus spilus along Blaschko’s miatrophy, CNS anomalies
lines
Didymosis Sebaceous nevus with aplasia
aplasticosebacea cutis congenita
SCALP syndrome Sebaceous nevus, congenital Aplasia cutis, CNS
melanocytic giant nevus anomalies
Brooke-Spiegler syndrome Cylindroma, trichoepithelioma, Increased carcinoma risk AD CYLD
milia, spiradenoma
Familial cylindromatosis Cylindroma, spiradenoma, Increased carcinoma risk AD CYLD
trichoepithelioma
Cowden syndrome Trichilemmoma Mucosal fibromas, AD PTEN
increased carcinoma risk,
intestinal polyps
Birt-Hogg-Dubé syndrome Fibrofolliculoma (≥ 5 are pa- Lung cysts, pneumothorax AD FLCN
thognomonic), trichodiscoma,
skin tags
Muir-Torre syndrome Sebaceoma, sebaceous carcino- Increased carcinoma risk AD MSH2+1, SH6
ma, keratoacanthoma
Gardner syndrome Infundibular cysts, hybrid cysts Soft tissue fibromas, AD APC
with matrix component, miliary colorectal adenomas/
osteoma cutis adenocarcinoma
Oldfield syndrome Steatocystoma, trichilemmal Multiple colon polyps
cysts
Multiple pilomatricomas Pilomatricoma Myotonic dystrophy
(Steinert’s disease)
Bazex-Dupré-Christol Basal cell carcinoma, milia Hypotrichosis, follicular XD BZX
syndrome atrophoderma
Rombo syndrome Basal cell carcinoma, Atrophoderma vermicularis AD
trichoepithelioma with follicular keratosis
Costello syndrome Syringoma Woolly hair, cardiomyopa- AD HRAS
thy, mental retardation
Abbr.: AR, autosomal recessive; AD, autosomal dominant; XD, X-linked dominant.

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be diffi cult to make the distinction between PTC and squamous cell carcinoma as
desmoplasia and/or proliferation of the epithelium into the cyst lumen may mimic
pseudoinvasion. Trichilemmal keratinization, sharp demarcation, presence of a
preexisting cyst and peripheral palisading are suggestive of PTC, whereas histolo-gical
signs of malignancy include nuclear pleomorphism, increased mitotic activity, necrosis
and ulceration [33, 35].
Virtually every benign adnexal tumor has a malignant counterpart. Though
uncommon, given their aggressive local growth pattern and increased risk of lymph
node (7.4 %) and distant metastasis (12.2 %), they are associated with a poor
prognosis. The subgroup of malignant adnexal tumors has a 5-year overall and disease-
specifi c survival rate of 73 % and 98 %, respectively [36–38].
Characterized by infi ltrative local growth, high recurrence yet low metastatic
rates, primary cutaneous adenoid cystic carcinoma also has a predilection for the scalp.
So does mucinous eccrine carcinoma, which primarily occurs in elderly in-dividuals.
The latter frequently presents as a soft, non-ulcerated nodule that tends to recur; it
metastasizes in 15–20 % of all cases. Given their overlapping characte-ristics, it is
often challenging to make an unequivocal clinical and histological dis-tinction between
(primary) malignant adnexal tumors of the scalp and cutaneous metastases (for
example, sebaceous carcinoma versus a breast cancer metastasis with sebaceous
differentiation) [35, 39, 40].

Merkel cell carcinoma


Compared with melanoma, this tumor
– pathogenetically associated predomi- The scalp is one of the predilection sites for Merkel cell carcinoma (MCC), too. nantly (> 80
%) both with UV radiation Compared with melanoma, this tumor – pathogenetically associated predominant-and with Merkel cell
polyomavirus – is ly (> 80 %) both with UV radiation and with Merkel cell polyomavirus – is less
less common but has a higher mortality common but has a higher mortality rate (> 15 %). The embryonic origin of Merkel rate (> 15
%). cells remains subject to controversial debate. Initially, they were thought to origi-nate in the neural crest, which is why MCC used to
be classifi ed within the group of neuroendocrine tumors. Recent studies, however, point to an epidermal origin of these pale oval cells,
which are located in the basal layer; the majority of Merkel

While, unlike melanoma, a tumor thick- cells are in close contact with sensory nerve endings [41]. Apart from ectodermal ness of less
than 10 mm correlates only keratinocytes, it has also been suggested that mesodermal fi broblasts and even slightly with clinical stage
and rate of pre-/pro-B cells might be the origin of this tumor [42, 43]. While, unlike mela-metastasis, the primary location on the
noma, a tumor thickness of less than 10 mm correlates only slightly with clinical scalp is a distinct risk factor for larger stage and rate
of metastasis, the primary location on the scalp is a distinct risk fac-MCCs associated with distant metas- tor for larger MCCs
associated with distant metastasis (8.7 %), which reduces the tasis (8.7 %), which reduces the median median survival to nine months.
By comparison, the 5-year survival rate is 51 %
survival to nine months. for localized MCCs and 35 % in case of nodal involvement [44–46].

Lymphomas
Even though the skin is (after the gastrointestinal tract) the second most common site
of extranodal non-Hodgkin’s lymphoma (NHL) (estimated incidence in the skin 1 in
100,000), scalp lymphomas are very uncommon [47].
The most common B-cell lymphomas occurring on the scalp are primary cuta-
neous follicle center lymphoma and primary cutaneous marginal zone lymphoma, both
of which have an excellent prognosis, with a 5-year survival rate of > 95 % and
< 100 % respectively (Figures 2, 3). In rare cases, primary cutaneous diffuse large B-
cell lymphoma can present as a painless, slowly growing scalp tumor, associated with a
poor prognosis and a median survival of two to five years. Lymphoma cells can infi
ltrate the cancellous bone (of the skull) and spread via communicating veins into

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Figure 2 64-year-old patient. Asymptomatic, coalescing erythematous plaques on


the forehead / around the hairline as well as in the left parietal region, some with
central pseudovesicles; diagnosis: cutaneous follicle center lymphoma (a). Histolo-
gy shows dense lymphocytic infiltration of the dermis and formation of germinal
centers (CD-20 +, CD-21 +, bcl-6 +) (hematoxylin-eosin stain, original magnification
x 40 (b). Colonization of germinal centers by bcl-2 positive cells from the mantle
zone (insert).

Figure 3 Multifocal follicle center lymphoma on the scalp of a 69-year-old female


patient, presenting as disseminated, rather subtle, asymptomatic violaceous
plaques.

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the soft tissues on both sides of the cranium. Progressive intracranial expansion sub-
Among T-cell lymphomas, mycosis fun- sequently leads to headaches, neurological defi cits and epileptic seizures [3, 48–50].
goides (predominantly with large-cell Among T-cell lymphomas, mycosis fungoides (predominantly with large-cell
transformation) is the most common transformation) is the most common type occurring on the scalp, not rarely in
type occurring on the scalp, not rarely the form of the prognostically more unfavorable folliculotropic variant (follicu-
in the form of the prognostically more lar papules; with/without mucin). Finally, primary cutaneous large-cell anaplastic
unfavorable folliculotropic variant (folli- lymphoma, with a good 10-year survival rate of approximately 90 %, also occurs
cular papules; with/without mucin). in this region [51, 52].

Sarcomas
Typical clinical features of this (usually Among the heterogeneous group of sarcomas, all of which are of mesenchymal
sporadic) tumor are a highly aggressive origin, angiosarcoma in particular (1 % of all sarcomas) has a predilection for
course associated with a tendency for the scalp. Typical clinical features of this (usually sporadic) tumor are a highly ag-
multifocal spread and early hemato- gressive course associated with a tendency for multifocal spread and early hemato-
genous dissemination. In the head genous dissemination. In the head region in particular, there is a high rate of local
region in particular, there is a high rate recurrences, even if the tumor was initially excised with wide (2–3 cm) surgical
of local recurrences, even if the tumor margins. Accordingly, the 5-year survival rate is only 10–30 %. Not infrequently
was initially excised with wide (2–3 cm) is angiosarcoma initially diagnosed following a pneumothorax, which is due to its
surgical margins. tendency for subpleural metastasis [53, 54].
There have also been reports of liposarcoma arising on the scalp, with the
It must also be borne in mind that the myxoid subtype being the most common variant. Rapid growth and potential tis-
distinction between well-differenti- sue necrosis can make it diffi cult to distinguish such a lesion from an infected be-
ated liposarcoma and lipoma using nign trichilemmal cyst. It must also be borne in mind that the distinction between
fine-needle aspiration or tissue biopsies well-differentiated liposarcoma and lipoma using fi ne-needle aspiration or tissue
may at times be challenging or even biopsies may at times be challenging or even impossible [55].
impossible.
Metastases
The scalp’s vascularity likely facilitates The scalp’s vascularity likely facilitates the relatively common occurrence of me-
the relatively common occurrence of tastases in this region, predominantly from visceral tumors [56]. In women, over
metastases in this region, predominant- 70 % of metastases are attributable to breast cancer, followed by ovarian, lung,
ly from visceral tumors. gastrointestinal and oropharyngeal cancer. In men, 10 % of metastases origina-
te from the lung and gastrointestinal tract, followed (in decreasing frequency) by
ENT/esophageal tumors and renal tumors [57]. Clinically, metastases typically
present as highly indurated, indolent lesions. Alopecia neoplastica is a distinct,
site-specifi c clinical presentation, which is usually caused by hematogenous breast
Histological assessment of metastases cancer metastases. Tumor infi ltration and the infl ammation associated therewith
always requires correlation with the pri- lead to the destruction of hair follicles, clinically characterized by hair loss, indura-
mary tumor, given that different tumor ted skin and telangiectasia.
types – especially on the scalp – can show Histological assessment of metastases always requires correlation with the
morphological and immunohistochemi- primary tumor, given that different tumor types – especially on the scalp – can
cal overlap with metastases originating show morphological and immunohistochemical overlap with metastases origina-
from tumors of other organ systems. ting from tumors of other organ systems [58].

Pediatric scalp tumors


Lack of lesional mobility or a midline Pediatric scalp tumors are relatively rare and fortunately mostly (> 98 %) benign.
location should prompt an initial radio- Tumorous scalp lesions in this age group therefore predominantly have no neopla-
graphic evaluation to rule out cranial stic origin (Table 4). In this context, lack of lesional mobility or a midline location
(meningo-)encephalocele. should prompt an initial radiographic evaluation to rule out cranial (meningo-)
encephalocele [59].

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Table 4 Differential diagnoses of (commonly benign) scalp tumors in children [94].

Congenital Epidermal cyst, dermoid cyst, meningocele,


encephalocele, craniosynostoses
Neoplastic – benign Neurofibroma, osteoma, fibrous dysplasia, osteoid
osteoma, ossifying fibroma, giant cell tumor, aneurys-
mal bone cyst, lymphangioma, meningioma
Neoplastic – malignant Neuroblastoma, lymphoma, sarcoma (Ewing’s,
osteogenic, meningeal), glioma
Posttraumatic Scalp hematoma, traumatic leptomeningeal cyst,
subgaleal hematoma, subdural hygroma
Vascular Hemangioma, pericranial sinus, arteriovenous fistula/
cirsoid aneurysm
Inflammatory Lymphadenopathy, abscesses, necrobiotic nodules,
cranial fasciitis, osteomyelitis
Idiopathic Langerhans cell histiocytosis, myofibromatosis,
osteitis deformans (Paget’s disease)

With an incidence of 0.5–1 %, nevus sebaceus (NS) is among the most com-mon
benign scalp tumors in children. A complex hamartoma, NS is characterized
– among other things – by sebaceous and sweat gland hyperplasia with absence of
terminal hair follicles within the lesion. The lesion is caused by postzygotic muta-tions
in the HRAS (95 % of cases) and KRAS genes (5 %), which also explains its linear
arrangement along Blaschko’s lines (mosaic RASopathy). While only about 1.7 % of
pediatric sebaceous nevi give rise to – largely benign – tumors, approxi-mately 10–30
% of adult cases are associated with complications in the form of syringocystadenoma,
trichoblastoma or trichilemmoma (Figure 4). Basal cell carci-noma (occurs in 3.5 % of
all sebaceous nevi) and other malignant epithelial tumors such as malignant sebaceous
gland carcinoma tend to be an exception in adults, too; female patients are
predominantly affected [60]. Occasionally, sebaceous nevi

Figure 4 Sebaceous nevus of the left temple in a 23-year-old patient presenting as


characteristic waxy reddish-yellow papules; there is a nodular basal cell carcinoma
arising in the medial aspect of the lesion.

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are associated with various syndromes (including RASopathies) (Table 3). While
prophylactic excision is not always necessary for the aforementioned reasons, pro-
minent alopecia due to a sebaceous nevus may still be highly stigmatizing. Given the
relatively small size of lesions during infancy, early surgical removal (usually
associated with very good cosmetic outcomes) may therefore be considered in such
cases [61].

Pathogenetic principles of scalp tumors


UV exposure and reduced UV protecti- UV exposure and reduced UV protection due to sparse hair (early childhood,
on due to sparse hair (early childhood, [androgenetic] alopecia) or Fitzpatrick skin types I and II are considered the most
[androgenetic] alopecia) or Fitzpatrick important risk factors for developing (malignant) scalp tumors. Gender-specifi c
skin types I and II are considered the differences in average hair length and in the incidence and clinical pattern of (e.g.,
most important risk factors for develo- androgenetic) alopecia with resultant differences in the intensity of cumulative UV
ping (malignant) scalp tumors. exposure of the scalp also have an impact with respect to typical gender-related
predilection sites for scalp tumors. According to Chiu et al., the frontal and vertex
region are affected in women in particular, whereas men, who are more exposed
overall, frequently develop lesions on the temples and in the postauricular and occipital
regions; malignant scalp tumors are four times more common in men [5].
In addition, repeated contact with po- In addition, repeated contact with polycyclic hydrocarbons, chronic wounds, scars
lycyclic hydrocarbons, chronic wounds, and human papillomaviruses (especially HPV types 16, 18 and 31) also pro-mote the
scars and human papillomaviruses development of these malignant tumors [4, 62, 63]. Previous radiation therapy in the
(especially HPV types 16, 18 and 31) scalp region is also associated with an increased risk of malignant tumors. For
also promote the development of these example, the relative risk of basal cell carcinoma in particular is in-creased 3.6-fold (95
ma-lignant tumors. % CI) for those patients who were exposed to a cumulative dose of 4.8 Gy during
radiation therapy for tinea capitis, which was popular in the
fi rst half of the last century. Moreover, 40 % patients thus treated exhibit a more
unfavorable multifocal growth pattern [64].
There is a marked increase in the in- There is a marked increase in the incidence of nonmelanoma skin cancer (NMSC)
cidence of nonmelanoma skin cancer with age due to less effective immune surveillance; this also applies to
(NMSC) with age due to less effective immunosuppressed patients (including iatrogenic immunosuppression). For in-stance,
immune surveillance; this also applies to about 70 % of Australian transplant recipients develop a squamous cell carcinoma
immunosuppressed patients (inclu-ding within 20 years (Figure 5) [65]. In addition, there is an increased risk of infi ltrative
iatrogenic immunosuppression). growth and tumor dedifferentiation. Although the mutation profi le

Figure 5 Disseminated actinic keratoses (carcinoma in situ) with progression to


poorly differentiated squamous cell carcinoma (G2; arrows) in the parietal region
of an 82-year-old patient with a history of longstanding immunosuppression with
azathioprine for Crohn’s disease.

740 © 2018 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2018/1606
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is heterogeneous overall, UV signature mutations in p53-, CDKN2A or Ras genes


confi rm a direct correlation between UV exposure, DNA damage and skin car-
cinogenesis [13]. These kinds of mutations have been found in nonmelanoma skin
cancer and atypical fi broxanthoma (AFX, dermal sarcoma). The latter is a rare
mesenchymal tumor characterized by pseudosarcomatous proliferation of dermal
fi broblasts. It has been classifi ed as a superfi cial variant of (the more invasive and
aggressive) pleomorphic dermal sarcoma [66].
Finally, photosensitizing genetic disorders and congenital DNA repair and im-
mune defects (such as albinism, xeroderma pigmentosum) may predispose to a
remarkably early occurrence of these epithelial malignancies [62].

Diagnosis
Relatively frequently are scalp tumors Relatively frequently are scalp tumors diagnosed only at an advanced stage when
diagnosed only at an advanced stage they present as visible/palpable masses. Early detection is often rendered diffi cult
when they present as visible/palpable because of the (dense) hair and the inaccessibility of certain regions to self-inspec-
masses. Early detection is often ren- tion. Tumors can therefore remain undetected for a prolonged period of time. For
dered difficult because of the (dense) the same reasons, the patient’s own account with regard to duration and changes
hair and the inaccessibility of certain in color and shape is less reliable. This circumstance underscores the importance
regions to self-inspection. Tumors can of regular and thorough scalp exams. In this context, special training programs
therefore remain undetected for a pro- for certain professions such as hairdressers appears useful. Given that hairdressers
longed period of time. regularly inspect the scalp of a large part of the population, they can detect suspi-
cious lesions and thus play a key role in early diagnosis [67].
In light of the astonishingly low rate of Although most benign scalp tumors require surgical intervention only when
correct preoperative diagnoses as re- they become infl amed or for cosmetic reasons, distinguishing them from malig-
gards scalp tumors (13–27 %), indistinct nant tumors purely on clinical grounds is often challenging or even impossible.
and suspicious lesions should always In light of the astonishingly low rate of correct preoperative diagnoses as regards
warrant histological confirmation. scalp tumors (13–27 %), indistinct and suspicious lesions should always warrant
histological confi rmation [3]. For example, angiosarcoma can mimic numerous
(more banal) skin conditions (hematoma, rosacea, erysipeloid, radiodermatitis)
and should therefore always be considered in the differential diagnosis (Figure 6)
[53]. With respect to the interpretation of clinical symptoms, it should be noted
that even histomorphologically benign scalp tumors may pose a differential diag-
nostic challenge. Given the limited space for expansion in this region, they can lead
to compression and (early) infi ltration/erosion of neighboring structures, with as-
sociated symptoms such as facial swelling and proptosis. Initial clinical symptoms
may therefore be misleading.
Noninvasive methods (such as dermoscopy and confocal laser microscopy)
may also be useful in the (differential) diagnostic workup (as well as for follow-up,
preoperative determination of tumor borders and assessment of excision margins)
in the cosmetically delicate scalp region.
Non-neoplastic lesions must always be Non-neoplastic lesions must always be distinguished (Table 4, Figure 7), given
distinguished (Table 4, Figure 7), given that tumors/tumorous lesions of the scalp may not only originate in the skin but
that tumors/tumorous lesions of the scalp (as noted above, especially in children) may also indicate an underlying process
may not only originate in the skin but (as of the cranium or point to craniocephalic malformations (“tip of the iceberg”).
noted above, especially in children) may Imaging studies such as ultrasound (index lesion, lymph nodes), CT (bone, lymph
also indicate an underlying process of the nodes), MRI (soft tissue, nerves) are important especially for large, hard immobile
cranium or point to craniocephalic mal- lesions, yet also for those with a midline location (e.g., subdural empyema, der-
formations (“tip of the iceberg”). moid cyst). These tests help narrow down the differential diagnosis and determine
the extent or depth of a given lesion (also during follow-up). Large congenital
nevi (> 20 cm) and those associated with satellite nevi should be worked up for
neurocutaneous melanocytosis (in 5–10 %) as soon as possible using cranial MRI,

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Figure 6 72-year-old patient. Indolent, rapidly growing, partly ulcerated, firm


erythematous nodules in the left temporo-occipital region (hematoxylin-eosin
stain, original magnification x 40) (a). Moderately differentiated angiosarcoma,
showing diffuse infiltration by irregular aggregates of atypical endothelial cells
intermingled with bizarre slit-like vascular structures (hematoxylin-eosin staining,
original magnification x 200) (b, c).

Figure 7 A 57-year-old patient presenting with a sharply demarcated erosion (7 ×


3 cm) in the central parietal region. In addition, there are smaller, sharply defined
erosions on the right side of the scalp (within reach of the right hand). Diagnosis:
artifact associated with psychiatric comorbidity.

742 © 2018 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2018/1606
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especially if there are neurological symptoms (signs of increased intracranial pres-


sure) [68, 69].
As regards the histological evaluation As regards the histological evaluation of scalp tumors, it should be borne in
of scalp tumors, it should be borne in mind that non-mesenchymal lesions in this region frequently exhibit spindle-cell
mind that non-mesenchymal lesions morphology (e.g., squamous cell carcinoma, [desmoplastic] melanoma) or pseu-
in this region frequently exhibit spind- domalignant features; this phenomenon is artifi cially augmented in small, crushed
le-cell morphology (e.g., squamous cell biopsies. Given these micromorphological peculiarities, shave biopsies should be
carcinoma, [desmoplastic] melanoma) avoided to ensure that the tissue sample is as representative as possible [70]. Reliab-
or pseudomalignant features; this phe- le and representative assessment of adnexal tumors in particular frequently requires
nomenon is artificially augmented in complete excision as these lesions can present considerable diagnostic problems due
small, crushed biopsies. to their morphological variety and essential clinicopathological overlap (Table 2).
If there is evidence of malignancy, current guidelines recommend a comple-
If there is evidence of malignancy, cur- te diagnostic workup including clinical examination (thorough skin exam, pal-
rent guidelines recommend a complete pation of lymphatic drainage pathways/regional lymph nodes), imaging studies
diagnostic workup including clinical (ultrasound, CT/MRI, PET-CT) and possibly further biopsies, depending on the
examination (thorough skin exam, pal- primary tumor type (see, for example, www.awmf.org, www.ado-homepage.de,
pation of lymphatic drainage pathways/ www.eado.org, www.euroderm.org/edf).
regional lymph nodes), imaging studies The signifi cance of sentinel lymph node biopsy (SLNB) for malignant scalp tu-
(ultrasound, CT/MRI, PET-CT) and pos- mors remains unclear, not least because of the high rate of false-negative results (see
sibly further biopsies, depending on the above). Nevertheless, SLNB should be considered in the case of satellite and in-tran-
primary tumor type. sit metastases and for melanomas with a Breslow thickness ≥ 0.8 mm [71–73].

Treatment principles
Topical field therapy of early forms of nonmelanoma skin cancer

For the treatment of superficial basal Considered to be noninvasive squamous cell carcinomas in situ, actinic keratoses
cell carcinoma, the aforementioned are among the most common early forms of malignant scalp tumors in everyday
topical agents (with the exception of clinical practice; one in three patients over the age of 30 (seen by a dermatologist)
imiquimod and conventional photody- is affected [74]. As regards the therapeutic approach to actinic keratoses, it has
namic therapy) are only available for proven useful to make a distinction between the treatment of individual lesions
off-label use. and fi eld therapy; the latter is employed for multiple lesions or those with indistinct
tumor borders. However, there is only limited qualitative evidence from studies
comparing the effectiveness of the various treatment options available. In addition,
response rates of 70–80 % contrast with high recurrence rates [75]. Given that
certain one-time treatments are not suffi ciently effective (depending on extent, site,
tolerability and compliance, among other things), it is important that methods em-
ployed for a longer period of time have a low side effect profi le. One of the classic
modalities used for destroying localized and hypertrophic actinic keratoses and su-
perfi cial basal cell carcinomas is cryotherapy. A relatively time-effi cient and inex-
pensive procedure, healing is often associated with hypopigmented scars [76]. Field
therapy plays a signifi cant role especially for prevention as it allows for subclinical
and incipient in situ carcinomas to be treated, thus preventing potential progressi-
on to invasive cancer [9, 77, 78]. Therapeutic agents include imiquimod, 5-fluoro-
uracil, diclofenac/hyaluronic acid and ingenol mebutate as well as conventional or
daylight photodynamic therapy [75]. However, topical immunomodulators such
as imiquimod may have a limited therapeutic effect in the scalp region, possibly
because they cannot be applied as evenly due to the presence of hair [75, 79]. For
the treatment of superfi cial basal cell carcinoma, the aforementioned topical agents
(with the exception of imiquimod and conventional photodynamic therapy) are
only available for off-label use; they should not be used for other basal cell carci-
noma subtypes because of insuffi cient effi cacy [75].

© 2018 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2018/1606 743
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Figure 8 Recommended surgical margins for the most common malignant skin
tumors.

Primary excision
In this context, for example, congenital Excision of benign lesions is sometimes indicated for cosmetic and psychosocial nevi used
to be surgically removed as reasons, if they cannot be unequivocally distinguished from malignant lesions, or soon as possible to
reduce the allege- if they become symptomatic, for instance by compressing other structures.
dly increased melanoma risk. Today, In this context, for example, congenital nevi used to be surgically removed however, a
limited surgical approach is as soon as possible to reduce the allegedly increased melanoma risk. Today, ho-recommended. wever, a
limited surgical approach is recommended [80, 81]. Surgical removal of congenital nevi up to 20 cm in size may be considered between the
ages of one and two if surgically feasible, and mainly for cosmetic reasons, given the low overall risk of malignant transformation (0.1 %
for nevi up to 10 cm in diameter and 1 % for nevi 10–20 cm in size). In addition, congenital nevi of the scalp usually tend to become lighter
over time and thus cosmetically less bothersome, even though the infi ltration persists. However, (serial partial) excision is defi nitely
indicated for all irregular and otherwise suspicious scalp lesions (for example, thick, non-homogeneous nodular nevi) as follow-up is more
diffi cult because of the hair

[80–82].
At any rate, surgical intervention is required if malignancy is clinically suspec-ted
or if there is deeper infi ltration or increased invasiveness (for example microcy-stic,
sclerosing basal cell carcinoma). Clinical signs include induration, bleeding, increase in
diameter, erythema, ulceration, hyperkeratosis, pruritus, pigmentation, pain).

Complete primary excision (with/wi- Complete primary excision (with/without micrographic margin control), thout micrographic
margin control), possibly followed by re-excision with tumor-specifi c surgical margins, is the possibly followed by re-excision with
treatment of choice for all malignant scalp tumors[8, 15, 17, 83] (Figure 8). Fol-tumor-specific surgical margins, is the lowing
excision, “simple” primary defect closure is preferable to more complex treatment of choice for all malignant fl ap repairs because of
the higher complication rates and greater subsequent scalp tumors. diffi culty in determining the excision margins if the primary
excision was in-
complete [84].

744 © 2018 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2018/1606
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Strategies for local tumor control


Treatment options for inoperable tu- Treatment options for inoperable tumors predominantly include radiation therapy and –
mors predominantly include radiation increasingly – also electrochemotherapy. Using brief intense electric pulses, the latter
therapy and – increasingly – also elec- involves the delivery of a chemotherapeutic agent such as bleomycin via
trochemotherapy. Using brief intense electroporation directly into the (tumor) cells, where it achieves a high local con-
electric pulses, the latter involves the centration. This method is suitable for all inoperable yet accessible skin tumors such as
delivery of a chemotherapeutic agent squamous cell carcinoma, cutaneous metastases, Merkel cell carcinoma and
such as bleomycin via electroporation angiosarcoma [85, 86]. In addition, there is an approved oncolytic tumor vac-cine
directly into the (tumor) cells, where it available for the treatment of advanced melanoma, which exerts both local and
achieves a high local concentration. systemic anti-tumor effects [87].

Systemic therapies
Systemic treatment options for locally Systemic treatment options for locally advanced and metastatic tumors include advanced and
metastatic tumors inclu- immunotherapies (for example, PD-1 inhibitors for melanoma and Merkel cell de immunotherapies (for
example, PD-1 carcinoma) and targeted molecular therapies based on the genetic tumor profi le. inhibitors for melanoma and Merkel
See current recommendations and guidelines issued by various scientifi c societies: cell carcinoma) and targeted molecular
www.awmf.org, www.ado-homepage.de, www.eado.org, www.euroderm.org/edf therapies based on the genetic tumor (Table 5). To
some extent, the latter have become routinely available for certain profile. tumors (for example, melanoma, basal cell carcinoma, dermatofi
brosarcoma protu-berans). Classic cytotoxic chemotherapies continue to be used, largely for palliation.

Given the complexity of these tumors, which often require multidisciplinary


expertise, treatment regimens should be agreed on in a quality-assured manner and
always involve an interdisciplinary tumor board.

Prevention
Irrespective of improvements and the increase in treatment options in recent ye-ars,
preventive measures (UV avoidance [textile, chemical and physical UV pro-tection],
consistent follow-up for early detection of recurrence) continue to be critically
important. Raising public awareness for this issue remains a key task of our specialty.

Prognosis
Malignant scalp tumors are generally characterized by a more aggressive biological
behavior than their counterparts at other sites. Basal cell and squamous cell carci-noma,
angiosarcoma and dermatofi brosarcoma protuberans, as well as Merkel cell carcinoma,
microcystic adnexal carcinoma, metastases, melanoma and, in child-ren,
rhabdomyosarcoma have considerable destructive potential and are associated with
signifi cant complications due to infi ltration/invasion of the periosteum, bone, dura and
brain [17, 31, 88]. The risk of osseous infi ltration correlates with disease duration and
tumor size, prior therapies (radiation therapy) and (aggressive) histo-logical growth
pattern (for example, sclerosing, micronodular, metatypical basal cell carcinoma) [15,
89].
Factors that potentially have a negative effect on prognosis not only include the
often-advanced age of the patients (comorbidities, “surgical resilience”), but also the
inherently limited accessibility for surgery and radiation therapy. This is the reason why
– in the scalp region – local tumor control is conside-red prognostically more signifi
cant overall than the tumor-specifi c potential for metastasis [90, 91].

© 2018 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2018/1606

745
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Table 5 Overview of established and experimental therapies for the treatment of malignant scalp tumors

Scalp tumors Topical Excision (with Radiation Electrochemotherapy Targeted therapy Immunotherapy Chemotherapy Other
agents sufficient
surgical
margins)
Basal cell x x x x* Hedgehog inhibitor x* Itraconazole*
carcinoma (vismodegib, sonidegib)
Squamous cell x x x x* EGFR inhibitor (cetuximab, Anti-PD1 Ab* x*
carcinoma panitumumab)
Melanoma x x x* BRAF, MEK inhibitor Anti-CTLA4 Ab (ipi- x
(dabrafenib, vemurafenib, limumab), anti-PD1
cobimetinib, trametinib) Ab (pembrolizumab,
nivolumab), on-
colytic virus T-VEC
Adnexal x x* x*
tumors
Merkel cell x x x* Tyrosine kinase inhibitor Anti-PD-L1 Ab x Somatostatin
carcinoma (pazopanib)* (avelumab)* analogs*
Lymphoma x x x* Fusion proteins, synthetic Interferon alpha x PUVA (psoralen
retinoids, monoclonal Ab, + UVA), ECP, he-
histone deacetylase inhi- matopoietic stem
bitor, and others cell transplantati-
on, and others
Sarcoma x x x* PDGFR-α inhibitor, x* x
tyrosine kinase inhibitor,
VEGF inhibitor, endoglin
Ab*, growth factor (VEGF),
and others
*Limited data.
Abbr.: Ab, antibody; T-VEC, talimogene laherparepvec; EGFR, epidermal growth factor receptor; PDGRF, platelet-derived growth factor; VEGF, vascular endothelial
growth factor; ECP, extracorporeal photopheresis [95–97].
CME Article

Conclusion
An interdisciplinary approach is desirable in the management of scalp tumors, given the
complex and delicate location as well as the variety of clinical and mor-phological
manifestations and the diagnostic and therapeutic challenges associ-ated therewith. This
must be emphasized all the more as independent care of patients with rare scalp tumors
by various medical specialties (such as pediatrics, dermatology, plastic and
neurosurgery, ENT or ophthalmology), which has been common practice, limits the
generation of individual expertise and comprehensi-ve data collection. However, such
epidemiological data is necessary for reliable conclusions to be drawn about the
incidence of these tumors and possible patho-genetic factors.

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CME Article

Fragen zur Zertifizierung durch die DDA


1. Nichmelanozytäre Hauttumoren stellen 3. Welcher Tumor mit Prädilektion für die 6. Eine erhöhte UV-Exposition spielt in der
die größte Gruppe der malignen Kopfhaut besitzt auch bei in-itial Pathogenese vieler (Kopfhaut-) Tumoren
Kopfhauttumoren dar. Welche Aussage ist durchgeführter, weiter Exzision (2–3 cm eine entscheidende Rolle. Wel-cher
für diese Subgruppe zutreffend? Sicherheitsabstand) eine star-ke Tendenz Kopfhauttumor ist (nach aktuellem
a) Plattenepithelkarzinome werden an zum Lokalrezidiv und kann zudem Erkenntnisstand) am wenigsten mit
der Kopfhaut erfreulicherweise zahlreiche (banale) Hautverän-derungen diesem externen Risikofaktor assoziiert?
meist schon in einem frühen Stadi- imitieren? a) Basaliom
um erstdiagnostiziert. a) Dermatofibrosarcoma protuberans b) Plattenepithelkarzinom
b) Basalzellkarzinome rezidivieren an b) Lentigo-maligna-Melanom c) Melanom
der Kopfhaut nur in Ausnahmefällen. c) Angiosarkom d) kutanes Lymphom
c) Melanozytäre Muster im Auflicht d) multifokales Basaliom e) Merkelzellkarzinom
sind für das Plattenepithelkarzinom e) Merkelzellsarkom
der Kopfhaut typisch.
d) Kryotherapie kann als verhältnismä- 7. Viele maligne Tumoren, die an der
ßig zeiteffiziente und kostengüns- 4. Sämtliche genetische Syndrome sind Kopfhaut auftreten, haben im Ver-gleich
tige Modalität zur Destruktion von mit Kopfhauttumoren assoziiert. Bei zu anderen Lokalisationen eine
invasiven Plattenepithelkarzinomen welchem Syndrom ist diese Assozi-ation schlechtere Prognose. Bei welchem der
sowie nodulären Basalzellkarzino- nicht typisch? folgenden Tumoren ist eine frühzeitige
men herangezogen werden. a) Brooke-Spiegler-Syndrom Diagnose und Therapieeinleitung ent-
e) Risikofaktoren für die Entstehung b) familiäre Zylindromatose scheidend, um potenzielle Komplikati-
nichtmelanozytärer Kopfhauttumo- c) SCALP-Syndrom onen durch Infiltration/Invasion tiefe-rer
ren inkludieren UV-Exposition, statt- d) Netherton-Syndrom Strukturen (z. B. Schädelknochen) zu
gehabte Radiotherapie, chronische e) Muir-Torre-Syndrom vermeiden?
Wunden und Immunsuppression.
a) mikrozystisches Adnexkarzinom
b) superfizielles Basaliom
5. Eine chirurgische Exzision von
c) Lentigo maligna Melanom
2. Kopfhauttumoren bei Kindern sind kleinen und mittelgroßen kongenita-len
d) Nävus sebaceus mit Trichoblastom
zwar selten und überwiegend benigne, Nävi wird im Unterschied zu vor einigen
e) proliferierende tricholemmale Zyste
die Liste an Differenzialdia-gnosen ist Jahren mittlerweile nur mehr sehr
jedoch groß. Bei welchem klinischen restriktiv vorgenommen. Was ist der
Kriterium sollte man früh-zeitig eine Grund für die Änderung des Vor-
8. Kopfhauttumoren stellen nicht zuletzt
radiologische Abklärung vornehmen? gehens?
aufgrund ihrer Heterogenität eine
a) Ein chirurgischer Eingriff erhöht das
diagnostische Herausforderung. Welche
a) Lokalisation okzipital lateral Melanomrisiko.
der folgenden Aussagen trifft nicht über
b) fehlende Verschieblichkeit bezie- b) Das maligne Transformationsrisiko
die diagnostischen Maß-nahmen zu?
hungsweise Lokalisation in der wurde fälschlicherweise zu hoch
Mittellinie angesehen.
a) Schildwächter-Lymphknoten-
c) langsames Wachstum c) Das kosmetische Ergebnis ist
Biopsien werden aufgrund der
d) unbehaarter Tumor postoperativ in den meisten Fällen
hohen falschpositiven Rate bei
e) Regressionszeichen von wenig zufriedenstellend.
Kopfhautmelanomen kritisch
vaskulären Läsionen d) Nach einer Exzision kommt es in
bewertet.
einem Großteil der Fälle zu einem
b) Diagnoseverzögerungen sind häufig,
Rezidiv.
da die Früherkennung von
e) Die meisten kongenitalen Nävi
Kopfhauttumoren durch (dichte)
werden heutzutage mittels Laser
Behaarung und (in Selbstinspektion)
therapiert.
schwer einsehbaren Bereichen an
der Kopfhaut erschwert wird.

752 © 2018 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2018/1606
CME Article

c) Shavingbiopsien im Rahmen der d) 1–2 % aller Kopfhauttumoren sind


Abklärung von Tumoren sollten maligne. Liebe Leserinnen und Leser,
vor allem aufgrund der e) Der Häufigkeitsgipfel für die Entste- der Einsendeschluss an die DDA
mikromorphologischen hung maligner Kopfhauttumoren für diese Ausgabe ist der 31. Juli
Besonderheiten an der Kopfhaut liegt zwischen dem 40. und 60. 2018. Die richtige Lösung zum
vermieden werden. Lebensjahr. Thema „Lipomatosen“ in Heft
d) Nichtinvasive diagnostische Metho- 3 (März 2018) ist: (1e, 2d, 3a, 4b, 5b, 6b,
den (z. B. Dermatoskopie) können 7e, 8a, 9c, 10a).
ergänzend zur differenzialdiagnos- 10. Welche Aussage zu melanozytären
Kopfhautumoren ist richtig? Bitte verwenden Sie für Ihre Einsen-
tischen Abklärung herangezogen
werden. a) Kopfhautnävi zeigen im Vergleich dung das aktuelle Formblatt auf der
e) Bei großen kongenitalen Nävi zur restlichen Körperhaut histo- folgenden Seite oder aber geben
(> 20 cm) und solchen, die mit Sa- pathologisch selten dysplastische Sie Ihre Lösung online unter
tellitennävi einhergehen, wird zur Merkmale. http://jddg. akademie-dda.de ein.
weiteren Abklärung eine kraniale b) Dermatoskopisch sind blau-graue
MR-Untersuchung empfohlen ovoide Nester typisch.
c) GNA11-mutierte blaue Nävi zeigen
eine Prädilektion für die Kopfhaut
9. Welche der folgenden Aussagen und haben ein erhöhtes Entartungs-
über Kopfhauttumoren ist richtig? risiko.
a) Kutane Metastasen führen die Rang- d) Die Rate falsch positiver regionaler
liste der häufigsten malignen Kopf- Schildwächterlymphknoten ist beim
hauttumoren an. Kopfhautmelanom höher.
b) Lipome und Fibrome bilden zusam- e) Kopfhautmelanome verantworten
men die größte Gruppe der benig- 45 % der Todesfälle durch schwar-
nen Kopfhauttumoren. zen Hautkrebs.
c) Die hohe Anzahl an apokrinen
Schweißdrüsen prädisponiert die
Kopfhaut als Prädilektionsort für
Atherome.

© 2018 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2018/1606 753

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