ARTICLE
Auteur(s) : Wolfram Sterry
Department of Dermatology, Venerology and Allergology, Charité
Campus Mitte,Universität Berlin Charitéplatz 1, 10117 Berlin,
Germany
Chairman of EDF Guideline CommiteeWolfram
SterrySubcommittee membersBill Bowers, Cornwall (United
Kingdom)Nicole Basset-Seguin, Paris (France)Graham Colver,
Chesterfield (United Kingdom)Andrew Finlay, Cardiff (United
Kingdom)Martino Neumann, Rotterdam (The Netherlands)Claas Ulrich,
Berlin (Germany)Wolfram Sterry, Berlin (Germany)Members of EDF
Guideline CommitteeWerner Aberer, Graz (Austria)Martine Bagot,
Créteil (France)Lasse Braathen, Bern (Switzerland)Sergio Chimenti,
Rome (Italy)José Luis Diaz-Perez, Bilbao (Spain)Vladimir Hegy,
Bratislava (Slovak Republic)Lajos Kemény, Szeged (Hungary)Gillian
Murphy, Dublin (Ireland)Martino Neumann, Rotterdam (The
Netherlands)Tony Ormerod, Aberdeen (UK)Annamari Ranki, Helsinki
(Finland)Fenella Wojnarowska, Oxford (UK)List of conflicts of
interest. Prof. Dr. Andrew Y. Finlay, Cardiff (UK) is on the UK 3M
Dermatology Advisory Board. Prof. Dr. Wolfram Sterry, Berlin
(Germany) is a consultant for 3M Pharmaceuticals. Prof. Dr. Nicole
Basset-Seguin, Paris (France) is a consultant for 3M
Pharmaceuticals and Galderma.
Definition
BCC is a slowly growing, locally invasive malignant epidermal skin
tumour, which exhibits a differentiation potential comparable to
the embryonic hair bud. BCC tends to infiltrate tissues in a
three-dimensional contiguous fashion through the irregular growth
of subclinical finger-like outgrowths [2]. Metastases are extremely
rare, and the morbidity associated with BCC is related to local
tissue invasion and destruction; growth pattern largely correlates
with the aggressiveness of the tumour [3-5]. The tumour is mainly
located on the head and neck and mainly affects Caucasians. The
multipotent differentiation potential is reflected by a larger
diversity in clinical appearance and morphology, i.e. nodular,
cystic, ulcerated, superficial, morphoeic (sclerosing), keratotic
and pigmented variants. Histological subtypes can also serve a
prognostic factor (table 1( Table 1
)).
Table 1 Prognosis and the concept of ‘low-risk’ and
‘high-risk’ basal cell carcinomas (modified from The Royal College
of Pathologists 2002) [99]
|
1. Risk stratification
|
|
1.1 Low risk. Nodular (cystic). May have follicular
differentiation and keratin cysts.
|
|
1.2 Special types and high risk
|
|
1.2.1 Superficial BCCs have multiple small collections of
follicular germ cells, with variable palisading. Associated with
increased recurrence, possibly because of incomplete excision.
There is debate on whether these are invasive.
|
|
1.2.2 Infiltrative and morphoeic BCCs may be aggressive.
Irregular groups of cells with islands and strands, atypicality and
poor palisading.
|
|
Note. Many BCCs contain more than one growth pattern.
|
|
ARRAY(0x2c5558)
|
|
2. Differentiation
|
|
2.1 Perineural invasion is mostly seen in atypical,
squamous, and infiltrative, morphoeic BCC, and is associated with a
high incidence of recurrence because of incomplete excision.
|
|
2.2 Vascular invasion seems to be of no importance.
|
|
Note. Moderate or severe squamous atypia or squamous malignancy.
These BCCs may have a higher incidence of recurrence [4, 5].
|
Incidence/prevalence
BCC is the most common cancer in the USA, Australia and Europe; its
incidence (new cases/100,000 inhabitants/year) increases worldwide
with the following numbers being reported: 1998: 128/105
male/female/100,000 in South Wales, Great Britain; 2058/1195
male/female/100,000 in Northern Australia [6-8]. The most
significant aetiological factor is exposure to ultraviolet
radiation. Sun exposure during childhood may be especially critical
in the development of BCC in adult life [9, 10].
Patients with BCC on head and neck show different phenotypes,
including one with continuous development of BCC clusters on the
trunk. Such patients are generally younger than patients with BCC
on head and neck only [11]. Further risk factors are increasing
age, male sex (18-40% more common in white men than women, skin
type I and II) and chronic immunosuppression [6, 12]. Multiple BCCs
may also arise in basal cell nevus (Gorlin’s) syndrome [13]. Once a
person has developed a BCC there is a significantly increased risk
of developing subsequent BCCs at other sites [14, 15].
There is evidence that an epidemic of BCCs in Australia is
beginning to abate, especially in younger people, possibly as a
result of adequate photoprotection [16].
Diagnosis and Investigation
Ideally the treatment of BCC is based upon a clinical diagnosis.
Where clinical doubt exists, or when patients are referred for
specialized forms of treatment, histology is crucial. Information
on the prognosis will be provided by the histological subtype of
the BCC. Clinically the extent of penetration of tumours is
impossible to judge. Scanning techniques such as CT and MRI may,
rarely, be needed [17].
Prognosis and the concept of “low-risk” and “high-risk” basal
cell carcinomas
The histological subtype may correlate with prognosis, and thereby
may be used to describe BCCs as low or high risk (table 1).
Management
The aim is to completely cure the tumour with the best cosmetic
result. The early detection and appropriate re-treatment of either
recurrent BCCs or new primary BCCs may help to increase the chances
of a permanent cure and to minimize morbidity. Patient education
together with close collaboration with colleagues in primary care
should allow the vast majority of adequately treated patients to be
discharged back to the care of their general practitioners or
dermatologists in private practice.
The relative value of the available forms of therapy for BCCs of
different sizes, clinical and histological subtypes and involving
both high- and low-risk body sites are summarized in table 3( Table 2 )( Table 3
) (primary BCCs) and table 4( Table 4 )
(recurrent BCCs).
Table 2 Primary (untreated) basal cell carcinoma:
influence of tumour type size (large ≥ 2 cm) and site on the
selection of available forms of treatment
|
Histology, size and site
|
Excision
|
Excision with margin control
|
Topical therapy incl. PDT
|
IRM
|
Curettage and cautery
|
Radiation
|
Cryo-surgery
|
|
Superficial, small and low-risk site
|
**
|
*
|
**
|
**
|
**
|
?
|
**
|
|
Superficial, large and low-risk site
|
**
|
*
|
**
|
**
|
**
|
*
|
**
|
|
Superficial, small and high-risk site
|
**
|
***
|
*
|
*
|
*
|
*
|
*
|
|
Superficial, large and high-risk site
|
**
|
***
|
x
|
x
|
-
|
**
|
*
|
|
Nodular, small and low-risk site
|
***
|
*
|
*
|
*
|
*
|
?
|
**
|
|
Nodular, large and low-risk site
|
***
|
**
|
x
|
x
|
*
|
**
|
*
|
|
Nodular, small and high-risk site
|
**
|
***
|
x
|
x
|
*
|
***
|
**
|
|
Nodular, large and high-risk site
|
**
|
***
|
x
|
x
|
x
|
**
|
x
|
|
Morphoeic, small and low-risk site
|
***
|
**
|
-
|
x
|
-
|
*
|
*
|
|
Morphoeic, large and low-risk site
|
***
|
**
|
x
|
x
|
-
|
*
|
*
|
|
Morphoeic, small and high-risk site
|
**
|
***
|
x
|
x
|
-
|
*
|
*
|
|
Morphoeic, large and high-risk site
|
*
|
***
|
x
|
x
|
x
|
*
|
x
|
Table 3 Recurrent BCC: influence of tumour type size
(large ≥ 2 cm) and site on the selection of available forms of
treatment
|
Histology, size and site
|
Excision
|
Excision with margin control
|
Topical therapy incl. PDT
|
IRM
|
|
Radiation
|
Cryo-surgery
|
|
Superficial, small and low-risk site
|
***
|
*
|
**
|
**
|
**
|
*
|
**
|
|
Superficial, large and low-risk site
|
**
|
***
|
**
|
**
|
**
|
**
|
**
|
|
Superficial, small and high-risk site
|
**
|
***
|
x
|
x
|
x
|
*
|
*
|
|
Superficial, large and high-risk site
|
**
|
***
|
x
|
x
|
x
|
*
|
-
|
|
Nodular, small and low-risk site
|
***
|
*
|
x
|
x
|
*
|
**
|
**
|
|
Nodular, large and low-risk site
|
***
|
**
|
x
|
x
|
x
|
*
|
*
|
|
Nodular, small and high-risk site
|
***
|
**
|
x
|
x
|
x
|
*
|
*
|
|
Nodular, large and high-risk site
|
**
|
***
|
x
|
x
|
x
|
*
|
x
|
|
Morphoeic, small and low-risk site
|
**
|
***
|
x
|
x
|
x
|
**
|
*
|
|
Morphoeic, large and low-risk site
|
**
|
***
|
x
|
x
|
x
|
*
|
*
|
|
Morphoeic, small and high-risk site
|
**
|
***
|
x
|
x
|
x
|
*
|
*
|
|
Morphoeic, large and high-risk site
|
*
|
***
|
x
|
x
|
x
|
*
|
-
|
Table 4 Basal cell carcinoma (BCC), indications for
Mohs micrographic surgery (modified from Telfer NR et al. [84])
|
– Site: eyes ears, lips, nose, nasolabial folds [85-89]
|
|
– Histological subtype: morphoeic, infiltrative,
micronodular, recurrent BCCs [26, 88]
|
|
– Size: > 2 cm, especially in high-risk sites [26,
41, 27, 64, 86, 88-97]
|
|
– Special sites: perineural spread [98]
|
Surgical techniques
The most commonly used surgical techniques can be divided into main
categories:
Excision with predetermined margins
The aim of any excisional procedure is to eradicate the tumour
entirely. Discussion of the surgical excision of BCC is divided
into the following sections:
- – Primary (previously untreated) BCC. Surgical excision
is a highly effective treatment for primary BCC [18, 19]. (Strength
of Evidence A, II-ii) The excised tissue can be examined
histologically [20, 21] and the peripheral and deep surgical
margins can be grossly assessed. The overall cosmetic results are
usually good. The use of thorough curettage prior to excision of
primary BCC may help to increase the cure rate by more accurately
defining the true borders of the BCC [22]. The size of the surgical
margins should correlate with the likelihood that subclinical
tumour extensions exist. Few data exist on the correct deep
surgical margin, as this will depend upon the local anatomy.
Studies using horizontal frozen sectioning Mohs micrographic
surgery (MMS) to detect accurately BCC at any part of the surgical
margin suggest that, for a small (< 20 mm) well defined
BCC, 3 mm peripheral surgical margins will clear the tumour in
85% of cases (and a 4-5 mm margin will increase the peripheral
clearance rate to approximately 95%, i.e. approximately 5% of
small, well-defined BCCs show a subclinical spread of >
4 mm [2, 23]). In contrast to small primary BCCs, morphoeic
and large BCCs require wider surgical margins for complete
histological resection. For primary morphoeic BCC, the rate of
complete excision with increasing peripheral surgical margins is as
follows: 3 mm margin: 66%, 5 mm margin: 82%,
13-15 mm margin: > 95% [23]. Positive margins are most
often seen in morphoeic and facial tumours and are associated with
a 26% recurrence rate over 5 years compared to 14% with free
margins [24]. In the eyelid or periorbital area incompletely
excised and morphoeic tumours were shown to have a 50% risk of
recurrence [25].
- – Recurrent (previously treated) BCC. The results of all
published series on the surgical excision of BCC show that cure
rates for recurrent BCC are inferior to those for primary lesions
[26]. Recurrent BCCs require wider peripheral surgical margins than
primary lesions with or without standard (non-Mohs) frozen section
control [20]. Peripheral excision margins for recurrent BCC of
5-10 mm have been suggested [27] (Strength of Evidence A,
II-ii).
- – Incompletely excised BCC (positive histological
margins). This scenario should not occur following excision with
histological control of margins. However if closure is completed
before histological assessment there will be instances when the
pathologist reports tumour presence at the lateral and/or deep
margin of excision. In some cases this may be apparent, not real,
and due to tangential slicing of the specimen or tissue shrinkage.
A study in which 43 incompletely excised BCCs were re-excised and
the tissue examined using standard tissue sectioning techniques
suggested that only 7% contained residual BCC [28]. However, when
78 incompletely excised BCCs were re-excised and examined using
horizontal frozen sectioning (Mohs micrographic surgery) in order
to detect BCC more accurately at any part of the surgical margin,
55% were found to contain residual BCC [29] (Strength of Evidence
A, II-iii). These findings were in accordance with another study
demonstrating a tumour persistence in 28% of cases following
incomplete excision of BCC [30].
Several studies have demonstrated that not all tumours will
recur despite positive margins but the recurrence rate varies from
17-58%. The lowest rates were for lateral margin involvement only.
The higher rates were for deep and lateral margin involvement, for
tumours which were previously recurrent and those which had been
treated by radiotherapy [28, 29, 31-34].
In a series of 187 incompletely excised BCCs, with 93% occurring
on the head and neck, 119 were immediately retreated with
radiotherapy, one was excised and 67 were not treated. After a
median follow-up period of 2.7 years, statistical analysis
suggested a 5-year probability of cure in the radiotherapy group of
91%, and in the untreated group of 61% [34].
So what advice should the patient be given? Several studies have
strongly recommended the immediate retreatment of incompletely
excised BCC especially those where the surgical defect has been
repaired using skin flaps or skin grafts [29, 30, 33, 35, 36].
There may be occasions when a patient with a low risk primary
tumour with possible lateral margin involvement opts for a period
of observation. However generally it seems appropriate to re-excise
with or without frozen section control or Mohs micrographic
surgery.
Stepwise excision with histology control of margins
Micrographic surgery might serve as a treatment of choice for large
or difficult primary BCC lacking distinct clinical boundaries [37].
The histological mapping of the tumour margins preserves
tumour-free adjacent tissue, optimizes wound reconstruction and
reduces the percentage of additional excisions in order to remove
the tumour completely [38] (Strength of Evidence A II-iii). This
specialized minimal surgery was initially developed by Mohs and
offers highly accurate yet conservative removal of BCC [39, 40].
The indications for using Mohs micrographic surgery are summarized
in table 2 (Strength of Evidence A, II-i).
A review of all studies published since 1947 suggested an
overall 5-year cure rate of 99% following Mohs micrographic surgery
for primary BCC [41] and a review of all studies published since
1945 suggested an overall 5-year cure rate of 94.4% following Mohs
micrographic surgery for recurrent BCC [26]. In addition to Mohs
micrographic surgery, further fresh tissue micrographic techniques
exist: both the margin strip method (“Tübinger Torte”) as well as
the “Munich” method follow the same aim of complete tumour
resection though they differ in some points, for example in the
preparation or technique of tumour excision [38]. The latter method
was the treatment of choice for the excision of 3065 BCCs in 2795
patients. In 53.3% of all BCC, the first excision led to a complete
tumour removal, another 36.9% were free of BCC after a second
excision. Interestingly, clearance with one step excision ranged
between 80% of adenoid-cystic, > 50% of solid but only 43% of
morphea-like BCC. The follow-up period over more than 5 years
resulted in recurrences of BCC in 41 of 1604 patients (2.6%). 36%
of these recurrent BCCs were re-recurrent tumours, 64% of these
tumours were initially primary BCCs.
When compared with other outpatient-based treatments for BCC,
these specialized methods undoubtedly offer high cure rates but are
relatively expensive and time consuming.
Non-surgical techniques
Destructive techniques without histological control
Curettage and cautery/electrodesiccation
There are wide variations in how this technique is performed
(e.g. type of curette used, number of cycles of treatment) and both
experience in the technique and appropriate selection of cases is
crucial to success [42]. Curettage and cautery is best used for
selected low-risk lesions (small, well defined primary lesions with
non-aggressive histology usually in non-critical sites [43, 44]
where 5-year cure rates of up to 97% are possible [43]. Curettage
and cautery are not recommended for the management of large [44]
and other ‘high-risk’ tumours [42, 45-48].
Tumour size is an important factor as the recurrence rate rises
dramatically with increasing tumour size [44] (Strength of Evidence
[Appendix 1, 2] A, II-iii).
A literature review of all studies published since 1947
suggested an overall 5-year cure rate of 92.3% following curettage
and cautery for primary BCC [41]. However, a similar review of all
studies published since 1945 suggested an overall 5-year cure rate
of 60% following curettage and cautery for recurrent BCC. This
supports the view that curettage and cautery is much less useful in
the treatment of recurrent BCC, especially in high-risk sites [26]
(Strength of Evidence A, II-ii).
Cryotherapy
Cryosurgery is widely used to treat solitary and multiple BCCs.
Individual technique can vary considerably, using the open or
closed spray techniques and single, double or triple freeze/thaw
cycles [49, 50].
Many large published series specifically exclude the treatment
of very high-risk BCCs, emphasizing the importance of careful
selection of appropriate lesions with non-aggressive histology,
away from critical facial sites in order to achieve high cure rates
[48, 50-52] (Strength of Evidence A, II-ii). There are reports in
the ophthalmological literature recommending the use of
cryo-surgery for periocular BCC, although full-thickness eyelid
defects may occasionally result and require subsequent plastic
surgical reconstruction [53, 54].
Thorough curettage immediately prior to cryosurgery may help to
increase the cure rate [55] (Strength of Evidence A, II-ii).
As with most treatment modalities, cryosurgery is less useful in
the treatment of recurrent BCC [41].
Post-operative wound care can be a problem. However, the
treatment is usually well tolerated when performed on a local
anaesthetic, outpatient basis and the cosmetic results can be
excellent [51, 56].
Carbon dioxide laser
Carbon dioxide (CO2) laser surgery is not a widely used
form of treatment and there is little published follow-up data. The
treatment is mainly recommended for low-risk lesions. When combined
with curettage, CO2 laser surgery may be useful in the
treatment of large or multiple superficial BCCs [57, 58].
Radiotherapy
Radiotherapy (RT) is an extremely efficient form of treatment, but
faces the same problem of accurately identifying tumour margins as
standard excisional surgery [34, 59, 60]. RT includes a range of
treatments using different types of equipment, each with its own
specific indications and side-effects. It is therefore best
performed by specialized dermatological centers or by clinical
oncologists with a specialist interest in skin cancer.
Careful patient selection can result in very high cure rates; in
a series of 412 BCCs treated with RT, 5-year cure rates of 90.3%
were achieved [18]. In a prospective trial, where 93 patients with
BCC were randomized to receive either cryosurgery or radiation
therapy; the 2-year cure rate for the RT group was 96% [52]. A
review of all studies published since 1947 suggested an overall
5-year cure rate of 91.3% following RT for primary BCC and a review
of all studies published since 1945 suggested an overall 5-year
cure rate of 90.2% following RT for recurrent BCC [26, 41].
Radiotherapy can be used to treat many types of BCC, even those
overlying bone and cartilage, although it is probably less suitable
for the treatment of large tumours in critical sites, as very large
BCC masses are often both resistant and require radiation doses
that closely approach tissue tolerance. However, surgery should be
preferred for BCC of the face measuring < 4 cm in diameter
[61].
Radiotherapy is also not indicated for BCCs on areas subject to
repeated trauma such as the extremities or trunk and for young
patients as the late-onset changes of cutaneous atrophy and
telangiectasis may result in a cosmetic result inferior to that
following surgery [59, 62]. It can also be difficult to use RT to
re-treat BCCs that have recurred following RT. Modern fractionated
dose therapy has many advantages but requires multiple visits to a
specialist centre. Late-onset fibrosis may cause problems such as
epiphora and ectropion following treatment of lower eyelid and
inner canthal lesions, where cataract formation is also a
recognized risk, although this can be minimized by the use of
protective contact lenses [63].
There is some suggestion that BCCs recurring following RT may
behave in a particularly aggressive and infiltrative fashion,
although this may simply reflect that these lesions were of an
aggressive, high-risk type from the very beginning [33, 64,
65].
Topical therapy
– Topical 5-fluorouracil (5FU). Treatment is especially useful for
low-risk, extrafacial BCC but it cannot be expected to eradicate
invasive BCC [66] (Strength of Evidence A, II-ii).
– Topical Imiquimod. Imiquimod is an immune response modifier
(IRM). It has been successfully used for the treatment of cutaneous
neoplasias as well as viral skin diseases and acts as an antitumour
agent principally by both production alpha interferon and other
cytokines and induction of apoptosis [67].
The use of imiquimod 5% cream for the treatment of superficial
(sBCC) and nodular BCC (nBCC) was recently investigated comparing
low frequency dosing with and without occlusion for 6 weeks. In
patients with superficial BCC the highest complete clearance rates
ranged between 87% with occlusion (20 of 23 patients) and 76%
without occlusion (19 of 25 patients). Several studies have shown a
clearance rate (judged at 3 months with histology) of 87.9% with
one application/day for 6 weeks [68] and of 80.8% (with one
application/day/5 days/w for 6 weeks) [69] in sBCC without
occlusion. If applied 5 times per week for 6 weeks (pooled analysis
of two double-blind vehicle controlled clinical trials) a
histological clearance was noted in 82% (152/185) of patients [70,
71]. Clearance was assessed by excisional histology at the
completion of treatment. Interim results from a long-term
open-label uncontrolled study indicate an estimated sustained
clearance of 92% at 12 months. The recommendation of prescription
of imiquimod for the treatment of sBCC is actually once/day, 5
times/week for 6 weeks [69] (tables 2 and 3) (Strength of Evidence
A,I).
In the nodular BCC group (7 days per week treatment, 12 weeks
therapy) the percentage of complete responders was only 76% (16 of
21) and 71% (7 days per week, 6 weeks) (25 of 35) [72]. Therefore
imiquimod is not actually recommended for the treatment of nodular
BCC.
A successful treatment of multiple BCCs with topical imiquimod
in nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome) was
also reported [73].
Photodynamic therapy
Mainly due to its good cosmetic outcome the use of topical
photodynamic therapy (PDT) in the management of BCC becomes more
and more available in dermatological centres. In a study of 151
BCCs treated with PDT without long-term follow-up, 88% demonstrated
a complete response [74] (Strength of Evidence C, II-iii).
Long-term follow-up data on large series is needed to demonstrate
whether or not topical PDT has a role in the management of BCC.
Methyl aminolevulinate PDT was recently shown to be an effective
treatment even for nodular BCC [75]. However, as depth of
penetration of the photosensitizer appears to be a limiting factor
with topical PDT, without previous curettage PDT is only likely to
be of benefit for the treatment of superficial BCC in low risk
areas [75, 76] (Strength of Evidence C, III). A report from the
British Photodermatology Group suggests topical ALA-PDT to be an
effective therapy for superficial (< 2 mm thick) BCC
(Strength of recommendation A, I) [77].
Chemotherapy
Chemotherapy has been used both for the management of uncontrolled
local disease and for patients with metastatic BCC, which is both
an extremely rare and a rapidly fatal condition [3]. A cisplatin
(CDDP)- based chemotherapy (including doxorubicin) as sole therapy
and as neoadjuvant (NA) therapy revealed a complete remission in
eight of 28 patients (28%) suffering from advanced basal cell and
squamous cell cancers, a partial remission was achieved in 11 of 28
patients (40%) [78].
Palliation and observation
In some instances the patient’s general health or condition may
indicate palliation and/or observation. The risk/benefit ratio must
be considered individually to assess whether the cases warrants
only palliation, observation or both [79]. In the debilitated
patient aggressive treatment may be inappropriate. Observation
alone or simple debulking or RT may achieve local control and
improve quality of life.
Retinoids
Oral retinoid therapy may prevent or delay the development of new
BCCs. Such therapy has mainly been used in patients with the basal
cell nevus (Gorlin’s) syndrome and may also have a lesser effect in
producing partial regression of existing BCCs [80]. A recent study
questioned the benefit of acetretin in preventing BCCs [81].
Unfortunately, the relatively high doses necessary mean that
compliance may be poor, and relapse occurs following the
discontinuation of treatment [80] (Strength of Evidence B, III).
Follow-up
Long-term hospital-based follow-up of all patients after treatment
of BCC is neither necessary nor recommended. However, follow-up can
be important for selected patients, although there is no clear
consensus on either the frequency or total duration or such review.
The main arguments for follow up are: (i) early detection of tumour
recurrence; (ii) early detection and treatment of new lesions; and
(iii) patient education, especially regarding sun protection
measures. Most evidence suggests that the majority of BCCs that
recur will present within 5 years of treatment [82], although up to
18% will recur after this [41]. A review of all studies published
since 1947 suggested that for primary (previously untreated) BCCs
treated by a variety of modalities less than one-third of all
recurrences occurred in the first year following treatment, 50%
appear within 2 years, and 66% within 3 years [41]. Patients who
have had one BCC are at significantly higher risk of developing new
primary lesions [14, 83], many of which may go unnoticed by
patients. In a 5-year prospective follow-up study of 1000 patients
following treatment for BCC, 36% developed new primary BCCs and 20%
of patients with very fair skin types and frequent sun exposure
went on to develop multiple BCCs [15]. Consequently, some authors
have recommended long-term, even lifetime follow-up, particularly
for patients with high-risk or multiple lesions [41, 83]. In
contrast to this special group, patients with single BCCs
completely excised in low risk sites possibly do not require follow
up [80].
Additional considerations
Once the tumour has been assessed the most appropriate treatment
options must be discussed with the patient. Although surgery is the
gold standard there are some circumstances, which will affect the
final decision. Patients reluctant to consider any form of surgery
might be referred for radiotherapy. Similarly, co-existing medical
conditions or drug medication may influence the choice between
surgical and non-surgical treatment. Aggressive treatment might be
inappropriate for patients of advanced age or poor general health,
especially when dealing with asymptomatic low-risk lesions that are
unlikely to cause significant morbidity. Some elderly or frail
patients with symptomatic or high-risk tumours might prefer less
aggressive treatments designed to palliate rather than cure their
tumours. Local availability of various specialized services,
together with the experience and preferences of the dermatologist
managing the case are also factors that will influence the
selection of therapy. A great variety of skills is needed to manage
the spectrum of problems thrown up by basal cell carcinoma.
Multidisciplinary teams are of great value in difficult high-risk
tumours in order to obtain both the highest clearance rates and
best cosmetic results. Essential members of such a team are likely
to cover the specialties of dermatology, diagnostic imaging,
histopathology, oncology, radiotherapy and specialised surgery e.g.
dermatologists, plastics, maxillofacial, ophthalmology. Such close
collaboration has an additional educational benefit for all
parties.
Guideline development standard operating procedure of
EDF
|
Step
|
Responsible
|
Task
|
|
1
|
EDF Guidelines Committee (EDF-GC)
|
Decision of topic of specific guideline
|
|
2
|
EDF Guidelines Committee
|
Foundation of subcommittee for specific guidelines, nomination of
EDF members (50%)
|
|
3
|
EDF Guidelines Subcommittee (EDF-GSubC)
|
Identify all existing guidelines for the specific guideline (active
process: literature survey plus contact to Dermatological
Societies)
|
|
4
|
EDF Guidelines Subcommittee
|
Select the guidelines with highest quality. Criteria for
selection:
|
|
1. Availability of strength of evidence
|
|
2. Availability of strength of recommendation
|
|
3. Evidence of mechanics of literature review
|
|
5
|
EDF Guidelines Subcommittee
|
Identification/nomination of additional 50% EDF members for the
EDF-GsubC from amongst the authors of the best guidelines
|
|
6
|
EDF Guidelines Subcommittee
|
Nomination of chairperson for EDF-GSubC from the GSubC members
|
|
7
|
Chairperson
|
Consider involvement of other disciplines and patients´
organisations
|
|
8
|
EDF Guidelines Subcommittee
|
Meet
|
|
1. to decide the author of the first draft and to discuss the
present guidelines, their strengths and weaknesses
|
|
2. 6 months later to discuss the draft (consensus conference)
|
|
9
|
Chairperson
|
Circulate the guideline draft to national dermatological societies
for comments
|
|
10
|
Guidelines Subcommittee
|
Circulate final version for approval among members of the guideline
subcommittee
|
|
11
|
Chairperson of Subcommittee
|
Deliver final version to EDF guideline chairperson
|
|
12
|
EDF Guidelines Committee
|
Review and comment guideline
|
|
13
|
EDF Guidelines Committee chairperson
|
Send guideline for official approval to UEMS (formal approval)
|
|
14
|
EDF secretary
|
Distribute guideline for in advance information to EDF members and
National Dermatological Societies
|
|
15
|
EDF
|
Publication
|
|
1. on EDF homepage
|
|
2. in European Journal of Dermatology, British Journal of
Dermatology and Journal of Deutsche Dermatologische
Gesellschaft
|
|
3. If publication in other national journals is requested by the
respective society, this will be encouraged by the EDF
|
Appendix 1. Strength of recommendations (according to Telfer NR
[84])
|
A
|
There is good evidence to support the use of the procedure
|
|
B
|
There is fair evidence to support the use of the procedure
|
|
C
|
There is poor evidence to support the use of the procedure
|
|
D
|
There is fair evidence to support the rejection of the use of the
procedure
|
|
E
|
There is good evidence to support the rejection of the use of the
procedure
|
Appendix 2. Quality of evidence
|
I
|
Evidence obtained from at least one properly designed, randomized
control trial
|
|
II-i
|
Evidence obtained from well-designed controlled trials without
randomization
|
|
II-ii
|
Evidence obtained from well-designed cohort or case-control
analytic studies
|
|
Preferably from more than one centre or research group
|
|
II-iii
|
Evidence obtained from multiple time series with or without the
intervention. Dramatic results in uncontrolled experiments (such as
the introduction of penicillin treatment in the 1940s) could be
regarded as this type of evidence
|
|
III
|
Opinions of respected authorities based on clinical experience,
descriptive studies or reports of expert committees
|
|
IV
|
Evidence inadequate owing to problems of methodology (e.g. sample
size, or length or comprehensiveness of follow-up or conflicts of
evidence)
|
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