A systematic review on external ear melanoma.

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Journal of Plastic, Reconstructive & Aesthetic Surgery (2015) xx, 1e12

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A systematic review on external ear melanoma Francesca Toia a,*, Giuseppe Garbo a, Massimiliano Tripoli a, Gaetana Rinaldi b, Francesco Moschella a, Adriana Cordova a a Plastic and Reconstructive Surgery, Department of Surgical, Oncological and Oral Sciences, University of Palermo, Palermo, Italy b Medical Oncology, Department of Surgical, Oncological and Oral Sciences, University of Palermo, Palermo, Italy

Received 25 April 2014; accepted 6 April 2015

KEYWORDS External ear; Melanoma; Ear melanoma; Melanoma treatment

Summary Background: External ear melanoma accounts for only 1% of all cutaneous melanomas, and data on its optimal management and prognosis are limited. Aim: We aim to review the literature on external ear melanoma to guide surgeons in the treatment of this uncommon and peculiar pathology. Materials and methods: A systematic review of English language studies on ear melanoma published from 1993 to 2013 was performed using the PubMed electronic database. Data on epidemiology, oncological treatment (tumor resection and regional lymph nodes management), and reconstruction were extrapolated from selected papers. Results: The total number of patients was 858 (30 studies). The helix was the most common location (57%); superficial spreading melanoma was the most common histopathological subtype (41%). The mean Breslow thickness was 2.01 mm, with 88% of stage IeII patients. Sentinel lymph node biopsy was performed in 45% of patients, with 8% of positive nodes. Available data on its prognosis are fragmentary and contrasting, but the Breslow thickness appears to be the main prognostic factor. There is a tendency towards reduced resection margins and preservation of the underlying perichondrium and cartilage. Local flaps are the most popular reconstructive option. Conclusion: To the best of our knowledge, this systematic review presents the largest data series on external ear melanoma. There is no general agreement on its surgical management, but a favorable prognosis seems to justify the tendency towards conservative treatments. ª 2015 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

* Corresponding author. Plastic and Reconstructive Surgery, Department of Surgical, Oncological and Oral Sciences, University of Palermo, Via del Vespro 129, 90127 Palermo, Italy. Tel.: þ39 091 6553771; fax: þ39 0916553776. E-mail address: [email protected] (F. Toia). http://dx.doi.org/10.1016/j.bjps.2015.04.003 1748-6815/ª 2015 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

Please cite this article in press as: Toia F, et al., A systematic review on external ear melanoma, Journal of Plastic, Reconstructive & Aesthetic Surgery (2015), http://dx.doi.org/10.1016/j.bjps.2015.04.003

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Introduction External ear melanomas are relatively rare, representing approximately 1% of all cutaneous melanomas.1 A limited number of papers address this topic, and most of them include a small number of patients.2e9 The external ear presents peculiar characteristics with respect to anatomy and lymphatic drainage: the presence of a thin skin and of a cartilaginous framework requires a specific approach for tumor resection and reconstruction; lymphatic drainage e as for other head and neck regions e is unpredictable and often reaches multiple basins.4,9 Furthermore, there is no agreement on the biological behavior of melanoma of this region: some authors claim a better prognosis and propose less invasive treatments,8,9 while others consider external ear melanoma more aggressive.2 As a consequence, its treatment is not standardized, which often differs from standard treatment for melanomas of other regions.10 The aim of this study was to review the literature on the epidemiology and surgical treatment of external ear melanoma in order to provide guidelines for its clinical management.

Materials and methods Search methodology A systematic review of the studies on ear melanoma was performed using the PubMed electronic database. Initial keywords included “ear melanoma.” Extended search using the Medical Subject Headings (MeSH) terms “malignant melanoma” and “ear” and the keywords “melanoma pinna” and “melanoma ear reconstruction” was performed. A review of abstracts was performed on articles published from 1993 to 2013. The year 1993 was chosen as a starting point because of the introduction of sentinel lymph node biopsy (SLNB) procedure in the clinical practice.11 Data on (a) epidemiology, (b) oncological treatment, and (c) reconstruction of external ear melanoma were extrapolated.

Selection criteria Studies in languages other than English, on animal models, and/or not concerning external ear melanoma were excluded based on abstract review; letters to the editor were also excluded. Papers containing no basic data (at least on the Breslow thickness, tumor resection, lymph node management, or defect reconstruction) were excluded based on full-text article review (Figure 1).

Data collection and analysis For each paper, we collected and analyzed data on the following variables (when available): 1. Epidemiology: study type, number of patients, sex, mean age, tumor location, mean Breslow thickness, ulceration, histopathological subtypes, TNM stage, follow-

F. Toia et al. up time, survival rate, recurrence type, and rate (Table 1); 2. Oncological treatment: tumor resection (excision margin, resection/preservation of perichondrium and/or cartilage, Table 2) and management of regional lymph nodes (SLNB: number of procedures, lymph node basins, number of sentinel lymph node (SLN) per patient, and positive SLN; lymph node dissection: number and type of procedure, and positive dissections-, Table 1); and 3. Reconstruction: defect location and size, and reconstructive technique (Table 3).

Results and discussion The initial search returned 448 papers; an extended search did not return any additional reference. Four hundred and ten papers were excluded and 38 papers were selected based on abstract review; four further papers were excluded because we were not able to find them despite consulting the archives of other institutions as well. Thirtyfour full-length papers were reviewed and four further articles were excluded based on the lack of basic data. A total of 30 papers were eventually selected for the review (Figure 1). Of the selected 30 papers, 23 provided data on ear melanoma epidemiology, 19 on tumor resection, and 18 on reconstruction techniques. Most studies were incomplete with regard to the collected data.

Epidemiology Of the selected 23 papers, 12 were retrospective studies, two were prospective studies, and nine were case reports. Table 1 shows the total number of studies and patients for each investigated variable, and the present data in detail. The total number of patients was 845, with a strong male predominance (78%); the mean age was 59.4 years. The helix was the most common location (57%), followed by the lobule (17%). Interestingly, only three cases of melanoma of the external auditory canal were reported. The mean Breslow thickness was 2.01 mm; the most common histopathological subtype was the superficial spreading melanoma (41%), followed by the nodular melanoma (22%) and the lentigo maligna melanoma (LMM, 21%). The occurrence of ulceration was reported in 20% of patients. Data on the TNM stage were explicitly stated only in three papers3,12,13; however, we were able to extrapolate data on the melanoma stage in 15 additional studies (total of 18 studies, 731 patients), which contain in their text relevant data on the Breslow thickness and eventual nodal or systemic metastases. Of note, despite a high mean Breslow thickness, most patients were in stage IeII at initial diagnosis (88%), and 7.1% were in stage III; stage 0 and IV accounted for only 1.4% and 0.1%, respectively. Follow-up ranged between 1 and 298.8 months (mean 45.3 calculated on 11 studies; median range 24e39.5). The literature data on the prognosis of external ear melanoma are fragmentary and not conclusive.


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A systematic review on external ear melanoma

Figure 1

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Systematic literature review strategy.

Interestingly, due to the lack of detailed data, while we extrapolated the 5-year survival rate from nine studies and 348 patients, we were able to correlate the 5-year survival rate with TNM stage at initial diagnosis only in three studies2,3,8 and 35 patients: the survival rate was 100% in stage 0, and 71% and 53% in stage I and II, respectively, while it dropped to 0% in stage III. Jahn et al.12 suggested a higher survival rate for patients in stage I and II, with a cumulative 5-year survival rate of 96%. The data suggest a poorer prognosis for patients presenting with node metastasis compared to melanomas of other regions, but the small number of patients does not allow for conclusions or for statistical considerations. Regional lymph nodes were the most common localization of metastases (10%); most studies distinguished between local, regional, and distant recurrences, but in two studies14,15 local and regional recurrence rates were reported as a single summation value (Table 1). Data on recurrence-free survival are limited. Jahn et al.12 reported a recurrence-free survival of 83% at 3-year follow up, and 79% at 5-year follow up (161 stage IeII patients). Lack and heterogeneity of data did not allow a KaplaneMeier

analysis to be performed for recurrence-free survival. Most studies only report mean or median follow-up time, and we were able to extrapolate data on time to recurrence for each patient only in nine studies and 99 patients2,4,6e9,16e18: recurrences was observed in five patients (5%) in the first year after surgery, three patients (3%) between 1 and 3 years, and two patients (2%) between 3 and 5 years. However, we could not calculate recurrence-free survival, because of a short minimal follow-up in most studies (6e12 months). For a long time, melanoma of the ear has been associated with poorer prognosis and lower survival rates than other anatomical regions.19 Benmeir et al.2 even advocate urgent removal of any nevus of the external ear in view of the aggressive nature of ear melanoma. More recently, McCarty et al.6 pointed out that e if adjusted for thickness e ear melanoma has a similar prognosis to other head and neck melanomas, excluding the scalp; in their review of the Sunbelt Melanoma Trial, Augenstein et al.15 also showed that location of a head and neck melanoma at the external ear is not an independent risk factor for recurrences, and overall and disease-free survival.


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F. Toia et al. Table 1

Data on epidemiology, regional lymph node management, and follow-up of external ear melanomas.


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Tumor thickness appears to be the most relevant prognostic factor2e4,12,20; several authors also agree on the negative prognostic role of ulceration.6,13,15 However, some contrasting data have been published: Augenstein et al.15 even claim ulceration as the strongest predictor of overall survival, while Jahn et al.12 showed that ulceration does not statistically influence the disease-specific survival. According to the literature, other significant prognostic factors for survival or recurrence rate are as follows: Clark level,6,12 SLN positivity, clinically positive nodes,6,13,15,20,21 and age (>59 years old).12 Histological subtype13 and surgical margins or type of resection3,12,20 do not appear to affect survival probability, supporting the tendency towards reduced excision margins (see below). Limited and somehow contrasting are the results of the sex prognostic value: Jahn et al.12 did not find it to be significant, while Ravin et al.13 confirmed this finding until approximately 7.5 years after diagnosis, but they pointed out higher long-term survival rates in women.

Tumor resection Excision margins Safe excision margins in melanoma of the ear are still controversial. This review points out that wide differences in excision margins exist and that, in the absence of specific evidence-based guidelines, these vary considerably on the only basis of surgeon preference. Moreover, it is not possible to draw any recommendation on safe excision margins based on an analysis of the recurrence rates: although several papers reported data on the recurrence

F. Toia et al.

rate, none of them correlated it to the excision margin, nor were complete patients’ data available to extrapolate relative influence. Generally, as it is frequent in head and neck melanomas,22 resection margins are often lower than general recommendations, with the aim of preserving function and improving cosmetic outcome: although the mean Breslow thickness was 2.01 mm, the resection margins were often between 1 and 2 cm. Most authors report a minimum excision margin of 1 cm,4,6,9,16,20e26 and Pockaj et al.20 claim that a 1-cm negative margin is associated with a low risk of local recurrence. An excision margin of 0.5 cm, however, has been used not only for melanoma in situ3,8 e as for other anatomical region e but also for melanomas with higher Breslow thickness: Bono et al.3 used a 0.5-cm margin for four non-in situ melanomas up to 5.92 mm in thickness, while Jahn et al.12 recommend a 0.5-cm margin for all LMMs, irrespective of the tumor thickness. They also report excision margins between 0.6 and 1 cm in other 37 patients with melanoma (data on the Breslow thickness not shown). Recommendations on excision margins based on the Breslow thickness are unclear and often diverging. According to Pockaj et al.,20 the treatment should be customized to the patient and conciliate a radical excision with preservation of cosmesis. Some authors recommend following standard melanoma treatment guidelines to improve local tumor control.12,21 As for melanomas of other anatomical regions, Jahn et al.12 recommend a 1-cm excision margin for SSM and nodular melanoma 1

6 (30%) 8 (40%) 6 (30%)

2 1 2

8 (42.1%) 1 documented

Unknown 0.5 0.6e1 1.1e1.5 >1.5 (Mohs Surgery patients)

Jones et al. (2013) Kruse-Lo ¨sler et al. (2006) Langman et al. (1996) McCarty et al. (2013)

60 1 1 18

1.01e2.00 >2.00 Narayan et al. (2001)

21

Mise6.34 (range)

O Lee et al. (2004) Pockaj et al. (2003)

2 78

/ 0.2e7.0

Ravin et al. (2006)

199

Sartore et al. (2012)

9

15

3.5 4.5-cm full-thickness resection Defect included skin and cartilage Cartilage resection 10 (52.6%) - Most patients partial auriculectomy - 2 patients treated conservatively (skin and perichondrium excision) Cartilage resection in some patients only for reconstructive reasons

24 (14.9%) 37 (23%) 43 (26.7%) 40 (24.8%) in 39/161

1

1e2 2 Unknown 1 NS

1 2

Cartilage included in resection

1 5 (27.8%)

Cartilage included in resection Cartilage-sparing approach, if not violated by direct tumor invasion or a previous biopsy

7 (38.9%) 5 (27.8%) (5.5%) Safer to excise full thickness including cartilage for patients with >1 mm 25 (33%) 29 (38%) 22 (29%)

0.18e18.2 Amputation Perichondrium Biopsy Total excision Subtotal excision No correlation 1

All melanomas

Full-thickness wedge resection 14 (70%) Partial amputation 6 (30%)

Mean 1.26 1.0e2.0*

0.5 1.5 Shpitzer et al. (2007)

17 (10.6%)

0.6e5.00 (range) 4.6 2.17 1.00

Approach to cartilage and perichondrium

Perichondrium preservation Full-thickness wedge resection Partial amputation Total amputation Mohs Wedge excision

7 (9%) 45 (59%) 8 (11%) 5 (7%) 11 (14%) 161 (80.9%)

5 (2.5%) preservation: 6 (3%) 26 (13.1%) 1 (0.5%) 6 (67%)

1 (11%) 2 (22%) 1

Cartilage and perichondrium preservation 2 (22%)

Full-thickness wedge resection 10 (66%)

*Margins were individualized at surgeon’s discretion, NS: not specified, Mis: melanoma in situ.


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F. Toia et al. Table 3

Data on defect reconstruction after removal of external ear melanomas.

Reference Benmeir et al. (1995)

Patients, n 13

Butler et al. (2002)

1


1


1

Cardoso et al. (2006)

1

Cole et al. (2003)c

19

Dessy et al. (2010)

1

Jahn et al. (2006)

Defect

3.5 4.5-cm full-thickness defect involving helical rim, concha, and retroauricular skin Large helical-rim defects extending onto antihelix and scapha Middle and lower third helical rim defects Extensive loss of helical cartilage Average size 10 cm2, Largest 24 cm2

Anterior auricular conchal defect

161

Johnson et al. (1997)

7

Katzbach et al. (2006) Kruse-Lo ¨sler et al. (2006)

1a 1

McCarty et al. (2013)

18

Narayan et al. (2001)

21

Pockaj et al. (2003)

78

Sartore et al. (2012)

9

Shpitzer et al. (2007)

15

Sleilati et al. (2006)

1

Helix defects range 1 2 to 4 6 cm average 2.4 3.3 cm Ablation of upper ear Subtotal ablation: loss of about 60% of the pinna tissue

Earlobe skin

Defect reconstruction n (%) Primary closure Skin graft Partial auriculectomy Total auriculectomy Extended retroauricular advancement flap reconstruction

7 2 1 3

(53.8) (15.4) (7.7) (23.1)

Chondrocutaneous helical rim advancement flap

Chondrocutaneous helical rim and lobular advancement flaps and a scaphal reduction cartilage graft. Kirschner wire and preauricular transposition flap Primary closure 3 (15.8) Locoregional flap 9 (47.5) Split skin graft 2 (10.5) Full skin graft 3 (15.8) Other methods 4 (21) Revolving-door flap versus full-thickness skin graft. None 15 Secondary healing 3 Primary closure 26 Locoregional flap 50 Wedge excision 8 Split skin graft 30 Full skin graft 7 Partial auriculectomy 8 Total auriculectomy 6 Combination of methods 8 Retroauricular to auricular direct pedicle (interpolation) flap.

(9.3) (2.1) (17.8) (34.2) (5.5) (20.5) (4.8) (5.5) (4.1) (5.5)

Bone-anchored prosthesis Bilobed flap from the retroauricular region

Full-thickness skin graft 14 (77.8) Split-thickness skin graft 2 (11.1) Advancement flap and full-thickness 1 (5.55) skin graft Transposition flap and full-thickness 1 (5.55) skin graft Primary closure 3 (14.3) Locoregional flap 15 (71.4) Split-thickness skin graft 3 (14.3) Most of the resections were allowed to close by secondary intention Others were skin-grafted Primary closure 1 (11.1) Locoregional flap 5 (55.6) Full-thickness graft 1 (11.1) Partial auriculectomy 2 (22.2) Locoregional flap 5 (33.3) Wedge excision 10 (66.7) Double-crossed skin flaps


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A systematic review on external ear melanoma

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Table 3 (continued ) Reference Talmi et al. (2002) Total

a b c

Patients, n b

1

Defect

Defect reconstruction n (%)

Conchal defects: range 1.5 2 to 5 6 cm

Flip-flop flap

350

Flaps Grafts Primary closure Wedge excision Combination of methods None/other Insufficient data

98 64 40 18 11 43 78

(28) (18.3) (11.4) (5.1) (3.1) (12.3) (22.3)

Of the 140 patients in this study, only the one with melanoma was considered. Of the 21 patients with skin cancer, only the one with melanoma was considered. Data on 19 patients and 21 procedures.

thickness, and a 2-cm excision margin for thicker melanomas; however, he reduced the excision margin to 0.5 cm for LMM, and he associated it with a multiple-step surgery and three-dimensional (3-D) histology. Other authors consider acceptable lower excision margins, in view of a better cosmetic outcome. Mc Carty et al.6 recommend an excision margin of 1e2 cm for melanomas up to 2 mm in thickness. Sartore et al.8 recommend a 1-cm excision margin for melanomas up to 4 mm in thickness, and Bono et al.3 even claim that a conservative excision margin of 1 cm may be safe for all melanomas, irrespective of tumor thickness. Depth of tumor excision External ear melanomas are often superficial, and “although this auricular dermis is thin, most spread is local via lateral extension rather than penetrating into the underlying cartilage.”6 Detailed data on perichondrium and cartilage removal were available in 13 studies on 409 patients; cartilage was removed in 333 patients (81%), with wedge resection performed in 230 of these patients (56%). A recent review article on the management of malignant melanoma recommends excision of the cartilage27; however, most of the reviewed studies recommend preservation of the underlying perichondrium and cartilage if not invaded by the tumor, and if removal is not indicated for reconstructive reasons.6,8,12,20,28 As for excision margins, recommendations are not based on evidence or outcomes but only based on the personal experience and opinion of the surgeon, as none of the reviewed studies correlated the depth of excision with recurrence rate. Sartore et al.8 and Craig et al.28 advocate a protective role of the perichondrial cartilage against tumor invasion. In retrospective histological review conducted by Craig et al. on 51 ear melanomas, the perichondrium was never violated by the tumor regardless of the Breslow thickness.28 Others authors2,7 believe that it is safer to remove the cartilage; Benmeir et al.2 report invasion of the cartilage in eight out of 13 patients with melanoma (all thicker than 2 mm)2; Narayan and Ariyan7 recommend cartilage excision for melanomas of 1.00 mm “to avoid the risk of submicroscopic residual malignant cell in the perichondrium.”

In the only three reports available in the literature on external auditory canal (EAC) melanomas, the lesion was always removed with a margin of surrounding cartilage and bone.26,17,18 Langman et al.26 and Milbrath et al.17 justify this aggressive treatment considering that tumors of this specific location are often extensive due to a late diagnosis.

Management of regional lymph nodes Although SLNB for melanoma was first introduced in 1992,11 data on SLNB are available only for studies published from 2003, with the exception of two procedures reported by Wei et al.29 in 1998 and a single procedure reported by Narayan et al.7 in 2001. SLNB was performed in 45% of patients; this rate is partially biased by the series published by Augenstein et al.,15 which included only patients with melanomas thicker than 1 mm (with 95% of patients undergoing SLNB). An average of 2.7 SLNs per patient was identified. The sentinel node positivity rate was 8.1%, which is lower than what is usually reported for other body regions (Table 1).30 This datum could reflect a less aggressive behavior or a higher rate of false negativity related to the variability of the lymphatic drainage patterns. Several authors support the idea that these patterns are highly unpredictable,4,6,14,15,20,21,25,27,29 but only a few studies focus on the lymphoscintigraphy and the drainage patterns of the external ear. Nine studies report information about the regional lymph nodes,4,6,9,14,16,20,24,25,29 but only four of them describe the exact location of the identified sentinel node according to Robbins’ topographic classification31 of the cervical node levels.6,9,14,16 These studies confirm that the drainage pattern of the external ear is variable and unpredictable; in Peach et al.’s series of 111 lymphoscintigraphy, lymphatic drainage occurred in a retrograde, antegrade, or transaural pattern, although never contralaterally.14 Despite the great variability, identification of the SLN is possible in >90% of cases.9,20,29 All the studies reported a predominance of drainage at level II, and Shpitzer et al.9 always found at least one SLN at levels II or III in 15 melanomas of the helix9 (Table 1). The study by Peach et al.14 provides the most detailed description of the lymphatic drainage of the external ear:


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10 “up to 30% of patients displayed lymphatic drainage to sentinel nodes anterior and posterior to the ear; the lobule was the only site to drain to the supra-clavicular region; levels II, V, and pre-auricular nodes were the only areas to receive drainage from all sites on the ear; the lobule and concha were the only sites to drain to level I; however, these nodes accounted for only 1.5% of all nodes retrieved and 4.5% of all nodes associated with these two sites. Postauricular drainage occurred from the concha, helical rim, and lobule in 28% of all nodes associated with these three sites, which accounted for 26% of all nodes retrieved.”14 Data on lymph node dissection are available from 17 studies; four of them (24%)2,3,23,27 do not include patients who underwent SLNB. Among lymph node dissections, elective lymph node dissection (ELND) was the most frequently performed (74% of all dissections), and it was required in 9.4% of all treated patients; six of the papers reporting ELND were published in the last 10 years,8,12e14,20,25 and they concern 43 out of a total of 73 procedures. This predominance of ELND in several recent papers is apparently bizarre, but it is partially explained by their retrospective nature. The series by Jahn et al.,12 Pockaj et al.,20 and Ravin et al.13 include patients operated starting from the 1970s or the 1980s. Other reasons for performing an ELND were as follows: patient’s refusal to undergo a two-stage procedure (SLNB and eventual dissection)25 and thick melanomas.8 The rate of metastasis was comparable among ELND and completion lymph node dissection (CLND) (30% and 33%, respectively). The much higher rate of ELND compared to SNB (30% vs. 8%, respectively) is apparently bizarre; however, it is likely to depend on the following factors: (1) the positivity rate of ELND is calculated for a limited sample (47 ELND, of which 14 were positive); (2) one of the two widest series with a high positivity rate2 included only patients with a high Breslow thickness (mean: 3.7 mm; range: 2.6e4.8); (3) in the other wide series with highly positive ELND,20 ELND was performed in a selected group of patients, while most patients in the same series underwent SNB or follow-up e Pockaj et al. admit that patients who underwent ELND had much thicker melanomas compared to those who did not, and that this is likely to reflect the surgeon’s decision to perform more aggressive surgery.

Reconstruction Only 19 studies on 351 patients (eight of which are case reports) specifically address reconstruction after ear melanoma removal (Table 3). In all but one study,32 the external ear was reconstructed with autologous tissues2,4e9,12,20,23,24,32e38: Katzbach et al.32 reported, instead, the use of a bone-anchored ear prosthesis, which they consider an elective indication after malignant tumor resection; they also found this technique to be advantageous because of its reversibility and predictable cosmetic result within a short period of time. Table 3 summarizes data on the type of reconstruction from the selected 18 papers. Local flaps are more commonly used than grafts (28% vs. 18%); split-thickness skin grafts are more commonly used than full-thickness skin grafts (58% vs. 39%). Skin grafts are usually taken from the contralateral ear, the neck, the supraclavicular

F. Toia et al. region, or the inner part of the arm.6,33 Primary closure or wedge excision accounts for 17%; combination methods account for 3.1%. Among studies on the type of reconstruction, one study accounting for 22.3% of patients reported insufficient data, as authors only stated that defects were allowed to close by secondary intention or skin grafts. As for external ear defects in general,39e43 retroauricular and mastoid skin is the most popular flap donor bank; several authors report on one-stage reconstruction with a modified Masson flap44 (“revolving door” flap33 and “flip-flop”34 flap), which is tunneled through an opening in the conchal cartilage. Sleilati et al.35 also reported on lobule reconstruction with double-crossed skin flaps from the preauricular and retromandibular regions. Kruse-Lo ¨sler et al.25 reported a one-stage reconstruction of the helical border with a bilobed flap from the retroauricular region, but if removal of the helical cartilage is required, two-stage reconstruction is often performed: Johnson et al.5 used a retroauricular “interpolation” flap in two stages. Butler et al.36 reconstructed the helical and antihelical defect with a conchal contralateral graft and a retroauricular flap, divided at a second stage 4 weeks later, when a skin graft is placed at the retroauricular surface; Cardoso et al.24 replaced the cartilage with a Kirschner wire to maintain helical shape and reconstructed the defect with a retroauricular flap in two stages.

Conclusions This systematic review provides epidemiological data on the distribution, location, and histological characteristics of external ear melanomas. It also highlights the lack of agreement on the surgical management of ear melanoma. The available literature does not allow drawing of clinical guidelines, but it only summarizes the most common approach to tumor excision, lymph node management, and reconstruction, based on authors’ opinions rather than on scientific evidence: - Tumor excision: For Tis and T1 melanomas, standard recommended margins of 0.5 and 1 cm, respectively, are commonly used. For T2eT4 melanomas, some authors recommend a standard 2-cm margin, while, as for other head and neck melanomas, most authors consider lower excision margins safe (1 cm minimum). With respect to the depth of excision, the most common approach is to preserve the perichondrium and cartilage if not invaded by the tumor. - Lymph node management: Indications for SNB follow those for other body regions, while ELND is no longer indicated. Despite a great variability of the lymphatic drainage patterns, identification of the sentinel lymph node is possible in >90% of cases. The lower sentinel node positivity compared to other body regions (8.1%) could reflect a less aggressive behavior or a higher rate of false negativity. Variability of the lymphatic drainage patterns and the proximity of the tumor to lymph nodes make this procedure more challenging, and recommend a longer training, as for other head and neck melanomas. - Reconstruction: Most authors do prefer flaps to grafts, and some of them advocate a better aesthetic


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A systematic review on external ear melanoma outcome.33 When cartilage resection is planned, wedge excision is another popular option. However, the choice of the reconstructive technique does not differ in the case of ear melanoma; rather, it reflects surgeon’s preferences. Available data on the prognosis of ear melanoma are contrasting: a favorable prognosis seems to justify the tendency towards a more conservative treatment, but there is no sufficient evidence or general agreement. Large multicentric studies are desirable to collect evidence on the effect of different treatments on recurrence and survival and to draw clinical evidence-based guidelines.

Conflict of interest None.

Funding None.

Ethical approval N/A.

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Malignant melanoma of the external ear.

Painless nodule on the external ear.

Microsurgical ear replantation-is venous repair necessary?-A systematic review.

[Paracoccidioidomycosis of the external ear].

Keloids of the external ear.

Coccidioidomycosis of the external ear.

Infections of the external ear.

Socioeconomic and lifestyle factors and melanoma: a systematic review.

Hepatic resection for metastatic melanoma: a systematic review.

Risk prediction models for melanoma: a systematic review.

The use of interferon in melanoma patients: a systematic review.

Effects of external cues on gait parameters of Parkinson's disease patients: a systematic review.

Ewing Sarcoma of the External Ear Canal.

Leiomyosarcoma of the external ear--case report.

[Leiomyosarcoma of the external ear canal].

[Reconstruction of the rudimentary external ear].

Congenital Abnormalities of the External Ear.

Dermatologic diseases of the external ear.

[A rare cause of an external ear canal stenosis].

External ear dermatitis in a Crohn's disease patient.

Gaint epidermoid cyst of external ear- a rare case report.

Middle ear cholesteatoma treated with a mastoidectomy: a systematic review of the measures used.

Systematic review and meta-analysis on outcomes for endoscopic versus external dacryocystorhinostomy.

Are head bandages really required after middle-ear surgery? A systematic review.