ORIGINAL PAPER

Eric Vandeweyer á Francois Sales á Rika Deraemaecker

Cutaneous malignant melanoma in children

Received: 25 August 1999 /Accepted: 9 February 2000

Abstract Although rare, malignant melanoma does occur in children. As in adults,prognosis clearly depends on rapid diagnosis and early treatment. This paper describeseight cases of con®rmed malignant melanoma in children, with full case history andpathological ®ndings. Mean follow-up was 109.8 months. Early diagnosis and low Bre-slow indices provide good results in the form of absence of recurrence and long-termsurvival prognosis. The paper also includes a discussion of speci®c risk factors a�ectingchildren. In order to encourage earlier diagnosis and improved prognosis, it must be keptin mind that in children too, the skin as an organ should be included in any physicalexamination.

Conclusion As for adult patients, early clinical detection, speedy histologicalcon®rmation and prompt surgery is the only way to achieve a long survival periodfor children su�ering from malignant melanoma.

Key words Children á Malignant melanoma á Surgery á Early diagnosis á Skin cancer

Introduction

Malignant melanoma is considered to be rare in children,although its incidence rises rapidly during adolescence. Itconstitutes 1%±3% of all malignant diseases in childrenbut less than 1% of all melanomas are found in childrenand adolescents. The same diagnostic criteria, histological®ndings, prognostic factors and treatment methods applyalike to children and adults. Because of its rarity, there issome confusion or hesitance in diagnosing malignantmelanoma in children, frequently causing treatment to bedelayed, therefore, late diagnosis is particularly unfa-vourable in such an aggressive skin tumour.

Subjects and methods

Only children under the age of 14 were considered for this study.From December 1978 to June 1990, eight children presenting

con®rmed malignant melanoma were treated at the Jules BordetCancer Institute. The sample included six males and two females.Their average age was 11 years (range 1.5±14 years) at the timethe disease was diagnosed. The main reasons for seeking a con-sultation are listed in Table 1. In three of the children, more thanone criterion was present. Table 2 identi®es the location of thelesions. On clinical examination, the diameter of lesions recordedwas 7.4 mm (range 4.5±10 mm). Excisional biopsy was performedin each case to con®rm the clinical diagnosis of malignantmelanoma and to ascertain the histological type, the Breslowindex and Clark level. The Breslow index is de®ned as the maxi-mum thickness of the tumour, measured in millimetres, on thehistological slides of the specimen. The Clark level representsthe maximum depth of invasion into the skin, related to thedermis.

Table 3 provides the tumour, nodes, metastasis (TNM) classi-®cation according to the American Joint Committee on Cancercriteria. The complete pre-operative screening included a physicalexamination and comprehensive skin investigation, liver ultra-sound examination, blood tests, chest CT scan, brain CT scan andbone scintigraphy. Mean follow-up in this series was 118.2 months(range 24±198 months).

Eur J Pediatr (2000) 159: 582±584 Ó Springer-Verlag 2000

E. Vandeweyer á F. Sales á R. Deraemaecker (&)Department of Plastic and Reconstructive Surgery,Institut Jules Bordet, Rue He ger-Bordet 1,

1000-Bruxelles, BelgiumTel.: +0032-2-5413168

Results

All patients presented con®rmed malignant melanomaafter excision biopsy. Table 3 summarises the histolog-ical evaluation of the specimens. The disease had spreadin only one patient at the time of consultation but acomplete regression was observed after eight coursesof systemic chemotherapy using cisplatin (50 mg/m2),vindesine (3 mg/m2) and dacarbazine (450 mg/m2). Allpatients underwent wide excision following diagnosis,ranging from 1±3 cm around the biopsy scar. No localrecurrence or dissemination of the disease were found onfollow-up.

Discussion

Malignant melanoma before the age of puberty is rareand represents 0.3%±0.4% of patients in two large seriesfrom Norway [7] and the United States [1]. For manyyears, it was thought that malignant melanoma inchildhood was benign. This misleading concept wasclari®ed by Spitz [12] when he de®ned the histologicalfeatures of the benign ``juvenile melanoma'' as not beinga true melanoma and distinguished this benign formfrom the true malignant melanoma. Prior to this, Spitz

nevi were frequently misdiagnosed as melanomas. Thesenevi follow a benign course, thus encouraging the mis-conception of a benign evolution of malignant melano-ma in children. Malignant melanoma in children iscapable of metastasis and may follow an aggressivepattern [4]. The connection between adult risk factorssuch as inability to tan or su�ering sunburn accompa-nied by blisters on the one hand and the development ofchildhood malignant melanoma on the other has notbeen proven [11].

In up to 66% of cases, increased mortality has beenattributed to late diagnosis. This situation most com-monly arises as the result of the physician's reluctance torecognise malignant melanoma in children [9, 11].Clinical symptoms indicative of malignant melanoma inchildren are lesions which are large or expanding, whichhave irregular edges and pigment distribution, andwhich undergo surface changes such as ulceration or lossof skin markings [11]. Malignant melanoma may beassociated with clinical symptoms such as pain, pruritusor bleeding. When these clinical symptoms are present,an early biopsy is essential in order to rule out amalignant melanoma.

Some speci®c risks have been recorded for childrensuch as giant congenital nevi, dysplastic nevus syndromeand xeroderma pigmentosum or immunode®ciency con-ditions [2, 11]. Trozak et al. [14] observed that at least33% of pre-puberty malignant melanomas develop fromlarge congenital nevi and that 50% of cases of malignantmelanoma within large congenital nevi occur in the ®rstdecade of life. Based on this danger of malignant pro-gression, some authors recommend the prophylactic re-moval of large congenital melanocytic nevi in early life[3]. At the very least, close clinical monitoring at frequentintervals is recommended to allow for early diagnosis.This signi®es yearly clinical examination with close at-tention being paid to any changes in size or colour.

Dysplastic nevi are known potential precursors ofmalignant melanoma [2, 11]. A dysplastic nevus is clin-ically de®ned as a nevus that tends to be larger thancommonly acquired nevi, with diameters of between 5and 10 mm. Typically, these nevi have fuzzy, indistinctedges and are multicoloured shades of tan, brown, pinkand black. They can vary from macular forms to truepapules [11]. In children with dysplastic nevi, malignantmelanoma has been documented as early as from theage of 10 years [2, 11].

Nevertheless, there are no guidelines for clinical fol-low-up in a�ected children but thorough and regularclinical examinations are recommended, i.e. a yearlyclinical examination. Xeroderma pigmentosum is a rareinherited disorder characterised by photosensitivity anddefective cellular repair to DNA damaged by ultravioletradiation [2]. Children with xeroderma pigmentosummust be monitored very closely. Protective clothing andthe regular use of e�ective sunscreens are essential. Hostimmunity plays an important role in the biologicalbehaviour of malignant melanoma. A melanocyticdysplasia can progress very rapidly to true malignant

Table 1 Diagnostic circumstances

Criteria Number of cases

Colour change 2Size change 1Bleeding 2Pruritus 2Screening 4

Table 2 Localisation of the lesions

Site Number of cases

Occipital area 1Foot 1Back 3Thigh 2Shoulder 1

Table 3 Correlation between histological ®ndings and TNMclassi®cation (according to the American Joint Committee onCancer classi®cation)

Case Breslow index (mm) Clark level TNM classi®cation

1 3.2 III T3N0M02 In situ I TISN0M03 0.5 II T1N0M04 0.71 II T1N0M05 0.28 II T1N0M06 0.43 II T1N0M07 3 IV T3N1M08 0.86 II T2N0M0

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melanoma in immuno-compromised children, a moreaggressive approach to the management of dysplasticnevi in such patients is recommended [2]. As in adults,early clinical detection or suspicion, rapid con®rmationby excisional biopsy and prompt surgical treatment isthe only way to obtain long survival times in childrena�ected by malignant melanoma.

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