Giant Congenital Melanocytic Naevi Research Articles

The surgical challenge of giant circumferential congenital naevi of the extremities: a 13-year follow-up of two cases

The surgical management of giant congenital melanocytic naevi involving extremities in a circumferential pattern may represent a surgical challenge with respect to their resection and subsequent reconstructive options. We present our experience in the management of circumferential giant congenital melanocytic naevi involving the upper limb in one patient and lower limb in another one. They were managed and followed up for 13 years. The lesion regenerated following dermabrasion in the first patient and regenerated following tangential excision and split-thickness skin graft in both patients. Therefore, another approach was used, full-thickness resection of the lesions and reconstruction with dermal regeneration template (Integra™), followed by ultrathin split-thickness skin graft. The last approach resulted in no recurrence during a 3-year follow-up. The complexity of these lesions and the limitation of different surgical approaches have been highlighted. Full-thickness excision of the affected integument and the use of dermal regeneration template is a useful technique which can be considered in the management of circumferential giant melanocytic naevi, particularly when other established methods have failed to provide satisfactory results.

Giant congenital melanocytic naevus associated with Dandy-Walker malformation, lipomatosis and hemihypertrophy of the leg

A 30-year-old woman presented with congenital hyperpigmented plaques and multiple tumoral lesions. Her left leg was hypertrophic, although the bones were normal. Dermatological examination revealed hyperpigmented macules and plaques with hair on the legs, abdominal and lumbar areas (covering > 60% of the total body surface) and multiple naevi on the face, arms, back and chest. Multiple soft masses of various sizes, some of which colocalized with hyperpigmented plaques, were seen on the trunk and legs. Malignant melanoma was excluded by histopathological examinations of multiple biopsy specimens obtained from the pigmented lesions. Histopathological examination of one of the soft masses showed that it was a lipoma. Cranial magnetic resonance imaging and computed tomography scans showed an enlarged fourth ventricule and vermis hypoplasia (Dandy-Walker malformation; DWM). Neurocutaneous melanosis is a rare combined abnormality of the skin and central nervous system. A few case reports relating to the coexistence of giant congenital melanocytic naevus, lipomatosis and hemihypertrophy have been published in the literature. We report this very rare case of giant melanocytic naevus with lipomatosis, hemihypertrophy of the leg, and DWM.

Early serial Q-switched ruby laser therapy for medium-sized to giant congenital melanocytic naevi

Medium-sized to giant congenital melanocytic naevi (CMN) are difficult to treat, especially if the lesions appear on the face or extremities where treated areas are visible and cosmesis is important. In infants, nests of pigmented naevus reside more superficially and the skin is more transparent than in adults, so we treated medium-sized to giant CMN with early serial Q-switched ruby laser therapy from infancy. We treated nine patients with medium-sized to giant CMN on the face or upper limbs from 1 month of age with early serial Q-switched ruby laser therapy. The laser power was initially 5 J cm(-2) and increased in 0.5 J cm(-2) steps to a maximum of 10 J cm(-2). There were three treatment sites on the forehead, one on the temple, one on the cheek and four on the upper arm. It took 8-15 treatments for the CMN to become a colour similar to the surrounding skin. The mean number of treatments was 9.6. The colour was reduced to 0-20% of the colour of the baseline lesion in all nine patients. Partial slight repigmentation occurred in eight of these patients. These naevi were treated with an additional one or two Q-switched ruby laser irradiations and successfully lightened for at least 1 year. In the remaining patient, pigmentation returned to a level similar to the original lesion within 1 month of the last treatment. Therefore, the lesion was excised for cosmetic reasons. After the treatment series, the skin texture was fine and no patients had hypertrophic scarring. Although treatment of one patient with the Q-switched ruby laser therapy failed, the remaining patients responded well and had good to excellent skin texture without hypertrophic scarring. Early serial Q-switched ruby laser treatment, starting from infancy, is a promising treatment method for this condition.

Cartilaginous differentiation in a giant congenital melanocytic naevus

Cartilaginous differentiation in a giant congenital melanocytic naevus

Results of the early use of tissue expansion for giant congenital melanocytic naevi on the scalp and face

Giant congenital melanocytic naevi (GCMN) are uncommon, have a significant morbidity and require extensive treatment. This paper presents results after complete excision of GCMN on the scalp, forehead or periorbita after early tissue expansion. Based on 15 years of experience, we want to show that performing tissue expansion at a young age is advisable. We included 17 consecutive patients in whom 38 tissue expanders were used. Early and late complications were noted. Patients were seen for a clinical follow up in which scars and re-pigmentation were evaluated with a validated scar scale (POSAS). All GCMN could be excised completely with early tissue expansion. The age at treatment ranged from 4 months to 2 years of age. With a mean follow-up period of 8.7 years, mild re-pigmentation was seen in only three patients and none of the patients developed a malignant melanoma. Complication rates are comparable with the literature. Tissue expansion is a good method for removing GCMN located at the scalp or face with good cosmetic and oncological results. Performing tissue expansion at a young age is advisable.

Rapid, severe repigmentation of congenital melanocytic naevi after curettage and dermabrasion: histological features

Curettage and dermabrasion are effective in treating giant congenital melanocytic naevi (GCMN). We report two patients with rapid, severe postoperative repigmentation. To the best of our knowledge this is the first report on the histological features of such patients. We wish to call attention to histological features that may cause rapid, severe repigmentation after curettage and dermabrasion of medium to giant CMN. From 1998 to 2002, we treated 23 patients with medium to giant CMN with curettage and dermabrasion. Patients being treated ranged in age from 1 month to 19 years. Histological samples were taken from the centre of naevi in all patients during surgery. Histological types were 12 intradermal and 11 compound. Follow-up after curettage lasted at least 3 years. Among our 23 patients only two showed repigmentation soon after surgery. Histological sections from these two patients indicated naevoid cells in the deep dermis along hair follicles or sebaceous glands. However, no such pigmented naevoid cells along hair follicles were observed in samples from patients successfully treated with curettage and dermabrasion with less repigmentation. Although we saw only two cases of repigmentation soon after curettage and dermabrasion, we suspect a correlation between pigmented naevoid cells around hair follicles and repigmentation. If histological sections of skin biopsies show pigmented cells along hair follicles in the deep dermis, other treatments such as total skin resection followed by skin grafting or tissue expansion may be better choices than curettage or dermabrasion.

Ulcerated sclerotic giant congenital melanocytic naevus: case report and review of the literature

We report a case of a white girl with an ulcerated giant congenital melanocytic naevus that initially had a hard, stony consistency but in which the pigmentation and the induration are progressively vanishing. Very few cases of this variant of GCMN, known as sclerodermoid GCMN or desmoplastic hypopigmented hairless naevus, have been reported to date, and clinical evolution seems to be heterogeneous. We review the published cases and propose the term 'sclerotic hypopigmented GCMN as a common descriptor of this type of congenital melanocytic naevus.

Congenital naevi treated with erbium:YAG laser (Derma K) resurfacing in neonates: clinical results and review of the literature

Giant congenital melanocytic naevi (CMN) are often disfiguring, potentially malignant pigmented lesions present at birth. Their management is based on two main considerations: attempt to minimize the risk of malignancy and to obtain an acceptable cosmetic result. In the past various approaches have been used to treat these naevi. To describe clinical and histopathological results after treatment of CMN in neonates with erbium:YAG (Er:YAG) laser resurfacing. Ten children with CMN were treated with Er:YAG laser resurfacing in the first weeks of life. Laser ablation was well tolerated by all children and immediate results were good. At a total follow-up ranging from 3 to 36 months we saw good results, with no or minimal repigmentation, in eight of 10 patients. Patients experienced minimal side-effects such as postoperative pain, bleeding and scar formation. Postoperative histopathology showed disappearance of heavily pigmented cells in the upper part of the dermis. Er:YAG laser resurfacing is an effective method of ablating CMN, with minimal scarring and postoperative complications.

Distinct phenotypic changes between the superficial and deep component of giant congenital melanocytic naevi: a rationale for curettage

Giant congenital melanocytic naevi (GCMN) convey a 14-fold increased melanoma risk. In contrast, medium congenital melanocytic naevi (MCMN) are rarely associated with malignant transformation. Management of patients with GCMN is challenging and there is no consensus on the most appropriate strategy for treating these patients. To provide a rationale for performing curettage of GCMN in the neonatal period in order to reduce the risk of malignant transformation to melanoma. Twenty-six infants with GCMN who underwent biopsies before excisional surgery (n = 7) or curettage (n = 19) during the past 14 years (Academic Hospital, Vrije Universiteit Brussel) and 10 MCMN patients who underwent excision biopsies (Radboud University Nijmegen Medical Centre) were included in this study. Using these biopsies, we performed genetic and detailed immunohistochemical evaluations of changes that are associated with malignant transformation. Variables of interest included melanoma-associated BRAF mutations, proliferative activity, vascularity, cellular context and extracellular matrix architecture. GCMN and MCMN did not show oncogenic BRAF mutation and displayed similar features with respect to the amount of nonmelanocytic cells within the naevus and matrix architecture. Naevus cells in the superficial component of the GCMN, however, were more proliferative, and this component was more vascular compared with its deep component and with MCMN. In this study, none of the 19 newborn patients who underwent curettage developed a melanoma within a mean follow-up time of 7 years. The data presented here support the idea that curettage of GCMN in neonates has the potential for lowering the risk of developing cutaneous melanoma by not only obtaining an important numerical reduction of naevus cells but also removing the 'active' melanocytes.

Giant congenital melanocytic naevus in association with hepatic melanin deposits

This report describes a neonate with a giant congenital pigmented naevus over most of the trunk surface area, along with multiple satellite lesions, especially over the legs. Magnetic resonance imaging of the abdomen showed large deposits of melanin in the glutei bilaterally, the rectus abdominis muscles and the liver. Treatment consisted of repeated dermabrasion of the naevus over the lower back with CO2 laser (Silk Touch), followed by autologous skin grafting. No evidence of malignant transformation was observed. Giant congenital melanocytic naevus may be associated with involvement of internal organs other than the central nervous system.

Cutaneous melanoma in childhood and adolescence: retrospective study of 32 patients

This study was performed to analyse the behaviour, risk factors, prognosis and evolution of cutaneous melanoma in childhood and adolescence treated in a single institution. A retrospective study was performed between 1980 and 2000 of patients aged 18 years or younger followed at the Hospital do Cancer de Sao Paulo, Brazil. Data included demographic status, risk factors, clinical and histopathological characteristics of the primary and metastatic lesions, stage and follow-up. Seventeen female (53.1%) and 15 male (46.9%) patients were studied. Twelve patients (37.5%) were aged 12 years or younger. The trunk was the most common location (14 patients; 43.8%). Five patients (15.6%) had giant congenital melanocytic naevus, three (9.4%) had xeroderma pigmentosum and one (3%) had dysplastic melanocytic naevus. Nodular melanoma was the most frequent histological type and 43.8% had a thickness of more than 4 mm. Five of the 32 patients (15.6%) were lost to follow-up and 15 (46.9%) were alive at the last year's follow-up, 11 (34.4%) without disease and four (12.5%) with active disease. The 5-year overall survival was 64.34%. An overall survival of 11.71% was found in patients with visceral metastasis with or without cutaneous and/or lymph node involvement, whereas the corresponding value was 90.48% (P value=0.0002) in patients with only cutaneous and/or lymph node metastasis. Cutaneous melanomas are uncommon in the young and are seldom diagnosed in the early stages, perhaps due to a reluctance to accept this diagnosis in this age group. Prevention and early stage diagnosis depend upon the recognition that this disease is present in the young.

Melanoma in children and adolescents

Childhood and adolescent melanoma is rare, accounting for only 1.3% for all cases of cancer in patients under the age of 20 years. However, in 15–19 year olds, melanoma accounts for up to 7% of all cancers. Review of reported cases in this age group reveals that predisposing ‘paediatric’ conditions such as a giant congenital melanocytic naevi or xeroderma pigmentosum are rarely present. Furthermore, inactivating germ-line mutations of the gene CDKN2A have only been reported in 1.5% of cases of early onset melanoma. Epidemiological studies suggest that interactions between solar exposure, development of naevi, pigmentary traits, and a family history of melanoma are the main determinants of melanoma development during the first 20 years of life. As yet, there are no available staging or treatment strategies for this group of patients so treatment recommendations are based on the adult experience. To improve our understanding of the natural history of melanoma and to identify the most appropriate therapies for young patients with this disease, practising physicians are encouraged to enroll their patients, especially those with advanced stage disease, in cooperative group trials which incorporate newer staging systems and promising therapies.

Psychosocial sequelae in 29 children with giant congenital melanocytic naevi.

Giant congenital melanocytic naevus (GCMN) may be expected to affect psychosocial functioning of children and their parents due to deviant appearance and painful treatment. To obtain insight into clinical aspects and psychosocial functioning of those suffering from GCMN, 29 children diagnosed with GCMN syndrome or single GCMN received a dermatological examination, were interviewed, and their mothers and teachers completed standardized questionnaires on the child's competence and behavioural/emotional problems and their own adjustment. Social problems were reported for 30% of the patients and behavioural/emotional problems for 25.9%. There was no correlation between visibility of the naevus, treatment or child age and psychological problems. Mothers reported considerable psychosocial burden. It is concluded that children with GCMN are at increased risk of social and behavioural/emotional problems, and mothers suffer considerable psychological impact of their child's condition.

DNA content and cell proliferation in giant congenital melanocytic naevi (GCMN). An analysis by image cytometry.

GCMN may be precursors of melanoma, and it has been suggested that the presence of atypical foci could increase the risk of malignant transformation. In order to better define atypical GCMN, we analyzed DNA content and proliferative activity in 21 samples of GCMN, with (n=13) or without (n=8) cytologic atypia. Six benign acquired naevi (AN) and 6 malignant melanoma (MM) were used as controls. DNA content was determined with the CAS 200 image analyzer, and DNA histograms were classified according to the Auer classification. The proliferative indices (PI) were measured after Ki 67 immunostaining using the CAS 200 system. All AN and GCMN without atypia showed class I histograms (normal DNA content) and low PI (mean 1.9 and 2.1). Atypical GCMN showed in 10 cases an abnormal DNA content (class III or IV histograms) with low PI (mean 2.7), and in 3 cases a normal DNA content (class I histograms) with higher PI (mean 16.2). All MM displayed abnormal DNA content and high PI (mean 32.6). In conclusion, abnormal DNA content seems to correlate with cytologic atypia in GCMN. Atypical GCMN exhibit an overall pattern of DNA content and cell proliferation intermediate between non-atypical naevi and MM.

Neonatal currettage of giant congenital melanocytic naevi

Neonatal currettage of giant congenital melanocytic naevi

Successful treatment of a giant congenital melanocytic naevus with the highenergy pulsed CO2 laser

Successful treatment of a giant congenital melanocytic naevus with the highenergy pulsed CO2 laser

Successful treatment of a giant congenital melanocytic naevus with the highenergy pulsed CO 2 laser

Summary The successful use of the high energy pulsed CO 2 laser to treat a giant congenital melanocytic naevus in an infant following unsuccessful neonatal curettage is reported.