Fig.2
Melanoma of the face in a 3-years-old girl
(from Handfield-Jones, 1996 [9])


Fig.3
Survival curves in patients < 20 years
(from Pappo, 2003 [8])

References

1.Naasan A, al-Nafussi A, Quaba A. Cutaneous malignant melanoma in children and adolescents in Scotland, 1979–1991. Plast Reconstr Surg 1996; 98:442–446
2. Schmid-Wendtner MH, Berking C, Baumert J, et al. Cutaneous melanoma in childhood and adolescence: an analysis of 36 patients. J Am Acad Dermatol 2002; 46: 874–879.
3. JJ Strouse et al, Pediatric Melanoma: Risk Factor and Survival Analysis of the Surveillance, Epidemiology and End Results Database J Clin Oncol 2005: 23:4735-4741
4. Berg P, Lindelof B. Differences in malignant melanoma between children and adolescents. A 35-year epidemiological study. Arch Dermatol 1997, 133, 295–297.
5. Bader JL, Li FP, Olmstead PM, et al. Childhood malignant melanoma: incidence and etiology. Am J Pediatr Hematol Oncol 1985;7: 341–345.
6. SK Richardson et al, Congenital and infantile melanoma: Review of the literature and report of an uncommon variant, pigment-synthesizing melanoma. J Am Acad Dermatol 2002;47: 77-90
7. Saenz NC, Saenz-Badillos J, Busam K, et al: Childhood melanoma survival. Cancer 1999: 85:750-754
8. Pappo AS: Melanoma in children and adolescents. Eur J Cancer 2003; 39:2651-2661
9. Handfield-Jones SE, Smith NP. Malignant melanoma in childhood. Br J Dermatol 1996; 134:607–16.


Fig.1
Paediatric melanoma: atypical melanocytes arranged in sheets.

MELANOMA IN PAEDIATRIC AGE

Paediatric melanoma [FIG. 1], although rare, represents about 2% of all melanomas [1], constituting a not negligible fraction (1-3%) of all paediatric malignancies [2]. This tumor, that displays a rising incidence of 2.9% per year [3], mostly affects patients of age > 15 years (85%) and less frequently (15%) children in prepubertal age [4]; the incidence in the second decade is in fact eightfold greater than in the first: 0.6 per milon in children aged 0-10 and 6.3 per milion in individuals aged 11-20 [5]. Prepubertal melanoma can be clinically subdivided into three categories based on the age at which the melanoma is diagnosed: congenital melanoma (in utero to birth), infantile melanoma (birth to the first birthday), and childhood melanoma (first birthday to the onset of puberty)[6]. Most paediatric melanomas (more than 60%) arise in a preexisting congenital nevus, some melanomas arise de novo, in apparently normal skin [7]; trans-placental melanoma, occurring by maternal transmission to the placenta, fetus, or both, is very rare [6]. Inasmuch as melanoma in children is uncommon, pigmented lesions of paediatric age may be underdiagnosed and more than half of paediatric melanomas appear to have a delayed diagnosis [7]. Clinical signs commonly used in the diagnosis can be not adequately evaluated [FIG. 2]; the most common signs are recent growth, color change, pain, bleeding, and ulceration [7]. The most frequent sites are the upper and lower limbs, followed by the trunk and head and neck [8]. Paediatric melanoma appears to have a prognosis similar to that of adult melanoma, depending on some basic parameters, as thickness, ulceration, and lympn node status [FIG. 3]. Patients with localized disease have an excellent outcome, with an estimated 10-year survivals of 90%, whereas those with nodal and distant metastases have estimated 10-year survivals of only 60 and 25%, respectively [8]. Recommended guidelines for the resection of paediatric and adolescent melanoma follow the same principles as those established for adult melanoma: in situ melanoma can be managed with simple excision; lesions thinner than 1 mm in depth can be resected with a 1-cm margin and thicker melanomas with a 2-cm margin. Sentinel node biopsy is used in melanomas thicker than 1 mm and/or in patients with lesions <1 mm in the presence of ulceration or a Clark’s level of invasion of IV or V. Among non surgical therapies of paediatric melanoma, interferon has been used; chemiotherapy and radiotherapy have not offered relevant results.

page 15

The other pages

Home Page: Dermatopathology - C. Urso, MD
page 1: Melanoma of the skin
page 2: Histologic diagnosis of cutaneous melanoma
page 3: Melanoma arising in a melanocytic nevus
page 4: Infiltration and pseudoinfiltration
page 5: Sweat gland carcinomas
page 6: The sentinel lymph node in cutaneous melanoma
page 7: Bullous cutaneous diseases
page 8: Spitz nevus
page 9: Prognostic factors in cutaneous melanoma
page 10: The dysplastic nevus
page 11: Dermatofibrosarcoma protuberans
page 12: Melanoma in situ
page 13: Merkel cell carcinoma
 page 14: Basal cell carcinoma
 page 15: Melanoma in paediatric age
last page: Questions, comments and opinions