Giant Congenital Melanocytic Nevus (GCMN) - A New Hope for Targeted Therapy?

  • Georgi Tchernev Medical Institute of Ministry of Interior (MVR), Department of Dermatology and Dermatologic Surgery, General Skobelev 79, 1606 Sofia
  • Ilia Lozev Medical Institute of Ministry of Interior, Department of General, Vascular and Abdominal Surgery, General Skobelev 79, 1606 Sofia
  • Ivan Pidakev Medical Institute of Ministry of Interior, Department of General, Vascular and Abdominal Surgery, General Skobelev 79, 1606 Sofia
  • Torello Lotti University of Rome “G. Marconi”, Rome
  • Uwe Wollina Department of Dermatology and Allergology, Academic Teaching Hospital Dresden-Friedrichstadt, Friedrichstrasse 41, 01067 Dresden
  • Serena Gianfaldoni University G. Marconi of Rome, Rome
  • Jacopo Lotti Department of Nuclear, Subnuclear and Radiation Physics, University of Rome "G. Marconi", Rome
  • Katlein França Institute for Bioethics & Health Policy; Department of Dermatology & Cutaneous Surgery; Department of Psychiatry & Behavioral Sciences, University of Miami Miller School of Medicine - Miami, FL
  • Atanas Batashki Abdominal and Thoracic Surgery, Department of Special Surgery, Medical University of Plovdiv, bul. "Peshtersko shose" Nr 66, 4000 Plovdiv
  • Georgi Konstantinov Maximov Onkoderma/ADCRSTR cooperation Group- Policlinic for Dermatology, Venereology and Dermatologic Surgery, General Skobelev 26, Sofia
  • Anastasiya Chokoeva Department of Dermatology and Venereology, Medical University of Plovdiv, 15A Vasil Aprilov blvd., 4002 Plovdiv
Keywords: nevi, congenital nevi, dermabrasion, surgery, NRAS, targeted therapy

Abstract

We present a 6-month-old male patient, who was consulted with dermatologist by his parents, because of a pigmented lesion, present since birth, covering almost the all skin of the back and buttocks.  A sharply bordered, unequally coloured congenital pigmented nevus, measuring approximately 21 cm in diameter was observed in the whole body skin examination. The lesion was affecting the lower 2/3 of the skin of the back and the top half of the gluteus area, extending to the lateral part of the tors, forward the abdomen and the upper lateral part of the hips, composed by multiple darker-pigmented nests and several lighter areas, with single depigmented zones, hairy surface, irregularly infiltrated on palpation. Congenital melanocytic nevi are presented in approximately 1% of newborns, while giant congenital melanocytic nevi (GCMN) are the most uncommon subtype of them; with occurrence rate 1 in 50,000 births. They affect 2% of a total body surface or presenting in a diameter larger than 20 cm in older children. Although not common, the possible malignant transformation remains one of the most important considerations related to them, as the related lifetime risk of melanoma is 4% to 10%. Treatment recommendations include non-surgical methods as dermabrasion only within the first two weeks of life, for prevention the possible melanocytic deeper migration, while serial surgical excisions or tissue expanders could be useful treatment tool even in later stages. Nevertheless, cosmetic result is not always satisfactory, and the risk of malignant changes remains, in cases of previous melanocytic migration in deeper layer. Recent article suggests the potential role in the treatment of GCMN with NRAS inhibitor trametinib, approved for treatment of advanced melanoma, associated with underlying NRAS mutations. Although promising, the drug could be useful in paediatric patients, only with associated NRAS gene mutation. It is still unclear whether it could be helpful, independent of the NRAS status.

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References

Ma T, Fan K, Li L, Xie F, Li H, Chou H, Zhang Z. Tissue expansion in the treatment of giant congenital melanocytic nevi of the upper extremity. Medicine (Baltimore). 2017; 96(13):e6358. https://doi.org/10.1097/MD.0000000000006358 PMid:28353563 PMCid:PMC5380247

Kopf AW, Bart RS, Hennessey P. Congenital nevocytic nevi and malignant melanomas. J Am Acad Dermatol 1979; 1:123–30. https://doi.org/10.1016/S0190-9622(79)70009-0

Recio A1, Sánchez-Moya AI2, Félix V3, Campos Y4. Congenital Melanocytic Nevus Syndrome: A Case Series. Actas Dermosifiliogr. 2017. pii: S0001-7310(16)30371-4. https://doi.org/10.1016/j.ad.2016.07.025

Martín-Carrasco P, Bernabeu-Wittel J, Dominguez-Cruz J, Zulueta Dorado T, Conejo-Mir Sanchez J. "Sky Full of Stars" Pattern: Dermoscopic Findings in a Desmoplastic Giant Congenital Melanocytic Nevus.Pediatr Dermatol. 2017. https://doi.org/10.1111/pde.13099

Published
2017-07-22
How to Cite
1.
Tchernev G, Lozev I, Pidakev I, Lotti T, Wollina U, Gianfaldoni S, Lotti J, França K, Batashki A, Maximov GK, Chokoeva A. Giant Congenital Melanocytic Nevus (GCMN) - A New Hope for Targeted Therapy?. Open Access Maced J Med Sci [Internet]. 2017Jul.22 [cited 2020Oct.30];5(4):549-50. Available from: https://www.id-press.eu/mjms/article/view/oamjms.2017.121

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