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Congenital Nevi

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Jun 10, 2016
 

Background

Congenital nevi are present at birth and result from a proliferation of benign melanocytes in the dermis, epidermis, or both (see the image below). Occasionally, nevi that are not present at birth but are histologically identical to congenital nevi may develop during the first 2 years of life. This is referred to as congenital nevus tardive.[1]

The photomicrograph shows a symmetrical broad prol The photomicrograph shows a symmetrical broad proliferation of melanocytes in the papillary and reticular dermis with maturation with progressive descent; splaying between collagen bundles; and permeation of muscles of hair erection, blood vessels, and adnexa (hematoxylin and eosin stain, 20X magnification).

See Mole or Melanoma? Test Yourself With These Suspicious Lesions, a Critical Images slideshow, to help identify various skin lesions.

Congenital melanocytic nevi are one of several known risk factors for the eventual development of melanoma. Fortunately, melanoma remains an uncommon malignancy in prepubertal children, with an annual incidence of 0.7 cases per million children aged 0-9 years. Patient concerns regarding changing or worrisome-looking nevi are, nonetheless, very common. Moreover, by the time a child reaches adolescence, the incidence of melanoma increases substantially, with a rate of 13.2 cases per million children aged 15-19 years.[2] These melanoma metastasize and are thus life-threatening.[3]

While many sources have noted the so-called melanoma epidemic in adults, only in recent years have data documented an alarming increase in melanoma in adolescents. This increase, combined with the recognition of clearly identifiable melanoma risk factors in childhood, allows physicians of the 21st century to play a crucial role in the identification of children at risk for melanoma and to aid in the prevention of melanoma through education regarding the risks of ultraviolet light exposure.

Congenital melanocytic nevus can be divided into two types, the first being the most common, usually a solitary plaque growing in proportion to that of the child, and a second type composed of many lesions, at least one being large and surrounded by many others and sometimes involving bone and central nervous system.[4]

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Pathophysiology

The etiology of congenital melanocytic nevi remains unclear. The melanocytes of the skin originate in the neuroectoderm, although the specific cell type from which they derive remains unknown.[5, 6, 7] One hypothesis is that pluripotential nerve sheath precursor cells migrate from the neural crest to the skin along paraspinal ganglia and peripheral nerve sheaths and differentiate into melanocytes upon reaching the skin.[8] One possible explanation for the presence of congenital melanocytic nevi is that an external insult results in a mutation that affects the morphogenesis of the embryonic neuroectoderm and migration of precursor cells to the skin. Multiple congenital melanocytic nevi may reflect a mosaic RASopathy, reflecting postzygotic activating mutations in NRAS.[9]

One study found that the MC1R (melanocortin-1-receptor) genotype, which corresponds to a red-haired genotype and a tendency to increased birthweight, was overrepresented in a cohort of congenital melanocytic nevi affected Northern European patients. How MC1R variants promote growth of congenital melanocytic nevi and the fetus itself is unknown as is the application of this finding to non-European and more darkly pigmented races.[10]

Congenital nevi have been stratified into 3 groups according to size. Small nevi are less than 1.5 cm in greatest diameter, medium nevi are 1.5-19.9 cm in greatest diameter, and large or giant nevi are greater than 20 cm in greatest diameter. Giant nevi are often surrounded by several smaller satellite nevi. An alternate definition is that a small congenital nevus is one for which primary closure is possible after excision.

Congenital nevi may also be seen as a component of neurocutaneous melanosis, a rare congenital syndrome characterized by the presence of congenital melanocytic nevi and melanotic neoplasms of the central nervous system. Rokitansky first described neurocutaneous melanosis in 1861.[11] The current diagnostic criteria for neurocutaneous melanosis are (1) large (>20 cm) or multiple (>3) congenital nevi in association with meningeal melanosis or melanoma, (2) no evidence of meningeal melanoma except in patients in whom cutaneous lesions are histologically benign, and (3) no evidence of cutaneous melanoma except in patients in whom meningeal lesions are histologically benign.[12]

Neurocutaneous melanosis may result from an error in the morphogenesis of the neuroectoderm, which gives rise to the melanotic cells of both the skin and meninges. Clinically, patients may present with increased intracranial pressure due to hydrocephalus or a mass lesion. The prognosis of patients with symptomatic neurocutaneous melanosis is very poor, even in the absence of malignancy. In one review of 39 reported cases of symptomatic neurocutaneous melanosis, death occurred in more than half the patients within 3 years of the onset of neurological symptoms, and most deaths were in patients younger than 10 years.[12]

Mutations in NRAS in congenital melanocytic nevi can cause mitogen-activated protein kinase activation and may represent early events in melanoma development.[13]

Oncogenic missense mutations in codon 61 of NRAS were found in affected neurological and cutaneous tissues in 12 of 15 patients with congenital melanocytic nevi with neurocutaneous melanosis, implying single postzygotic NRAS mutations were responsible.[14]

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Epidemiology

Frequency

Congenital nevi are present in 1-2% of newborn infants. One small study in Spain found a higher prevalence of congenital melanocytic nevi in preterm infants, females, and nonwhite infants, whereas maternal age, number of previous pregnancies, and birth weight did not appear to influence the prevalence.[15]

Race

No racial predilection is recognized for congenital nevi.

Sex

Congenital nevi occur in both sexes, with no known predilection.

Age

To be considered congenital nevi, lesions must be present at birth.

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Prognosis

The prognosis for patients with small or medium-sized congenital melanocytic nevi is good. Although the risk of developing melanoma in these lesions has not been quantified, it is generally regarded as only slightly higher than that of normal skin. Despite the increased risk for melanoma in patients with giant congenital melanocytic nevi, the vast majority of patients never develop melanoma. Therefore, prognosis remains good in these patients, especially if the lesions are examined regularly for signs of atypia.

Prognosis in cases of symptomatic neurocutaneous melanosis is quite poor.

Mortality/morbidity

Congenital nevi, depending on size and location, may have a significant impact on cosmesis. Giant congenital nevi place individuals at an increased risk for the development of melanoma at the site of the nevus. For giant congenital melanocytic nevi, the risk of developing melanoma has been reported to be as high as 5-7% by age 60 years.[16, 17] One study suggests that the risk of melanoma may be greater in those with giant congenital melanocytic nevi with more satellite lesions or a larger diameter.[18] Another suggests multiple satellite nevi alone or with associated posterior midline location of large congenital melanocytic nevi is linked with increased risk.[19] Additionally, melanoma developing within giant congenital nevi may develop during childhood and occur deeper in the tissue where it is harder to detect clinically.

While the general consensus regarding smaller nevi is that they pose a greater risk for the development of melanoma than normal skin, this risk has not been quantified. Also suggested is that melanoma developing within smaller congenital nevi usually occurs at puberty or later and develops more superficially in the skin, where it is easier to detect clinically.

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Patient Education

All people need to be educated on the importance of protection from ultraviolet light exposure. This is especially important in people who have congenital melanocytic nevi, particularly the giant type, because they are already at an increased risk for the development of melanoma.

For patient education resources, see the Skin, Hair, and Nails Center, as well as Skin Cancer and Mole Removal.

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Jerry Rothenberg, MD Clinical Associate Professor, Department of Dermatology, University of Medicine and Dentistry of New Jersey, New Jersey Medical School; President, Director, New Jersey Dermatopathology Laboratory, Inc

Disclosure: Nothing to disclose.

Isabelle Thomas, MD Associate Professor, Department of Dermatology, University of Medicine and Dentistry of New Jersey, New Jersey Medical School; Chief of Dermatology Service, Veterans Affairs Medical Center of East Orange

Isabelle Thomas, MD is a member of the following medical societies: American Academy of Dermatology, Sigma Xi

Disclosure: Nothing to disclose.

Christopher J Steen, MD Dermatologist, Private Practice

Christopher J Steen, MD is a member of the following medical societies: Alpha Omega Alpha, Sigma Xi

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD Associate Clinical Professor of Dermatology, University of Texas School of Medicine at San Antonio

Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Franklin Flowers, MD Department of Dermatology, Professor Emeritus Affiliate Associate Professor of Pathology, University of Florida College of Medicine

Franklin Flowers, MD is a member of the following medical societies: American College of Mohs Surgery

Disclosure: Nothing to disclose.

References
  1. Clemmensen OJ, Kroon S. The histology of "congenital features" in early acquired melanocytic nevi. J Am Acad Dermatol. 1988 Oct. 19(4):742-6. [Medline].

  2. Krengel S, Hauschild A, Schafer T. Melanoma risk in congenital melanocytic naevi: a systematic review. Br J Dermatol. 2006 Jul. 155(1):1-8. [Medline].

  3. Volejnikova J, Bajciova V, Sulovska L, Geierova M, Buriankova E, Jarosova M, et al. Bone marrow metastasis of malignant melanoma in childhood arising within a congenital melanocytic nevus. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2016 Apr 1. [Medline].

  4. Magaña M, Sánchez-Romero E, Magaña P, Beck-Magaña A, Magaña-Lozano M. Congenital Melanocytic Nevus: Two Clinicopathological Forms. Am J Dermatopathol. 2014 Aug 19. [Medline].

  5. Ansarin H, Soltani-Arabshahi R, Mehregan D, Shayanfar N, Soltanzadeh P. Giant congenital melanocytic nevus with neurofibroma-like changes and spina bifida occulta. Int J Dermatol. 2006 Nov. 45(11):1347-50. [Medline].

  6. Cruz MA, Cho ES, Schwartz RA, Janniger CK. Congenital neurocutaneous melanosis. Cutis. 1997 Oct. 60(4):178-81. [Medline].

  7. Silfen R, Skoll PJ, Hudson DA. Congenital giant hairy nevi and neurofibromatosis: the significance of their common origin. Plast Reconstr Surg. 2002 Oct. 110(5):1364-5. [Medline].

  8. Cramer SF. The melanocytic differentiation pathway in congenital melanocytic nevi: theoretical considerations. Pediatr Pathol. 1988. 8(3):253-65. [Medline].

  9. Waelchi R, Williams J, Cole T, Dattani M, Hindmarsh P, Kennedy H, et al. Growth and hormonal profiling in children with congenital melanocytic naevi. Br J Dermatol. 2015 Aug 19. [Medline].

  10. Kinsler VA, Abu-Amero S, Budd P, Jackson IJ, Ring SM, Northstone K, et al. Germline Melanocortin-1-Receptor Genotype Is Associated with Severity of Cutaneous Phenotype in Congenital Melanocytic Nevi: A Role for MC1R in Human Fetal Development. J Invest Dermatol. 2012 May 10. [Medline].

  11. Rokitansky J. Ein ausgezeichneter Fall von Pigment-mal mit ausgebreiteter Pigmentierung der inneren Hirn- und Ruchenmarkshaute. Allg Wien Med Z. 1861. 6:113-6.

  12. Kadonaga JN, Frieden IJ. Neurocutaneous melanosis: definition and review of the literature. J Am Acad Dermatol. 1991 May. 24(5 Pt 1):747-55. [Medline].

  13. Blokx WA, van Dijk MC, Ruiter DJ. Molecular cytogenetics of cutaneous melanocytic lesions - diagnostic, prognostic and therapeutic aspects. Histopathology. 2010 Jan. 56(1):121-32. [Medline].

  14. Kinsler VA, Thomas AC, Ishida M, Bulstrode NW, Loughlin S, Hing S, et al. Multiple Congenital Melanocytic Nevi and Neurocutaneous Melanosis Are Caused by Postzygotic Mutations in Codon 61 of NRAS. J Invest Dermatol. 2013 Sep. 133(9):2229-36. [Medline]. [Full Text].

  15. Monteagudo B, Labandeira J, Acevedo A, et al. Prevalence and Clinical Features of Congenital Melanocytic Nevi in 1,000 Spanish Newborns. Actas Dermosifiliogr. 2011 Mar. 102(2):114-120. [Medline].

  16. Rhodes AR, Wood WC, Sober AJ, Mihm MC Jr. Nonepidermal origin of malignant melanoma associated with a giant congenital nevocellular nevus. Plast Reconstr Surg. 1981 Jun. 67(6):782-90. [Medline].

  17. Bett BJ. Large or multiple congenital melanocytic nevi: occurrence of cutaneous melanoma in 1008 persons. J Am Acad Dermatol. 2005 May. 52(5):793-7. [Medline].

  18. Hale EK, Stein J, Ben-Porat L, et al. Association of melanoma and neurocutaneous melanocytosis with large congenital melanocytic naevi--results from the NYU-LCMN registry. Br J Dermatol. 2005 Mar. 152(3):512-7. [Medline].

  19. Lovett A, Maari C, Decarie JC, et al. Large congenital melanocytic nevi and neurocutaneous melanocytosis: one pediatric center's experience. J Am Acad Dermatol. 2009 Nov. 61(5):766-74. [Medline].

  20. Tcheung WJ, Bellet JS, Prose NS, Cyr DD, Nelson KC. Clinical and Dermoscopic Features of 88 Scalp Nevi in 39 Children. Br J Dermatol. 2011 Mar 17. [Medline].

  21. Chuah SY, Tsilika K, Chiaverini C, Fontas E, Ortonne JP, Lacour JP, et al. Dermoscopic features of congenital acral melanocytic nevi in children: a prospective comparative and follow-up study. Br J Dermatol. 2014 Jun 16. [Medline].

  22. Bastian BC, Xiong J, Frieden IJ, et al. Genetic changes in neoplasms arising in congenital melanocytic nevi: differences between nodular proliferations and melanomas. Am J Pathol. 2002 Oct. 161(4):1163-9. [Medline].

  23. Wu PA, Mancini AJ, Marghoob AA, Frieden IJ. Simultaneous occurrence of infantile hemangioma and congenital melanocytic nevus: Coincidence or real association?. J Am Acad Dermatol. 2008 Feb. 58(2 Suppl):S16-22. [Medline].

  24. Zaenglein AL, Heintz P, Kamino H, Zisblatt M, Orlow SJ. Congenital Spitz nevus clinically mimicking melanoma. J Am Acad Dermatol. 2002 Sep. 47(3):441-4. [Medline].

  25. Phadke PA, Rakheja D, Le LP, Selim MA, Kapur P, Davis A, et al. Proliferative Nodules Arising Within Congenital Melanocytic Nevi: A Histologic, Immunohistochemical, and Molecular Analyses of 43 Cases. Am J Surg Pathol. 2011 Mar 22. [Medline].

  26. Flux K, Hartschuh W. Congenital spindle cell naevus with unusual transformation: proliferative nodule or melanoma?. Acta Derm Venereol. 2012 Mar. 92(2):152-5. [Medline].

  27. Ambros T, Furian R, Riccardi F. The development of two different malignancies in a patient with large congenital melanocytic nevus. Pediatr Dermatol. 2011 Nov-Dec. 28(6):729-31. [Medline].

  28. Snyder JA, Khozeimeh N, Reyes C, Qureshi FG. Benign sacrococcygeal neoplasm arising at birth from a giant congenital melanocytic nevus. J Pediatr Surg. 2012 Feb. 47(2):408-11. [Medline].

  29. Bergman R, Ben-Arush MW, Bar-Shalom R, Gilboa M, Simon E, Hershkovitz D, et al. A Deep Penetrating Facial Congenital Melanocytic Tumor With Bone Involvement and Ipsilateral Eye Blindness. Am J Dermatopathol. 2014 Sep 12. [Medline].

  30. Changchien L, Dusza SW, Agero AL, et al. Age- and site-specific variation in the dermoscopic patterns of congenital melanocytic nevi: an aid to accurate classification and assessment of melanocytic nevi. Arch Dermatol. 2007 Aug. 143(8):1007-14. [Medline].

  31. Mark GJ, Mihm MC, Liteplo MG, Reed RJ, Clark WH. Congenital melanocytic nevi of the small and garment type. Clinical, histologic, and ultrastructural studies. Hum Pathol. 1973 Sep. 4(3):395-418. [Medline].

  32. Rhodes AR, Silverman RA, Harrist TJ, Melski JW. A histologic comparison of congenital and acquired nevomelanocytic nevi. Arch Dermatol. 1985 Oct. 121(10):1266-73. [Medline].

  33. Everett MA. Histopathology of congenital pigmented nevi. Am J Dermatopathol. 1989 Feb. 11(1):11-2. [Medline].

  34. Berg P, Lindelof B. Congenital nevocytic nevi: follow-up of a Swedish birth register sample regarding etiologic factors, discomfort, and removal rate. Pediatr Dermatol. 2002 Jul-Aug. 19(4):293-7. [Medline].

  35. Stanganelli I, Ascierto P, Bono R, De Giorgi V, Pimpinelli N, Chiarion-Sileni V, et al. Management of small and intermediate congenital nevi: a nationwide survey in Italy. Dermatology. 2013. 226 Suppl 1:7-12. [Medline].

  36. Margulis A, Adler N, Bauer BS. Congenital melanocytic nevi of the eyelids and periorbital region. Plast Reconstr Surg. 2009 Oct. 124(4):1273-83. [Medline].

  37. Hoffman D, Ratner D. Diagnosis and management of a changing congenital melanocytic nevus. Skinmed. 2006 Sep-Oct. 5(5):242-5. [Medline].

  38. Criscito MC, Correa LM, Koshenkov VP, Firoz BF. Recurrent Nevi in a Skin Graft Following Excision of Giant Congenital Melanocytic Nevus. Dermatol Surg. 2016 May 25. [Medline].

  39. Warner PM, Yakuboff KP, Kagan RJ, Boyce S, Warden GD. An 18-year experience in the management of congenital nevomelanocytic nevi. Ann Plast Surg. 2008 Mar. 60(3):283-7. [Medline].

  40. Demirseren ME, Ceran C, Demirseren DD. Treatment of a Congenital Melanocytic Nevus on the Forehead with Immediate Tissue Expansion Technique: A Three-Year Follow-Up. Pediatr Dermatol. 2012 Feb 3. [Medline].

  41. De Raeve LE, Roseeuw DI. Curettage of giant congenital melanocytic nevi in neonates: a decade later. Arch Dermatol. 2002 Jul. 138(7):943-7. [Medline].

  42. Michel JL, Caillet-Chomel L. [Treatment of giant congenital nevus with high-energy pulsed CO2 laser]. Arch Pediatr. 2001 Nov. 8(11):1185-94. [Medline].

  43. Michel JL. Laser therapy of giant congenital melanocytic nevi. Eur J Dermatol. 2003 Jan-Feb. 13(1):57-64. [Medline].

  44. Reynolds N, Kenealy J, Mercer N. Carbon dioxide laser dermabrasion for giant congenital melanocytic nevi. Plast Reconstr Surg. 2003 Jun. 111(7):2209-14. [Medline].

  45. Chong SJ, Jeong E, Park HJ, Lee JY, Cho BK. Treatment of congenital nevomelanocytic nevi with the CO2 and Q-switched alexandrite lasers. Dermatol Surg. 2005 May. 31(5):518-21. [Medline].

  46. Lapiere K, Ostertag J, Van De Kar T, Krekels G. A neonate with a giant congenital naevus: new treatment option with the erbium:YAG laser. Br J Plast Surg. 2002 Jul. 55(5):440-2. [Medline].

  47. Kono T, Ercocen AR, Kikuchi Y, Isago T, Honda T, Nozaki M. A giant melanocytic nevus treated with combined use of normal mode ruby laser and Q-switched alexandrite laser. J Dermatol. 2003 Jul. 30(7):538-42. [Medline].

  48. Kono T, Ercocen AR, Nozaki M. Treatment of congenital melanocytic nevi using the combined (normal-mode plus Q-switched) ruby laser in asians: clinical response in relation to histological type. Ann Plast Surg. 2005 May. 54(5):494-501. [Medline].

  49. Kono T, Ercocen AR, Chan HH, Kikuchi Y, Nozaki M. Effectiveness of the normal-mode ruby laser and the combined (normal-mode plus q-switched) ruby laser in the treatment of congenital melanocytic nevi: a comparative study. Ann Plast Surg. 2002 Nov. 49(5):476-85. [Medline].

  50. Kim S, Kang WH. Treatment of congenital nevi with the Q-switched Alexandrite laser. Eur J Dermatol. 2005 Mar-Apr. 15(2):92-6. [Medline].

  51. Kovalyshyn I, Braun R, Marghoob A. Congenital melanocytic naevi. Australas J Dermatol. 2009 Nov. 50(4):231-40; quiz 241-2. [Medline].

  52. MacLachlan WWG. Extensive pigmentation of the brain associated with nevi pigmentosi of the skin. J Med Res. 1913. 29:433-47.

  53. Patterson WM, Lefkowitz A, Schwartz RA, Lambert WC, Rao BK. Melanoma in children. Cutis. 2000 May. 65(5):269-72, 275. [Medline].

 
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The photomicrograph shows a symmetrical broad proliferation of melanocytes in the papillary and reticular dermis with maturation with progressive descent; splaying between collagen bundles; and permeation of muscles of hair erection, blood vessels, and adnexa (hematoxylin and eosin stain, 20X magnification).
Higher-power view of the previous image (hematoxylin and eosin stain, 40X magnification).
 
 
 
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