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Congenital Pigmented Nevi

Background

The cause of congenital nevi, which are present at birth, is an abundance of non-cancerous melanocytes in one or both of the two outer layers of the skin. Sometimes nevi that are that are identical in tissue structure to congenital nevi may develop within the first two years of life even though they were not present at birth. This later development is called congenital nevus tardive.

Congenital nevi are included among the risk factors for increased risk and later development of melanoma. Fortunately, malignant melanoma is still uncommon in children who have not reach puberty. Patient concerns about changing or any nevi seem to be of concern are quite common nonetheless. However, by the time a child arrives at puberty, the occurrences of melanoma rises considerably from a rate of .7 cases per million at ages 0-9 years to an alarming 13.2 cases per million for ages 15-19 years.
Although several sources have made notes of the alleged melanoma epidemic among the adult population, it has only been quite recently that the data has documented a startling rise in melanoma cases for young people. This increase, along with the identification of childhood risk factors for melanoma, allows physicians in the 21st Century to participate actively in identifying children at risk for melanoma and aiding its prevention by educating everyone to all of the risks pertaining to ultraviolet light exposure.

Pathophysiology

The cause of congenital melanocytic nevi is still not clear. The melanocytes of the skin begin in the neuroectoderm, but the exact type of cell of their origin is not known at this time. One reasonable theory for congenital melanocytic nevi is that a transformation caused by an external insult affects the development of the budding neural plate as well as migration of originator cells to the skin.

Congenital nevi fall into three groups based on their size: small nevi grow to less than 1.5 cm, medium nevi grow to 1.5-19.9 cm in diameter, and large or giant nevi grow larger than 20 cm in diameter. Smaller satellite nevi often surround the giant nevi.

Congenital nevi may also be thought of as one of the components of neurocutaneous melanosis, which is a rare congenital condition. The characteristics of neurocutaneous melanosis include the presence of congenital melanocytic nevi and melanotic tissue masses that encompass the central nervous system.

Neurocutaneous melanosis was first described in 1861, and the current criteria that is used for diagnosis of this condition is the presence of (1) large or multiple congenital nevi along with meningeal melanosis or melanoma; and (2) no evidence of meningeal melanoma other than in patients whose skin lesions are benign; and (3) no evidence of skin cancer except in patients whose meningeal lesions are benign.

From a clinical standpoint, patients may be born with increased intracranial pressure because of hydrocephalus or a mass lesion. The outlook for patients with symptomatic neurocutaneous melanosis is very meager even if it is not malignant. A study and review of 39 reported cases of symptomatic neurocutaneous melanosis showed that more than half the patients died within three years after the commencement of neurological symptoms, with most of the deaths occurring in patients under the age of ten.

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Mortality/Morbidity

Cosmesis may be impacted by congenital nevi based on its size and location. Patients diagnosed with giant congenital nevi have a greater risk of developing melanoma at the site of the nevus. Reports have shown the risk is as high as 5.7% by age 60 for patients with giant congenital melanocytic nevi. It has been suggested that those who have been diagnosed with congenital melanocytic nevi or who have a larger number of satellite lesions or are larger in diameter have a greater risk of developing melanoma. The general belief is that smaller nevi present an increased risk for the development of melanoma compared to normal skin, but this risk has not yet been quantified.

Medical Care

The treatment plan for management of congenital melanocytic nevi depends on a number of factors such as the following:
The lesion’s size
Where the lesion is located
How old the patient is
How it might affect cosmesis
What its potential is for malignant transformation

Surgical Care

It may be necessary to surgically remove congenital melanocytic nevi in order to improve the patient’s cosmetic appearance and to ensure the reduction in the likelihood of malignant transformation. It is well know that there is an increased risk of malignant transformation associated with giant congenital melanocytic, so these lesions are removed if possible. Situations that may prevent removal may include the lesions’ size and location in relation to vital structures. In those cases, other procedures may need to be used such as curettage of the lesions or laser treatment of the lesions.


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