Sandbox:Qurrat
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Qurrat-ul-ain Abid, M.D.[2]
Managemnet of Congenital melanocytic Nevi
MANAGEMENT
Small/medium CMN — Small and medium-sized CMN are managed on an individual basis depending on factors that affect ease of monitoring (eg, color, thickness/topography, and location), clinical history, parents' anxiety, and cosmetic concerns [4]. As an example, a multinodular black CMN on the scalp that is partially obscured by dense hair growth would be difficult to follow clinically, whereas a thin light brown lesion on the face would be relatively simple to observe. However, the latter might be removed for cosmetic reasons, and the former may spontaneously lighten during childhood.
Periodic evaluation of small- and medium-sized CMN is most important after puberty, since the risk of melanoma arising within these lesions during childhood is extremely low. Baseline photographs can be helpful, and dermoscopy represents a useful tool for assessing changes. (See "Dermoscopic evaluation of skin lesions".)
Patients and parents should be instructed to perform skin self-examinations and to bring focal changes in color, border, or topography (eg, a red or black papule, nodule, or crust) to the clinician's attention. (See "Screening and early detection of melanoma in adults and adolescents", section on 'Patient self-examination'.)
Large CMN — Early surgical removal is often desired for large CMN because of their cosmetic and psychosocial sequelae and concern for possible malignant transformation. Complete excision is difficult to achieve; however, resection of bulky and cumbersome portions of large CMN can be beneficial for some patients. Elimination of every nevus cell may be impossible because of the large area of skin affected, the anatomic site (eg, distal extremity, periocular area, genitalia), and involvement of deeper structures (eg, fat, fascia, muscle). Even theoretically complete surgical excision cannot completely eliminate future risk of melanoma, as some melanomas in these patients may develop in the CNS or retroperitoneum. In many cases, close clinical observation with no surgical removal of the lesion is a reasonable choice.
Factors that affect the decision to perform surgery as well as to determine the timing of surgery include the size and location of the large CMN, the technical difficulty of the procedure(s) required, and anesthesia options. When possible, complete removal of large CMN usually necessitates staged excision with the use of tissue expanders and, occasionally, skin grafting [45].
When surgical excision is not feasible, cosmetic benefit may potentially be obtained from procedures such as curettage, dermabrasion, and ablative laser therapy (eg, carbon dioxide or erbium:yttrium aluminum garnet lasers, sometimes combined with pigment-directed lasers). During the neonatal period, there is a lower risk of excessive scarring following such interventions, and nevus cells are more accessible because they are concentrated in the upper dermis [46,47]. Curettage can be performed during the first two weeks of life, taking advantage of a cleavage plane between the upper and mid-dermis exclusive to neonatal skin. However, nevus cells remain in the dermis after all of these procedures, as evidenced by frequent repigmentation as well as several reports of the subsequent development of melanoma in treated areas [48-52]. This underscores the need for lifelong clinical observation.
Regardless of the treatments employed, patients with large CMN (or scars after their excision) should be followed with periodic skin and general physical examinations. Palpation of the nevus and/or scars is essential for detection of focal induration. Histologic evaluation is indicated for firm nodules or indurated areas. Even theoretically complete removal of a large CMN does not eliminate the risk of melanoma, since melanoma of the CNS and other visceral primary sites (eg, the retroperitoneum) may still occur [53].
Proliferative nodules that develop within large CMN during infancy can have histologic features of melanoma yet behave in a benign manner. Techniques such as comparative genomic hybridization can help to distinguish proliferative nodules (usually having no chromosomal aberrations or only numeric changes) from melanoma (typically demonstrating gains/losses of chromosomal fragments) [40]. Mass spectroscopy imaging proteomic analysis may also help differentiate proliferative nodules from melanoma [29]. (See 'Proliferative nodules' above.)
Surveillance for neurocutaneous melanosis — Patients with a large CMN plus multiple (especially >20) satellite nevi or with multiple medium-sized CMN are at risk for NCM and should be followed with serial head circumference measurements, neurologic examinations, and developmental assessments [3,37,39]. This monitoring includes evaluation for signs and symptoms of increased intracranial pressure, mass lesions, and spinal cord compression [3,39].
Gadolinium-enhanced magnetic resonance imaging (MRI) of brain and spine should be performed in any high-risk patient exhibiting neurologic symptoms, and we suggest that asymptomatic high-risk patients also be screened for NCM with gadolinium-enhanced MRI of the brain and spine, ideally during the first six months of life before myelination, which may obscure evidence of melanosis [42]. For very young infants, it may be possible to obtain initial high-quality MRI images without general anesthesia using "feed and wrap" techniques that allow a swaddled infant to sleep during the imaging procedure [54].
Given the poor prognosis, aggressive surgical procedures for CMN removal should be postponed in patients with symptomatic NCM. NCM in an asymptomatic patient does not necessarily preclude skin surgery.
| Diseases | Clinical manifestations | Para-clinical findings | Gold standard | Additional findings | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Symptoms | Physical examination | ||||||||||||||
| Lab Findings | Imaging | Histopathology | |||||||||||||
| Symptom 1 | Symptom 2 | Symptom 3 | Physical exam 1 | Physical exam 2 | Physical exam 3 | Lab 1 | Lab 2 | Lab 3 | Imaging 1 | Imaging 2 | Imaging 3 | ||||
| Diseases | Symptom 1 | Symptom 2 | Symptom 3 | Physical exam 1 | Physical exam 2 | Physical exam 3 | Lab 1 | Lab 2 | Lab 3 | Imaging 1 | Imaging 2 | Imaging 3 | Histopathology | Gold standard | Additional findings |
| Differential Diagnosis 1 | |||||||||||||||
| Differential Diagnosis 2 | |||||||||||||||
| Differential Diagnosis 3 | |||||||||||||||
| Diseases | Symptom 1 | Symptom 2 | Symptom 3 | Physical exam 1 | Physical exam 2 | Physical exam 3 | Lab 1 | Lab 2 | Lab 3 | Imaging 1 | Imaging 2 | Imaging 3 | Histopathology | Gold standard | Additional findings |
| Differential Diagnosis 4 | |||||||||||||||
| Differential Diagnosis 5 | |||||||||||||||
| Differential Diagnosis 6 | |||||||||||||||
Table for Differential Diagnosis of Small Intestine Cancer
ABBREVIATIONS:
N/A: Not available, NL: Normal,