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Neonatal Curettage of Giant Congenital Melanocytic Nevi
Linda E. De Raeve, MD;
Arlette L. De Coninck, MD;
Patrick R. Dierickx, MD;
Diane I. Roseeuw, MD, PhD
Arch Fam Med. 1998;7:114-116.
REPORT OF CASES
Four newborns presented with giant congenital melanocytic nevi (GCMN). Curettage was performed during the first weeks of life.
THERAPEUTIC CHALLENGE
Giant congenital melanocytic nevi may lead to severe cosmetic deformities and subsequent psychosocial problems for the child. Another important problem is the relatively high potential of GCMN to undergo malignant transformation.1-3 Management of such lesions presents an enormous challenge to those caring for these patients. The choice of surgical procedures depends on the consideration of the importance of the patient's cosmetic appearance, the reduction of the risk of malignancy, and the maintenance of normal function. Excision is the surest method of removing all potentially malignant cells. However, although great strides have been made in excisional surgery, particularly with the use of tissue expanders, all of the tumor cannot always be removed easily, and cosmetic results are sometimes less than satisfactory. Our challenge was to use a procedure to treat GCMN that would provide good cosmetic and functional results and that may lessen the risk of melanoma developing within the GCMN.
SOLUTION
Curettage is a relatively new approach to the treatment of GCMN in the newborn. The use of curettage is based on the observation that, in the first few weeks of life of infants with GCMN, there is a cleavage plane between the upper dermis that contains most of the pigmented nevus cells and the deeper dermis.4
We performed debridement using a curette to dissect along this cleavage plane in four newborns with GCMN.
METHODS
Before performing curettage, one or more punch biopsy specimens were obtained from these infants with GCMN. Under general anesthesia, the GCMN were scraped with a Volkmann curette (diameter, 8 mm) from the center of the lesion to its periphery. In the first 2 weeks of life, the technique was easy to perform, as the cleavage plane between the upper and the lower dermis was easily found. The procedure was relatively atraumatic, with minimal bleeding. The wound was dressed with petrolatum-saturated gauze, and healing was uneventful.
RESULTS
Curettage yielded favorable cosmetic results (Figure 1 and Figure 2). The histologic features of the lesions before curettage were comparable for all four patients: the upper dermis consisted entirely of heavily pigmented nevus cells, while the lower dermis contained a less dense, diffuse infiltration of nonpigmented nevus cells, often showing a neuroid appearance (Figure 3). In some sections, scattered nests of heavily pigmented nevus cells were observed within the lower epidermis. The monoclonal antibody HMB-45 reacted with the junctional nests, occasionally with a melanocyte in the basal layer of the epidermis and the heavily pigmented nevus cells in the upper dermis. The deeply located nonpigmented nevus cells, however, did not stain for HMB-45. The curetted material consisted of epidermis and the underlying upper dermis of mostly pigmented nevus cells, as described by Johnson.5
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Figure 1. Preoperative appearance of a giant congenital melanocytic nevus in a newborn.
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Figure 2. Same patient as in Figure 1 after 4 years' follow-up. Note some repigmentation and hypertrichosis.
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Figure 3. The upper dermis is occupied by heavily pigmented nevus cells that stained positive for HMB-45, while the lower dermis contains nonpigmented nevus cells that stained negative for HMB-45 (hematoxylin-eosin, x40).
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A skin biopsy specimen obtained from an area that did not repigment 4 years after curettage showed that the entire upper dermis was composed of a dense connective tissue with some degree of sclerosis, with preservation of the skin appendages, and no heavily pigmented nevus cells. The deeper dermis still contained a diffuse infiltration of nonpigmented nevus cells, as was observed before curettage (Figure 4). The biopsy specimen stained negative for HMB-45.
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Figure 4. Histologic section 4 years after intervention. Note dense connective tissue with sclerosis and no pigmented nevus cells in upper dermis. Staining was negative for HMB-45 (hematoxylin-eosin, x40).
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In addition to the findings mentioned above, a biopsy specimen from the slightly repigmented area showed that the lower epidermis contained some scattered nests of nevus cells that stained positive for HMB-45. There was no immunoreactivity for HMB-45 in the deeper structures.
COMMENT
The recent approach to the management of GCMN has been changed considerably by more accurate information on the evaluation of the risk of malignant change1-3,6-9 and by the development of new surgical techniques. The incidence of malignant melanomas within GCMN was first reported to vary from 1.8% to 42%.2-3,6 Part of the discrepancy with the data presented in these reports results from the large variability in the criteria used to diagnose giant congenital nevi. According to Rhodes et al,10 the lifetime risk of melanoma arising in a GCMN is about 6.3%. Owing to this substantial increase in risk (17-fold relative risk compared with the general population), most authorities10-12 agree that GCMN should be removed whenever and as early as technically possible. However, the accepted surgical methods available for treating GCMN have many disadvantages.
Surgical excision in toto early in life is often not practical because of the cosmetic and functional disability caused by extensive surgery; multiple procedures increase the risks of general anesthesia.12 Sometimes, serial excisions with primary wound closing are not possible owing to localization of the nevus. Split-skin grafting can be limited by donor sites or lead to unacceptable cosmetic results. Some of the difficulties of surgery may be eased by the use of technical advances such as tissue expansion; however, most GCMN are too large, and all of the tumor cannot be removed.13 Another possible treatment is the use of cultured epithelial autografts or allografts.14 Neonatal dermabrasion of GCMN is based on the theory that nevus cells at birth lie predominantly in the papillary dermis and only later migrate into the deeper dermis.5, 15-16 This theory, however, is not generally accepted.
The histologic pattern of congenital melanocytic nevi is as follows: GCMN mostly show deep dermal involvement; smaller congenital melanocytic nevi have a variable pattern of histologic appearance with nevus cells at varying levels throughout the dermis; these patterns do not appear to change remarkably during the first years of life.17-18 Ruiz-Maldonado et al19 reported their experience with the management of smaller congential melanocytic nevi; they considered the results of dermabrasion to be poor.
We used the curettage technique of Moss4 to treat GCMN during the first 2 weeks of life. We found this technique superior to other treatments such as dermabrasion because it affords the surgeon tactile knowledge of the depth and lateral extension of the lesion that is not provided by other methods and because the surgeon has the ability to discern the level of destruction within the dermis when curettage is performed before 2 weeks of age. In terms of cosmetic results, this level of separation seems to be adequate. In our experience, the cosmetic results were favorable. Our histologic findings showed deep dermal involvement, but we distinguished 2 different populations of nevus cells: superficially located heavily pigmented nevus cells and deeper dermal nonpigmented nevus cells. Our findings of positive staining for HMB-4520-21 of superficially located dermal pigmented nevus cells and negative staining of deeper dermal nonpigmented nevus cells in patients with GCMN indicate that the biologic behavior of these 2 populations of nevus cells seems to be different, as their expression of melanocytic antigen differs. Follow-up biopsy specimens obtained after curettage showed a large reduction of pigmented nevus cells and no staining for HMB-45 in the dermis and in deeper structures. Thus, we achieved at least a numerical reduction of pigmented nevus cells together with good functional and cosmetic results.
Since curettage of GCMN in the neonatal period removes pigment from areas that cannot otherwise be excised easily, it may be a useful alternative to surgical excision, if used in the first 2 weeks of life. Later on, curettage becomes progressively more difficult.
It is premature to state that the risk of malignant degeneration is reduced, but it might be lessened by a reduction of the number of melanocytes. Certainly, the deeper nevus cells that remain after the debulking procedure can give rise to melanoma,22 and there is concern that the scar tissue induced by superficial surgical procedures may mask the ability to detect malignant growth in the deep component. Only long-term follow-up on a larger number of patients so treated will answer some of these critical questions.
AUTHOR INFORMATION
Presented at the Seventh International Congress of Pediatric Dermatology, Buenos Aires, Argentina, October 1, 1994.
We wish to thank A. Goossens, MD, for histopathologic advice, P. Verhavert for immunostaining, and C. Vleminckx for typing the manuscript.
Selected from Arch Dermatol. 1996;132:20-22. The Cutting Edge.
Academic Hospital Vrije Universiteit Brussel, Brussels, Belgium (Contributors); June K. Robinson, MD (Section Editor); Jerome M. Garden, MD; Amy S. Paller, MD (Assistant Section Editors)
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