CLINICAL ARTICLE
Treatment of a Giant Congenital Melanocytic Nevus in the Adult Review of the Current Management of Giant Congenital Melanocytic Nevus Jeannie J. Su, MAS, Daniel K. Chang, MD, Brian Mailey, MD, and Amanda Gosman, MD Abstract: Giant congenital melanocytic nevi (GCMNs) create cosmetic disfigurements and pose risk for malignant transformation. Adult GCMN cases are uncommon because most families opt for surgical treatment during childhood. We review the current literature on GCMN and present an interesting case of an adult with a GCMN encompassing the entire back with painful nodules exhibiting gross involvement of his back musculature, without pathologic evidence of malignancy. Surgical management was deferred in childhood because of parental desires to allow the patient to make his own decision, and treatment in adulthood was pursued on the basis of the significant impairment of the patient's quality of life and self-esteem due to the massive size and deforming nature of the nevus. The treatment strategy used for this young adult male patient involved a massive en bloc excision of the GCMN with partial resection of the latissimus dorsi, followed by a 5-week staged reconstructive process using dermal regenerative matrices and split-thickness skin grafting. Because of the shift in GCMN management from early surgical management to more conservative management, we may see an increase in adult cases of GCMN. Thus, it is critical to better understand the controversy surrounding early versus delayed management of GCMN. Key Words: adult, skin neoplasms, melanosis, reconstructive surgical procedures, skin grafting (Ann Plast Surg 2015;74: S57–S61)
G
iant congenital melanocytic nevi (GCMNs) occur in approximately 1 in 20,000 live births.1 Giant congenital melanocytic nevi create various medical, cosmetic, and psychological complications, the most concerning of which are transformation to a malignant melanoma and neurocutaneous melanocytosis.2–6 Earlier reports of melanoma transformation rates ranged up to 42%7; however, recent data suggest that previous reports overestimated the risk for transformation and the true risk lies between 0.7% and 2.8%.6,8–10 Because most malignant transformations occur before the age of 3 years, historically, the optimal management strategy of GCMN has been prophylactic excision performed during early childhood. The recent evidence supporting a lower risk for malignant transformation, however, has shifted views regarding the preferred management of GCMN. However, surgical excision can be indicated for medical issues outside malignant transformation including physical discomfort and psychosocial distress.11 Here, we review the current literature on GCMN while presenting a case of an adult with a GCMN encompassing the patient's entire back, which was initially managed during childhood with watchful waiting but ultimately resulted in a multiprocedure surgical excision. Received July 7, 2014, and accepted for publication, after revision, December 6, 2014. From the Division of Plastic Surgery, Department of Surgery, University of California, San Diego, San Diego, CA. Conflicts of interest and sources of funding: none declared. Reprints: Amanda Gosman, MD, Division of Plastic Surgery, Department of Surgery, University of California, San Diego, 200 W. Arbor Dr, San Diego, CA 921038890. E-mail: [email protected]. Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. ISSN: 0148-7043/15/7401–S057 DOI: 10.1097/SAP.0000000000000433
Annals of Plastic Surgery • Volume 74, Supplement 1, May 2015
Presentation Congenital melanocytic nevi vary in size, color, shape, and location. They are classified according to size as small, medium, and large/ giant with diameters of less than 1.5 cm, 1.5 to 20 cm, and greater than 20 cm, respectively, as anticipated in adulthood. Although most nevi are thought to grow proportionally with the child, other lesions can grow rapidly during the first few years of life.12 Color is usually shades of brown or black but can also be pink or nonpigmented.13,14 The nevi can become more defined and darken over time or even become hypopigmented and regress. They can be speckled or homogeneous and, as time progresses, can alter between the two. The nevus is usually oval but can also assume other shapes with well- or ill-defined borders. It can be located anywhere on the skin including the trunk, the extremities, and the face. The surface is usually elevated with ridges and grooves but can also be flat, especially in the early years of life. Although 1 lesion is typically present, multiple nevi can be present. Proliferative nodules can develop, which can be a potential sign of malignancy but also be benign.15 A 23-year-old white man with a 42 46-cm speckled brown GCMN and multiple satellite lesions presented with his parents for evaluation in July 2012 (Figs. 1A, B). The nevus had been present at birth, covering the patient's back, with a dark ring encircling it as well as a 2.5 2.5-cm nodule over his spine (Fig. 2). The patient was followed throughout childhood by a pediatric dermatologist. His initial management before presentation to us included a biopsy at 4.5 months of age, after which a more conservative, watch-and-wait approach was assumed, primarily because of his parents' desire to include the patient in the decision-making process. Family history was positive for basal cell carcinoma in his mother in her late 40s. The patient had no history of malignancy before presentation. On presentation, the nevus spanned his back from the T1 to L4 level and crossed his posterior axillary lines bilaterally. Several satellite lesions marked his lower left back, chest, left third metacarpal, right arm, bilateral legs, and feet. The patient complained of the progression of pain and pruritus in the area of the nevus and the development of 2 palpable nodules. The nodules included a red 8-mm papule on the right border near the axillae and a 6-cm rounded raised nodule on the left mid-to-lower back.
Diagnosis and Evaluation Diagnosis of GCMN is performed via microscopic analysis of biopsies. Classic histologic features include nevus cell extension into the deep reticular dermis and/or superficial subcutaneous fat; tracking of melanocytes around or within vascular, neural, or appendageal structures; arrangement of nevus cells in the reticular dermis as single cells, rows, cords, and sheets; and splaying of melanocytes in between collagen bundles.16,17 In our patient, punch biopsies of the 2 nodular sites were performed. The right lateral back nodule punch biopsy demonstrated mildly thickened epidermis with overlying orthokeratosis and spongiosis with epidermal ulceration, follicular rupture, and atypical intradermal melanocytic proliferation with spitzoid features. The left lower back nodule punch biopsy revealed atypical junctional melanocytic proliferation www.annalsplasticsurgery.com
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FIGURE 1. Initial presentation of GCMN spanning 42 46 cm with 2 palpable nodules. Posterior view (A). Right lateral view (B).
with spitzoid features and dermal fibrosis consistent with hypertrophic scar. In February 2013, repeated biopsies of 2 other enlarging sites were most consistent with compound nevus with congenital features, demonstrating mildly thickened epidermis with overlying basket-weave stratum corneum, hyperpigmented basilar keratinocytes, melanocytic hyperplasia of mildly enlarged melanocytes along the dermal-epidermal junction, focal sclerosis, an increased number of vessels, and dermal edema. Single bland-appearing melanocytes were admixed between sclerotic collagen bundles in the superficial and deep dermis. There was no significant evidence of mitotic activity in the dermal melanocytic population. Evaluation of GCMN often includes a screening magnetic resonance image, a thorough neurologic examination, and serial clinical observations of the progression of the nevi to determine possible development of cutaneous melanoma. Magnetic resonance imaging of the thoracic spine demonstrated the presence of multiple soft tissue masses extending beyond the superficial subcutaneous layer (Fig. 3). On neurologic examination, the patient complained of the progression of pain and pruritus in the area of the nevus and intermittent upper extremity weakness. As stated above, repeated biopsies did not demonstrate evidence of malignancy.
transformation. Contrarily, other studies have suggested a more conservative and less invasive approach to treating GCMN. De Raeve et al20 and Rasmussen et al21 suggested the use of curettage to decrease the number of melanocytic cells and potentially lower the risk for cutaneous melanoma. Recent literature has also commented that previous literature overestimated the risk for melanoma because of reporting bias and less accurate histological methods of diagnosis and that the true risk lies between 0.7% and 2.8%.6,8–10 Moreover, several studies have not been able to demonstrate a reduction in melanoma transformation risk with surgical management.21–24 Granted, these studies have been limited in both sample size and follow-up time. In fact, there have been 5 cases in the literature in which melanoma has developed in a site of
Treatment The management of GCMNs is controversial and highly dependent on the individual patient. A largely debated issue in literature is when and on whom to perform surgery. Treatment of GCMN ranges from watchful waiting to less invasive procedures (dermabrasion, carbon dioxide laser ablation, curettage, chemical peels) to more extensive procedures (tissue expansion, en bloc or serial excision with staged direct closure, skin grafting, free tissue transfer, or skin substitutes).18 Factors that need to be taken into account when considering GCMN management include the presence of high-risk phenotypic features linked to malignant transformation such as size greater than 20 cm in diameter, trunk location, presence of 3 or more lesions, and irregular morphology due to the inability to determine morphological changes.19 Additional considerations unrelated to malignancy risk include the possibility of psychosocial impact and cosmetic deformity.2 Historically, treatment goals have centered on complete excision of the GCMN before the age of 2 years because of increased risk for melanoma transformation. Arneja and Gosain18 presented an algorithm for the treatment of GCMN with surgical excision as the optimal treatment strategy to remove pigmented nevus cells and reduce malignant S58
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FIGURE 2. GCMN spanning the trunk as a child. © 2015 Wolters Kluwer Health, Inc. All rights reserved.
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Annals of Plastic Surgery • Volume 74, Supplement 1, May 2015
Adult GCMN: Review of Management
FIGURE 3. Thoracic magnetic resonance imaging demonstrating the giant superficial congenital nevus extending from approximately the level of T1 down to L4, with a central defect at the level of T8 to T10 from presumed prior resection. Several masslike regions including a 2.8 1.8-cm lesion on the right at the level of T12 (A) and a 4.0 2.3-cm lesion on the left at the level of L1 (B) extend slightly beyond the superficial layer.
excision and grafting.25 Because of these new data, Arad and Zuker26 recently argued that treatment should focus on esthetics and not on complete excision. However, surgical excision can be indicated for medical issues outside malignant transformation including painful ulceration and bleeding,
physical discomfort, concerning dermatologic changes, multiple expected surgical procedures, concomitant surgical procedures, and psychosocial distress.11 Early excision potentially decreases recovery time and psychological trauma because of the patient's lack of recollection of not only the nevus but also the surgical procedure.27 Therefore, in
FIGURE 4. Wide local excision, 42 46 cm, of GCMN and multiple subcutaneous masses. Massive en bloc excision of GCMN spanning the trunk over the site of excision (A). Excision down to the level of the paraspinal musculature with partial resection of the right latissimus dorsi muscle (B). Isolation of main nevus from the patient (C). © 2015 Wolters Kluwer Health, Inc. All rights reserved.
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FIGURE 5. Staged reconstructive process involved the use of Integra (A) followed by split-thickness skin grafting from the buttocks and the posterolateral thighs (B).
considering the case for prophylactic resection, the nonmalignant sequelae of GCMN must be factored into the equation. In our patient's case, although the patient did not possess malignant transformation on biopsy, surgical excision of the nevus was recommended for 2 main reasons: (1) the progressive enlargement and development of the soft tissue masses into the superficial back muscles and (2) the inability to completely rule out malignancy, despite multiple reassuring biopsies, given the enormous size of the nevus. In addition, he had debilitating painful nodules and complained about the social and psychological distress caused by the lesion and his unwillingness to remove his shirt in public. A massive en bloc excision of the GCMN was performed, including partial resection of the right latissimus dorsi muscle, because of gross infiltration of the tumor into the muscle (Fig. 4). Massive en bloc excision was performed over serial excision because of distortion and invasion of the adjacent musculature. Pathology of this subcutaneous involvement revealed multiple nodules of spindle-shaped cells with schwannian features and pseudomeissnerian corpuscles consistent with congenital nevus with neuritization without
evidence of malignancy. Skin flaps were advanced circumferentially to decrease the overall size of the wound. The wound was covered with a meshed Integra dermal regenerative matrix bilayer (Integra LifeSciences, Plainsboro, NJ), Acticoat (Smith & Nephew, Hull, United Kingdom), an antimicrobial silver barrier dressing, and negative-pressure wound therapy with a vacuumassisted closure (VAC) device (KCI, San Antonio, Tex). After the excision, the patient underwent a 5-week staged reconstructive process during which he underwent 7 VAC changes under anesthesia. The patient was unable to tolerate VAC changes at the bedside because of pain. During these operations, the wound edges were inspected and debrided as necessary and small portions of the Integra were changed. After 5 weeks, the Integra became well integrated and the wound's depth decreased from 4-cm deep to 0.5 cm in most areas within the level of the skin margin. Split-thickness skin grafting (0.010-in thick, approximately 2000 cm2, meshed 1:1.5) was performed using the bilateral buttocks and the posterolateral thighs as donor sites (Fig. 5). Use of Integra, a bilayer matrix dermal substitute consisting
FIGURE 6. Postoperative results after excision, Integra placement, and split-thickness skin grafting. Overall aspect of the patient's back 5 months after completion of procedure (A). The patient demonstrated full range of motion (B) and adequate response to pinch stress (C). S60
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Annals of Plastic Surgery • Volume 74, Supplement 1, May 2015
of glycosaminoglycans and chondroitin-6-sulfate covered by a silicon layer,28 allowed for immediate closure and induced recapillarization of the excision site. This enabled us to perform a more stable reconstruction with skin grafts, similar to that described by Kopp et al.28
Outcome The disfigurement and painful treatment resulting from a GCMN can negatively affect the psychosocial functioning of not only the patients but also their families. Social problems and behavioral/ emotional problems have been reported in 30% and 26% of children with GCMN, respectively.29 Children with GCMN often avoid social situations requiring public exposure of body parts, with the view that a burnlike scar resulting from excision is more socially acceptable than a nevus.29 A majority of parents feel that their children are rejected because of the deformity, and many refuse to photograph their child with the nevus in hopes to erase all memories of the nevus.27 Contrarily, although a majority of families report satisfaction with the postsurgical cosmetic result,23,27 a significant proportion (11%–14%) feels that surgical excision worsened their appearance at ages 1, 5, and 10 years.23 Given the disfiguring nature of both the nevus itself and the postoperative scar, the patient's quality of life and the cosmetic outcome of surgery must be taken into consideration when considering timing of surgical excision. At 2 months after split-thickness skin grafting, the patient demonstrated good neodermis formation with adequate elastic response to both pinch and shear stress. The patient tolerated the operation and the reconstructive process well and has had no major complications at 5 months of follow-up (Fig. 6). The patient underwent physical therapy and was able to return to all activities with full range of motion. The procedure resulted in both a cosmetically and functionally satisfactory outcome as deemed by both the operating physician and the patient. The patient was much happier with his cosmetic result, and his symptoms of discomfort and itching were relieved. Despite the favorable outcomes, waiting until adulthood may have resulted in a more painful reconstructive course than if he had undergone early childhood prophylactic surgical excision. The surgery and healing process also disrupted several months of his young adult life.
CONCLUSIONS We present an interesting case of an adult with a GCMN encompassing the patient's entire back with painful nodules exhibiting gross involvement of his back musculature, without pathologic evidence of malignancy. The patient experienced significant impairment in his quality of life and self-esteem because of the massive size and deforming nature of the nevus. The treatment strategy used for this young adult male patient involved a massive en bloc excision of the GCMN with partial resection of the latissimus dorsi followed by a multiweek reconstruction process using dermal regenerative matrices and split-thickness skin grafting. Because of the shift in GCMN management from early surgical management to nonoperative management, we may see an increase in adult cases of GCMN. While illustrating a successful surgical approach to the immense surface area of lesions encountered in adulthood GCMN, this case provides a scenario in which early treatment may have reduced the need for a more morbid operation including partial resection of muscles with localized spread even without malignant transformation. Early surgical excision should be considered not only for presence of high-risk phenotypical features concerning for development of malignant transformation but also for likelihood of improved outcomes including morbidity of delayed excision, improved cosmetic result, and quality of life.
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Adult GCMN: Review of Management
REFERENCES 1. Castilla EE, da Graca Dutra M, Orioli-Parreiras IM. Epidemiology of congenital pigmented naevi: I. Incidence rates and relative frequencies. Br J Dermatol. 1981;104:307–315. 2. Kaplan EN. The risk of malignancy in large congenital nevi. Plast Reconstr Surg. 1974;53:421–428. 3. Quaba AA, Wallace AF. The incidence of malignant melanoma (0 to 15 years of age) arising in “large” congenital nevocellular nevi. Plast Reconstr Surg. 1986;78: 174–181. 4. Solomon LM. The management of congenital melanocytic nevi. Arch Dermatol. 1980;116:1017. 5. Trozak DJ, Rowland WD, Hu F. Metastatic malignant melanoma in prepubertal children. Pediatrics. 1975;55:191–204. 6. Watt AJ, Kotsis SV, Chung KC. Risk of melanoma arising in large congenital melanocytic nevi: a systematic review. Plast Reconstr Surg. 2004;113: 1968–1974. 7. Bergfeld W, Helwig WB. Risk of Developing Melanoma in Congenital Nevi. In Annual Meeting of the American Academy of Dermatology. Miami, FL; 1972. 8. Krengel S, Hauschild A, Schafer. Melanoma risk in congenital melanocytic naevi: a systematic review. Br J Dermatol. 2006;155:1–8. 9. Kinsler VA, Birley J, Atherton DJ. Great Ormond Street Hospital for Children Registry for congenital melanocytic naevi: prospective study 1988–2007. Part 1—epidemiology, phenotype and outcomes. Br J Dermatol. 2009;160:143–150. 10. Heffel DF, Thaller S. Congenital melanosis: an update. J Craniofac Surg. 2005; 16:940–944. 11. Lopez Gutierrez JC, Garcia Palacios M, Diaz M, et al. Indication for early excision of the giant congenital nevus. Cir Pediatr. 2010;3:165–169. 12. Rhodes AR, Albert LS, Weinstock MA. Congenital nevomelanocytic nevi: proportionate area expansion during infancy and early childhood. J Am Acad Dermatol. 1996;34:51–62. 13. Boente Mdel C, Asial RA. Desmoplastic hairless hypopigmented nevus (DHHN). A distinct variant of giant melanocytic nevus. Eur J Dermatol. 2005;15:451–453. 14. Wu JJ, Markus RF, Orengo IF. Nonpigmented nodule on the shoulder: amelanotic congenital nevus. Int J Dermatol. 2004;43:312. 15. Han JS, Won CH, Chang SE, et al. Giant congenital melanocytic nevus with proliferative nodules mimicking a congenital malignant melanoma. Ann Dermatol. 2014;26:554–556. 16. Mark GJ, Mihm MC, Liteplo MG, et al. Congenital melanocytic nevi of the small and garment type. Clinical, histologic, and ultrastructural studies. Hum Pathol. 1973;4:395–418. 17. Zayour M, Lazova R. Congenital melanocytic nevi. Clin Lab Med. 2011;31: 267–280. 18. Arneja JS, Gosain AK. Giant congenital melanocytic nevi of the trunk and an algorithm for treatment. J Craniofac Surg. 2005;16:886–893. 19. Lyon VB. Congenital melanocytic nevi. Pediatr Clin North Am. 2010;57: 1155–1176. 20. De Raeve LE, Claes A, Ruiter DJ, et al. Distinct phenotypic changes between the superficial and deep component of giant congenital melanocytic naevi: a rationale for curettage. Br J Dermatol. 2006;154:485–492. 21. Rasmussen BS, Henriksen TF, Kolle SF, et al. Giant congenital melanocytic nevus: report from 30 years of experience in a single department. In: Ann Plast Surg. 2013; 74:223–229. 22. Bett BJ. Large or multiple congenital melanocytic nevi: occurrence of cutaneous melanoma in 1008 persons. J Am Acad Dermatol. 2005;52:793–797. 23. Kinsler VA, Birley J, Atherton DJ. Great Ormond Street Hospital for Children Registry for congenital melanocytic naevi: prospective study 1988–2007. Part 2—evaluation of treatments. Br J Dermatol. 2009;160:387–392. 24. Manganoni AM, Belloni Fortina A, Pavoni L, et al. The controversial management of giant congenital melanocytic nevi. When would it be better “to wait and see”? G Ital Dermatol Venereol. 2013;148:203–207. 25. Coughlin CC, Council ML, Gru AA, et al. Malignant melanoma arising at the site of a previously excised giant congenital melanocytic nevus. JAMA Dermatol. 2014;150:100–101. 26. Arad E, Zuker RM. The shifting paradigm in the management of giant congenital melanocytic nevi: review and clinical applications. Plast Reconstr Surg. 2014; 133:367–376. 27. Bellier-Waast F, Perrot P, Duteille F, et al. Surgical treatment for giant congenital nevi: what are the psychosocial consequences for the child and family? Ann Chir Plast Esthet. 2008;53:408–414. 28. Kopp J, Magnus Noah E, Rubben A, et al. Radical resection of giant congenital melanocytic nevus and reconstruction with meek-graft covered integra dermal template. Dermatol Surg. 2003;29:653–657. 29. Koot HM, de Waard-van der Spek F, Peer CD, et al. Psychosocial sequelae in 29 children with giant congenital melanocytic naevi. Clin Exp Dermatol. 2000;25:589–593.
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Treatment of a Giant Congenital Melanocytic Nevus in the Adult Review of the Current Management of Giant Congenital Melanocytic Nevus Jeannie J. Su, MAS, Daniel K. Chang, MD, Brian Mailey, MD, and Amanda Gosman, MD Abstract: Giant congenital melanocytic nevi (GCMNs) create cosmetic disfigurements and pose risk for malignant transformation. Adult GCMN cases are uncommon because most families opt for surgical treatment during childhood. We review the current literature on GCMN and present an interesting case of an adult with a GCMN encompassing the entire back with painful nodules exhibiting gross involvement of his back musculature, without pathologic evidence of malignancy. Surgical management was deferred in childhood because of parental desires to allow the patient to make his own decision, and treatment in adulthood was pursued on the basis of the significant impairment of the patient's quality of life and self-esteem due to the massive size and deforming nature of the nevus. The treatment strategy used for this young adult male patient involved a massive en bloc excision of the GCMN with partial resection of the latissimus dorsi, followed by a 5-week staged reconstructive process using dermal regenerative matrices and split-thickness skin grafting. Because of the shift in GCMN management from early surgical management to more conservative management, we may see an increase in adult cases of GCMN. Thus, it is critical to better understand the controversy surrounding early versus delayed management of GCMN. Key Words: adult, skin neoplasms, melanosis, reconstructive surgical procedures, skin grafting (Ann Plast Surg 2015;74: S57–S61)
G
iant congenital melanocytic nevi (GCMNs) occur in approximately 1 in 20,000 live births.1 Giant congenital melanocytic nevi create various medical, cosmetic, and psychological complications, the most concerning of which are transformation to a malignant melanoma and neurocutaneous melanocytosis.2–6 Earlier reports of melanoma transformation rates ranged up to 42%7; however, recent data suggest that previous reports overestimated the risk for transformation and the true risk lies between 0.7% and 2.8%.6,8–10 Because most malignant transformations occur before the age of 3 years, historically, the optimal management strategy of GCMN has been prophylactic excision performed during early childhood. The recent evidence supporting a lower risk for malignant transformation, however, has shifted views regarding the preferred management of GCMN. However, surgical excision can be indicated for medical issues outside malignant transformation including physical discomfort and psychosocial distress.11 Here, we review the current literature on GCMN while presenting a case of an adult with a GCMN encompassing the patient's entire back, which was initially managed during childhood with watchful waiting but ultimately resulted in a multiprocedure surgical excision. Received July 7, 2014, and accepted for publication, after revision, December 6, 2014. From the Division of Plastic Surgery, Department of Surgery, University of California, San Diego, San Diego, CA. Conflicts of interest and sources of funding: none declared. Reprints: Amanda Gosman, MD, Division of Plastic Surgery, Department of Surgery, University of California, San Diego, 200 W. Arbor Dr, San Diego, CA 921038890. E-mail: [email protected]. Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. ISSN: 0148-7043/15/7401–S057 DOI: 10.1097/SAP.0000000000000433
Annals of Plastic Surgery • Volume 74, Supplement 1, May 2015
Presentation Congenital melanocytic nevi vary in size, color, shape, and location. They are classified according to size as small, medium, and large/ giant with diameters of less than 1.5 cm, 1.5 to 20 cm, and greater than 20 cm, respectively, as anticipated in adulthood. Although most nevi are thought to grow proportionally with the child, other lesions can grow rapidly during the first few years of life.12 Color is usually shades of brown or black but can also be pink or nonpigmented.13,14 The nevi can become more defined and darken over time or even become hypopigmented and regress. They can be speckled or homogeneous and, as time progresses, can alter between the two. The nevus is usually oval but can also assume other shapes with well- or ill-defined borders. It can be located anywhere on the skin including the trunk, the extremities, and the face. The surface is usually elevated with ridges and grooves but can also be flat, especially in the early years of life. Although 1 lesion is typically present, multiple nevi can be present. Proliferative nodules can develop, which can be a potential sign of malignancy but also be benign.15 A 23-year-old white man with a 42 46-cm speckled brown GCMN and multiple satellite lesions presented with his parents for evaluation in July 2012 (Figs. 1A, B). The nevus had been present at birth, covering the patient's back, with a dark ring encircling it as well as a 2.5 2.5-cm nodule over his spine (Fig. 2). The patient was followed throughout childhood by a pediatric dermatologist. His initial management before presentation to us included a biopsy at 4.5 months of age, after which a more conservative, watch-and-wait approach was assumed, primarily because of his parents' desire to include the patient in the decision-making process. Family history was positive for basal cell carcinoma in his mother in her late 40s. The patient had no history of malignancy before presentation. On presentation, the nevus spanned his back from the T1 to L4 level and crossed his posterior axillary lines bilaterally. Several satellite lesions marked his lower left back, chest, left third metacarpal, right arm, bilateral legs, and feet. The patient complained of the progression of pain and pruritus in the area of the nevus and the development of 2 palpable nodules. The nodules included a red 8-mm papule on the right border near the axillae and a 6-cm rounded raised nodule on the left mid-to-lower back.
Diagnosis and Evaluation Diagnosis of GCMN is performed via microscopic analysis of biopsies. Classic histologic features include nevus cell extension into the deep reticular dermis and/or superficial subcutaneous fat; tracking of melanocytes around or within vascular, neural, or appendageal structures; arrangement of nevus cells in the reticular dermis as single cells, rows, cords, and sheets; and splaying of melanocytes in between collagen bundles.16,17 In our patient, punch biopsies of the 2 nodular sites were performed. The right lateral back nodule punch biopsy demonstrated mildly thickened epidermis with overlying orthokeratosis and spongiosis with epidermal ulceration, follicular rupture, and atypical intradermal melanocytic proliferation with spitzoid features. The left lower back nodule punch biopsy revealed atypical junctional melanocytic proliferation www.annalsplasticsurgery.com
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FIGURE 1. Initial presentation of GCMN spanning 42 46 cm with 2 palpable nodules. Posterior view (A). Right lateral view (B).
with spitzoid features and dermal fibrosis consistent with hypertrophic scar. In February 2013, repeated biopsies of 2 other enlarging sites were most consistent with compound nevus with congenital features, demonstrating mildly thickened epidermis with overlying basket-weave stratum corneum, hyperpigmented basilar keratinocytes, melanocytic hyperplasia of mildly enlarged melanocytes along the dermal-epidermal junction, focal sclerosis, an increased number of vessels, and dermal edema. Single bland-appearing melanocytes were admixed between sclerotic collagen bundles in the superficial and deep dermis. There was no significant evidence of mitotic activity in the dermal melanocytic population. Evaluation of GCMN often includes a screening magnetic resonance image, a thorough neurologic examination, and serial clinical observations of the progression of the nevi to determine possible development of cutaneous melanoma. Magnetic resonance imaging of the thoracic spine demonstrated the presence of multiple soft tissue masses extending beyond the superficial subcutaneous layer (Fig. 3). On neurologic examination, the patient complained of the progression of pain and pruritus in the area of the nevus and intermittent upper extremity weakness. As stated above, repeated biopsies did not demonstrate evidence of malignancy.
transformation. Contrarily, other studies have suggested a more conservative and less invasive approach to treating GCMN. De Raeve et al20 and Rasmussen et al21 suggested the use of curettage to decrease the number of melanocytic cells and potentially lower the risk for cutaneous melanoma. Recent literature has also commented that previous literature overestimated the risk for melanoma because of reporting bias and less accurate histological methods of diagnosis and that the true risk lies between 0.7% and 2.8%.6,8–10 Moreover, several studies have not been able to demonstrate a reduction in melanoma transformation risk with surgical management.21–24 Granted, these studies have been limited in both sample size and follow-up time. In fact, there have been 5 cases in the literature in which melanoma has developed in a site of
Treatment The management of GCMNs is controversial and highly dependent on the individual patient. A largely debated issue in literature is when and on whom to perform surgery. Treatment of GCMN ranges from watchful waiting to less invasive procedures (dermabrasion, carbon dioxide laser ablation, curettage, chemical peels) to more extensive procedures (tissue expansion, en bloc or serial excision with staged direct closure, skin grafting, free tissue transfer, or skin substitutes).18 Factors that need to be taken into account when considering GCMN management include the presence of high-risk phenotypic features linked to malignant transformation such as size greater than 20 cm in diameter, trunk location, presence of 3 or more lesions, and irregular morphology due to the inability to determine morphological changes.19 Additional considerations unrelated to malignancy risk include the possibility of psychosocial impact and cosmetic deformity.2 Historically, treatment goals have centered on complete excision of the GCMN before the age of 2 years because of increased risk for melanoma transformation. Arneja and Gosain18 presented an algorithm for the treatment of GCMN with surgical excision as the optimal treatment strategy to remove pigmented nevus cells and reduce malignant S58
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FIGURE 2. GCMN spanning the trunk as a child. © 2015 Wolters Kluwer Health, Inc. All rights reserved.
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Annals of Plastic Surgery • Volume 74, Supplement 1, May 2015
Adult GCMN: Review of Management
FIGURE 3. Thoracic magnetic resonance imaging demonstrating the giant superficial congenital nevus extending from approximately the level of T1 down to L4, with a central defect at the level of T8 to T10 from presumed prior resection. Several masslike regions including a 2.8 1.8-cm lesion on the right at the level of T12 (A) and a 4.0 2.3-cm lesion on the left at the level of L1 (B) extend slightly beyond the superficial layer.
excision and grafting.25 Because of these new data, Arad and Zuker26 recently argued that treatment should focus on esthetics and not on complete excision. However, surgical excision can be indicated for medical issues outside malignant transformation including painful ulceration and bleeding,
physical discomfort, concerning dermatologic changes, multiple expected surgical procedures, concomitant surgical procedures, and psychosocial distress.11 Early excision potentially decreases recovery time and psychological trauma because of the patient's lack of recollection of not only the nevus but also the surgical procedure.27 Therefore, in
FIGURE 4. Wide local excision, 42 46 cm, of GCMN and multiple subcutaneous masses. Massive en bloc excision of GCMN spanning the trunk over the site of excision (A). Excision down to the level of the paraspinal musculature with partial resection of the right latissimus dorsi muscle (B). Isolation of main nevus from the patient (C). © 2015 Wolters Kluwer Health, Inc. All rights reserved.
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Annals of Plastic Surgery • Volume 74, Supplement 1, May 2015
Su et al
FIGURE 5. Staged reconstructive process involved the use of Integra (A) followed by split-thickness skin grafting from the buttocks and the posterolateral thighs (B).
considering the case for prophylactic resection, the nonmalignant sequelae of GCMN must be factored into the equation. In our patient's case, although the patient did not possess malignant transformation on biopsy, surgical excision of the nevus was recommended for 2 main reasons: (1) the progressive enlargement and development of the soft tissue masses into the superficial back muscles and (2) the inability to completely rule out malignancy, despite multiple reassuring biopsies, given the enormous size of the nevus. In addition, he had debilitating painful nodules and complained about the social and psychological distress caused by the lesion and his unwillingness to remove his shirt in public. A massive en bloc excision of the GCMN was performed, including partial resection of the right latissimus dorsi muscle, because of gross infiltration of the tumor into the muscle (Fig. 4). Massive en bloc excision was performed over serial excision because of distortion and invasion of the adjacent musculature. Pathology of this subcutaneous involvement revealed multiple nodules of spindle-shaped cells with schwannian features and pseudomeissnerian corpuscles consistent with congenital nevus with neuritization without
evidence of malignancy. Skin flaps were advanced circumferentially to decrease the overall size of the wound. The wound was covered with a meshed Integra dermal regenerative matrix bilayer (Integra LifeSciences, Plainsboro, NJ), Acticoat (Smith & Nephew, Hull, United Kingdom), an antimicrobial silver barrier dressing, and negative-pressure wound therapy with a vacuumassisted closure (VAC) device (KCI, San Antonio, Tex). After the excision, the patient underwent a 5-week staged reconstructive process during which he underwent 7 VAC changes under anesthesia. The patient was unable to tolerate VAC changes at the bedside because of pain. During these operations, the wound edges were inspected and debrided as necessary and small portions of the Integra were changed. After 5 weeks, the Integra became well integrated and the wound's depth decreased from 4-cm deep to 0.5 cm in most areas within the level of the skin margin. Split-thickness skin grafting (0.010-in thick, approximately 2000 cm2, meshed 1:1.5) was performed using the bilateral buttocks and the posterolateral thighs as donor sites (Fig. 5). Use of Integra, a bilayer matrix dermal substitute consisting
FIGURE 6. Postoperative results after excision, Integra placement, and split-thickness skin grafting. Overall aspect of the patient's back 5 months after completion of procedure (A). The patient demonstrated full range of motion (B) and adequate response to pinch stress (C). S60
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Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
Annals of Plastic Surgery • Volume 74, Supplement 1, May 2015
of glycosaminoglycans and chondroitin-6-sulfate covered by a silicon layer,28 allowed for immediate closure and induced recapillarization of the excision site. This enabled us to perform a more stable reconstruction with skin grafts, similar to that described by Kopp et al.28
Outcome The disfigurement and painful treatment resulting from a GCMN can negatively affect the psychosocial functioning of not only the patients but also their families. Social problems and behavioral/ emotional problems have been reported in 30% and 26% of children with GCMN, respectively.29 Children with GCMN often avoid social situations requiring public exposure of body parts, with the view that a burnlike scar resulting from excision is more socially acceptable than a nevus.29 A majority of parents feel that their children are rejected because of the deformity, and many refuse to photograph their child with the nevus in hopes to erase all memories of the nevus.27 Contrarily, although a majority of families report satisfaction with the postsurgical cosmetic result,23,27 a significant proportion (11%–14%) feels that surgical excision worsened their appearance at ages 1, 5, and 10 years.23 Given the disfiguring nature of both the nevus itself and the postoperative scar, the patient's quality of life and the cosmetic outcome of surgery must be taken into consideration when considering timing of surgical excision. At 2 months after split-thickness skin grafting, the patient demonstrated good neodermis formation with adequate elastic response to both pinch and shear stress. The patient tolerated the operation and the reconstructive process well and has had no major complications at 5 months of follow-up (Fig. 6). The patient underwent physical therapy and was able to return to all activities with full range of motion. The procedure resulted in both a cosmetically and functionally satisfactory outcome as deemed by both the operating physician and the patient. The patient was much happier with his cosmetic result, and his symptoms of discomfort and itching were relieved. Despite the favorable outcomes, waiting until adulthood may have resulted in a more painful reconstructive course than if he had undergone early childhood prophylactic surgical excision. The surgery and healing process also disrupted several months of his young adult life.
CONCLUSIONS We present an interesting case of an adult with a GCMN encompassing the patient's entire back with painful nodules exhibiting gross involvement of his back musculature, without pathologic evidence of malignancy. The patient experienced significant impairment in his quality of life and self-esteem because of the massive size and deforming nature of the nevus. The treatment strategy used for this young adult male patient involved a massive en bloc excision of the GCMN with partial resection of the latissimus dorsi followed by a multiweek reconstruction process using dermal regenerative matrices and split-thickness skin grafting. Because of the shift in GCMN management from early surgical management to nonoperative management, we may see an increase in adult cases of GCMN. While illustrating a successful surgical approach to the immense surface area of lesions encountered in adulthood GCMN, this case provides a scenario in which early treatment may have reduced the need for a more morbid operation including partial resection of muscles with localized spread even without malignant transformation. Early surgical excision should be considered not only for presence of high-risk phenotypical features concerning for development of malignant transformation but also for likelihood of improved outcomes including morbidity of delayed excision, improved cosmetic result, and quality of life.
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Adult GCMN: Review of Management
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