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Indications for excision of nevi and melanoma diagnosed in a pediatric surgical unit
Journal of Pediatric Surgery (2007) 42, 1412 – 1416
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Indications for excision of nevi and melanoma diagnosed in a pediatric surgical unit Andrea Zangaria,*, Maria Luisa Bernardinib, Rosella Tallaricoa, Michele Ilaria Mirella Giangiacomic, Anna Maria Offidanib, Ascanio Martinoa a
Paediatric Surgery Unit, Salesi Children’s Hospital, Ancona, Italy Dermatology Clinic, University Hospital, Ancona, Italy c Pathology Department, University Hospital, Ancona, Italy b
Index words: Melanoma; Nevi; Child
Abstract Background/Purpose: Childhood melanoma is rare but increasing in incidence. Its management relies on early diagnosis. The purpose of this study is to discuss surgical indications of nevi and diagnosis of melanoma in a pediatric surgical unit. Methods: Data relative to the patients who underwent removal of nevi in our pediatric surgical unit from 1999 to 2005 were reviewed to identify indications, histology, and melanoma occurrence. Results: The most frequent indication was atypical nevus. Compound nevus was the most common finding, followed by congenital and Spitz nevi. Melanoma was diagnosed in 3 excised nevi, and in 1 case it occurred as a metastatic disease. Conclusions: Our data showed a pattern of indications for surgery similar to that described in the literature, with a high detection rate of melanoma, nonetheless showing that some rare conditions may delay diagnosis. D 2007 Elsevier Inc. All rights reserved.
Malignant melanoma (MM) accounts for 1% to 3% of all human malignant tumors. Only 0.3% to 0.4% of all melanomas occur in prepubertal age [1]; 1.3% occur in patients younger than 20 years [2]. The incidence of MM in children is estimated to be 0.7 per million per year in children aged 0 to 9 years, whereas it is 13.2 per million per year in people aged between 15 and 19 years [3]. This incidence is continuing to rise. Management of melanoma relies on prevention and early diagnosis. Diagnostic criteria include the distinction from MM mimics, especially atypical * Corresponding author. Unita` Operativa Chirurgia Pediatrica, Ospedale bG. Salesi,Q Via Filippo Corridoni, 11 60123 Ancona, Italy. Tel.: +39 071 5962218; fax: +39 071 5962316. E-mail address: [email protected] (A. Zangari). 0022-3468/$ – see front matter D 2007 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2007.03.044
nevi, Spitz tumors, and lesions presenting clinical changing. Identification of lesions resembling melanoma or prone to melanoma development is essential to obtain early treatment and to avoid unnecessary surgery. We report our experience from 1999 to 2005 concerning indications for surgery, histologic types, treatment of nevi, and occurrence of melanoma in a pediatric surgical unit.
1. Methods Data relative to the patients who underwent removal of nevi in our pediatric surgical unit from 1999 to 2005 were retrospectively reviewed. Eighty-two children aged 1 month to 14 years underwent surgical excision of 91 nevi.
Indications for excision of nevi and melanoma diagnosed in a pediatric surgical unit
Fig. 1
Indications for excision of nevi.
Indications for excision were recorded and grouped by clinical aspects and signs, either relevant to MM diagnosis or not. Groups of indications included clinical appearance of atypical and Spitz nevi, other isolated warning signs of MM (rapid onset and growth, bleeding, itching, pain), preventive excision of congenital nevi, site of difficult monitoring, and indications unrelated to MM diagnosis, as in the case of exposure to frequent trauma. Surgical therapy followed the most recent recommendations [4]. Excision of the primary tumor was performed with 2-mm margins of normal skin. After diagnosis of MM, the patient underwent sentinel lymph node biopsy (SLNB) for melanoma thickness of 1 mm or more, along with wide excision of the tumor site with 2-cm margins and primary closure or skin graft. Sentinel lymph node biopsy was performed using preoperative lymphoscintigraphy, intraoperative blue dye injection around the site of excision, and handheld gamma probe for radiolocalization. Elective regional lymph node dissection (ELND) was subsequently performed if the result of the SLNB was positive for metastases. Pathologic reports were reviewed for standard pathologic variables including Clark level, Breslow thickness, lymph node involvement, and histologic diagnosis for lesions other than melanoma. The study was approved by the institutional review board.
difficult monitoring, congenital nevi, and clinical appearance of Spitz nevi; less frequent reasons for removal were bleeding, exposure to frequent trauma, and itching (Fig. 1). In all cases, first surgical excision included 2-mm margins of normally appearing skin around the lesion. Primary skin closure was obtained in all cases except in 3 congenital nevi: a large congenital nevus on the left gluteus was treated by complete excision after skin expansion; an intermediate-sized congenital nevus on the plantar region required skin closure by local flaps after complete removal; a large bgarmentQ nevus in an infant affected by von Recklinghausen disease underwent partial excision at 1 month of age. After the removal of a dorsal schwannoma when 16 months old, at the age of 24 months this baby also underwent an excisional biopsy of an inguinal nodal mass resulting in metastatic melanoma. Three patients underwent scar excision with 2-cm margins and SLNB for MM diagnosis after excision of a nevic lesion. In 2 patients, primary skin closure was obtained. In 1 patient, an inguinal skin graft was used to cover the residual defect on his foot. In the same patient, the result of the SLNB was positive, and inguino-iliac-obturatory ELND was required. One of these cases of MM was diagnosed in an 11-year-old girl after excision of a bleeding nevic lesion frequently irritated by brassieres; it was a superficial spreading-type MM of Clark level IV, 3.22 mm thick; the result of the SLNB was negative. A 12-year-old girl underwent excision of a clinically atypical nevus of the dorsal region; histologic examination revealed a Clark level III melanoma, 1 mm thick; she underwent wide local excision, SLNB, and a second axillary nevus removal; the result of the SLNB was negative. A 3-year-old boy underwent surgical excision of a small congenital nevus on his foot that showed rapid growth in the last 6 months from 3 to 4 mm in diameter to 1 cm; after diagnosis of nodular MM of Clark level V with 7-mm thickness, the result of the SLNB was positive, and regional inguino-iliac-obturatory lymph node dissection showed micrometastases in a single femoral node. With the exception of 1 case of MM, diagnosed when already
2. Results Indications for excision were assessed by dermatologists in 71 (78%) of 91 cases, in 15 cases by pediatric surgeons (16.5%), in 4 cases by pediatricians (4.4%), and in 1 case by a physician (1.1%). Sites of the lesions were trunk in 43 cases (47%), extremities in 40 (44%), and head and neck in 8 (8.8%). Clinical signs of atypical nevi were main surgical indications, followed by abnormal growth, sites of
1413
Fig. 2
Histologic findings for excised nevi.
1414
A. Zangari et al.
Fig. 3
Histologic findings related to indications.
metastatic, the diagnosis of MM after excision of suspected lesions was obtained in the 3 cases described previously (3.3%). Histologic examination of the remaining lesions revealed 26 (29%) compound nevi, 15 (16%) congenital nevi, 14 (15%) Spitz nevi of which 3 showed atypical features, 11 (12%) dermal nevi, 9 (10%) junctional nevi, 4 (4.4%) atypical nevi, 2 (2.2%) blue nevi, 2 (2.2%) hyperpigmentation of the basal layer, 1 (1.1%) seborrheic keratosis, 1 (1.1%) lentigo simplex, 1 (1.1%) combined blue and junctional nevus, 1 (1.1%) angiokeratoma, and 1 (1.1%) combined blue and Spitz nevus; in this last case, diagnosis was revealed by an additional histologic evaluation after a first diagnosis of melanoma. Histologic findings are shown in Fig. 2. Distribution of most frequent and relevant histologic findings related to different indications for surgery is shown in Fig. 3. All patients who underwent surgery did not show complications or residual deformities from scarring. The baby with giant nevus and von Recklinghausen disease died of cerebral metastasis 1 month after diagnosis of nodal metastatic melanoma. The 11-year-old girl with 3.22-mm-thick MM and the 12-year-old girl with 1-mm-thick MM are disease free after 6 years and 3 years, respectively. The 3-year-old boy with nodular, 7-mm-thick MM of the foot and femoral nodal micrometastases had negative results at 3 and 6 months’ follow-up by blood tests and thoracic x-ray.
3. Discussion The main predisposing conditions for development of melanoma are xeroderma pigmentosum, pigmentary traits, sunburns during infancy with discontinuous and intense
exposure, nevus density, familial history of MM, dysplastic nevi, large congenital nevi, and, with some controversy, intermediate congenital nevi [2,5,6]. Diagnostic criteria include the distinction from MM mimics, especially atypical nevi, Spitz tumors, and lesions presenting asymmetry, border notching, color variegation, diameter of more than 5 mm, rapid onset or increase in diameter, as well as bleeding, ulceration, itching, and pain [7]. Proper surgical excision, appropriate use of staging techniques, such as SLNB, and ELND are important for correct management [8-10], but prevention is still the best strategy. Education about warning signs of melanoma, adequate sun protection, follow-up for clinical changing, the use of dermoscopy, and, according to many authors, removal of large (N20 cm) congenital melanocytic nevi play an important role in the prevention of MM, whereas the risks associated with medium and small congenital melanocytic nevi and their preventive excision are still a matter of controversy [5,6]. Large congenital nevi show an increased risk for development of MM, varying from 1% to 31% in the literature [11,12]. This was confirmed by the clinical evolution and outcome in our case. Nonetheless, in similar cases, malignancy is reported to occur despite complete excision and may not be preventable [13]. In addition, our case was affected by the increased risk of malignancy generally related to von Recklinghausen disease and by its possible association with MM [14,15]. Although intermediate congenital nevi are not demonstrated to be at risk [6], according to some studies, incidence of MM in intermediate and even small lesions is increased [16-18]. Because melanoma may also develop within small congenital nevi, some authors advise prophylactic excision of these lesions [12,19]. Nevertheless, melanoma risk in small congenital nevi is still a matter of controversy, although in some reports it seems to be of importance [20-25]. In our unit, the treatment of choice for congenital nevi was surgical excision of all giant and intermediate-sized lesions. The occurrence of MM in a patient with a small congenital nevus, in the lack of consensus about systematic removal, confirms the importance of careful dermatologic monitoring of these lesions and prompt excision after clinical changing. Indications for surgical excision of acquired nevi are mainly related to lesions resembling MM, such as atypical nevi, Spitz nevi, and lesions presenting clinical signs and symptoms as diameter of more than 5 mm or increasing diameter, irregular margins or pigmentation, ulceration, bleeding, and itching [26]. Patients with atypical nevi are demonstrated to be at increased risk for development of melanoma. This risk is higher in familial atypical nevi subgroups, but it has been found even in patients with nonfamilial atypical nevi [27]. In fact, features of atypical nevi were the most frequent indication to surgery in our unit. Moreover, 1 case of MM was diagnosed within this group of clinical entities. Some special consideration is attributed to Spitz nevus for possible misdiagnosis with MM [28]. Most dermatolo-
Indications for excision of nevi and melanoma diagnosed in a pediatric surgical unit gists and physicians recommend biopsy, either excisional or incisional. In an interview with dermatologists, most responding specialists (93%) recommended the biopsy of suspected Spitz nevi: 43% of dermatologists in this group recommended excisional biopsy, and 55% recommended incisional biopsy; 2% chose either excisional or incisional biopsy, depending on the clinical situation. Sixty-nine percent of physicians would completely excise a lesion that was histologically diagnosed as an incompletely removed Spitz nevus. Seventy percent of general dermatologists and 80% of pediatric dermatologists would recommend excision with a 1- to 2-mm margin of normal-appearing skin around a Spitz nevus. The lack of consensus about the nature and the ideal management of Spitz nevus reflects the uncertainty in histopathologic distinction between Spitz nevus and melanoma, and such a concern influences management [29]. Complete excision with 2-mm margins was performed in all cases that referred to our unit with a clinical diagnosis of Spitz nevus. Particular concern regarded a patient who referred to our center for an enlarging and oozing atypical nevus, which at first histologic examination was considered to be a melanoma and after additional evaluation resulted to be a combined blue and Spitz nevus. Criteria for distinguishing melanoma from Spitz nevus are not always reliable. Most of these lesions occur in children and can be diagnosed with confidence; nevertheless, great caution should be taken in rendering this diagnosis when we observe excessive mitotic activity, deep mitoses, atypical mitoses, clear lack of maturation, and bpushingQ rather than infiltrative lower border. In our opinion, the difficulty in histopathologic diagnosis implies wide local excision with 2-cm margins. It is of interest that, in most cases, clinical diagnosis of Spitz nevus was confirmed by histology. In 1 case of MM, spontaneous bleeding had been the indication for excision. Bleeding is included in minor signs suggesting transformation to melanoma [30]. Surgical excision should be seriously considered in these cases, as bleeding, recent growth, pain, ulceration, and color change have been reported as presentation symptoms of MM in 85% of cases [31]. In 13% of cases, indication for excision was location in a site of difficult examination and monitoring. Although this is not classically described among standard criteria for melanoma prevention, it seems that melanoma concern has extended preventive surgery beyond standard indications. In fact, only in 1 case of excision with this indication was histologic diagnosis of atypical nevus, whereas in all other cases histopathologic findings were negative. Our findings suggest that selection of cases was satisfactory, as detection rate of MM among all excised nevi (3/91) was high compared to the rarity of MM occurrence in the pediatric population. Concurrent with this result is the finding that, in 78% of cases, a dermatologist was the referring specialist. Early excision of all suspect lesions is the best treatment, especially when surgical indications are assessed by the dermatologist. Indications
1415
for surgery, when selected and appropriate, lead to early detection and treatment of MM; but some lesions, such as small congenital nevi in which the entity of risk is unclear, should undergo strict monitoring while further investigation on their clinical behavior is still needed. Prevention is still the best strategy against MM: patient and family education, sun protection, and early excision of large congenital nevi.
References [1] Strojan P, Lamovec J. Prepubertal malignant melanoma: report of three cases. Pediatr Hematol Oncol 2000;17(2):163 - 9. [2] Pappo AS. Melanoma in children and adolescents. Eur J Cancer 2003;39:2651 - 61. [3] Makkar HS, Frieden IJ. Congenital melanocytic nevi: an update for the paediatrician. Curr Opin Pediatr 2002;14(4):397 - 403. [4] Swetter SM. Malignant melanoma. eMedicine.com, Inc, 2004. [5] Rompel R, Moser M, Peters J. Dermabrasion of congenital nevocellular naevi: experience in 215 patients. Dermatology 1997;194:261 - 7. [6] Sahin S, Levin L, Kopf AW, et al. Risk of melanoma in medium-sized congenital melanocytic nevi: a follow-up study. J Am Acad Dermatol 1999;41(1):131 - 2. [7] Yesudian PD, Parslew RAG. A guide to the management of pigmented skin naevi in children. Curr Paediatr 2003;13:407 - 12. [8] Neville HL, Andrassy RJ, Lally KP, et al. Lymphatic mapping with sentinel node biopsy in pediatric patients. J Pediatr Surg 2000;35(6):961 - 4. [9] Durrani AJ, Moir GC, Diaz-Cano SJ, et al. Malignant melanoma in an 8-year-old Caribbean girl: diagnostic criteria and utility of sentinel lymph node biopsy. Br J Dermatol 2003;148(3):569 - 72. [10] Shen P, Guenther JM, Wanek LA, et al. Can elective lymph node dissection decrease the frequency and mortality rate of late melanoma recurrences? Ann Surg Onc 2000;7(2):114 - 9. [11] Ruiz-Maldonado R, Tamayo L, Laterza AM, et al. Giant pigmented naevi: clinical, histopathologic and therapeutic considerations. J Pediatr 1992;120:906 - 11. [12] Zaal LH, Mooi WJ, Sillevis Smitt JH, et al. Classification of congenital melanocytic naevi and malignant transformation: a review of the literature. Br J Plast Surg 2004;57(8):707 - 19. [13] Davies DM. Benign skin tumors. Br Med J 1985;291:217 - 8. [14] Zoller ME, Rembeck B, Oden A, et al. Malignant and benign tumors in patients with neurofibromatosis type 1 in a defined Swedish population. Cancer 1997;79(11):2125 - 31. [15] Gallino G, Belli F, Tragni G, et al. Association between cutaneous melanoma and neurofibromatosis type 1: analysis of three clinical cases and review of the literature. Tumori 2000;86(1):70 - 4. [16] Rhodes AR, Sober AJ, Day CL, et al. The malignant potential of small congenital nevocellular nevi. An estimate of association based on a histologic study of 234 primary cutaneous melanomas. J Am Acad Dermatol 1982;6:230. [17] Mackie RM, Watt D, Doherty V, et al. Malignant melanoma occurring in those aged under 30 in the west of Scotland 1976-1986: a study of incidence, clinical features, pathologic features and survival. Br J Dermatol 1991;124:560 - 4. [18] Illig L, Waidner F, Hundeiker M, et al. Congenital nevi b10 cm as precursors to melanoma. Arch Dermatol 1985;121:1274 - 81. [19] Rhodes AR. Melanocytic precursors of cutaneous melanoma: estimated risks and guidelines for management. Med Clin North Am 1986;70:3 - 37. [20] Rhodes AR, Melski JW. Small congenital nevocellular nevi and the risk of cutaneous melanoma. J Pediatr 1982;100:219 - 24. [21] Amagai N, Williams CM. Malignant melanoma arising from a small congenital nevus in a black child. Arch Dermatol 1993;129(9):1215 - 7.
1416 [22] Tannous ZS, Mihm Jr MC, Sober AJ, et al. Congenital melanocytic nevi: clinical and histopathologic features, risk of melanoma, and clinical management. J Am Acad Dermatol 2005;52(2): 197 - 203. [23] Michel JL, Chalencon F, Gentil-Perret A, et al. Congenital pigmented nevus: prognosis and therapeutic possibilities. Arch Pediatr 1999;6(2):211 - 7. [24] Clemmensen O, Ackerman AB. All small congenital nevi need not be removed. Am J Dermatopathol 1984;6(Suppl):189 - 94 [Summer]. [25] Paull WH, Polley D, Fitzpatrick JE. Malignant melanoma arising intradermally in a small congenital nevus of an adult. J Dermatol Surg Oncol 1986;12(11):1176 - 8. [26] Kocialkowski K, Prazynski A, Urbanski G, et al. Removal of melanocytic nevi as a prophylaxis and early recognition of cutaneous and mucous malignant melanoma. Ginekol Pol 1993;64(1):16 - 8.
A. Zangari et al. [27] Dyon GCTM, Snels MD, Elyse´e TM, et al. Risk of cutaneous malignant melanoma in patients with nonfamilial atypical nevi from a pigmented lesions clinic. Leiden, The Netherlands. J Am Acad Dermatol 1999;40:686 - 93. [28] Zaenglein AJ, Heintz P, Kamino H, et al. Congenital Spitz nevus clinically mimicking melanoma. J Am Acad Dermatol 2002;47: 441 - 4. [29] Gelbard SN, Tripp JM, Marghoob AA, et al. Management of Spitz nevi: a survey of dermatologists in the United States. J Am Acad Dermatol 2002;47(2):224 - 30. [30] Champion RH, Burton JL, Burns DA, et al, editors. Melanocytic nevi and malignant melanoma. Textbook of Dermatology. Oxford7 Blackwell Science Ltd; 1998. p. 1743 - 9. [31] Saenz NC, Saenz-Badillos J, Busam K, et al. Childhood melanoma survival. Cancer 1999;85:750 - 4.
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Indications for excision of nevi and melanoma diagnosed in a pediatric surgical unit Andrea Zangaria,*, Maria Luisa Bernardinib, Rosella Tallaricoa, Michele Ilaria Mirella Giangiacomic, Anna Maria Offidanib, Ascanio Martinoa a
Paediatric Surgery Unit, Salesi Children’s Hospital, Ancona, Italy Dermatology Clinic, University Hospital, Ancona, Italy c Pathology Department, University Hospital, Ancona, Italy b
Index words: Melanoma; Nevi; Child
Abstract Background/Purpose: Childhood melanoma is rare but increasing in incidence. Its management relies on early diagnosis. The purpose of this study is to discuss surgical indications of nevi and diagnosis of melanoma in a pediatric surgical unit. Methods: Data relative to the patients who underwent removal of nevi in our pediatric surgical unit from 1999 to 2005 were reviewed to identify indications, histology, and melanoma occurrence. Results: The most frequent indication was atypical nevus. Compound nevus was the most common finding, followed by congenital and Spitz nevi. Melanoma was diagnosed in 3 excised nevi, and in 1 case it occurred as a metastatic disease. Conclusions: Our data showed a pattern of indications for surgery similar to that described in the literature, with a high detection rate of melanoma, nonetheless showing that some rare conditions may delay diagnosis. D 2007 Elsevier Inc. All rights reserved.
Malignant melanoma (MM) accounts for 1% to 3% of all human malignant tumors. Only 0.3% to 0.4% of all melanomas occur in prepubertal age [1]; 1.3% occur in patients younger than 20 years [2]. The incidence of MM in children is estimated to be 0.7 per million per year in children aged 0 to 9 years, whereas it is 13.2 per million per year in people aged between 15 and 19 years [3]. This incidence is continuing to rise. Management of melanoma relies on prevention and early diagnosis. Diagnostic criteria include the distinction from MM mimics, especially atypical * Corresponding author. Unita` Operativa Chirurgia Pediatrica, Ospedale bG. Salesi,Q Via Filippo Corridoni, 11 60123 Ancona, Italy. Tel.: +39 071 5962218; fax: +39 071 5962316. E-mail address: [email protected] (A. Zangari). 0022-3468/$ – see front matter D 2007 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2007.03.044
nevi, Spitz tumors, and lesions presenting clinical changing. Identification of lesions resembling melanoma or prone to melanoma development is essential to obtain early treatment and to avoid unnecessary surgery. We report our experience from 1999 to 2005 concerning indications for surgery, histologic types, treatment of nevi, and occurrence of melanoma in a pediatric surgical unit.
1. Methods Data relative to the patients who underwent removal of nevi in our pediatric surgical unit from 1999 to 2005 were retrospectively reviewed. Eighty-two children aged 1 month to 14 years underwent surgical excision of 91 nevi.
Indications for excision of nevi and melanoma diagnosed in a pediatric surgical unit
Fig. 1
Indications for excision of nevi.
Indications for excision were recorded and grouped by clinical aspects and signs, either relevant to MM diagnosis or not. Groups of indications included clinical appearance of atypical and Spitz nevi, other isolated warning signs of MM (rapid onset and growth, bleeding, itching, pain), preventive excision of congenital nevi, site of difficult monitoring, and indications unrelated to MM diagnosis, as in the case of exposure to frequent trauma. Surgical therapy followed the most recent recommendations [4]. Excision of the primary tumor was performed with 2-mm margins of normal skin. After diagnosis of MM, the patient underwent sentinel lymph node biopsy (SLNB) for melanoma thickness of 1 mm or more, along with wide excision of the tumor site with 2-cm margins and primary closure or skin graft. Sentinel lymph node biopsy was performed using preoperative lymphoscintigraphy, intraoperative blue dye injection around the site of excision, and handheld gamma probe for radiolocalization. Elective regional lymph node dissection (ELND) was subsequently performed if the result of the SLNB was positive for metastases. Pathologic reports were reviewed for standard pathologic variables including Clark level, Breslow thickness, lymph node involvement, and histologic diagnosis for lesions other than melanoma. The study was approved by the institutional review board.
difficult monitoring, congenital nevi, and clinical appearance of Spitz nevi; less frequent reasons for removal were bleeding, exposure to frequent trauma, and itching (Fig. 1). In all cases, first surgical excision included 2-mm margins of normally appearing skin around the lesion. Primary skin closure was obtained in all cases except in 3 congenital nevi: a large congenital nevus on the left gluteus was treated by complete excision after skin expansion; an intermediate-sized congenital nevus on the plantar region required skin closure by local flaps after complete removal; a large bgarmentQ nevus in an infant affected by von Recklinghausen disease underwent partial excision at 1 month of age. After the removal of a dorsal schwannoma when 16 months old, at the age of 24 months this baby also underwent an excisional biopsy of an inguinal nodal mass resulting in metastatic melanoma. Three patients underwent scar excision with 2-cm margins and SLNB for MM diagnosis after excision of a nevic lesion. In 2 patients, primary skin closure was obtained. In 1 patient, an inguinal skin graft was used to cover the residual defect on his foot. In the same patient, the result of the SLNB was positive, and inguino-iliac-obturatory ELND was required. One of these cases of MM was diagnosed in an 11-year-old girl after excision of a bleeding nevic lesion frequently irritated by brassieres; it was a superficial spreading-type MM of Clark level IV, 3.22 mm thick; the result of the SLNB was negative. A 12-year-old girl underwent excision of a clinically atypical nevus of the dorsal region; histologic examination revealed a Clark level III melanoma, 1 mm thick; she underwent wide local excision, SLNB, and a second axillary nevus removal; the result of the SLNB was negative. A 3-year-old boy underwent surgical excision of a small congenital nevus on his foot that showed rapid growth in the last 6 months from 3 to 4 mm in diameter to 1 cm; after diagnosis of nodular MM of Clark level V with 7-mm thickness, the result of the SLNB was positive, and regional inguino-iliac-obturatory lymph node dissection showed micrometastases in a single femoral node. With the exception of 1 case of MM, diagnosed when already
2. Results Indications for excision were assessed by dermatologists in 71 (78%) of 91 cases, in 15 cases by pediatric surgeons (16.5%), in 4 cases by pediatricians (4.4%), and in 1 case by a physician (1.1%). Sites of the lesions were trunk in 43 cases (47%), extremities in 40 (44%), and head and neck in 8 (8.8%). Clinical signs of atypical nevi were main surgical indications, followed by abnormal growth, sites of
1413
Fig. 2
Histologic findings for excised nevi.
1414
A. Zangari et al.
Fig. 3
Histologic findings related to indications.
metastatic, the diagnosis of MM after excision of suspected lesions was obtained in the 3 cases described previously (3.3%). Histologic examination of the remaining lesions revealed 26 (29%) compound nevi, 15 (16%) congenital nevi, 14 (15%) Spitz nevi of which 3 showed atypical features, 11 (12%) dermal nevi, 9 (10%) junctional nevi, 4 (4.4%) atypical nevi, 2 (2.2%) blue nevi, 2 (2.2%) hyperpigmentation of the basal layer, 1 (1.1%) seborrheic keratosis, 1 (1.1%) lentigo simplex, 1 (1.1%) combined blue and junctional nevus, 1 (1.1%) angiokeratoma, and 1 (1.1%) combined blue and Spitz nevus; in this last case, diagnosis was revealed by an additional histologic evaluation after a first diagnosis of melanoma. Histologic findings are shown in Fig. 2. Distribution of most frequent and relevant histologic findings related to different indications for surgery is shown in Fig. 3. All patients who underwent surgery did not show complications or residual deformities from scarring. The baby with giant nevus and von Recklinghausen disease died of cerebral metastasis 1 month after diagnosis of nodal metastatic melanoma. The 11-year-old girl with 3.22-mm-thick MM and the 12-year-old girl with 1-mm-thick MM are disease free after 6 years and 3 years, respectively. The 3-year-old boy with nodular, 7-mm-thick MM of the foot and femoral nodal micrometastases had negative results at 3 and 6 months’ follow-up by blood tests and thoracic x-ray.
3. Discussion The main predisposing conditions for development of melanoma are xeroderma pigmentosum, pigmentary traits, sunburns during infancy with discontinuous and intense
exposure, nevus density, familial history of MM, dysplastic nevi, large congenital nevi, and, with some controversy, intermediate congenital nevi [2,5,6]. Diagnostic criteria include the distinction from MM mimics, especially atypical nevi, Spitz tumors, and lesions presenting asymmetry, border notching, color variegation, diameter of more than 5 mm, rapid onset or increase in diameter, as well as bleeding, ulceration, itching, and pain [7]. Proper surgical excision, appropriate use of staging techniques, such as SLNB, and ELND are important for correct management [8-10], but prevention is still the best strategy. Education about warning signs of melanoma, adequate sun protection, follow-up for clinical changing, the use of dermoscopy, and, according to many authors, removal of large (N20 cm) congenital melanocytic nevi play an important role in the prevention of MM, whereas the risks associated with medium and small congenital melanocytic nevi and their preventive excision are still a matter of controversy [5,6]. Large congenital nevi show an increased risk for development of MM, varying from 1% to 31% in the literature [11,12]. This was confirmed by the clinical evolution and outcome in our case. Nonetheless, in similar cases, malignancy is reported to occur despite complete excision and may not be preventable [13]. In addition, our case was affected by the increased risk of malignancy generally related to von Recklinghausen disease and by its possible association with MM [14,15]. Although intermediate congenital nevi are not demonstrated to be at risk [6], according to some studies, incidence of MM in intermediate and even small lesions is increased [16-18]. Because melanoma may also develop within small congenital nevi, some authors advise prophylactic excision of these lesions [12,19]. Nevertheless, melanoma risk in small congenital nevi is still a matter of controversy, although in some reports it seems to be of importance [20-25]. In our unit, the treatment of choice for congenital nevi was surgical excision of all giant and intermediate-sized lesions. The occurrence of MM in a patient with a small congenital nevus, in the lack of consensus about systematic removal, confirms the importance of careful dermatologic monitoring of these lesions and prompt excision after clinical changing. Indications for surgical excision of acquired nevi are mainly related to lesions resembling MM, such as atypical nevi, Spitz nevi, and lesions presenting clinical signs and symptoms as diameter of more than 5 mm or increasing diameter, irregular margins or pigmentation, ulceration, bleeding, and itching [26]. Patients with atypical nevi are demonstrated to be at increased risk for development of melanoma. This risk is higher in familial atypical nevi subgroups, but it has been found even in patients with nonfamilial atypical nevi [27]. In fact, features of atypical nevi were the most frequent indication to surgery in our unit. Moreover, 1 case of MM was diagnosed within this group of clinical entities. Some special consideration is attributed to Spitz nevus for possible misdiagnosis with MM [28]. Most dermatolo-
Indications for excision of nevi and melanoma diagnosed in a pediatric surgical unit gists and physicians recommend biopsy, either excisional or incisional. In an interview with dermatologists, most responding specialists (93%) recommended the biopsy of suspected Spitz nevi: 43% of dermatologists in this group recommended excisional biopsy, and 55% recommended incisional biopsy; 2% chose either excisional or incisional biopsy, depending on the clinical situation. Sixty-nine percent of physicians would completely excise a lesion that was histologically diagnosed as an incompletely removed Spitz nevus. Seventy percent of general dermatologists and 80% of pediatric dermatologists would recommend excision with a 1- to 2-mm margin of normal-appearing skin around a Spitz nevus. The lack of consensus about the nature and the ideal management of Spitz nevus reflects the uncertainty in histopathologic distinction between Spitz nevus and melanoma, and such a concern influences management [29]. Complete excision with 2-mm margins was performed in all cases that referred to our unit with a clinical diagnosis of Spitz nevus. Particular concern regarded a patient who referred to our center for an enlarging and oozing atypical nevus, which at first histologic examination was considered to be a melanoma and after additional evaluation resulted to be a combined blue and Spitz nevus. Criteria for distinguishing melanoma from Spitz nevus are not always reliable. Most of these lesions occur in children and can be diagnosed with confidence; nevertheless, great caution should be taken in rendering this diagnosis when we observe excessive mitotic activity, deep mitoses, atypical mitoses, clear lack of maturation, and bpushingQ rather than infiltrative lower border. In our opinion, the difficulty in histopathologic diagnosis implies wide local excision with 2-cm margins. It is of interest that, in most cases, clinical diagnosis of Spitz nevus was confirmed by histology. In 1 case of MM, spontaneous bleeding had been the indication for excision. Bleeding is included in minor signs suggesting transformation to melanoma [30]. Surgical excision should be seriously considered in these cases, as bleeding, recent growth, pain, ulceration, and color change have been reported as presentation symptoms of MM in 85% of cases [31]. In 13% of cases, indication for excision was location in a site of difficult examination and monitoring. Although this is not classically described among standard criteria for melanoma prevention, it seems that melanoma concern has extended preventive surgery beyond standard indications. In fact, only in 1 case of excision with this indication was histologic diagnosis of atypical nevus, whereas in all other cases histopathologic findings were negative. Our findings suggest that selection of cases was satisfactory, as detection rate of MM among all excised nevi (3/91) was high compared to the rarity of MM occurrence in the pediatric population. Concurrent with this result is the finding that, in 78% of cases, a dermatologist was the referring specialist. Early excision of all suspect lesions is the best treatment, especially when surgical indications are assessed by the dermatologist. Indications
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for surgery, when selected and appropriate, lead to early detection and treatment of MM; but some lesions, such as small congenital nevi in which the entity of risk is unclear, should undergo strict monitoring while further investigation on their clinical behavior is still needed. Prevention is still the best strategy against MM: patient and family education, sun protection, and early excision of large congenital nevi.
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