Vulvar nevi in children

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<ul><li><p>J Pediatr Adolesc Gynecol (2004) 17:353356</p><p>enlarge in proportion with the growth of the skin of</p><p>the involved body area. Congenital nevi reach theirfinal size when children reach their final size. A con-genital nevus on infant girls vulva can be expectedto increase by approximately two to three times itsoriginal size by the time she is an adult.1 If this is acongenital nevus, growth of the lesion by a few milli-meters since birth would be considered normal but adoubling or tripling in size by age 3 would not be.</p><p>Two pertinent negatives in the history should be high-lighted: the lack of symptoms and the negative familyhistory. Women with vulvar melanoma may presentwith bleeding, a mass, discharge, pruritus, pain, dys-uria, a non-healing sore, alteration of the urinarystream, and a foul odor.2 One premenarchal girl with</p><p>Vulvar melanoma has been reported, albeit rarely, inpremenarchal girls. In a recent series of 20 patientswith vulvar melanoma, one patient was a 10-year-old premenarchal girl who had two primary vulvarmelanomas with a positive sentinel lymph node.2</p><p>Other reported cases of vulvar melanoma in child-hood, however, seem less clear. In 1997, two premen-archal girls, ages 9 and 11, were reported to havevulvar melanoma; each girl had an acquired, asymp-tomatic enlarging hyperpigmented macule on thelabium minor and in each lesion there were alsohistological changes of lichen sclerosus.4 Experts indermatopathology later wrote in to disagree with thediagnosis of melanoma in these cases.5 The dis-Management QuandaryVulvar Nevi in ChildrenEditor: Elisabeth H. Quint, MDUniversity of Michigan Health Systems, Ann Arbor, Michigan</p><p>Discussant: Jonathan D.K. Trager, MDThe Mount Sinai Medical School, New York, NY</p><p>Case Presentation</p><p>The patient is a 3-year-old girl who was noticed tohave a small nevus on her right vulva at birth. Thiswas asymptomatic for her. She developed normallyand was followed by her local pediatrician. She met allher developmental milestones. Her mother then noticeda change in size over a 3-month period and came toour clinic. Past medical history is negative. She neverhas had any surgery. Family history revealed no skincancer; her grandmother had breast cancer.</p><p>On physical exam she is a well-appearing 3-year-old. Her general physical exam was in the normalrange. Her skin exam revealed no lesions. Her exter-nal genitalia revealed a right labial lesion 2 12 mm,adjacent to the clitoris (see Fig. 1). The lesion is flatwith sharp edges. The color is dark brown and homoge-neous. No inguinal lymphadenopathy was appreciated.</p><p>Dr.Tragers comments:</p><p>Were any prior measurements of the lesion taken bythe pediatrician or by a dermatologist? It might behelpful to know how quickly this lesion was growing.Congenital nevi normally grow as children grow andvulvar melanoma was reported to present with pruri-tus.2 Women with vulvar melanoma may also have a</p><p> 2004 North American Society for Pediatric and Adolescent GynecologyPublished by Elsevier history of melanoma.2 Of course, the lack ofsymptoms and the negative family history in this pa-tient do not by themselves determine that this isa benign lesion.</p><p>The differential diagnosis of this pigmented vulvarlesion includes a small congenital nevus and vulvarmelanosis,3 both of which are benign. The possibilitythat this lesion is a melanoma is exceedingly un-likely because of the extreme rarity of this entityin childhood.</p><p>The only relevant testing is testing of the lesion itselfand the questions are: should the lesion be biopsied andif so, should a portion of the lesion be biopsied (apunch biopsy or an incisional biopsy) or should theentire lesion be removed (an excisional biopsy)?There are multiple issues that factor into the decisionto surgically remove this lesion or not. There are noabsolute criteria which determine the need for surgicalexcision of small congenital nevi (those less than 1.5cm) in general or of small congenital vulvar nevi inparticular. Rather, each vulvar nevus, congenital oracquired, must be evaluated individually while takinginto account the following:</p><p>Has vulvar melanoma been reported in premenar-chal girls?1083-3188/04/$22.00doi:10.1016/j.jpag.2004.06.012</p><p>agreement focused on two issues: (1) vulvar nevi havehistological features, such as mild cytologic atypia of</p></li><li><p>354 Quint and Trager: Management QuandaryFig. 1. Vulvar nevus in a 3-year-old.</p><p>intraepidermal melanocytes, that in other sites couldbe interpreted as melanoma but which are con-sidered normal in nevi from vulvar skin; and (2)co-existent changes of lichen sclerosus can producehistological features in a benign vulvar nevus whichmimic melanoma. This latter issue has been expandedupon recently.6,7 While vulvar melanomas occuralmost universally in adult women (average age, 50s60s),2 it is possible that a rare case of vulvar melanomacould occur in a premenarchal girl.</p><p>What is the risk of melanoma occurring in a smallcongenital nevus?</p><p>The lifetime risk of melanoma developing in a smallcongenital nevus (defined as less than 1.5 cm) is low.It is estimated to be around 1% which is about the riskof developing melanoma in the general population.1</p><p>Does the fact that this congenital nevus is on the vulvamean it is more likely to develop into a melanoma thanif the nevus were on a non-genital site?</p><p>There is no evidence that a small congenital nevuson the vulva is at any greater risk of developing mela-noma than a small congenital nevus elsewhere onthe body.</p><p>Nonetheless, can vulvar melanoma occur in pre-ex-isting nevi?</p><p>Yes, they can. Authors of a 25-year study of malignantmelanoma of the vulva in Swedish women8 reportedthat of 123 women with Stage 1 vulvar melanoma(tumor confined to the vulva with or without satel-lite(s) within a distance of 2 cm from the primarytumor), nine were associated with pre-existing neviand that this association adversely affected prognosis.By pre-existing nevi, the authors meant the histologi-cal presence of benign appearing groups of dermalnevomelanocytic proliferations in close conjunctionto and sometimes gradually merging with a melanoma.Of note, such pre-existing nevi were exclusively foundin conjunction with melanoma on hairy skin, neverwith melanoma on glabrous skin. (This patients lesionis on glabrous skin.) What is not mentioned in theSwedish study is whether or not any of these pre-existing nevi were congenital.</p><p>Is this lesion, in fact, a melanoma now?The likelihood that this lesion is a melanoma now isextremely low. Of course, the only way to know ifthis lesion is a melanoma or not is to biopsy it.</p><p>Should I follow the lesion clinically or should I biopsythe lesion now?</p><p>Following the lesion clinically for any significantchanges is an option here, ideally with a baselinephotograph to help out. The parents, and you, mustbe comfortable with this since, after all, the parentscame to you because the lesion was changing. Themain issue with following a congenital or even anacquired vulvar nevus clinically is not that they are atany higher risk for developing a melanoma than nevielsewhere on the body, but that there may be somedifficulties with the followup itself: (1) since the lesionis in a hidden area, the patient may not be able todetect changes herself and may someday forget aboutit and not have it checked regularly, or, if thepatient seesanother dermatologist for followup, she may forgetto mention it and the dermatologist may not examinethe vulva closely; (2) vulvar nevi may be coveredwith hair, which, if the patient does not shave herpubic hair, may obscure its features; (3) it may becomeembarrassing to older girls and young women tohave this area checked regularly by a dermatologist;(4) some gynecologists may not be comfortable withfollowing vulvar nevi for significant changes; and (5)someday the child will be of an age at which vulvarmelanoma is more common (and vulvar melanomadoes carry a worse prognosis than melanoma on non-genital skin) and the question may then arise: is thisa melanoma?</p><p>As far as following congenital nevi, Ive had manyparents ask me that if Im worried enough to recom-mend examining their childs nevus once a year in-definitely, why cant I just remove the lesion andbe done with it? On the other hand, Ive had manyparents who are quite relieved to have their child avoida surgery and are happy to bring in their child foryearly skin checks. Discussion of this issue withparents is critical.</p><p>If I am going to biopsy the lesion, should I biopsy aportion of the lesion or excise the lesion in its entirety?</p><p>It is a well-accepted practice among dermatologiststhat if there is concern about a particular pigmentedlesion and that lesion is large or in an anatomicallydifficult or cosmetically sensitive area, a representa-tive biopsy of the lesion may be performed.9</p><p>In this patient, a punch biopsy (3 mm) of the lesioncould be done to determine the basic pathology of thelesion. If it turns out that this lesion is vulvar melano-sis, which is benign macular pigmentation, then subse-quent excision of the lesion would not be necessary.If the lesion is diagnosed as a benign congenital nevus,then the lesion could be followed clinically and possi-bly excised when the child was older, for reasonsstated above. If the pathology shows melanoma,clearly the lesion would be excised with appropriatemargins.There are drawbacks to performing a punchbiopsy or incisional biopsy of this lesion. First, it is</p></li><li><p>Quint and Trager: Management Quandary 355possible that there could be a sampling error, i.e., thebiopsy might miss a melanoma. Second, performinga punch biopsy or incisional biopsy will leave a scarwhich may make followup and interpretation of subse-quent clinical changes more difficult because now youwill have to follow a lesion with a scar.</p><p>A careful discussion with the parents about the limita-tions of this approach is necessary but it can be anacceptable alternative to complete excision if the par-ents were worried about putting the child through abigger procedure. Performing a complete excision ofthe lesion; however, would obviously eliminate thelimitations of the partial-lesion biopsy.</p><p>Other factors</p><p>There are several other factors which may make youmore likely to recommend removal of vulvar nevi ingeneral, even if they appear to be benign:</p><p> A history of a prior melanoma elsewhere on thepatients skin</p><p> A positive family history of melanoma in a first-degree relative</p><p> Parental anxiety</p><p>This last issuethat of parental anxietymust begiven consideration. If the girls parents are going tolose sleep over a congenital nevus on their daughtersvulva, even though the odds are overwhelmingly inthe childs favor that nothing will ever happen to thelesion, it may be best to have the lesion excised. It isa relatively simple procedure and can place parents attremendous ease.</p><p>Taking all of what I have said into account, this lesionlooks dark enough to be of concern, and has changedenough over a period of 3 months to be noted by themother, that I would recommend excision of the lesionby a gynecologist or pediatric surgeon in the operatingroom. This way the lesion would be removed onceand for all, the histopathology would be ascertained,and the difficulties inherent in followup would beavoided.</p><p>If there were concern on the parents part about thissort of procedure, I would consider doing a punchbiopsy of the lesion in the office after reviewingwith the parents the limitations of this approach. Per-forming a punch biopsy on the vulva of a 3-year-oldgirl in the office would be a challenge and would takethe most cooperative of patients and probably somepre-medication (e.g., with acetaminophen with codeine).If I did perform a punch biopsy and the pathologywere that of a benign congenital nevus, I would followthe lesion clinically. As the child got older and werebetter able to tolerate surgery, I would discuss withher and her parents the issue of removing it completelyso as to avoid the difficulties inherent in followup.</p><p>The patient was taken to the operating room andthe lesion was removed under general anesthesia. Anelliptical incision was made around the lesion and itwas removed in toto.The patient healed well and the final pathology wasa compound nevus.</p><p>Dr. Tragers comments:</p><p>The margin to take when excising a suspicious pig-mented lesion for diagnostic purposes is 1 to 1.5 mmaround the pigmented area with excision down tofascia.9 Taking a larger margin, as you would forexcision of melanoma, is neither indicated (becausethis lesion is most likely benign) nor practical(because of the anatomic location of the nevus) inthis patient.</p><p>The true incidence of vulvar nevi in premenarchalgirls is not known. Studies of nevi in children gener-ally exclude nevi in the genital area.10,11</p><p>In clinical practice, vulvar nevi in premenarchalgirls are common enough and I see them regularlyas an incidental findings. These nevi may occur onglabrous and non-glabrous skin and may be presentat birth (congenital nevi) or arise later in childhood(acquired nevi). All of the issues I have discussed comeinto play each time I see a girl with a vulvar nevus.Most of the small, common nevi that I see on thevulva of premenarchal girls are so clearly benign that Ijust follow them clinically and possibly think aboutexcision when the girls are older. When a lesion lookssuspiciousirregular, very dark, changing, symptom-aticthen I recommend removal of the lesion forhistopathological analysis.</p><p>Exciting developments in molecular biology may soonhelp us determine if a particular pigmented lesion isa melanoma or not and reduce some of the clinical andpathological uncertainties we routinely face whendealing with pigmented lesions. These developmentsinclude evaluating nevus biopsy specimens for DNAcontent, cell cycle aberrations, clonality, gene muta-tions, or the overexpression or underexpression ofspecific proteins.12</p><p>References</p><p>1. Marghoob AA, Kopf AW, Bittencourt FV: Moles presentat birth: Their medical significance. Primary Care &amp; Cancer2000; 20:1</p><p>2. Wechter ME, Gruber SB, Haefner HK, et al: Vulvar mela-noma: A report of 20 cases and review of the literature. JAm Acad Dermatol 2004; 50:554</p><p>3. Sison-Torre EQ, Ackerman AB: Melanosis of the vulva.A clinical simulator of malignant melanoma. Am J Der-matopathol 1985; 7(suppl):51</p><p>4. Egan CA, Bradley RR, Logsdon VK, et al: Vulvar mela-noma in childhood. Arch Dermatol 1997; 133:345</p><p>5. Carlson JA, Mihm MC: Vulvar nevi, lichen sclerosus etatrophicus, and vitiligo [letter]. Arch Dermatol 1997; 133:1314</p><p>6. Carlson JA, Mu XC, Slominski A, et al: Melanocytic prolif-erations associated with lichen sclerosus. Arch Dermatol2002; 138:77</p></li><li><p>356 Quint and Trager: Management Quandary</p><p>7. El Shabrawi-Caelen L, Soyer HP, Schaeppi H, et al: Geni-tal lentigines and melanocytic nevi with superimposedlichen sclerosus: a diagnostic challenge. J Am Acad Derma-tol 2004; 50:690</p><p>8. Ragnarsson-Olding BK, Nilsson BR, et al: Malignant mela-noma of the vulva in a nationwide, 25-year study of 219Swedish females. Cancer 1999; 8...</p></li></ul>