Vulvar Melanoma Associated with Lichen Sclerosus

Kostaki M, Plaka M, Champsas G, Christofidou E, Dimitrakopoulou A, Polydorou D, Stratigos A.

First Department of Dermatology-Venereology, Medical School of Athens University, Andreas Syggros Hospital

 Case Presentation:

A 59-year-old woman presented to our department with intense pruritus in the vulvar area. Clinical examination revealed the presence of slightly white shiny plaques of the vulva and an atypical pigmented lesion, which the patient was not aware of. The lesion was asymmetric, darkly pigmented, heterogeneous in color and irregular in shape.

Diagnostic work-out and treatment:

The suspicious pigmented lesion was excised under local anesthesia. Histological examination revealed the presence of an in situ melanoma arising on a dysplastic nevus. Moreover, a relatively thin epidermis and dermal hyalinization were observed in association with a band-like inflammation, suggestive of lichen sclerosus (LS). 

A wide local excision with a 5mm margin was also performed. No residual tumor was detected but slight skin alterations suggestive of LS were still present.

A local treatment with emollients and potent corticosteroids was recommended for one month for the LS.

Comments:

Vulvar melanoma is rather rare, with clinical, histologic and genetic features that differ from those of cutaneous melanoma 1,2. It affects mostly post-menopausal women but its etiology remains unknown, probably involving a genetic basis.

Lichen sclerosus is a chronic inflammatory disease of unknown origin usually affecting post-menopausal women but also reported in prepubertal girls, boys and men. It may be associated with auto-immune diseases and is the main precursor of HPV-negative squamous cell carcinoma of the vulva.

Until today only 4 cases of vulvar melanoma associated with LS in adult women have been reported and 6 cases in girls 2,3. The distinction between benign pigmented lesions and vulvar melanoma in the setting of LS is quite challenging 4.

Is there an association between LS and vulvar melanoma? Although their co-occurrence is probably rare and unrelated, the occurrence of LS may represent a pattern of immune host response to melanoma.

References:

  1. Trone JC, Guy JB, Mery B et al. Melanomas of the female genital tract: state of the art. Bull Cancer 2014;101:102-106
  2. Viola A,  Heinzelmann-Schwartz VA, Nixorf S et al. A clinicopathological review of 33 patients with vulvar melanoma identifies c-KIT as a prognostic marker. Int J Mol Med 2014;33:784-794
  3. La Spina M, Meli MC, De Pasquale R et al. Vulvar melanoma associated with lichen sclerosus in a child: Case Report and Literature Review. PediatrDermatol 2016;33:e190-194
  4. El Shabrawi-Caelen L, Soyer HP, Schaeppi H et al. Genital lentigines and melanocytic nevi with superimposed lichen sclerosus: a diagnostic challenge. J Am AcadDermatol 2004;50:690-694

 

Figure 1: Clinical image of vulvar melanoma and shiny plaques of labia majora and minora, indicative of lichen sclerosus

Figure 2: Dermoscopic image of melanoma with atypical pigment network and irregular dots. Shiny areas indicative of lichen sclerosus

Figure 3: Presence of in situ melanoma arising on a dysplastic nevus, in a background histology of lichen sclerosus (10X)

Figure 4: Dermal hyalinization and a band-like inflammatory infiltrate underneath (40X)

Figure 5: Immunochemistry with HMB-45

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