EXTRAORDINARY CASE REPORT

Amelanotic Vulvar Melanoma: A Case Report Rossella Filippetti, MD and Rossella Pitocco, MD

Abstract: A 14-year-old girl stated that she has vulvar pruritus, pain, and bleeding and underwent a complete gynecological evaluation during which a suspicious 2-cm achromic lesion on her vulva was observed. Initially, it was suspected to be a pyogenic granuloma, in relation to the patient’s age. A biopsy was taken, and a histopathological diagnosis of amelanotic mucosal melanoma was made. Pyogenic granuloma is a reactive hyperproliferation vascular response to trauma or other stimuli. It predominantly occurs in the second decade of life in young females, in relation to the vascular effects of female hormones. Primary mucosal melanoma is a rare and aggressive neoplasm, characterized by a higher aggressiveness and a worse prognosis than her cutaneous counterpart. The female genital tract is the second most common site of onset of mucosal melanoma; it represents the 3% of melanomas diagnosed in women. Key Words: amelanotic mucosal melanoma, vulvar melanoma, pyogenic granuloma (Am J Dermatopathol 2015;37:e75–e77)

INTRODUCTION Pyogenic granuloma can be defined as a reactive hyperproliferation vascular response to trauma or other stimuli.1 It predominantly occurs in the second decade of life in young females, in relation to the vascular effects of female hormones. It presents itself as a smooth or lobulated exophytic lesion, small, red erythematous papule on a pedunculated or, sometimes, sessile base, which is usually hemorrhagic and compressible.2 Clinical characteristic of pyogenic granuloma are a slow growth, no symptoms, and without pain. The surface is ulcerated and friable with a fibrinous membrane. The size varies in diameter from a few millimeters to several centimeters. Common sites involved are fingers, feet, lips, head, upper trunk, oral mucosa, and perianal area. Histologically, it appears as a circumscribed proliferation of small capillaries in a lobular pattern.3–5 Primary mucosal melanoma is a rare and aggressive neoplasm,6 characterized by a higher aggressiveness and a more serious prognosis than her cutaneous counterpart. Mucosal melanoma is often diagnosed at advanced stages in relation to the unusual anatomical locations in which it occurs and the lack of early presenting signs and symptoms: 70% of patients present a clinically localized disease.7,8 The female genital tract is the second most common site of onset of mucosal melanoma; it represents the 3% of melanomas diagnosed in From the Department of Dermatology, Azienda Ospedaliera San Camillo-Forlanini, Rome, Italy. The authors declare no conflicts of interest. Reprints: Rossella Pitocco, MD, via Colle n866, CAP 03010, Vico nel Lazio (Fr), Italy (e-mail: [email protected]). Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.

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women. Usually affecting postmenopausal women between 60 and 70 years, only 500 cases have been reported in the literature. In the most of cases, the lesions are black or gray–black in color, whereas only 6% are amelanotic. Recent data showed a positive family history of cutaneous melanoma in 15% of cases of vulvar melanoma. Most frequent locations for vulvar melanoma are the labia minora, followed by the labia majora and clitoris.

CASE REPORT A 14-year-old girl presented with a 2-cm nodular, achromic, pedunculated, soft lesion on the labia minora (Fig. 1). She reported pruritus and pain associated to bleeding in the days before our evaluation. No regional lymphadenopathy was observed at the clinical examination. No sexual exposure at any time and no history of trauma other than that caused by pruritus was noted. We excluded viral and bacterial genital infection using Gram smear and serologic tests. Dark ground illumination for Treponema pallidum was also negative. We decided to surgically excise the lesion and to sent it for histologic examination, which revealed a vulvar melanoma, IV Clark level, Breslow thickness 3 mm, characterized by epithelioid cells, lymphocytic infiltration, and vertical growth (Fig. 2). We then performed the sentinel lymph node technique obtaining a negative result.

DISCUSSION Primary mucosal melanoma is a rare and aggressive disease, accounting for 0.03% of all new cancer diagnoses. Due to the unusual anatomical locations and the lack of early presenting signs and symptoms, mucosal melanoma are very often diagnosed at advanced stages. The rich vascular and lymphatic submucosal network contributes greatly to the early development of metastases in mucosal melanoma.9 Approximately one-third of patients have nodal involvement at presentation, and the overall 5-year survival is only 25%.10 A lot of adjuvant treatments, including several chemotherapy regimens and radiotherapy, obtained no success in improving the dismal prognosis; the aggressive surgical resection is the primary

FIGURE 1. Two-centimeter nodular, achromic, pedunculated, soft lesion on the labia minora in a 14-year-old girl. www.amjdermatopathology.com |

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Filippetti and Pitocco

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tumors, pigmented dermatofibroma, and adnexal tumors.23 Nowadays, extensive local resection is recommended as the primary treatment for vulvar melanoma by gynecologists. There are no differences in recurrence or survival between women treated with excision or hemivulvectomy compared with those who underwent radical vulvectomy and lymphadenectomy.24,25 Prognostic factors in vulvar melanoma are thickness and depth of invasion, lymph node status, ulceration (it reflects a very aggressive tumor growth), satellite lesions, and the local extension of melanoma to the urethra or vagina.7,11 In conclusion, we reported a rare case of amelanotic melanoma in a young girl, originally suspected to be a pyogenic granuloma. Further studies are required to define the biologic characteristics of this disease. FIGURE 2. Histological view of vulvar melanoma characterized by a IV Clark level, Breslow thickness of 3 mm, epithelioid cells, lymphocytic infiltration, and vertical growth.

REFERENCES

modality of choice. Despite all, the prognosis remains severe and the early detection gives the best hope for a cure. Vulvar malignant melanomas are extremely rare, counting ,3% of all cancers in women, 9% of all external genital tract malignancies, and 9% of all primary vulvar malignancies. Because the vulva is covered by squamous epithelium, approximately 85% of primary vulvar malignancies are squamous cell carcinomas. Other malignancies of the vulva include malignant melanoma, carcinoma arising from the Bartholin gland, basalcell carcinoma, and soft tissue sarcoma.11 In the female genital tract, the vulva is the most common location of melanoma, accounting for 1.3% of all melanomas, followed by vaginal melanoma, which accounts for 0.3%.12 Mean age at the diagnosis of vulvar melanoma is 60–70 years; it has rarely been reported in young females, most patients were postmenopausal. This is in contrast with the cutaneous counterpart whose mean age is 35 years.12–14 The overall 5-year survival rate for vulvar melanoma ranges from 25% to 85%.15 Usual presentation includes an asymptomatic pigmented lesion or a bleeding painful mass, related to the ulceration. Less frequently, patients may report pruritus, local discomfort, and cystic lesion.12,13,16 Vulvar melanomas are often pigmented, and recent data report the lack of pigment in ,10% of cases.17 Only few previous cases of amelanotic vulvar melanomas have been reported in the recent literature.18 Labia minora and clitoris are the most frequent involved sites.19,20,21 Vulvar melanoma tends to have a local recurrence as well as distant metastases. This aggressive behavior is primary related to the advanced stage of disease at the moment of diagnosis.16 The most common site of recurrence is the groin, followed by perineum, rectum, vagina, urethra, and cervix. Distant metastases occur most often in the lung, liver, and brain.22 In case of amelanotic melanoma, the diagnosis is very hard: nodular and ulcerated lesions are often misidentified as basal cell carcinoma or Kaposi sarcoma or pyogenic granuloma. Other differential diagnosis are other vulvar pathologic lesions including Paget disease, vulvar intraepithelial neoplasia, melanocytic nevi especially dysplastic nevi, vascular

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Am J Dermatopathol  Volume 37, Number 6, June 2015 20. Ragnarsson–Olding BK, Kanter-Lewensohn LR, Lagerlof B, et al. Malignant melanoma of the vulva in a nationwide, 25 year study of 219 Swedish females: clinical observations and histopathologic features. Cancer. 1999; 86:1273–1284. 21. Isenberg A, Shackelford PD. The value of tan lines: vulvar melanoma and ultraviolet rays. Obstet Gynecol Surv. 2001;56:377–380. 22. Sugiyama VE, Chan JK, Shin JY, et al. Vulvar melanoma: a multivariable analysis of 644 patients. Obstet Gynecol. 2007; 110:296–301.

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Amelanotic Vulvar Melanoma 23. An JS, Wu LY, Li N, et al. Clinical analysis of 42 cases of primary malignant melanoma in female tract. Zhonghua Fu Chan Ke Za Zhi. 2007;42:320–324. 24. Trimble EL. Melanomas of the vulva and vagina. Oncology (Williston Park). 1996;10:1017–1023. 25. Martinez-Palones JM, Perez-Benavente MA, Gil-Moreno A, et al. Comparison of recurrence after vulvectomy and lymphadenectomy with and without sentinel node biopsy in early stage vulvar cancer. Obstet Gynecol Surv. 2007;62:240–242.

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Amelanotic vulvar melanoma: a case report.

A 14-year-old girl stated that she has vulvar pruritus, pain, and bleeding and underwent a complete gynecological evaluation during which a suspicious...
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