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Genital Localization of Malignant Melanoma

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Genital Localization of Malignant Melanoma ginecologie Genital localization of malignant melanoma Localizări genitale ale melanomului malign 1 Elvira Brătilă , Abstract Rezumat Adelina Dan1, Genital melanomas are the second most common Melanoamele genitale sunt al doilea cel mai frecvent cancer Claudia cancers of the female external genitalia after al organelor genitale externe feminine, după carcinomul cu Mehedințu2, squamous cell carcinoma. It is a rare pathology celule scuamoase. Este o patologie rară, având în vedere că Roxana considering that vulvar, vaginal, and cervical localizările vulvare, vaginale și cervicale constituie mai puțin Bohîlțea3, melanomas represent less than 1­3% of all melanomas de 1­3% din totalul melanoamelor la femei. Printre acestea, in women. Amongst these, vulvar melanoma is the melanomul vulvar este cel mai frecvent. Scopul acestui Monica most common. The aim of this article is to present a articol este de a prezenta o colecție de date menționate în Cîrstoiu3, collection of data stated in literature about genital literatura de specialitate despre melanomul malign genital, Diana-Elena malignant melanoma since the interest in this disease deoarece interesul pentru această boală a crescut în mod Comandașu1, has significantly increased, although, until now, there semnificativ, cu toate că, până în prezent, au existat doar have been few studies made about it. It is known câteva studii efectuate cu privire la aceasta. Este cunoscut Costin that melanomas arising from urogenital mucosa faptul că melanoamele care rezultă din mucoasa urogenitală Berceanu4 have many diferrences from cutaneous melanomas, prezintă multe diferențe comparativ cu melanoamele 1. “Carol Davila” University including higher aggressiveness, worse prognosis cutanate, inclusiv agresivitate mai mare, prognostic mai prost of Medicine and Pharmacy, and, not at least, different molecular abnormalities, și, nu în ultimul rând, diferite anomalii moleculare, cum ar Department of Obstetrics and Gynecology, such as c­KIT mutations, that modify the therapeutic fi mutațiile c­KIT, care modifică managementul terapeutic. “St. Pantelimon” management. Most of the data regarding treatment Cele mai multe date cu privire la tratament sunt preluate din Emergency Clinical are assumed from cutaneous malignant melanoma, conduita în melanomul malign cutanat, dar studiile recente Hospital, Bucharest 2. “Carol Davila” University but recent studies show that care should be taken arată că trebuie avut grijă atunci când se aplică același of Medicine and Pharmacy, when applying the same conduct. Even more, the comportament. Chiar mai mult, prognosticul și tratamentul Department of Obstetrics and Gynecology, prognosis and treatment are guided by the depth of sunt ghidate mai degrabă de profunzimea tumorii, decât “Nicolae Malaxa” tumor rather than the lesion diameter, which means de diametrul acesteia, însemnând că stadializarea FIGO Emergency Clinical Hospital, Bucharest that FIGO staging used for squamos cell carcinoma folosită pentru carcinomul cu celule scuamuase nu are 3. “Carol Davila” University has no use for this type of genital neoplasm. nici o utilitate pentru acest tip de neoplasme genitale. of Medicine and Pharmacy, Keywords: genital malignant melanoma, Cuvinte-cheie: melanom malign genital, Department of Obstetrics and Gynecology, staging, treatment, review stadializare, tratament, review University Emergency Clinical Hospital, Bucharest 4. University of Medicine and Pharmacy from Craiova, (5) Department of Obstetrics Primary mucosal malignant melanoma is a rare and is in the lower one-third of the vagina , mostly on the and Gynecology, aggressive neoplasm, characterized by a higher aggres- anterior vaginal wall(6) (Figure 1). Emergency Clinical County Hospital of Craiova siveness and a worse prognosis than its cutaneous The presenting symptoms are not particular at all, counterpart(1). This pathology accounts for only 1.4% most of them being related to vaginal bleeding, the Corespondență: Dr. Diana-Elena of all melanomas, and of these, only about 20% are appearance of a vaginal tumor and vaginal discharge. Comandașu e-mail: diana.comandasu@ diagnosed in the female genital tract. The prognosis is among the worst of vaginal malig- yahoo.com Among all melanomas in women, vulvar, vaginal and nancies, with a reported 5-year survival rate ranging cervical malignant melanomas account for 1% to 3 %(2). from 10% to 20%(3). The most common site is the vulva (70%), followed On rare occasions, melanosis was seen in the cervix. by the vagina (21%) and the cervix (9%)(3). Malignant Thus, malignant melanoma may arise de novo in this melanoma of the female urethra remains rare, accoun- area. Histopathologically, it is similar to melanoma ting for approximately 0.2% of primary melanomas(4). elsewhere. The prognosis depends on the depth of Malignant melanoma is the second most common invasion into the cervical stroma(5). For propper dia- cancer of the vagina, representing 2.8% to 5% of all gnosis, immunohistochemical methods, especially for vaginal neoplasms. Vaginal malignant melanoma is the exclusion of other primary sites of melanoma, are extremely lethal, occurring most often in white women necessary(7). population. The average age of the patients is 58-years- There have been reported rare cases of malignant me- old. The most common location of these malignancies lanoma metastatic to the ovaries. 34 Anul IV • Nr. 14 (4/2016) ginecologia ro Vulvar malignant melanoma Malignant melanoma of the vulva represents the most frequent localization of melanotic lesions in the female genital tract and accounts for 2-4% of invasive vulvar cancers(5), having an estimated annual incident rate of 1 per 1,000,000 women (Figures 2 and 3). The disease can affect women of all ages, but is more common in the older population, with almost half of the patients aged 70 or older. More than 90% of malignant melanomas occur in white women(2). The histopathological types of vulvar cancer are the following(5): squamous (92%), melanoma (2-4%), basal cell (2-3%), Bartholin gland (adenocarcinoma, squamous cell, transitional cell, adenoid cystic) - 1%, metastatic (1%), verrucous (<1%), sarcoma (<1%). Clinically, most patients with vulvar malignant me- Figure 1. Vaginal melanoma (source: http://es.slideshare.net/Pamizu/pato- logas-del-aparato-reprodutor-femenino) lanoma have no symptoms except for a pigmented lesion that seems to be enlarging. Local itching and bleeding may be acused, and just a small percent have In these circumstances, the melanomas are commonly groin adenopathy(5). widely disseminated. Therefore, surgical removal is to be Pigmented vulvar lesions are present in approxi- done for palliation of abdominal or pelvic pain, bleeding, mately 1 in 10 women and include melanocytic and or preventing torsion. Malignant melanoma can rarely nonmelanocytic tumors(9). Various benign pigmented arise in a mature cystic teratoma(8). lesions including lentigo simplex, vulvar melanosis, Primary urethral malignant melanomas are often amela- acanthosis nigricans, seborrheic keratosis, and junc- notic, leading to difficulties in early clinical diagnosis and tional, compound, intradermal, or dysplastic nevi may biopsy delays. The distal urethra is most often involved. also be found in these areas. We consider pigmented The average peak incidence is 65-years-old. Symptoms lesions on the vulva suspicious if the color is blue-black, are associated with the development of a urethral mass, they present a fuzzy border, are raised or ulcerated or hematuria, local bleeding, perineal pain, dysuria, increased are larger than 1 cm(10). Vulvar nevi, melanosis and urinary frequency and urinary incontinence. Generally, melanoma are particularly challenging because of the primary urethral malignant melanomas carry a worse similarity of their clinical and/or histopathological prognosis than their cutaneous counterparts, most pro- presentation(9). Thus, tissue sampling is mandatory, and bably because of the following reasons: advanced stage immunohistochemical studies or electron microscopy at diagnosis, often presenting one decade later, richly may help to clarify the diagnosis(2). vascularized anatomic location and unique genetic mu- Lichen sclerosus is also suspected to cause vulvar ma- tational spectrum(4). lignant melanoma, although it is konown to be an in- Figure 2. Vulvar melanoma (ulcerated) - source: http://es.slideshare.net/ Figure 3. Melanocytic lesion of the vulva diagnosed as melanoma on biopsy Pamizu/patologas-del-aparato-reprodutor-femenino - source: http://es.slideshare.net/Pamizu/patologas-del-aparato-reprodutor- femenino 35 Anul IV • Nr. 14 (4/2016) ginecologie flammatory dermatosis of unknown origin that presents mas (BRAF, NRAS), indicating that these two diseases as a pruriginous whitening lesion of the skin(11), being the have a different origin(14). The identification of mutated most common precursor of HPV-negative squamous cell genes, such as c-KIT, or increased levels of c-KIT in vul- carcinoma of the vulva. var malignant melanomas seems to support the current Three histologic subtypes of vulvar malignant melano- consensus that vulvar melanomas arise de novo from the ma have been described: superficial spreading melanoma malignant transformation of a single junctional melano- (SS), nodular melanoma (NM), and acral lentiginous me- cyte in situ(15). Therefore, c-KIT expression is a valuable lanoma (AL)(3): SS tends to remain relatively superficial predictor of prognosis and survival, especially in tick (>4 early in its development;
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