Malignant melanoma of the vulva is a rare malignant disease. Women in their 50s, 60s, and 70s are predominantly affected. The clinical presentation can be in the form of an asymptomatic pigmented lesion, or with manifestations of vaginal bleeding, discharge, local pain, itching, and urinary discomfort. The disease has the aggressive nature and poor prognosis. A detailed physical examination followed by a histopathological evaluation of the lesion is crucial for making the diagnosis.
Presentation
Malignant melanoma of the vulva often has an insidious course and carries a poor prognosis because of its high potential for spreading to distant tissues [1] [2] [3]. One of its peculiar characteristics is the absence of ultraviolet (UV) light exposure as a risk factor [2] [4]. This rare tumor (< 5% of all melanomas in women) most often presents as an asymptomatic pigmented lesion, with the principal locations being the labia majora and the clitoral region, but the labia minora, the periurethral area, and, very rarely, the introitus of the vagina are also possible locations [4] [5] [6]. The glabrous skin is much more frequently the site of malignant melanoma of the vulva compared to hairy skin [2]. When manifestations are present, vaginal bleeding is reported as the most common, whereas vaginal discharge, pruritus, pain, vaginal irritation, and a stinging sensation are other notable symptoms and signs [5] [6] [7] [8]. Weight loss may be reported in some cases [4]. Malignant melanoma of the vulva is mainly identified in elderly women in their sixth, seventh, and eighth decades of life [1] [5] [6].
Cardiovascular
- Hypertension
The patient was hypertensive, diabetic and had total abdominal hysterectomy with bilateral salpingo oophorectomy 20 years ago. [journal.sajc.org]
Associated disorders found most frequently with carcinoma of the vulva are obesity, hypertension, and chronic vulvar irritation secondary to diabetes mellitus, granulomatous venereal disease, or vulvar dystrophy. [health.am]
Hypertension, diabetes mellitus, and obesity have been found to coexist in up to 25 percent of patients, although they are not considered independent risk factors. 7 In the past, syphilis and other granulomatous diseases have been associated with vulvar [aafp.org]
There is no evidence for the association with hypertension, diabetes, or obesity that had been considered in the past. The association between chronic vulvar inflammatory conditions and invasive carcinoma is unclear. [glowm.com]
Skin
- Keratosis
Clinical differential diagnoses may include a wide range of pathologic conditions, including condyloma acuminata, basal cell carcinoma, pyogenic granuloma, lymphangioma, nevi, condyloma lata, nodular prurigo, seborrheic keratosis, granuloma inguinale, [mdedge.com]
Hi Cindy, I used Aldera for about 6 weeks for an area of actinic keratosis ..on my cheek just below the left eye. It works with your immune system and tends to make things worse before they get better. [melanoma.org]
[…] pigmented lesions, including common or atypical vulvar nevi of the genital type: have architectural disorder but lack cytologic atypia, dermal mitosis and other features of melanoma including ulceration Pigmented epidermal neoplasms, including seborrheic keratosis [pathologyoutlines.com]
Other benign pigmented lesions include lentigines, also known as freckles; melanosis, the accumulation of pigment deposits; post-inflammatory hyperpigmentation, a collection of pigment in reaction to trauma and other skin conditions; seborrheic keratosis [womensvoicesforchange.org]
- Hyperpigmentation
[…] tyrosinase but often express S100, SOX10 and p75 NGFR Melanomas, regardless of histology, may not express typical melanocytic markers ( Hum Pathol 2005;36:1016, Dermatol Online J 2009;15:7 ) Differential diagnosis Dermatofibroma : epithelial induction with hyperpigmentation [pathologyoutlines.com]
Other benign pigmented lesions include lentigines, also known as freckles; melanosis, the accumulation of pigment deposits; post-inflammatory hyperpigmentation, a collection of pigment in reaction to trauma and other skin conditions; seborrheic keratosis [womensvoicesforchange.org]
Thus, it is advisable that maculous lentiginous hyperpigmentated areas should be excised. [casereports.in]
- Hyperkeratosis
To the Editor: Angiokeratoma is a benign vascular tumor characterized by several dilated vessels in the superficial dermis accompanied by epidermal hyperplasia and hyperkeratosis. 1 Angiokeratoma of the vulva is a rare clinical finding, usually involving [mdedge.com]
Pathologists identify lichen sclerosus by hyperkeratosis, edema, loss of vascularity, and loss of rete ridges. Treatment has changed somewhat over the years. [healthieryou.com]
White lesions are caused by hyperkeratosis, whereas red ones represent hypervascularity. Gray or brown lesions are produced by melanocytic overactivity and pigment incontinence. [glowm.com]
- Skin Lesion
Non-cancerous skin conditions of the vulva - Women who have non-cancerous skin conditions of the vulva like lichen sclerosis are at an increased risk of vulvar cancer. Around 1 in 50 women with these skin lesions go on to develop vulvar cancer. [news-medical.net]
If a pigmented skin lesion is suspected of being malignant melanoma, it should be surgically removed by a dermatologist or gynecologist and examined at a pathology lab. Early recognition and removal of a malignant melanoma can be lifesaving. [bottomlineinc.com]
- Skin Disease
[…] about skin diseases See the DermNet NZ bookstore. [dermnetnz.org]
Neurologic
- Irritability
When manifestations are present, vaginal bleeding is reported as the most common, whereas vaginal discharge, pruritus, pain, vaginal irritation, and a stinging sensation are other notable symptoms and signs. [symptoma.com]
Among the factors that can predispose to developing melanoma of the vulva, we can distinguish chronic inflammatory diseases, virus infections (including HPV virus), factors causing vulva irritation and genetic susceptibility. [termedia.pl]
Vulvar cancer symptoms include vulvar itching, bleeding, vulvar irritation, increasing pain with intercourse, and new pigmentation. [swwomensoncology.com]
The cause is unknown, but may be due to a vulvar irritant callusing thickening of the skin. Pruritus is commonly found and may bring the patient to her health care provider. Diagnosis is by biopsy. [healthieryou.com]
Side Effects of Vaginal/Vulvar Radiation •Vaginal and vulva skin burning irritation or discharge •Urethra irritation or frequency •Anus burning 60. Cancer of the Vulva www.aboutcancer.com [slideshare.net]
Workup
A detailed patient history and a full physical examination are crucial steps of the workup for patients in whom malignant melanoma of the vulva is suspected. Signs and symptoms are often missed during the initial workup [4], which may significantly affect the prognosis and the efficacy of therapy. The physician must ask about the presence of vaginal-related symptoms, such as bleeding, discharge, or pruritus, whereas a detailed genital examination is necessary to identify a pigmented lesion. The exact type of tumor must be identified as soon as the lesion is detected and a biopsy with subsequent histopathological examination is vital in this regard [2] [4] [9] [10]. Typical mutations include C-KIT and NRAS genes, whereas BRAF gene mutations are rarely seen [9]. When a suspicion toward the existence of metastases exists (the urethra, vagina, perianal area, regional and distant lymph nodes, liver, and lungs are frequently involved locations), imaging studies need to be conducted in order to determine the extent of tumor spread [4] [7]. Positron emission tomography-computed tomography (PET-CT) is regarded as the optimal study for evaluating metastatic malignant melanoma of the vulva [4].
Treatment
With a clinical three-stage system, lesion types and treatment modalities were assessed as survival factors in univariate and multivariate analyses. [ncbi.nlm.nih.gov]
The treatment is based on surgery, the extent of which depends on the stage of tumour advancement. Supplemental treatment makes use of interleukin-2, interferon alpha, dacarbazine or paclitaxel with cisplatin or carboplatin. [termedia.pl]
Prognosis
Radical vulvectomy with inguinal lymph node dissection is of uncertain value and the prognosis usually is poor. Wide local excision alone is suggested for level II melanomas, and in such instances the prognosis may be favorable. [ncbi.nlm.nih.gov]
Signs and symptoms are often missed during the initial workup, which may significantly affect the prognosis and the efficacy of therapy. [symptoma.com]
Etiology
Rosenberg SA (eds): AIDS: Etiology, Diagnosis. Treatment, and Prevention. الصفحة 190 - Nonspecific vaginitis. Diagnostic criteria and microbial and epidemiologic associations. Am J Med. الصفحة 193 - Henson D, Tarone R. [books.google.com]
TERMINOLOGY Abbreviations Malignant melanoma (MM) Definitions Malignant tumor composed of melanocytes ETIOLOGY/PATHOGENESIS Genetic Associations KIT mutation most frequent genetic alteration Melanocortin type I receptor germline mutation (rare) Familial [basicmedicalkey.com]
GYNECOLOGIC NEOPLASMS ENDOMETRIAL CANCER ¬Etiology and Pathology ¬Symptoms, Signs, and Diagnosis ¬Staging, Prognosis, and Treatment OVARIAN CANCER ¬Etiology and Pathology ¬Symptoms, Signs, and Diagnosis ¬Treatment CERVICAL CANCER ¬Pathology ¬Symptoms [health.am]
Etiologic factors Until now, the etiologic factors of mucosal melanomas have not been identified. Sun exposure, which is a well-known risk factor for cutaneous melanomas, seems to play no role in the pathogenesis of nonskin melanomas. [dovepress.com]
Although human papillomavirus (HPV) has been suspected in the etiology of squamous carcinoma of the vulva, in truth, the cause of cancer of the vulva is unknown. [csh.org.tw]
Epidemiology
Diagnostic criteria and microbial and epidemiologic associations. Am J Med. الصفحة 193 - Henson D, Tarone R. An epidemiologic study of cancer of the cervix, vagina, and vulva based on the Third National Cancer Survey in the United States. [books.google.com]
BACKGROUND: In an epidemiologic study of 219 Swedish females with vulvar melanoma, the authors previously established the incidence of this disease as 0.19 per 100,000 women, with a 3% annual decrease from 1960 to 1984 and a 5-year relative survival rate [ncbi.nlm.nih.gov]
The tumour was re... read more Mentioned in this Paper Vulva Neoplasm, Residual Examination of Vulva Epidemiology Neoplasm of Uncertain or Unknown Behavior of Urethra Malignant Neoplasm of Vulva Urethra Melanoma Benign Neoplasm of Vulva Benign Neoplasm [meta.org]
Pathophysiology
Anatomy and Pathophysiology The vulva is the exterior of the female genitalia and includes the external opening of the vagina, the labia minora and majora and the clitoris. Malignancy of the vulva affects this part of the female genitalia. [cancerwall.com]
Adenocarcinoma can arise from the Bartholin gland and present with a painful lump. [11] Causes [ edit ] Two main pathophysiological pathways are currently understood to contribute to development of vulvar cancer - human papillomavirus (HPV) infection [en.wikipedia.org]
View Article : Google Scholar : PubMed/NCBI 23 Smith YR and Haefner HK: Vulvar lichen sclerosus: pathophysiology and treatment. Am J Clin Dermatol. 5:105–125. 2004. [spandidos-publications.com]
Prevention
[…] and early detection of cervical cancer: American Cancer Society, American Society for Colposcopy and Cervical Pathology, and American Society for Clinical Pathology screening guidelines for the prevention and early detection of cervical cancer. [nccn.org]
The HPV vaccine can prevent the strains of HPV responsible for most cervical, vaginal and vulvar cancers. [hopkinsmedicine.org]
Treatment, and Prevention. الصفحة 190 - Nonspecific vaginitis. Diagnostic criteria and microbial and epidemiologic associations. Am J Med. الصفحة 193 - Henson D, Tarone R. [books.google.com]
These steps may also help prevent precancerous changes in the vulva. Treatment The treatment of vulvar cancer depends upon the type of cancer, its stage, and its location. [drugs.com]
Centers for Disease Control and Prevention. https://www.cdc.gov/std/HPV/STDFact-HPV.htm. Accessed Aug. 17, 2017. Aerts L, et al. [mayoclinic.org]
References
- Chokoeva A, Tchernev G, Wollina U. Vulvar melanoma [Article in Bulgarian] Akush Ginekol (Sofiia). 2015;54(2):56-60.
- Ragnarsson-Olding BK. Primary malignant melanoma of the vulva--an aggressive tumor for modeling the genesis of non-UV light-associated melanomas. Acta Oncol. 2004;43(5):421-435.
- Androutsopoulos G, Terzakis E, Ioannidou G, et al. Vaginal primary malignant melanoma: a rare and aggressive tumor. Case Rep Obstet Gynecol 2013;2013:137908.
- Skovsted S, Nielsen K, Blaakær J. Melanomas of the vulva and vagina. Dan Med J. 2017;64(3). pii: A5336.
- Aulmann S, Sinn HP, Penzel R, et al. Comparison of molecular abnormalities in vulvar and vaginal melanomas. Mod Pathol 2014;27:1386-1393.
- Lemańska A, Banach P, Magnowska M, Frankowski A, Nowak-Markwitz E, Spaczyński M. Vulvar melanoma with urethral invasion and bladder metastases – a case report and review of the literature. Arch Med Sci. 2015;11(1):240-252.
- Verschraegen CF, Benjapibal M, Supakarapongkul W, et al. Vulvar melanoma at the M. D. Anderson Cancer Center: 25 years later. Int J Gynecol Cancer. 2001;11:359–364
- Baiocchi G, Duprat JP, Neves RI, et al. Vulvar melanoma: report on eleven cases and review of the literature. Sao Paulo Med J. 2010;128:38–41.
- Rouzbahman M, Kamel-Reid S, Al Habeeb A, et al. Malignant Melanoma of Vulva and Vagina: A Histomorphological Review and Mutation Analysis--A Single-Center Study. J Low Genit Tract Dis. 2015;19(4):350-353.
- Frumovitz M, Etchepareborda M, Sun CC, et al. Primary malignant melanoma of the vagina. Obstet Gynecol 2010;116:1358-1365.