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Malignant Melanoma of the Vulva

Malignant Melanoma of Vulva

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].

Weight Loss
  • Weight loss may be reported in some cases. Malignant melanoma of the vulva is mainly identified in elderly women in their sixth, seventh, and eighth decades of life.[symptoma.com]
  • Symptoms of recurrent cancer may include bleeding, pain in the genitals, groin, pelvis or legs, leg swelling (edema), weight loss, chronic cough and chest pain.[womenscancercenter.com]
  • Other symptoms described in the literature are weight loss and secondary anemia. 7 Small bowel is the election site of cutaneous melanomas metastasis, so the presence of a melanocytic tumor at this level should alert gastroenterologists to search for[dovepress.com]
Rigor
  • Histopathologic specimens and clinical histories of the 198 patients who qualified for this study were reanalyzed and the tumors rigorously subtyped.[ncbi.nlm.nih.gov]
Localized Pain
  • 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.[symptoma.com]
Subcutaneous Nodule
  • Clinically, sarcoma may present as a subcutaneous nodule or may be exophytic and fleshy. Prognosis is usually poor and depends on histologic type, extent of local invasion, and treatment.[health.am]
  • Stage II included tumors with a spread to the regional lymph nodes and also cutaneous or subcutaneous nodules more than 2 cm from the primary tumor but not beyond the site of the primary lymph node drainage (i.e., in‐transit metastases).[onlinelibrary.wiley.com]
Angiokeratoma
  • angiokeratoma on the vulva that was misdiagnosed as malignant melanoma.[mdedge.com]
  • […] mitosis and other features of melanoma including ulceration Pigmented epidermal neoplasms, including seborrheic keratosis, vulvar intraepithelial neoplasia, condyloma, basal cell carcinoma Postinflammatory pigmentary alteration Vascular lesions, such as angiokeratoma[pathologyoutlines.com]

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]
  • Due to the rarity of this disease, treatment recommendations do not exist.[ncbi.nlm.nih.gov]
  • Partial vulvectomy was performed, and no metastases were detected at the time of treatment. The patient has been disease free for the 12 months after treatment.[ncbi.nlm.nih.gov]
  • In many patients, less radical surgical treatment offers equal cure rates with decreased morbidity.[ncbi.nlm.nih.gov]
  • Many patients had advanced tumours and the results of surgical treatment were very poor.[ncbi.nlm.nih.gov]

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]
  • Radical surgery does not improve prognosis and is not recommended when the inguinal lymph nodes are clinically negative.[ncbi.nlm.nih.gov]
  • Patients with Clark level 2 or less and less than 1.5 mm depth of penetration had the best prognosis. Lymph node involvement was present in 25% of the patients, and there were no survivors in this group.[ncbi.nlm.nih.gov]
  • Because radical surgery did not improve long-term prognosis, wider local excision or vulvectomy seems to be the recommended surgical approach.[ncbi.nlm.nih.gov]

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 and Signs[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

  • 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]
  • 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]
  • 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]
  • Based on 1442 patients with vulvar melanomas the tumors' epidemiology, clinical manifestations, histopathology, molecular genetics, treatment strategies, and prognosis were surveyed.[ncbi.nlm.nih.gov]
  • Institutionen för onkologi-patologi / Department of Oncology-Pathology Abstract From a consecutive, nationwide series of 219 females with primary vulvar malignant melanomas diagnosed in Sweden during 1960 to 1984 and followed up until 1994, we analyzed epidemiological[openarchive.ki.se]
Sex distribution
Age distribution

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

  • 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]
  • […] 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]
  • 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]
  • Encyclopedia) Cancer - vulva (Medical Encyclopedia) Pelvic (between the hips) radiation - discharge (Medical Encyclopedia) Understanding Chemotherapy - NIH - Easy-to-Read (National Cancer Institute) Vaginal and Vulvar Cancer (Centers for Disease Control and Prevention[icdlist.com]

References

Article

  1. Chokoeva A, Tchernev G, Wollina U. Vulvar melanoma [Article in Bulgarian] Akush Ginekol (Sofiia). 2015;54(2):56-60.
  2. 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.
  3. Androutsopoulos G, Terzakis E, Ioannidou G, et al. Vaginal primary malignant melanoma: a rare and aggressive tumor. Case Rep Obstet Gynecol 2013;2013:137908.
  4. Skovsted S, Nielsen K, Blaakær J. Melanomas of the vulva and vagina. Dan Med J. 2017;64(3). pii: A5336.
  5. Aulmann S, Sinn HP, Penzel R, et al. Comparison of molecular abnormalities in vulvar and vaginal melanomas. Mod Pathol 2014;27:1386-1393.
  6. 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.
  7. 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
  8. 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.
  9. 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.
  10. Frumovitz M, Etchepareborda M, Sun CC, et al. Primary malignant melanoma of the vagina. Obstet Gynecol 2010;116:1358-1365.

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Last updated: 2019-07-11 20:10