Vaginal melanoma

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Vaginal melanoma
Specialty Oncology/gynecology

Vaginal melanoma is a rare malignancy that originates from melanocytes in the vaginal epithelium. It is also known as a melanocytic tumor or as a malignant melanoma. [1] It is aggressive and infrequently cured. The median overall survival is 16 months. [2] Vaginal melanoma accounts 5.5% of all vaginal cancers and only 1% of all melanomas diagnosed in women. [2] Vaginal melanomas are frequently diagnosed in advanced stages of the disease. The prognosis is poor and the most important risk factor is the presence of lymph node metastases. [2] [3]

Contents

Presentation

This cancer most often develops on the lowest third of the vagina. It is darkly pigmented and of an irregular T-shape, but amelanotic melanomas have been described in 7% of cases. Melanoma of the vagina can be several centimeters in size. [2] [3]

Histology

When the tissue is assessed, the histological characteristics include:[ citation needed ]

Other cancers

Other cancerous conditions arise from vaginal epithelium: [4]

Diagnosis

A biopsy should be obtained from all suspicious lesions and Immunocytochemistry can reveal positive results for S-110 protein, HMB 45 and melan A. [8] Once the diagnosis of vaginal melanoma is established, additional examinations should be performed to exclude the spread to regional lymph nodes or distant organs, as the diagnosis of vaginal melanoma is often delayed. [2] Lymph-node involvement is the most important prognostic factor. [2]

Treatment

Surgery represents the primary treatment modality. Chemotherapy may be ineffective, but checkpoint inhibitors and BRAF and MEK inhibitors have been recently tested in vaginal melanomas. [8] Less than 10% of vaginal melanomas have BRAF-mutations. [8] [9] Therefore BRAF-inhibitors play only a minor role in vaginal melanomas (unlike in skin melanomas). However, a recent study has shown that checkpoint inhibitors (inkluding CTLA-4 inhibitors and PD-1 inhibitors) are effective in the treatment of advanced vulvovaginal melanomas. [9]

Related Research Articles

<span class="mw-page-title-main">Metastasis</span> Spread of a disease inside a body

Metastasis is a pathogenic agent's spread from an initial or primary site to a different or secondary site within the host's body; the term is typically used when referring to metastasis by a cancerous tumor. The newly pathological sites, then, are metastases (mets). It is generally distinguished from cancer invasion, which is the direct extension and penetration by cancer cells into neighboring tissues.

<span class="mw-page-title-main">Bartholin's gland</span> Mucous glands located near the vaginal opening

The Bartholin's glands are two pea sized compound alveolar glands located slightly posterior and to the left and right of the opening of the vagina. They secrete mucus to lubricate the vagina.

<span class="mw-page-title-main">Esophageal cancer</span> Gastrointestinal system cancer that is located in the esophagus

Esophageal cancer is cancer arising from the esophagus—the food pipe that runs between the throat and the stomach. Symptoms often include difficulty in swallowing and weight loss. Other symptoms may include pain when swallowing, a hoarse voice, enlarged lymph nodes ("glands") around the collarbone, a dry cough, and possibly coughing up or vomiting blood.

<span class="mw-page-title-main">Endometrial cancer</span> Uterine cancer that is located in tissues lining the uterus

Endometrial cancer is a cancer that arises from the endometrium. It is the result of the abnormal growth of cells that have the ability to invade or spread to other parts of the body. The first sign is most often vaginal bleeding not associated with a menstrual period. Other symptoms include pain with urination, pain during sexual intercourse, or pelvic pain. Endometrial cancer occurs most commonly after menopause.

<span class="mw-page-title-main">Anal cancer</span> Medical condition

Anal cancer is a cancer which arises from the anus, the distal opening of the gastrointestinal tract. Symptoms may include bleeding from the anus or a lump near the anus. Other symptoms may include pain, itchiness, or discharge from the anus. A change in bowel movements may also occur.

<span class="mw-page-title-main">Melanoma</span> Cancer originating in melanocytes

Melanoma, also redundantly known as malignant melanoma, is a type of cancer that develops from the pigment-producing cells known as melanocytes. Melanomas typically occur in the skin, but may rarely occur in the mouth, intestines, or eye. In women, they most commonly occur on the legs, while in men, they most commonly occur on the back. About 25% of melanomas develop from moles. Changes in a mole that can indicate melanoma include an increase in size, irregular edges, change in color, itchiness, or skin breakdown.

<span class="mw-page-title-main">Extramammary Paget's disease</span> Medical condition

Extramammary Paget's disease (EMPD) is a rare and slow-growing malignancy which occurs within the epithelium and accounts for 6.5% of all Paget's disease. The clinical presentation of this disease is similar to the characteristics of mammary Paget's disease (MPD). However, unlike MPD, which occurs in large lactiferous ducts and then extends into the epidermis, EMPD originates in glandular regions rich in apocrine secretions outside the mammary glands. EMPD incidence is increasing by 3.2% every year, affecting hormonally-targeted tissues such as the vulva and scrotum. In women, 81.3% of EMPD cases are related to the vulva, while for men, 43.2% of the manifestations present at the scrotum.

<span class="mw-page-title-main">Vulvar cancer</span> Cancer involving the vulva

Vulvar cancer is a cancer of the vulva, the outer portion of the female genitals. It most commonly affects the labia majora. Less often, the labia minora, clitoris, or vaginal glands are affected. Symptoms include a lump, itchiness, changes in the skin, or bleeding from the vulva.

Vaginal cancer is an extraordinarily rare form of cancer that develops in the tissue of the vagina. Primary vaginal cancer originates from the vaginal tissue – most frequently squamous cell carcinoma, but primary vaginal adenocarcinoma, sarcoma, and melanoma have also been reported – while secondary vaginal cancer involves the metastasis of a cancer that originated in a different part of the body. Secondary vaginal cancer is more common. Signs of vaginal cancer may include abnormal vaginal bleeding, dysuria, tenesmus, or pelvic pain, though as many as 20% of women diagnosed with vaginal cancer are asymptomatic at the time of diagnosis. Vaginal cancer occurs more frequently in women over age 50, and the mean age of diagnosis of vaginal cancer is 60 years. It often can be cured if found and treated in early stages. Surgery alone or surgery combined with pelvic radiation is typically used to treat vaginal cancer.

Clear-cell adenocarcinoma of the vagina is a rare adenocarcinoma often linked to prenatal exposure to diethylstilbestrol (DES), a drug which was prescribed in high-risk pregnancy.

Mucosal melanoma is a rare condition characterized by a melanoma of the mucous membranes. This subtype is associated a worse prognosis than those arising from the skin. Mucosal melanomas occur in the head and neck (55%), anorectal (24%) and vulvovaginal region (18%), and in the urinary tract (3%). Based on the histopathologic and clinical features, melanomas of the vulva and vagina are often considered a separate disease entity. The prognosis of vulvovaginal melanomas is poor, especially for vaginal melanomas and has not improved over the last decades. While chemotherapy has not shown capacity to improve survival in clinical and observational studies, immune checkpoint inhibitors have been tested in mucosal melanomas and have shown promising response rates.

Neuroendocrine carcinoma of the cervix is best defined separately:Neuroendocrine: Of, relating to, or involving the interaction between the nervous system and the hormones of the endocrine glands.Carcinoma: An invasive malignant tumor derived from epithelial tissue that tends to metastasize to other areas of the body.

<span class="mw-page-title-main">Vulva</span> External genital organs of the female mammal

In humans, the vulva consists of the external female sex organs. The vulva includes the mons pubis, labia majora, labia minora, clitoris, vestibular bulbs, vulval vestibule, urinary meatus, the vaginal opening, hymen, and Bartholin's and Skene's vestibular glands. The urinary meatus is also included as it opens into the vulval vestibule. Other features of the vulva include the pudendal cleft, sebaceous glands, the urogenital triangle, and pubic hair. The vulva includes the entrance to the vagina, which leads to the uterus, and provides a double layer of protection for this by the folds of the outer and inner labia. Pelvic floor muscles support the structures of the vulva. Other muscles of the urogenital triangle also give support.

<span class="mw-page-title-main">Adenocarcinoma of the lung</span> Medical condition

Adenocarcinoma of the lung is the most common type of lung cancer, and like other forms of lung cancer, it is characterized by distinct cellular and molecular features. It is classified as one of several non-small cell lung cancers (NSCLC), to distinguish it from small cell lung cancer which has a different behavior and prognosis. Lung adenocarcinoma is further classified into several subtypes and variants. The signs and symptoms of this specific type of lung cancer are similar to other forms of lung cancer, and patients most commonly complain of persistent cough and shortness of breath.

<span class="mw-page-title-main">Nivolumab</span> Cancer drug

Nivolumab, sold under the brand name Opdivo, is a medication used to treat a number of types of cancer. This includes melanoma, lung cancer, malignant pleural mesothelioma, renal cell carcinoma, Hodgkin lymphoma, head and neck cancer, urothelial carcinoma, colon cancer, esophageal squamous cell carcinoma, liver cancer, gastric cancer, and esophageal or gastroesophageal junction (GEJ) cancer. It is used by slow injection into a vein.

Conjunctival squamous cell carcinoma and corneal intraepithelial neoplasia comprise ocular surface squamous neoplasia (OSSN). SCC is the most common malignancy of the conjunctiva in the US, with a yearly incidence of 1–2.8 per 100,000. Risk factors for the disease are exposure to sun, exposure to UVB, and light-colored skin. Other risk factors include radiation, smoking, HPV, arsenic, and exposure to polycyclic hydrocarbons.

<span class="mw-page-title-main">Bartholin gland carcinoma</span> Medical condition

Bartholin gland carcinoma is a type of cancer of the vulva arising in the Bartholin gland. It typically presents with a painless mass at one side of the vaginal opening in a female of middle-age and older, and can appear similar to a Bartholin cyst. The mass may be big or small, may be deep under skin or appear nearer the surface with overlying ulceration. Average age at presentation is 53-years.

<span class="mw-page-title-main">Vaginal epithelium</span>

The vaginal epithelium is the inner lining of the vagina consisting of multiple layers of (squamous) cells. The basal membrane provides the support for the first layer of the epithelium-the basal layer. The intermediate layers lie upon the basal layer, and the superficial layer is the outermost layer of the epithelium. Anatomists have described the epithelium as consisting of as many as 40 distinct layers. The mucus found on the epithelium is secreted by the cervix and uterus. The rugae of the epithelium create an involuted surface and result in a large surface area that covers 360 cm2. This large surface area allows the trans-epithelial absorption of some medications via the vaginal route.

Vulvar tumors are those neoplasms of the vulva. Vulvar and vaginal neoplasms make up a small percentage (3%) of female genital cancers. They can be benign or malignant. Vulvar neoplasms are divided into cystic or solid lesions and other mixed types. Vulvar cancers are those malignant neoplasms that originate from vulvar epithelium, while vulvar sarcomas develop from non-epithelial cells such as bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Epithelial and mesenchymal tissue are the origin of vulvar tumors.

References

  1. "Vulva and Vagina tumors: an overview". atlasgeneticsoncology.org.
  2. 1 2 3 4 5 6 Wohlmuth C, Wohlmuth-Wieser I, May T, Vicus D, Gien LT, Laframboise S (2019-11-29). "Malignant Melanoma of the Vulva and Vagina: A US Population-Based Study of 1863 Patients". American Journal of Clinical Dermatology. 21 (2): 285–295. doi: 10.1007/s40257-019-00487-x . ISSN   1179-1888. PMC   7125071 . PMID   31784896.
  3. 1 2 Kalampokas E, Kalampokas T, Damaskos C (January 2017). "Primary Vaginal Melanoma, A Rare and Aggressive Entity. A Case Report and Review of the Literature". In Vivo. 31 (1): 133–139. doi:10.21873/invivo.11036. PMC   5354139 . PMID   28064232.
  4. Chen L, Xiong Y, Wang H, Liang L, Shang H, Yan X (October 2014). "Malignant melanoma of the vagina: A case report and review of the literature". Oncology Letters. 8 (4): 1585–1588. doi:10.3892/ol.2014.2357. PMC   4156219 . PMID   25202372.
  5. "Vaginal Cancer Treatment". National Cancer Institute. 1980-01-01. Retrieved 2018-02-08.
  6. "About DES". Centers for Disease Control and Prevention. Retrieved February 8, 2018.
  7. "Known Health Effects for DES Daughters". Centers for Disease Control and Prevention. Retrieved February 8, 2018.
  8. 1 2 3 Wohlmuth C, Wohlmuth-Wieser I (December 2019). "Vulvar malignancies: an interdisciplinary perspective". Journal der Deutschen Dermatologischen Gesellschaft. 17 (12): 1257–1276. doi:10.1111/ddg.13995. PMC   6972795 . PMID   31829526.
  9. 1 2 Wohlmuth, Christoph; Wohlmuth-Wieser, Iris; Laframboise, Stéphane (2020-11-24). "Clinical Characteristics and Treatment Response With Checkpoint Inhibitors in Malignant Melanoma of the Vulva and Vagina". Journal of Lower Genital Tract Disease. 25 (2): 146–151. doi:10.1097/LGT.0000000000000583. ISSN   1526-0976. PMC   7984764 . PMID   33252450.