関: vulvar cancer

WordNet

of or relating to the vulva (同)vulval
external parts of the female genitalia

PrepTutorEJDIC

(体内にできる)新生物;腫瘍(しゅよう)
陰門,外陰部

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2012/11/06 18:03:27」(JST)

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Vulvar cancer, a malignant invasive growth in the vulva, accounts for about 4% of all gynecological cancers and typically affects women in later life. It is estimated that in the United States in 2006 about 3,740 new cases will be diagnosed and about 880 women will die as a result of vulvar cancer Vulvar carcinoma is separated from vulvar intraepithelial neoplasia (VIN), a non-invasive lesion of the epithelium that can progress via carcinoma-in-situ to squamous cell cancer, and from Paget disease of the vulva.

Types

Squamous cell carcinoma

The vast majority of vulvar cancer (approximately 90%)^[1] is squamous cell carcinoma, which originates from the epidermis of the vulvar tissue. Carcinoma-in-situ is a precursor stage of squamous cell cancer prior to invading through the basement membrane. Most lesions originate in the labia, primarily the labia majora. Other areas affected are the clitoris, and fourchette, and the local glands. While the lesion is more common with advancing age, younger women who have risk factors (v.i.) may also be affected. In the elderly treatment may be complicated by the interference of other medical conditions.

Squamous lesions tend to be unifocal, growing with local extension, and spreading via the local lymph system. The lymphatic drainage of the labia proceeds to the upper vulva and mons, then to the inguinal and femoral nodes with both superficial and deep lymph nodes. The last deep femoral node is called the Cloquet’s node; spread beyond this node affects the lymph nodes of the pelvis. The tumor may also invade adjacent organs such as the vagina, urethra, and rectum and spread via their lymphatics.

A verrucous carcinoma of the vulva is a subtype of the squamous cell cancer and tend to appear as a slowly growing wart.

Melanoma

About 5% of vulvar malignancy is caused by melanoma of the vulva. Such melanoma behaves like melanoma in other locations and may affect a much younger population. Contrary to squamous carcinoma, melanoma has a high risk of metastasis.

Basal cell carcinoma

Basal cell carcinoma affects about 1-2% of vulvar cancer is a slowly growing lesion and affects the elderly. Its behavior is similar to basal cell carcinoma in other locations that is it tends to grow locally with a low potential of deep invasion or metastasis

Other lesions

Vulvar cancer can be caused by other lesions such as adenocarcinoma or sarcoma.

Signs and Symptoms

Typically a lesion is present in form of a lump or ulceration, often associated with itching, irritation, sometimes local bleeding and discharge. Also dysuria, dyspareunia and pain may be noted. Because of modesty or embarrassment, symptoms may not be heeded in a timely fashion. Melanomas tend to display the typical dark discoloration. Adenocarcinoma can arise from the Bartholin gland and results in a lump that may be quite painful.

Diagnosis

Examination of the vulva is part of the gynecologic evaluation and may reveal ulceration, a lump, or a mass. A suspicious lesion needs to undergo a biopsy that generally can be performed in an office setting under local anesthesia. Small lesion can be excised under local anesthesia. Examination of the vulva should include a thorough inspection of the perineal area, including areas around the clitoris and urethra. Palpation of the Bartholin's glands should be performed as well.^[2] Supplemental evaluation may include a chest X-ray, an IVP, cystoscopy and proctoscopy, as well as blood counts and metabolic assessment.

Differential diagnosis

Other neoplastic lesions that need to be considered in the differential diagnosis are Paget disease of the vulva and VIN. Non-neoplastic vulvar disease includes lichen sclerosus, squamous cell hyperplasia, and vulvar vestibulitis. Infectious disease lesions can be caused by a number of diseases including herpes genitalis, human papillomavirus, syphilis, chancroid, granuloma inguinale, and lymphogranuloma venereum.

Etiology

The etiology of the cancer is unclear; however, some condition such as condyloma or squamous dysplasias may have preceded the cancer. Human papillomavirus (HPV) is suspected to be a possible risk factor in the etiology of vulvar cancer. Patients infected with HIV tend to be more susceptible to vulvar malignancy. Also, smokers tend to be at higher risk.

Staging

Preclinical staging has been supplemented by anatomical staging since 1988. FIGO’s revised staging TNM classification system uses criteria of tumor size (T), involvement of lymph nodes (N), and metastasis (M). Stage I and stage II describe the early stage of the cancer that still appears to be confined to the site of origin, stage III defines less or more extensive extensions to neighboring tissue and unilateral inguinal lymph nodes, while stage IV indicates metastatic disease to inguinal nodes bilaterally or distant metastases.^[3]

Treatment

Staging and treatment are generally handled by an oncologist familiar with gynecologic cancer. The extent of the surgery is dictated by the anatomical staging.^[3] Surgery is a mainstay of therapy and usually accomplished by use of a radical vulvectomy, removal of vulvar tissue as well as the removal of lymph nodes from the inguinal and femoral areas. Complications of such surgery include wound infection, sexual dysfunction, edema and thrombosis. Sentinel lymph node (SLN) dissection may eventually replace routine groin node dissection.

Surgery is significantly more extensive when vulvar cancer has spread to adjacent organs such as urethra, vagina, and rectum. In cases of early vulvar carcinoma the surgery may be less radical and disfiguring and consist of wide excision or a simple vulvectomy.

Radiation therapy and chemotherapy are usually not a primary choice of therapy but may be used in selected cases of advanced vulvar cancer.

Prognosis

The prognosis of vulvar cancer shows overall about a 75% five year survival rate, but, of course, individually affected by many factors, notably stage and type of the lesion and age and general medical health. Five-year survival is down to about 20% when pelvic lymph nodes are involved but better than 90% for patients with stage I lesions. Thus early diagnosis is imperative.

References

^ "Vulvar Cancer - October 1, 2002 - American Family Physician". http://www.aafp.org/afp/2002/1001/p1269.html. Retrieved 2010-03-06.
^ "Vulvar Cancer". Gynecologic Neoplasms. Armenian Health Network, Health.am. 2005. http://www.health.am/cr/vulvar-cancer/. Retrieved 2007-11-08.
^ ^a ^b International Federation of Gynecologists and Obstetricians (FIGO) (2000). "Staging classification and clinical practice guidelines of gynaecologic cancers". Archived from the original on 2006-04-23. http://web.archive.org/web/20060423064053/http://www.igcs.org/guidelines/guideline_staging-booklet.pdf. Retrieved 2006-10-13.

External links

Guide by the American Cancer Society
Information from the National Cancer Institute
Vulvar Cancer. Timothy P. Canavan and Donna Cohen. Am Fam Physician 2002;66:1269-74,1276. Review article for general practitioners.
Cancerbackup site
Anne’s Site about Vulvar Cancer
Vulva Awareness Campaign Organisation
Women Conquering Cancer
Cancer.Net: Vulvar Cancer

UpToDate Contents

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1. 外陰上皮内腫瘍 vulvar intraepithelial neoplasia
2. 外陰部病変：形態に基づく鑑別診断 vulvar lesions differential diagnosis based on morphology
3. 外陰癌：臨床症状、診断、および病理 vulvar cancer clinical manifestations diagnosis and pathology
4. 外陰部の広範囲局所切除術、単純外陰部切除術、および外陰表皮切除術 vulvar wide local excision simple vulvectomy and skinning vulvectomy
5. 外陰癌：病期分類、治療、および予後 vulvar cancer staging treatment and prognosis

English Journal

Coxalgia? Call the gynecologist.

Mandato VD1, Aguzzoli L2, Cavazza A3, Abrate M2, Silvotti M4, La Sala GB5.
Journal of minimally invasive gynecology.J Minim Invasive Gynecol.2015 May-Jun;22(4):663-7. doi: 10.1016/j.jmig.2015.01.008. Epub 2015 Jan 19.
Cellular angiofibroma is a rare mesenchymal tumor that usually affects the vulvar region. It is commonly asymptomatic and treated with radical excision. We describe the first case of cellular angiofibroma located in the retroperitoneum presenting with coxalgia and laparoscopically treated. At the 6-
PMID 25614345

An unusual case of vulvar schwannoma.

Park ST1, Kim HM2, Shin MK3, Kim JW4.
World journal of surgical oncology.World J Surg Oncol.2015 Apr 8;13:139. doi: 10.1186/s12957-015-0556-z.
Schwannoma is a benign, solitary, slow-growing neoplasm of the peripheral nerve sheath. These tumors are rarely found in the external genital system, and only a few cases of vulvar schwannoma have been reported. Herein, we report a case of a vulvar schwannoma. A 37-year-old woman presented with a 3-
PMID 25879436

Spindle cell sarcoma of the vulva with myofibroblastic differentiation.

Adeleye AJ1, Palmeri N, Wang SH, Liu-Jarin X, Wright JD.
Journal of lower genital tract disease.J Low Genit Tract Dis.2015 Apr;19(2):e38-9. doi: 10.1097/LGT.0000000000000070.
OBJECTIVE: Primary vulvar sarcomas are rare lesions of the lower genital tract. We report the case of a patient with a spindle cell sarcoma of the vulva.MATERIALS AND METHODS: A 44-year-old woman presented with a painless vulvar mass. Vulvar biopsy demonstrated a spindle cell sarcoma with myofibrobl
PMID 25062403

Japanese Journal

移行上皮癌の上皮内進展との鑑別が困難であった再発外陰Paget病の1例

野首光弘,鈴木良夫,佐藤良重,林光雄,及川剛宏,松嵜理
日本臨床細胞学会雑誌 38(2), 183-186, 1999-03-22
NAID 110001225651

外陰部に発症した稀な類上皮肉腫の1例

島田賢一,石倉直敬,塚田貞夫,上野輝夫,服部邦之
Skin cancer : official organ of the Japanese Society for Skin Cancer = 皮膚悪性腫瘍研究会機関誌 13(1), 42-47, 1998-06-30
… Epithelioid sarcoma is rare neoplasm and was first recognized as distinctive entity in 1970 by Enzinger. … Only 13 cases of vulvar epithelioid sarcoma have been reported. …
NAID 10017218953