Tumors of the vulva can either be benign (non-cancerous) or malignant (cancerous). There are certain tumors that are classified as atypical (uncertain or unspecified behavior) meaning that some behave in a benign manner, or some behave in a malignant manner. A majority of the vulvar tumors diagnosed via lab studies (biopsy) are benign.

The World Health Organization (WHO) is an authoritative body that compiles, prepares, and publishes histological and molecular classification of tumors, via their Blue Books, based on research inputs from experts worldwide. These reference books chiefly aid in global cancer research, provide gold standards for tumor diagnosis, and help in treating cancer patients.

According to the WHO Classification of Female Genital Tumors (2020), 5th Ed., Vol. 4, benign and malignant tumors of the vulva are classified in the following manner:

Tumors of the vulva:

• Tumors of the vulva

Epithelial tumors

• Benign squamous lesions
  • Seborrheic keratosis: It is a rare tumor that normally presents as a flesh-colored or dark-pigmented papule on the vulva. It is usually seen in adult women
  • Condyloma acuminatum: It is one of the most commonly transmitted sexual infections caused by the human papilloma virus (HPV). It manifests as a benign vulvar skin growth, and commonly occurs in sexually-active females
• Squamous cell tumors and precursors
  • Squamous intraepithelial lesions, HPV-associated, of the vulva
  • Vulvar intraepithelial neoplasia, HPV-independent
  • Squamous cell carcinoma, HPV-associated, of the vulva
  • Squamous cell carcinoma, HPV-independent, of the vulva
  • Squamous cell carcinoma NOS of the vulva: It is a rare, malignant condition affecting the skin and mucosal membranes of the vulva, generally in middle-aged and older women
  • Basal cell carcinoma: It is a slow-growing malignancy affecting the vulva that is observed in older women
• Glandular tumors and cysts - Mammary-type glandular lesions
  • Papillary hidradenoma: It is a rare, benign tumor that is observed on the vulva, commonly in middle-aged women
  • Chondroid syringoma
  • Fibroadenoma: It is an extremely rare, benign mesenchymal tumor that resembles a breast fibroadenoma. It is mostly observed in young and middle-aged women
  • Phyllodes tumor: It is a rare tumor that arises in the vulva of adult women. Based on its behavior, phyllodes tumor may be benign, malignant, or of uncertain malignancy potential
  • Adenocarcinoma of mammary gland type
• Glandular tumors and cysts - Bartholin gland lesions
  • Bartholin gland cyst: It is a painless enlargement of the Bartholin gland. The condition affects women who are in their reproductive age
  • Bartholin gland hyperplasia
  • Bartholin gland adenoma: It is a benign proliferation (tumor/adenoma) of glandular cells on the Bartholin gland. It typically affects women who are in their reproductive age
  • Bartholin gland adenomyoma: It is a benign proliferation (tumor/adenoma) of glandular and muscle cells on the Bartholin gland. It typically affects women who are in their reproductive age
  • Bartholin gland carcinomas
• Other cysts of the vulva
• Adenocarcinomas of other types
  • Paget disease: It is a very rare form of Paget’s disease that arises from a location outside the breast (extramammary site). The condition is most commonly seen in women following menopause
  • Carcinomas of sweat gland origin
  • Adenocarcinoma of intestinal type: It is an extremely rare malignancy of intestinal type that arises in the vulva. The cancer may affect a wide age range of women

Germ cell tumors

• Germ cell tumors of the vulva

Additional vulvar tumor articles (on DoveMed) that are not part of WHO Classification of Female Genital Tumors:

Adenocarcinoma of Bartholin Gland: It is a malignant tumor that typically affects middle-aged and elderly women.

Adenoid Cystic Carcinoma of Bartholin Gland: It is a very rare and unusual form of cancer. It is typically observed in middle-aged and older women.

Adenosquamous Carcinoma of Bartholin Gland: It is a malignant tumor that typically affects middle-aged and elderly women. It is a rare subtype of squamous cell carcinoma.

Aggressive Angiomyxoma of Vulva: It is a rare and benign, soft tissue tumor with subcutaneous and deep tissue involvement. It is generally present in young and slightly middle-aged women.

Alveolar Rhabdomyosarcoma of Vulva: It is an uncommon histological subtype of rhabdomyosarcoma of vulva. The malignancy is generally seen in older girls and young adult women.

Alveolar Soft Part Sarcoma of Vulva: It is an extremely infrequent, but highly malignant soft tissue tumor observed in girls and young adult women.

Angiokeratoma of Vulva: It is a benign condition that results in the formation of small papules on the vulva. The condition affects both girls and women.

Angiomyofibroblastoma of Vulva: It is a benign tumor of the soft tissues that occurs in the vulva. The tumor is mostly seen in young and middle-aged women.

Angiosarcoma of Vulva: It is an uncommon, but aggressively developing tumor that presents no signs and symptoms in its early stages. These tumors are seen in adult women.

Apocrine Cystadenoma of Vulva: It is a rare condition in which there is a dilated cyst of the apocrine sweat glands. It is an uncommon tumor that occurs on the vulva as a painless nodule. It can occur at any age in females.

Basaloid Squamous Cell Carcinoma of Vulva: It is a rare histological variant of vulvar squamous cell carcinoma. The tumor is seen in women between the ages of 30 and 60.

Benign Phyllodes Tumor of Vulva: It is a rare tumor that arises in the vulva. It may affect a wide age range of women.

Borderline Phyllodes Tumor of Vulva: It is a rare tumor of uncertain malignancy potential that arises in the vulva. It may affect a wide age range of women.

Cellular Angiofibroma of Vulva: It is a rare, benign soft tissue tumor that is seen to originate below the skin of the vulva. It is generally present in middle-aged adults.

Ciliated Cyst of Vulva: It is a very rare type of fluid-filled vulvar cyst that is mostly seen in young adult women.

Dermatofibrosarcoma Protuberans of Vulva: It is a very rare intermediate-grade malignant tumor of the subcutaneous tissue of vulvar skin. It is typically seen in middle-aged women.

Embryonal Rhabdomyosarcoma of Vulva: It is a common histological subtype of vulvar rhabdomyosarcoma. It is a rare and invasive tumor that is mostly seen in girls below age 10.

Epithelial Inclusion Cysts of Vulva: It is a type of vulvar cyst that forms due to entrapment of surrounding epithelial tissue. It is a benign cyst mostly observed in adult women.

Epithelioid Sarcoma of Vulva: It is a highly infrequent soft tissue tumor involving the epithelioid cells and connective tissues. The malignancy is observed in a wide age range of females.

Ewing Sarcoma of Vulva: It is a rare, but highly-malignant tumor that is mostly observed in young adult women.

Fibroepithelial Polyp of Vulva: It is a benign tumor that has its origin in the vulvar skin and its appearance is similar to a polyp. The tumor is commonly observed in adult women.

Granular Cell Tumor of Vulva: It is a common and ‘mostly’ benign tumor of the soft tissue that occurs in the vulva. It is observed both in adult women and young girls.

Hidradenoma Papilliferum of Vulva: It is a rare, benign tumor that is observed on the vulva, commonly in middle-aged women.

Inverted Follicular Keratosis of Vulva: It is a subtype of seborrheic keratosis that is seen in the vulva, usually in adult women.

Keratinizing Squamous Cell Carcinoma of Vulva: It is a rare histological variant of vulvar squamous cell carcinoma. It is usually seen in women between 30 and 60 years’ old.

Leiomyoma of Vulva: It is a benign smooth muscle tumor that is present in the vulva. The tumor may be found in young and middle-aged women.

Leiomyosarcoma of Vulva: It is the most common subtype of adult vulvar soft tissue sarcoma found in middle-aged women. Leiomyosarcoma is a rare form of connective tissue cancer.

Lichen Planus of Vulva: It is a benign condition that affects the vulva. It is not a sexually contracted disease. The condition is observed in young and middle-aged women.

Lichen Sclerosus of Vulva: It is the presence of white spots on the that usually does not ulcerate and bleed. The condition affects young girls and older women.

Lipoma of Vulva: It is an uncommon benign tumor of fat tissues occurring in the vulva. Young and middle-aged adult women are usually affected by this tumor type.

Liposarcoma of Vulva: It is a rare type of vulvar carcinoma affecting women of a wide age category. A liposarcoma is a rare, malignant tumor of the fat cells.

Malignant Granular Cell Tumor of Vulva: It is an uncommon malignancy of the soft tissue that occurs in the vulva, typically in middle-aged women.

Malignant Peripheral Nerve Sheath Tumor of Vulva: It is an extremely rare vulvar tumor observed in adult women. A malignant peripheral nerve sheath tumor is a type of soft tissue sarcoma.

Malignant Phyllodes Tumor of Vulva: It is a rare tumor that arises in the vulva. The tumor is mostly observed in adult women.

Mixed Tumor of Vulva: It is a rare, benign tumor that occurs beneath the skin (subcutaneously) on the vulva. It affects middle-aged to elderly women.

Mucinous Carcinoma of Vulva: It is a very rare malignant tumor. It is typically observed in middle-aged and older women.

Mucinous Cyst of Vulva: It is a type of vulvar cyst that is filled with mucin. It occurs due to blockage of vulvar gland duct and is typically seen in adult women.

Neurofibroma of Vulva: It is an uncommon benign tumor that appears as a small bump on the vulvar skin. It is observed in young and middle-aged adult women.

Nodular Hidradenoma of Vulva: It is a common, benign tumor of the sweat glands of the vulva. It is often seen in adult women.

Nodular Hyperplasia of Bartholin Gland: It is a very uncommon nodular lesion that is caused by a benign growth of the Bartholin gland. The condition may be seen in a wide age range of adult women.

Non-Keratinizing Squamous Cell Carcinoma of Vulva: It is a rare histological variant of vulvar squamous cell carcinoma. It is usually seen in women between 30 and 60 years’ old.

Pacinian Neuroma of the Vulva: It is an uncommon tumor found in women that occurs after a trauma, such as due to a surgical procedure. In this condition, painful lesions form on the vulva. The condition can affect females of any age group.

Postoperative Spindle-Cell Nodule of Vulva: It is a rare and benign tumor that forms due to an injury (tissue damage); particularly because of a surgical procedure involving the female genital tract. It is generally observed in young and middle-aged women.

Primary Diffuse Large B-Cell Lymphoma of Vulva: It is a rare, B-cell non-Hodgkin’s lymphoma that affects middle-aged and older women. It is the most common subtype of lymphoma of vulva.

Proliferating Trichilemmal Cyst of Vulva: It is a rare, hair follicle tumor of vulvar skin that is generally observed in women during middle and old age.

Prominent Sebaceous Glands of Vulva: It is a common condition in girls and women that appear as small, raised papules.

Rhabdomyoma of Vulva: It is an uncommon benign tumor of the connective tissues. It is observed in young and middle-aged women.

Rhabdomyosarcoma of Vulva: It is a very uncommon, high-grade malignancy of the vagina. It typically affects girls and women.

Sebaceous Carcinoma of Vulva: It is a very rare malignant tumor, arising from the sebaceous gland of the vulva. It is mostly seen in older women.

Sebaceous Cyst of Vulva: It is a type of benign vulvar cyst that forms when the sebaceous glands that lubricate the skin and hair get obstructed due to various reasons. It is mostly observed in women of a wide age range.

Small Cell Carcinoma of Bartholin Gland: It is a malignant tumor that typically affects middle-aged and elderly women.

Solitary Fibrous Tumor of Vulva: It is a mostly benign overgrowth arising from the mesenchymal tissue. The tumor may be present in women between 20 and 70 years of age.

Squamous Cell Carcinoma In Situ of Vulva: It is a malignant condition affecting the skin or mucosal membranes of the vulva. It usually occurs in women between the ages of 30 and 60 years.

Squamous Cell Carcinoma of Bartholin Gland: It is a malignant tumor that typically affects middle-aged and elderly women.

Superficial Angiomyxoma of Vulva: It is a benign tumor located in the skin and mucosal surface of the vulva with subcutaneous tissue involvement. It is mostly found in young adult women.

Superficial Myofibroblastoma of Vulva: It can be described as an unusual and benign, soft tissue tumor affecting both the vulva and vagina. This tumor is generally observed in adult women.

Sweat Gland Type Adenocarcinoma of Vulva: It is a rare type of carcinoma that develops from the sweat glands on the skin of the vulva. The cancer may affect a wide age range of women.

Synovial Sarcoma of Vulva: It is an extremely rare malignancy of the vulva. Most cases have been reported in young adult women.

Syringoma of Vulva: It is a benign skin tumor of a type of sweat gland (eccrine sweat gland) of the female reproductive part known as the vulva. It can occur at any age but is usually seen in young women.

Transitional Cell Carcinoma of Bartholin Gland: It is a malignant tumor that typically affects middle-aged and elderly women.

Trichoepithelioma of Vulva: It is a slow-growing, benign tumor of the hair follicles that occurs on the vulva. It is most commonly observed in females during puberty, adolescence, and early adulthood.

Verrucous Carcinoma of Vulva: It is a rare histological variant of vulvar squamous cell carcinoma. The malignancy usually affects women between the ages of 30 and 60.

Vestibular Papillomatosis of Vulva: It is a benign tumor that occurs in the vestibule of the vulva. It I generally present in adult women.

Vulvar Keratoacanthoma: It is a low-grade, malignant tumor of the hair follicles of the vulva that usually affects elderly women.

Vulvar Melanotic Macules: These are observed on or around the vulva in girls or women. Melanotic macules are dark spots that may be also observed on the genitalia.

Warty (Condylomatous) Carcinoma of Vulva: It is a rare histological variant of vulvar squamous cell carcinoma. It usually develops in young and middle-aged women.