What are the other Names for this Condition? (Also known as/Synonyms)

• Vulvar Sebaceous Gland Carcinoma

What is Sebaceous Carcinoma of Vulva? (Definition/Background Information)

• Sebaceous Carcinoma of Vulva is a very rare malignant tumor, arising from the sebaceous gland of the vulva. It is mostly seen in older women
• The specific factors that may influence the development of Sebaceous Carcinoma of Vulva are currently unknown. However, in a small number of cases, Muir-Torre syndrome may be a risk factor
• The tumor may not present any symptoms initially, but later there may be pain and itching sensation (scratching), which can lead to ulceration with bleeding
• The treatment of choice of this malignant skin tumor is surgical intervention; however, there is a chance of recurrence. The prognosis is poor for metastatic Sebaceous Gland Carcinomas of the Vulva

Who gets Sebaceous Carcinoma of Vulva? (Age and Sex Distribution)

• Sebaceous Carcinoma of Vulva is usually seen in adult women, in their 60s. However, the condition may affect individuals of any age
• All racial and ethnic groups are prone to the condition
• Typically, the carcinoma is more common in certain Asian countries, such as India

What are the Risk Factors for Sebaceous Carcinoma of Vulva? (Predisposing Factors)

• The risk factors of Sebaceous Carcinoma of Vulva are unknown in a majority of cases
• However, in a minority of cases, the involvement of Muir-Torre syndrome, a genetic condition, is observed

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Sebaceous Carcinoma of Vulva? (Etiology)

• A majority of cases of Sebaceous Carcinoma of Vulva occur in a sporadic manner, without any genetic defect or abnormality
• Though, some cases occur due to genetic defects, caused by mutations in the DNA mismatched repair gene

What are the Signs and Symptoms of Sebaceous Carcinoma of Vulva?

The signs and symptoms of Sebaceous Carcinoma of Vulva include:

• They can occur as a single tumor or multiple tumors
• Sebaceous carcinomas usually occur as a painless mass on the vulva (external opening of the vagina), which may ulcerate as they become larger
• These tumors are generally slow-growing; but, sometimes they may grow rapidly
• Individuals with immunocompromised states have more aggressive tumors

Additionally, the tumor may cause the following signs and symptoms:

• Abnormal vaginal bleeding, vaginal discharge
• Pain during sex and bleeding after sexual intercourse
• Anemia (due to bleeding)
• Loss of weight, loss of appetite

How is Sebaceous Carcinoma of Vulva Diagnosed?

Sebaceous Carcinoma of Vulva may be diagnosed using the following tools:

• Physical exam and evaluation of medical history
• Examination by a dermatologist using a dermoscopy, a special device to examine the skin
• Radiological studies, such as ultrasound scan, CT and MRI scans, to determine the extent of tumor spread

Although the above modalities can be used to make an initial diagnosis, a tissue biopsy of the tumor is necessary to make a definitive diagnosis to begin treatment.

Tissue biopsy:

• A tissue biopsy of the tumor is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
• Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
• Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, and very rarely, electron microscopic studies to assist in the diagnosis

In case of metastatic tumors, the following diagnostic procedures can be used to procure the tissue sample: 

• Fine needle aspiration (FNA) biopsy of the tumor
• Core biopsy or open biopsy of the tumor

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Sebaceous Carcinoma of Vulva?

The main complications that arise from Sebaceous Carcinoma of Vulva include:

• Recurrence of the tumor, if incompletely removed
• Discomfort and pain during sex in some individuals
• The tumor can metastasize to the inguinal lymph nodes in the groin and other regions of the body
• Damage of vital nerves, blood vessels, and surrounding structures, during surgery
• Complications that arise from cancer therapy (such as due to chemotherapy or radiation therapy)

How is Sebaceous Carcinoma of Vulva Treated?

The treatment of Sebaceous Carcinoma of Vulva may include the following:

• In most cases, a wide surgical excision and removal of the entire tumor is the preferred treatment option. This may be followed by radiation therapy and/or chemotherapy
• The surgical removal of the tumor depends on the size and stage of the Vulvar Sebaceous Carcinoma. The surgical procedures performed could include:
  • Hemivulvectomy, which is partial removal of vulva
  • Total vulvectomy, which is complete removal of vulva: Sometimes, local lymph nodes may also be removed using total vulvectomy, with bilateral inguinal and femoral lymphadenectomy
  • If the tumor size is large, then partial pelvic exenteration may be performed. During this procedure, all the cancer tissue and surrounding pelvic tissue is removed to decrease the bulk of the tumor. This is often followed by radiation therapy and chemotherapy
• If the tumor has metastasized (in rare cases), then a combination of chemotherapy, radiation therapy, and invasive procedures may be used to treat the tumor
• Post-operative care is important: A minimum activity level is to be ensured until the surgical wound heals
• Follow-up care with regular screening and check-ups are important

How can Sebaceous Carcinoma of Vulva be Prevented?

• Currently, there are no definitive ways to prevent Sebaceous Carcinomas of Vulva
• Regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory for those who have already endured the tumor, due to its metastasizing potential and possibility of recurrence. Often several years of active vigilance is necessary

What is the Prognosis of Sebaceous Carcinoma of Vulva? (Outcomes/Resolutions)

• The prognosis of Sebaceous Carcinoma of Vulva depends on the tumor stage. Higher-staged carcinomas, especially those with distant metastasis, have poorer prognoses
• In cases of metastasis, its prognosis depends upon a set of several factors that include:
  • Stage of tumor: With lower-stage tumors, when the tumor is confined to site of origin, the prognosis is usually excellent with appropriate therapy. In higher-stage tumors, such as tumors with metastasis, the prognosis is poor
  • The surgical respectability of the tumor (meaning, if the tumor can be removed completely) 
  • Overall health of the individual: Individuals with overall excellent health have better prognosis compared to those with poor health
  • Age of the individual: Older individuals generally have poorer prognosis than younger individuals
  • Whether the tumor is occurring for the first time, or is a recurrent tumor. Recurring tumors have a poorer prognosis compared to tumors that do not recur
  • Response to treatment: Tumors that respond to treatment have better prognosis compared to tumors that do not respond so well to treatment
• Those tumors that have higher cell proliferation rates, as determined by pathology, after microscopic exam of the tumor, also have poorer prognoses
• The prognosis is generally better with early detection and treatment of the condition

Additional and Relevant Useful Information for Sebaceous Carcinoma of Vulva:

• Individuals with sebaceous carcinoma should be encouraged to be tested for Muir-Torre Syndrome
• Vulvar cancer is the fourth most common malignancy of the female reproductive tract. Vulvar cancers are very uncommon