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Vulvar cancer

What's new

Added 2023 ESGO, 2020 BGCS, and 2016 ACOG guidelines for the diagnosis and management of vulvar cancer.

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of vulvar cancer are prepared by our editorial team based on guidelines from the European Society of Gynaecological Oncology (ESGO 2023), the American College of Obstetricians and Gynecologists (ACOG 2020,2016), the British Gynaecological Cancer Society (BGCS 2020), the American Academy of Family Physicians (AAFP 2019), the Society of Obstetricians and Gynaecologists ...
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Screening and diagnosis

Indications for screening, general population: as per BGCS 2020 guidelines, insufficient evidence to recommend screening tests to prevent vulvar cancer.
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  • Indications for screening (patients with vulvar intraepithelial neoplasia)

Classification and risk stratification

Staging: as per ESGO 2023 guidelines, consider using the AJCC TNM 8th edition classification system for staging vulvar cancer. Insufficient evidence to support the use of the FIGO 2021 classification system.
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TNM classification for vulvar cancer
Tumor classification
Tx: Primary tumor cannot be assessed
T0: No evidence of primary tumor
T1a: Tumor limited to uterus, ≤ 5 cm in greatest dimension
T1b: Tumor limited to uterus, > 5 cm
T2a: Tumor involving the adnexa
T2b: Tumor involving other pelvic tissues
T3a: Tumor infiltrating abdominal tissues, 1 site
T3b: Tumor infiltrating abdominal tissues, ≥ 2 sites
T4: Tumor invading bladder or rectum
Lymph node classification
Nx: Regional lymph nodes cannot be assessed
N0: No regional lymph node metastasis
N0(i+): Isolated tumor cells in regional lymph nodes < 0.2 mm
N1: Regional lymph node metastasis present
Metastasis classification
M0: No distant metastasis
M1: Distant metastasis present
Stage cannot be fully assessed
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Diagnostic investigations

History and physical examination: as per ESGO 2023 guidelines, inspect the vulva in patients with vulvar symptoms. Obtain clinical drawings and/or photographs.
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  • Imaging for staging

  • Cytology and HPV testing

  • Screening for HIV

Diagnostic procedures

Vulvar biopsy
As per ESGO 2023 guidelines:
Perform a punch/incision biopsy to establish the diagnosis in patients with suspected vulvar cancer. Avoid performing an excision biopsy for initial diagnosis, as it may hinder further treatment planning.
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Perform a biopsy of all lesions separately with clear documentation of mapping in patients with multiple vulvar lesions.
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  • Sentinel lymph node biopsy

  • Histopathology

Medical management

General principles: as per ESGO 2023 guidelines, refer patients with vulvar cancer to a center specializing in vulvar disease and manage them with a multidisciplinary gynecological oncology team, a dedicated team of specialists experienced in the diagnosis and management of vulvar cancers.
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  • Management of locoregional disease (neoadjuvant chemotherapy)

  • Management of locoregional disease (surgical excision)

  • Management of locoregional disease (lymph node dissection, indications)

  • Management of locoregional disease (lymph node dissection, technical considerations)

  • Management of locoregional disease (adjuvant radiotherapy)

  • Management of locoregional disease (primary chemoradiotherapy)

  • Management of advanced/metastatic disease

  • Management of recurrent disease (general principles)

  • Management of recurrent disease (local recurrence)

  • Management of recurrent disease (unresectable recurrence)

  • Management of recurrent disease (regional recurrence)

  • Management of recurrent disease (distant recurrence)

  • Palliative care

Nonpharmacologic interventions

Psychosocial support: as per ESGO 2023 guidelines, offer supportive care and psychological support in all patients with vulvar cancer throughout their pathway.
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Specific circumstances

Patients with vulvar melanoma: as per BGCS 2020 guidelines, obtain combination imaging (MRI and CT) in patients with vulvar melanoma to provide information on the extent of local disease and metastatic disease. Consider obtaining PET-CT in selected cases.
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  • Patients with vulvar basal cell carcinoma

  • Patients with vulvar Paget's disease

  • Patients with Bartholin gland carcinoma

Patient education

General counseling: as per ESGO 2023 guidelines, counsel patients about the suggested diagnostic and treatment plan and potential alternatives, including the risks and benefits of all options.
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Preventative measures

Smoking cessation: as per ACOG 2016 guidelines, advise smoking cessation as it is strongly associated with vulvar high-grade squamous intraepithelial lesions (vulvar intraepithelial neoplasia usual type).
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  • HPV vaccination

  • Management of vulvar intraepithelial neoplasia

  • Management of vulvar lichen sclerosus

Follow-up and surveillance

Long-term surveillance: as per ESGO 2023 guidelines, individualize follow-up strategy in terms of intensity, duration, and procedures based on individual risk assessment.
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