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Teaching pack: Vulvar cancer

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9 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.

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  1. Teaching pack · Cancer · gynaecologic

    Vulvar cancer

    An uncommon cancer of the external genitalia with two distinct causes: HPV infection in younger women and chronic skin inflammation in older women. Surgery is the mainstay, and sentinel-node biopsy has made it far less mutilating.

    Teaching pack: Vulvar cancer · OnCo, CC BY 4.0 · not medical advice1 / 9
  2. What it is

    In two paragraphs

    Vulvar squamous cell carcinoma has two pathways: HPV-associated (usual-type VIN, p16-positive, younger patients, better prognosis) and HPV-independent (differentiated VIN arising in lichen sclerosus, p53-mutant, older patients, higher recurrence). Rarer histologies include melanoma, Bartholin gland adenocarcinoma, Paget disease and basal cell carcinoma. Nodal status is the dominant prognostic factor.

    Early disease is treated with radical local excision and sentinel lymph node biopsy (GROINSS-V I established safety for tumours <4 cm with unifocal disease, replacing inguinofemoral lymphadenectomy and its lymphoedema in most); GROINSS-V II showed radiotherapy can replace lymphadenectomy for micrometastases ≤2 mm. Locally advanced disease receives chemoradiation (cisplatin-based, GOG 205/279) to avoid exenteration. Metastatic or recurrent disease has limited options: platinum-based chemotherapy, pembrolizumab for PD-L1-positive or TMB-high disease (KEYNOTE-158), cemiplimab in trials, and, for HPV-independent p53-mutant disease, no targeted therapy.

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  3. Standard of care

    What is given today, by setting

    SettingApproachGuideline
    Early (T1, <4 cm, unifocal)Radical local excision with 1 cm margin and sentinel lymph node biopsy (GROINSS-V); radiotherapy for sentinel micrometastases ≤2 mm, lymphadenectomy for macrometastases.NCCN Category 2A
    Node-positive after surgeryAdjuvant radiotherapy to groins and pelvis (± concurrent cisplatin) for ≥2 nodes or extracapsular spread (AGO-CaRE-1 supports chemoradiation).NCCN Category 2A
    Locally advanced (T3 / fixed nodes)Definitive or neoadjuvant chemoradiation with weekly cisplatin (GOG 279: ~70% complete response), reserving exenterative surgery for residual disease.NCCN Category 2A
    Metastatic or recurrentCarboplatin-paclitaxel ± bevacizumab (by cervical analogy); pembrolizumab for PD-L1 CPS ≥1, TMB-H or MSI-H; clinical trials.NCCN Category 2A
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  4. State of the art

    Where the field stands

    • Sentinel-node biopsy and radiotherapy for micrometastases (GROINSS-V I/II) have removed lymphadenectomy morbidity for most early patients.
    • Molecular classification (HPV/p16 vs p53) is entering staging and predicts recurrence better than stage alone.
    • Chemoradiation avoids exenteration in most locally advanced disease.
    • Systemic therapy remains borrowed from cervical cancer; dedicated trials are few.
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  5. History

    How we got here

    1. 1912Basset describes radical vulvectomy with en bloc lymphadenectomy
    2. 1990Separate groin incisions replace en bloc dissection
    3. 2008GROINSS-V I: sentinel node biopsy safe in early vulvar cancer
    4. 2016HPV-independent vs HPV-associated pathways defined (WHO 2020 adopts)
    5. 2021GROINSS-V II: radiotherapy for sentinel micrometastases
    6. 2021GOG 279: cisplatin-gemcitabine chemoradiation for locally advanced disease
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  6. Pipeline

    What is coming

    • Pembrolizumab (product)
    • Cemiplimab (product)
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  7. Open problems

    What nobody has solved

    • HPV-independent p53-mutant disease recurs often and has no targeted therapy.
    • Lichen sclerosus surveillance and prevention of malignant transformation.
    • Few dedicated trials; therapy extrapolated from cervical cancer.
    • Psychosexual morbidity after treatment.
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  8. Quiz

    Check understanding

    1. What is the standard treatment for stage II-III triple-negative breast cancer today?
      Answer
      Neoadjuvant pembrolizumab with carboplatin/paclitaxel then anthracycline chemotherapy, surgery, and adjuvant pembrolizumab (KEYNOTE-522); adjuvant olaparib for germline BRCA carriers with residual disease (OlympiA); capecitabine for residual disease without BRCA.
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  9. Sources

    Read the primary sources

    • NCCN Guidelines: Vulvar Cancer: https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1476
    • GROINSS-V II (JCO 2021): https://doi.org/10.1200/JCO.21.00006
    • NCI PDQ: vulvar cancer: https://www.cancer.gov/types/vulvar/patient/vulvar-treatment-pdq
    • Wikipedia: https://en.wikipedia.org/wiki/Vulvar_cancer
    • Guideline: https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1476
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