The first 60 days: Vaginal adenocarcinoma (including DES-associated clear cell adenocarcinoma)
Vaginal adenocarcinoma is a rare glandular form of vaginal cancer, best known through the clear cell type that struck young women whose mothers took the hormone DES in pregnancy. Unlike the common squamous form it is not caused by HPV, it is treated with surgery where possible because it often affects young women, and radiotherapy and platinum chemotherapy are used when it is advanced. Below, week by week, is what OnCo's record of Vaginal adenocarcinoma (including DES-associated clear cell adenocarcinoma) says about the first two months: the order is typical, the timing is yours to ask about. Sections appear only where the record has something to say. Orientation, not medical advice.
What happens now
Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.
Biopsy with immunohistochemistry to exclude metastasis; MRI and PET-CT staging; DES exposure history.
Ask which of these were tested and what the results were; see the report reader for what each value means.
These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.
- PathologistNamed in the standard of care for: Diagnosis.
- RadiologistNamed in the standard of care for: Diagnosis.
- SurgeonNamed in the standard of care for: Early stage, DES-exposed women.
- Medical oncologistNamed in the standard of care for: Early stage, Locally advanced disease, Recurrent or metastatic disease.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Early stage, Locally advanced disease.
- Palliative and supportive care teamNamed in the standard of care for: Early stage.
A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.
Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.
Radical vaginectomy or radical hysterectomy with lymphadenectomy, with vaginal reconstruction and ovarian preservation where appropriate; adjuvant radiotherapy for close margins or positive nodes.
External beam radiotherapy with brachytherapy, with concurrent cisplatin by extrapolation from cervical cancer.
Platinum-based chemotherapy (carboplatin and paclitaxel); checkpoint inhibitors for mismatch-repair-deficient or PD-L1-positive tumours; pelvic exenteration for isolated central recurrence.
Lifelong annual gynaecological examination with cytology of the cervix and vagina and colposcopy of adenosis.
Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example Prenatal DES exposure history, Vaginal adenosis on examination and biopsy, Immunohistochemistry to separate primary from metastatic adenocarcinoma, HPV and p16 status, FIGO stage), and what were the results?These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Recognised subtypes for this cancer include DES-associated clear cell adenocarcinoma of the vagina, Sporadic clear cell adenocarcinoma of the vagina, Endometrioid and mucinous vaginal adenocarcinoma.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Diagnosis
- For my situation (diagnosis), which of the standard options do you recommend and why?Guideline options include: Biopsy with immunohistochemistry to exclude metastasis; MRI and PET-CT staging; DES exposure history.
Early stage
- For my situation (early stage), which of the standard options do you recommend and why?Guideline options include: Radical vaginectomy or radical hysterectomy with lymphadenectomy, with vaginal reconstruction and ovarian preservation where appropriate; adjuvant radiotherapy for close margins or positive nodes.
Locally advanced disease
- For my situation (locally advanced disease), which of the standard options do you recommend and why?Guideline options include: External beam radiotherapy with brachytherapy, with concurrent cisplatin by extrapolation from cervical cancer.
- Am I a candidate for Cisplatin, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Recurrent or metastatic disease
- For my situation (recurrent or metastatic disease), which of the standard options do you recommend and why?Guideline options include: Platinum-based chemotherapy (carboplatin and paclitaxel); checkpoint inhibitors for mismatch-repair-deficient or PD-L1-positive tumours; pelvic exenteration for isolated central recurrence.
- Am I a candidate for Carboplatin, Paclitaxel / nab-paclitaxel, Pembrolizumab, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
DES-exposed women
- For my situation (des-exposed women), which of the standard options do you recommend and why?Guideline options include: Lifelong annual gynaecological examination with cytology of the cervix and vagina and colposcopy of adenosis.
Any stage
- Are there clinical trials I could join, for example of Pembrolizumab?Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Supportive care improves quality of life and helps patients complete treatment.
- I read that “No trial-based treatment exists; everything is extrapolated from cervical and ovarian cancer”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “The natural history of late recurrence in clear cell tumours means decades of follow-up”. How does that affect my plan?Open problems are where trials and second opinions matter most.
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Vaginal adenocarcinoma (including DES-associated clear cell adenocarcinoma): the full pageVaginal adenocarcinoma is a rare glandular form of vaginal cancer, best known through the clear cell type that struck young women whose mothers took the hormone DES in pregnancy. Unlike the common squamous form it is not caused by HPV, it is treated with surgery where possible because it often affects young women, and radiotherapy and platinum chemotherapy are used when it is advanced.
- One-page appointment sheetYour questions, the words you may hear, what to bring, and space for the answers. Print it.
- Treatment sequencingWhich treatment tends to follow which, line by line.
- NavigatorStandard of care for your stage, what you have tried, and trials near you.
- HPV status (HPV-positive / HPV-negative): Whether a cancer is caused by human papillomavirus.
- Lymphadenectomy (lymph node dissection): Surgically removing the lymph nodes that drain a tumour, both to stage the cancer and to clear any spread.
- Chemoradiation (chemoradiotherapy, CRT): Radiotherapy given at the same time as chemotherapy, which sensitises the cancer to radiation.
Every term links to the glossary.