The first 60 days: Placental-site trophoblastic tumour and epithelioid trophoblastic tumour
Placental-site and epithelioid trophoblastic tumours are the rare, slow-growing forms of gestational trophoblastic neoplasia, arising from the intermediate trophoblast cells that anchor the placenta rather than the hormone-producing cells behind choriocarcinoma. They make little hCG and respond poorly to chemotherapy, so hysterectomy comes first, with platinum chemotherapy when they have spread. Below, week by week, is what OnCo's record of Placental-site trophoblastic tumour and epithelioid trophoblastic tumour 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.
Expert pathology with immunohistochemistry (human placental lactogen, p63, Ki-67) and genotyping; pelvic MRI, chest CT and FDG-PET; registration with a trophoblastic disease centre.
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, Resistant disease, Follow-up.
- SurgeonNamed in the standard of care for: Disease confined to the uterus, Metastatic disease or interval over four years, Resistant disease.
- Medical oncologistNamed in the standard of care for: Metastatic disease or interval over four years, Resistant disease.
- Transplant and cell therapy teamNamed in the standard of care for: Metastatic disease or interval over four years, Resistant disease.
- Palliative and supportive care teamNamed in the standard of care for: Disease confined to the uterus.
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.
Total hysterectomy with ovarian preservation; pelvic node sampling considered; fertility-sparing local resection only in exceptional cases with close follow-up.
- 2.Metastatic disease or interval over four yearsNCCN Guidelines: Gestational Trophoblastic Neoplasia
Multi-agent platinum-based chemotherapy (EP-EMA or TP/TE) with resection of residual disease; EMA-CO alone is insufficient.
Clinical review with hCG and imaging, because hCG alone can miss recurrence; prolonged surveillance for late relapse.
Surgical excision of chemoresistant deposits; high-dose chemotherapy with autologous stem cell rescue in selected cases; pembrolizumab in trials or on a case basis.
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 Serum hCG, Human placental lactogen by immunohistochemistryand p63, Interval since the antecedent pregnancy, Stage, depth of myometrial invasion and mitotic count, Genotyping to confirm gestational origin and identify the causative pregnancy), 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 Placental-site trophoblastic tumour confined to the uterus, Epithelioid trophoblastic tumour of the uterus or cervix, Metastatic placental-site or epithelioid trophoblastic tumour.
- 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: Expert pathology with immunohistochemistry (human placental lactogen, p63, Ki-67) and genotyping; pelvic MRI, chest CT and FDG-PET; registration with a trophoblastic disease centre.
Disease confined to the uterus
- For my situation (disease confined to the uterus), which of the standard options do you recommend and why?Guideline options include: Total hysterectomy with ovarian preservation; pelvic node sampling considered; fertility-sparing local resection only in exceptional cases with close follow-up.
Metastatic disease or interval over four years
- For my situation (metastatic disease or interval over four years), which of the standard options do you recommend and why?Guideline options include: Multi-agent platinum-based chemotherapy (EP-EMA or TP/TE) with resection of residual disease; EMA-CO alone is insufficient.
- Am I a candidate for Etoposide, Cisplatin, Methotrexate or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Resistant disease
- For my situation (resistant disease), which of the standard options do you recommend and why?Guideline options include: Surgical excision of chemoresistant deposits; high-dose chemotherapy with autologous stem cell rescue in selected cases; pembrolizumab in trials or on a case basis.
- Am I a candidate for Pembrolizumab, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Follow-up
- For my situation (follow-up), which of the standard options do you recommend and why?Guideline options include: Clinical review with hCG and imaging, because hCG alone can miss recurrence; prolonged surveillance for late relapse.
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 “Numbers are too small for any trial; all treatment rests on registry series”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “hCG is an unreliable marker, so recurrence can be missed”. 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.
- Placental-site trophoblastic tumour and epithelioid trophoblastic tumour: the full pagePlacental-site and epithelioid trophoblastic tumours are the rare, slow-growing forms of gestational trophoblastic neoplasia, arising from the intermediate trophoblast cells that anchor the placenta rather than the hormone-producing cells behind choriocarcinoma. They make little hCG and respond poorly to chemotherapy, so hysterectomy comes first, with platinum chemotherapy when they have spread.
- 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.
- Tumour markers (CEA, LDH, chromogranin, thyroglobulin): Substances released into the blood by some cancers that can be measured with a simple test, useful for tracking whether treatment is working or the cancer is coming back, but rarely good enough to diagnose or screen.
- Lymphadenectomy (lymph node dissection): Surgically removing the lymph nodes that drain a tumour, both to stage the cancer and to clear any spread.
Every term links to the glossary.