The first 60 days: Retroperitoneal sarcoma
Retroperitoneal sarcomas grow silently at the back of the abdomen until they are the size of a melon. The treatment is one carefully planned operation in a sarcoma centre that removes the tumour with the neighbouring organs it touches. The STRASS trial showed radiotherapy before surgery does not help overall; a second trial is testing chemotherapy before surgery in the most aggressive types. Below, week by week, is what OnCo's record of Retroperitoneal sarcoma 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.
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: Primary, resectable, Recurrent or metastatic.
- RadiologistNamed in the standard of care for: Primary, resectable.
- SurgeonNamed in the standard of care for: Primary, resectable, High-risk dedifferentiated liposarcoma or leiomyosarcoma, Recurrent or metastatic.
- Medical oncologistNamed in the standard of care for: Primary, resectable, High-risk dedifferentiated liposarcoma or leiomyosarcoma, Recurrent or metastatic.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Primary, resectable.
- Palliative and supportive care teamNamed in the standard of care for: High-risk dedifferentiated liposarcoma or leiomyosarcoma.
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.
Single-stage complete en bloc resection with adjacent organs in a sarcoma reference centre, after core biopsy and multidisciplinary planning; preoperative radiotherapy not routine after STRASS, considered in well-differentiated and low-grade dedifferentiated liposarcoma.
Neoadjuvant chemotherapy within STRASS2 (doxorubicin-ifosfamide or doxorubicin-dacarbazine) or up-front surgery; no proven adjuvant therapy.
Repeat resection for slowly growing, unifocal recurrence; histology-driven chemotherapy (doxorubicin plus trabectedin for leiomyosarcoma, LMS-04; eribulin or trabectedin for liposarcoma); MDM2 inhibitor trials.
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 Histological subtype and grade on preoperative core biopsy, MDM2 amplification, Multifocality and growth rate at recurrence, Sarculator nomogram risk), 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 Well-differentiated liposarcoma of the retroperitoneum, Dedifferentiated liposarcoma of the retroperitoneum, Retroperitoneal leiomyosarcoma.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Primary, resectable
- For my situation (primary, resectable), which of the standard options do you recommend and why?Guideline options include: Single-stage complete en bloc resection with adjacent organs in a sarcoma reference centre, after core biopsy and multidisciplinary planning; preoperative radiotherapy not routine after STRASS, considered in well-differentiated and low-grade dedifferentiated liposarcoma.
- How do the results of STRASS (EORTC 62092) apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
High-risk dedifferentiated liposarcoma or leiomyosarcoma
- For my situation (high-risk dedifferentiated liposarcoma or leiomyosarcoma), which of the standard options do you recommend and why?Guideline options include: Neoadjuvant chemotherapy within STRASS2 (doxorubicin-ifosfamide or doxorubicin-dacarbazine) or up-front surgery; no proven adjuvant therapy.
- Am I a candidate for Doxorubicin, Ifosfamide, Dacarbazine, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Recurrent or metastatic
- For my situation (recurrent or metastatic), which of the standard options do you recommend and why?Guideline options include: Repeat resection for slowly growing, unifocal recurrence; histology-driven chemotherapy (doxorubicin plus trabectedin for leiomyosarcoma, LMS-04; eribulin or trabectedin for liposarcoma); MDM2 inhibitor trials.
- Am I a candidate for Trabectedin, Eribulin, Pazopanib, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of LMS-04 apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Any stage
- Are there clinical trials I could join, for example of MDM2 inhibitors, Eribulin, Trabectedin?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 “Liposarcoma recurs in the abdomen in most patients even after complete surgery”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Neither radiotherapy nor chemotherapy has a proven adjuvant benefit”. 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.
- Retroperitoneal sarcoma: the full pageRetroperitoneal sarcomas grow silently at the back of the abdomen until they are the size of a melon. The treatment is one carefully planned operation in a sarcoma centre that removes the tumour with the neighbouring organs it touches. The STRASS trial showed radiotherapy before surgery does not help overall; a second trial is testing chemotherapy before surgery in the most aggressive types.
- 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.
- FNCLCC grade (soft-tissue sarcoma): The FNCLCC grade is a 1-to-3 score for soft-tissue sarcomas based on how abnormal, how fast-dividing, and how much dead tissue the tumour shows; grade drives whether chemotherapy is considered.
Every term links to the glossary.