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NOTE:  TESTING YOUR TUMOR FOR CANCER-RELATED GENES:

Molecular/genomic testing for sarcoma-related genes may not be for everyone, but by staying informed and continuing to ask important questions about such testing availability allows you to self-advocate. Make sure that every avenue for testing your tumor tissue is explored – potentially making a difference in the cutting-edge treatment options and targeted therapies/immunotherapies that might be available to you.

The Role of Genetic Test in Soft Tissue Sarcoma Video Presentation: https://www.youtube.com/watch?v=Dmb8fyzKUgQ

HIstorical information on genomic testing for sarcomas:https://pubmed.ncbi.nlm.nih.gov/16359533/

Leiomyosarcoma Staging

How is Leiomyosarcoma Staged?

The process of finding out how far leiomyosarcoma has spread is called staging. In sarcoma staging, doctors also evaluate the appearance of the tumor under the microscope and judge how fast the cancer seems to be growing. The stage of a sarcoma is the most significant factor in determining each patient’s prognosis (the course of the disease and the chances of survival) and in selecting treatment options.The information needed to stage sarcomas includes biopsies, and imaging tests of the main tumor (usually with CT or MRI scans) other parts of the body where the cancer may have spread.When examining the biopsy sample, the pathologist (doctor who specializes in diagnosing diseases by looking at the tissue under a microscope) takes into account the number of cells that are actively dividing and how closely the cancer resembles normal tissue. He or she determines the cell type and grade and estimates how rapidly it will grow and spread.A staging system is a standard way for the cancer care team to summarize the extent of a cancer’s spread. The system often used to stage sarcomas is the TNM system of American Joint Committee on Cancer.

In soft tissue sarcomas, an additional factor, called grade (G), is also part of the tumor’s stage. The grade is based on how the sarcoma cells look under the microscope.Grade (G)The grade is a sign of how likely it is the cancer will spread. Previously, the grade of a sarcoma was only based on how normal the cells looked under the microscope (called differentiation). This was not very helpful, and under a new system (known as the French or FNCLCC system), grade is based on 3 factors:

The scores for each factor are added to determine the grade for the cancer. Higher-grade cancers tend to grow and spread faster than lower-grade cancers.

STAGING THE TUMOR ONCE IT IS FOUND:

From the NCCN Guidelines for Patients (2020)

Home

FOR PATIENTS:   https://www.nccn.org/patients/guidelines/sarcoma/files/assets/basic-html/page-1.html

Note:  Treatment Planning is important in the first steps of care.  All such planning is customized always case-specific   – it is not a “one size fits all” situation.  Being proactive in your care is important-  which means knowing as much as you can, and asking the right questions along the way.

NOTE:  For Quality of Life maintenance/rebuilding:  Discuss Integrative Medicine  & Survivorship Care Planning with your Oncologist –  the sarcoma center may have a wealth of supportive resources available in a dedicated survivorship clinic, with program resources available patients and their families.

StagePrimary Treatment OptionsFollow Up Care
Stage 1: (1A/1B grade)                         Low-Grade tumor Surgery.  Recommendations for more treatment is based on size of margin is 1 cm or less and if the fascia was cut. Clean margins around the tumor site (always the goal)Will begin when treatment is completed. Rehabilitation if needed Imaging – CT Scan, CT/PET scan Note:  CT scan for lung surveillance is best Genetic test of the tumor Regular imaging of primary tumor site Follow up appointments with blood work done
STAGE 2: (2 – 3 Grade)  Treatment options are based on whether surgery is possible. Location, size of tumor and overall health are always taken into consideration in treatment options to be considered. Primary Treatment  Options: Surgery, Surgery following radiation therapy, Radiation followed by surgeryRehabilitation if needed Regular Imaging  – CT scans, CTG/PET Scans to check for tumor metastasis:  usually 3 – 6 months for 2 – 3 years;  then every 6 months for 2 years;  then repeat every year.
STAGE 3Primary Treatment Options (Stage lllA or lllB grade 2 – 3) Surgery followed by radiation therapy /Adjuvant chemotherapy. Chemotherapy or radiation therapy followed by surgery; then radiation therapy boost with or without adjuvant chemo. Chemotherapy followed by surgery, then Radiation therapy with or without adjuvant chemotherapy.Same as Stage 2 indicated above
Stage 4 (confined)Primary Treatment Options: Treatment as indicated for stages 2 or 3  apply. Surgery to remove metastases with or without chemotherapy before or after surgery; with or without radiation therapy – such as Ablation, SBRT, embolization, other modalities; observationRehabilitation if needed Imaging tests for surveillance same cycles as indicated for Stages 2 and 3. Consider baseline and regular imaging of primary tumor site
STAGE 4 (WIDESPREAD)    Primary Treatment Options: Chemotherapy, radiation therapy /SBRT surgery.   Observation if no symptomsSame as indicated for stage 4 above

Recurrence

LocationTreatment Options
LocalTesting; Treatment based on stage (extent of disease),More treatment as listed above
IsolatedRegional disease or lymph nodes,  Regional node dissection with or without radiation therapy, with or without chemotherapy SBRT Isolated limb infusion/per- fusion plus surgery
Widespread                                                                Palliative chemotherapy, Palliative radiation therapy SBRT, ablation, embolization. Observation if no symptoms