Soft Tissue Sarcoma with Jonathan C. Trent, MD, PhD and Shreyaskumar R. Patel, MD: Case 1 - Episode 1
How does this patient’s leiomyosarcoma (IVC) differ from those arising at other sites (eg; GI tract)?
It is important to understand that this is a very heterogeneous group of diseases. In the subset of patients with smooth muscle tumors, you have benign tumors like leiomyoma and you have tumors that look benign but have metastatic potential, which are called metastasizing leiomyoma. Then there are tumors that do not have malignant characteristics but behave in a malignant fashion, which are called smooth muscle tumors of uncertain malignant potential. You also have leiomyosarcoma. Even before we get into the term leiomyosarcoma, it is important to recognize that this is a very heterogeneous group of tumors.
Within leiomyosarcomas, the site of origin does make a difference in terms of biologies, clinical behavior, and even their therapeutic sensitivity to different drugs. The common one arising in the uterus of the gynecologic tract of a young woman would be the more sensitive tumors to chemotherapy. You then have tumors arising from vascular structures like the one in this case, which you then have to differentiate from the ones arising in the GI tract. Tumors arising in GI tract have been renamed as GI stromal tumors. We know what the activiating mutations are, we know what drives those tumors to grow, and the therapeutic implications are therefore different.
CASE: Soft-Tissue Sarcoma (Part 1)
Rachel F is a 58-year-old school teacher from Roanoke, Virginia. Her medical history is notable for mild hypertension and total knee replacement in 2011
Follow-up CT scan in January 2014 showed progression at multiple sites; at the time of follow up, her ECOG performance status was 1, with renal and hepatic function within normal limits
In September of 2014 she returns for follow-up, unable to work with increasing fatigue and abdominal pain, and her CT scan was consistent with progressive disease
After 4 months of therapy, she presents with worsening abdominal pain and declining performance status