! I’m your host today, Courtney Flaherty.

, which provides oncology professionals with the resources and information they need to provide the best patient care. In both digital and print formats, 

 covers every angle of oncology practice, from new technology to treatment advances to important regulatory decisions.

In today’s episode, sponsored by Aadi Biosciences, we had the pleasure of speaking with Kristen N. Ganjoo, MD, about the evolving treatment paradigm for patients with advanced malignant perivascular epithelioid cell tumors (PEComa), as well as remaining diagnostic and treatment challenges for this disease. Dr Ganjoo is a sarcoma specialist and medical oncologist at Stanford Health Care, as well as a professor of medicine at Stanford Medicine in California.

In our exclusive interview, Dr Ganjoo discussed remaining unmet needs for patients with malignant PEComa, patient characteristics and disease factors to consider when creating a treatment plan, and ways to have productive discussions with patients about treatment-associated adverse effects in the event that nab-sirolimus (Fyarro) is selected as the optimal therapeutic route.

That’s all we have for today! Thank you for listening to this episode of 

, sponsored by Aadi Biosciences. Check back on Mondays and Thursdays for exclusive interviews with leading experts in the oncology field.

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Dr Ganjoo discusses unmet needs for patients with malignant PEComa, treatment plan considerations, and the use of nab-sirolimus in this disease. 

Ganjoo Discusses Treatment Plans, Patient Conversations, and the Role of Nab-Sirolimus in Malignant PEComa

Kristen N. Ganjoo, MD, associate professor of medicine, Stanford University Medical Center, Stanford Medicine, discusses the treatment of patients with metastatic uterine leiomyosarcoma.

For patients who have metastatic disease, there are many different classes of therapies that are available, explains Ganjoo. Within the past 5 years, several chemotherapies have been approved by the FDA. In addition, immunotherapy agents are being tested in clinical trials. Although immunotherapy has yet to show a substantial benefit for patients with uterine leiomyosarcomas, it remains a potential option, she says.

Additionally, there are targeted therapies, such as pazopanib [Votrient], which is a VEGF inhibitor approved by the FDA for the treatment of patients with sarcomas. Most patients with leiomyosarcoma will receive all these treatments at some point, adds Ganjoo, as these patients live a long time. Throughout a patient’s course of treatment, they’re likely to receive [doxorubicin] first and gemcitabine/docetaxel second, or vice versa. However, one of the biggest challenges in practice is deciding which treatment to start patients on.

Videos

Dr. Ganjoo on the Treatment of Metastatic Uterine Leiomyosarcoma

Kristen N. Ganjoo, MD, associate professor of medicine, Stanford University Medical Center, Stanford Medicine, discusses the risks of morcellation.

Uterine leiomyosarcoma mostly presents in the metastatic stage, as it is often seen in younger women who are initially diagnosed incorrectly. By the time that uterine leiomyosarcoma is discovered, the tumor has already grown enough to show a mass in the abdomen, and there is bleeding. At this point, the disease is metastatic, Ganjoo says. Many women who have uterine leiomyosarcoma are diagnosed as having fibroids, which are leiomyomas—a benign tumor of the smooth muscle. Patients who are presumed to have fibroids undergo a procedure called morcellation.

During morcellation, the uterus is sectioned into parts and then suctioned out, Ganjoo explains. If a patient has uterine leiomyosarcoma instead of fibroids, morcellation causes the cancer to spread throughout the abdomen. Of every 350 patients diagnosed with fibroids, 1 will have uterine leiomyosarcoma, says Ganjoo. Almost all patients with undiagnosed uterine leiomyosarcoma who undergo morcellation will recur.

Kristen N. Ganjoo, MD, associate professor of medicine, Stanford University Medical Center, Stanford Medicine, discusses the risks of morcellation.
 

Dr. Ganjoo Discusses the Risks of Morcellation

Kristen N. Ganjoo, MD, associate professor of medicine, Stanford University Medical Center, Stanford Medicine, discusses the treatment of patients with early-stage uterine leiomyosarcoma.

Currently, the standard of care for this patient population is surgery. In early-stage disease, there is insufficient evidence to prove that adjuvant chemotherapy would help prevent the recurrence of tumors, Ganjoo explains. There were some phase II studies of neoadjuvant chemotherapy in patients with early-stage uterine leiomyosarcoma that showed promise, but the subsequent phase III study was abandoned due to low accrual.

Chemotherapy is not recommended following surgery for patients with stage I or II disease, she adds. However, if a patient with stage II uterine leiomyosarcoma has an aggressive tumor, chemotherapy is often recommended to prevent metastatic lesions from growing. Ganjoo explains that the chemotherapy will attack any microscopic cells that are circulating post-surgery, warding off recurrence or metastatic disease.

Dr. Ganjoo on the Treatment of Early-Stage Uterine Leiomyosarcoma

Kristen N. Ganjoo, MD, associate professor of medicine, Stanford University Medical Center, Stanford Medicine, discusses therapies for patients with uterine leiomyosarcomas.

The combination of adriamycin (doxorubicin) and olaratumab (Lartruvo) is the standard frontline therapy for patients with uterine leiomyosarcomas. A lot of physicians still believe that patients should be treated with gemcitabine and docetaxel because that was the combination that was tested in clinical trials. However, when adriamycin was compared with gemcitabine and docetaxel, it showed fewer side effects, says Ganjoo. For that reason, the combination of adriamycin and olaratumab is Ganjoo’s go-to therapy.

Additionally, she notes that it is important to factor in patient comorbidities, as not everyone can receive adriamycin and olaratumab due to congestive heart failure or other side effects that may be exacerbated by the therapy. Later lines of therapy typically include trabectedin (Yondelis), pazopanib (Votrient), or dacarbazine. Most patients with uterine leiomyosarcomas will receive all of these therapies at some point in their course of treatment, Ganjoo concludes.

Dr. Ganjoo on Therapies for Patients With Uterine Leiomyosarcomas

Kristen N. Ganjoo, MD, associate professor of medicine, Stanford University Medical Center, Stanford Medicine, discusses the rarity and diagnosis of uterine sarcomas.

Uterine sarcomas are a very rare type of tumor found in the uterus, explains Ganjoo. About 5% of all carcinomas are sarcomas, which are comprised of leiomyosarcomas, undifferentiated sarcoma, and endometrial stromal sarcoma. About 5,000 cases of uterine sarcomas are diagnosed in the United States annually. The only potentially curative option is surgery, says Ganjoo. Early diagnosis is extremely important because if it becomes metastatic it is then incurable.

It is very difficult to diagnose uterine sarcomas using ultrasound or MRI, notes Ganjoo. Moreover, a biopsy can come back as non-diagnostic. Rapid growth of the tumor is generally an indication that it is a sarcoma rather than a fibroid. Therefore, if a patient gets an ultrasound and 6 months later the mass doubles in size, physicians can be confident that it is a uterine sarcoma, Ganjoo concludes.

Kristen N. Ganjoo, MD

Articles

Ganjoo Discusses Treatment Plans, Patient Conversations, and the Role of Nab-Sirolimus in Malignant PEComa

Dr. Ganjoo on the Treatment of Metastatic Uterine Leiomyosarcoma

Dr. Ganjoo Discusses the Risks of Morcellation

Dr. Ganjoo on the Treatment of Early-Stage Uterine Leiomyosarcoma

Dr. Ganjoo on Therapies for Patients With Uterine Leiomyosarcomas

Dr. Ganjoo on the Rarity of Uterine Sarcomas