In October, Minneola, Florida resident and 50-year-old mother of six, Arlene Jones, noticed she was gaining weight, especially in her stomach, and often felt bloated after meals. She knew something wasn’t right but didn’t immediately think it was anything too serious. Like many women, especially during the COVID-19 pandemic, she just kept going.
Jones works nights as a certified nurse assistant in a group home and after her shift a few weeks before Thanksgiving, she felt exceptionally uncomfortable and short of breath.
“I just didn’t feel right,” Jones explained. “I said a prayer before I got in the shower that morning after work and asked God to show me a sign if I needed to go to the hospital.”
When she stepped out of the shower, Jones looked down at her feet and realized they were extremely swollen.
“That was my sign,” she said. “I threw on my clothes, called my dad and sister, and drove to the AdventHealth Winter Garden hospital.”
After an evaluation in the ER, Jones was transferred to AdventHealth’s Orlando campus.
Upon reviewing all of the diagnostic imaging, AdventHealth gynecologic oncologist Robert Holloway, MD, could see a large abdominopelvic tumor mass that extended from Jones’ pelvic veins up through her vena cava (a large vein that carries blood to the heart from other areas of the body) and into the right atrium of her heart. He diagnosed intravenous leiomyomatosis, an extremely rare condition where a smooth muscle uterine fibroid tumor extends into the veins.
“The tumor had grown up through her body like a tentacle,” Dr. Holloway explained. “In my 26-year career at AdventHealth, this was the first time I had seen this type of mass with benign leiomyomas make it all the way into the heart and cause symptoms.”
Leiomyoma, also known as fibroids, is the most common reason women have a hysterectomy in the US, accounting for over 70% of the approximately 300,000 hysterectomies performed per year in the most recent Centers for Disease Control and Prevention (CDC) data. However, there have been fewer than 300 cases of intravenous leiomyomatosis reported worldwide, with only 100 cases that extended into the right heart as in Jones’ case.
Dr. Holloway knew he needed to perform surgery to remove the tumor, and he needed to assemble the right team of vascular and cardiovascular surgeons to assist with this unique case. He called AdventHealth vascular surgeon David Varnagy, MD, and cardiothoracic surgeon Tomer Karas, MD.
The three surgeons teleconferenced, talked and texted over the course of three days to plan what was ultimately a complex, single surgery that included both an exploratory laparotomy (open abdominal surgery) and simultaneous thoracotomy (open chest surgery) with cardiopulmonary bypass so that the team could completely remove the tumor all in one surgery. Dr. Holloway first performed a radical hysterectomy, then Dr. Karas removed the atrial portion of the tumor in the heart, and finally, Dr. Varnagy removed the remaining section of the tumor from the vena cava while the patient was on cardiovascular bypass to control blood loss and maintain perfusion (the circulation of blood through the tissue and organs).
The surgery took about four and a half hours and was a success, with the surgeons able to completely remove the tumor. Fortunately for Jones, all sections of the tumor from the uterus to the right atrium were benign. In addition, because the team used the cardiac bypass and carefully timed the use of heparin (a blood thinner), she didn’t require a blood transfusion or experience a pulmonary embolism (blood clot) at any point during the procedure. Along with the surgical team’s extensive pre-operative planning and preparation, Dr. Holloway credits the success of the surgery to AdventHealth’s multi-disciplinary teams approach to care.
“This was a very rare case and procedure that would only be possible in a tertiary care center with expert teams of surgeons, anesthesiologists, nurses and technicians,” explained Dr. Holloway. “In addition to my team’s 25 years of gynecologic oncology experience, we had access to an expert cardiothoracic center with surgeons like Dr. Karas and vascular specialists like Dr. Varnagy. We also benefited from a highly specialized anesthesia team, outstanding imaging professionals and a dynamite OR staff that all worked together to ensure our complex plan was meticulously executed.”
Dr. Karas believes the team’s extensive planning was critical to achieving the successful outcome.
“We had not managed a case like this before so there was no standard approach,” he said. “The various teams had managed other malignancies invading the vena cava and atrium associated with renal cancers and sarcomas, but the presentation with a large tumor blocking atrial blood flow that resulted in congestive heart failure was a unique challenge. Our biggest concerns were the risk of a pulmonary embolism and potential blood loss so planning and communication was essential. We were fortunate to have expertise in performing complex procedures within our individual areas and had the willingness to work across them to think through each contingency and step of the surgery."
As for Jones, she had an uneventful postoperative stay in the hospital following her surgery and is now home recovering.
“I feel 100 percent better,” she recently shared. “The doctors explained everything to me and really made me feel comfortable. I tell everyone about my amazing medical team and how they saved my life.”
Like Dr. Holloway and Dr. Karas, Dr. Varnagy credits this surgical success to teamwork and planning. “We were able to combine three different specialties and achieve a phenomenal outcome that changed this patient’s life,” he shared. “It is an amazing example of multidisciplinary care, good communication and a surgical team that accepted the challenge.”
Robert Holloway, MD, FACOG, FACS, is internationally recognized for his pioneering skills in robotic surgery, post-graduate training programs and excellence in clinical research. In addition to serving as Medical Director of the Gynecologic Oncology Program at AdventHealth in Orlando, he is a founding member of the AdventHealth Global Robotics Institute. He routinely assists in the training of physicians from around the world in new surgical techniques and is a principal investigator for several clinical trials and projects aimed at advancing effective treatments for gynecologic cancers.
In his 13th year at AdventHealth, David Varnagy, MD, FACS, is a board-certified and fellowship-trained vascular surgeon who also serves as President-Elect of the Medical Staff. He specializes in treating patients with circulation disorders, arterial aneurysms, venous disease, peripheral vascular disease, carotid artery disease and more via minimally invasive interventions. He is a certified vascular technologist and leading provider of vascular surgery in Central Florida.
Tomer Z. Karas, MD, FACS, is a board-certified cardiothoracic surgeon with 14 years of experience. He received his medical degree from the University of Texas Health Science Center in Houston. Staying in Houston for his residency, Dr. Karas completed his general surgery residency at St. Joseph Hospital and his fellowship in cardiovascular and thoracic surgery at the Texas Heart Institute.
For more information or to refer a patient, call GYN Oncology Nurse Navigator Althea Buckner, MSN, APRN, AOCNP, at Call407-303-5909.