1. Galamba, Elizabeth Rose

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Virgil Abloh, Artistic Director of Louis Vuitton men's wear and founder of Off-White, was a self-proclaimed "maker"-a nickname that points to his multifaceted creativity. With a bachelor's degree in civil engineering and a master's in architecture, Abloh worked on side projects as a DJ and a furniture designer, rocking the music and interior design industries. Yet, it was his dream to revitalize fashion that conquered the fashion industry.

Cardiac Angiosarcoma... - Click to enlarge in new windowCardiac Angiosarcoma. Cardiac Angiosarcoma

Born in Rockville, IL, to Ghanaian immigrants, Abloh learned how to sew from his mother, who was a seamstress. Despite a lack of formal education, he pursued his career in fashion, a career that exploded when Abloh was 22 years old, after meeting Kanye West. As a part of West's creative team, Abloh found his way to Paris in 2009 to assist in West's sneaker collaboration with Louis Vuitton. This collaboration marked the beginning of Abloh's work with LVMH Moet Hennessy Louis Vuitton and lead to his July 2020 promotion, which allowed him to work across LVMH's 75 fashion brands.


A year before this promotion, before Abloh became the most powerful Black executive in fashion, he was diagnosed with a rare and aggressive cancer. For 2 years, he quietly and privately fought his battle with primary cardiac angiosarcoma (PCAS), before succumbing to the disease on November 28, 2021. He was 41 years old.


In Abloh and in many other patients, including a 30-year-old, unnamed patient who found himself less tolerable of exercise (J Am Coll Cardiol Case Rep 2021;3(6)950-953), PCAS announced its presence through exhaustion. However, exhaustion is one of many symptoms that alert the body to this cancer. Other common symptoms include dyspnea, chest pain, and heart failure; the rarer symptoms being pericardial effusion, vena cava obstruction, pulmonary embolism, and hemoptysis. Unfortunately, before many patients experience these symptoms, the tumor has already metastasized-approximately 66-89 percent of individuals. This is due, in part, to the tumor's type and its location in the heart.


Sarcoma is a broad term used to describe about 60-70 different cancer types that arise from the connective tissue in the body: muscle, bone, blood vessels, nerves, really any fibrous tissue that is not a part of organs but rather fills the gaps between them. Angiosarcoma is one cancer type that falls under this umbrella term, specifically affecting endothelial cells. It can affect blood vessels throughout the body, including the heart.


This is not to say that the entire heart is affected upon the introduction of PCAS. Rather, this cancer type specifically affects the endocardium, located below the epicardium and the myocardium. The very smooth cells of the endocardium create a streamline flow of blood through the heart without the creation of clots. Due to its close proximity to the cardiac chambers, PCAS arises in the perfect location for metastasis.


"When you have a tumor that is arising in any part of the body, it kind of has to find a way to travel through the local blood vessels, reach the heart, and then get pumped to some part of the body where it can establish its colony and start growing all over again," noted Vinod Ravi, MD, MS, MBA, an oncologist specializing in sarcomas at The University of Texas MD Anderson Cancer Center. "In angiosarcoma, it has a huge strategic advantage because it is sitting in an organ that is beating at 70/80 times a minute. That sheer agitation and shedding of cells is much more likely, and it scatters the cells across the rest of the body, including sites which get a lot of blood supply."


For this reason, lung, brain, liver, and bone metastases are common. "It can go anywhere," he said. "I have seen angiosarcoma in the heart metastasized to the gum line."


The location of PCAS not only heightens its malignancy, but it also makes it extremely difficult to treat. Radiation, for example, is an extremely useful and oftentimes successful form of cancer treatment. However, because radiation is hostile to normal cardiac muscle in addition to cancer cells because the heart is in constant motion, radiation therapy at this location adds more challenges. Heart transplantation and the subsequent use of immunosuppressive therapies is not an option in the treatment of PCAS because immunosuppressive drugs could encourage tumor recurrence and metastasis.


"We generally just start off with systemic therapy, because the likelihood of the tumor having spread from the heart to other organs is extraordinarily high," Ravi said, outlining a typical treatment plan for a patient with PCAS. "So, we start with chemotherapy. We administer chemotherapy in cycles of 21 days. We evaluate the tumor every 42 days, or every 2 cycles, or every 6 weeks. We evaluate the size, and we confirm and make sure that the tumor is continuing to respond. We continue to treat them to a point of getting to around 6 cycles of treatment, and at that particular point, we'll reevaluate the potential for taking the patient to surgery. Keep in mind that, in the evaluation after every 2 cycles of treatment, the surgeon is also involved to determine the timing of the surgery."


Support groups also play an invaluable part in the treatment of and quality of life for patients facing such a scary prognosis. These groups, ranging from the general sarcoma support groups to the more specific angiosarcoma groups, provide a lot of information to patients that remains unavailable to them online. Patients will learn what to expect, how to manage, and ways to adjust to their treatment and its side effects-knowledge that is extremely important for coping with such a rare and lethal tumor type.


"Rare diseases rely so heavily on the community to really generate interest and support in order to advance its research because this is not a high-volume problem," Ravi said. "Most funding agencies drive past this particular cancer, which is why it continues to remain fairly lethal. I'm glad that there is interest coming into the disease, and I'm hoping that our continued joint efforts across multiple academic institutions, patients, patient support groups, and industry partners continue to seek out novel treatments in this disease."


Elizabeth Rose Galamba is a contributing writer.