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Imitating the normal: In deep with the intricacies of sarcomas

Lurking within the connective tissues of human anatomy, sarcomas are complex cancers that are difficult to treat and diagnose.

Enter, cancer: the second leading cause of death worldwide, the cause of every one in six deaths, and the culprit behind millions of deaths every year. Born from uncontrollable cell growth, the disease has proven itself to be difficult. How does one differentiate between the cancer cell and its predecessor? How does a building block of life lose control and become its end?

Cancers are often divided into two types: benign and malignant. Benign cancers are tumors that stay in one place, while malignant cancers spread to distant sites of the body.

Of all malignant cancers, however, there is one that is particularly complex. They are found in particularly fragile pieces of the human anatomy through magnetic resonance imaging and computerized tomography scans. Showing a tinge of light on one side of the affected area and another until the cancer cells have lit up the body like a Christmas tree. Red, yellow, white. A sarcoma.

Tip of the iceberg

Sarcoma is an umbrella term for rare, malignant tumors that develop in bones and connective tissues of the body—also known as soft tissues, which hold the body together.

Dr. Kevin Elomina, a pathologist at the De La Salle University Medical Center, explains that sarcomas may differentiate into various lines such as skeletal muscles, blood vessels, and nerves. Differentiation describes how similar the affected tissues are to their normal counterparts. “[However], some sarcomas do not show any evidence of differentiation toward these previously mentioned tissues, and are called undifferentiated sarcomas,” he explains.

Due to the wide array of tissues that sarcomas may develop in, there are about a hundred different known types. Each type is expected to arise and to behave uniquely, sometimes leading to mistaken identification. To bring order to this chaos, Danielle Sacdalan, a medical oncologist in the Philippines and a doctorate medical student in the University of Toronto, explains that sarcomas are categorized by the area of the body that they affect. For instance, a “liposarcoma” is a fat sarcoma that arises in the abdominal cavity.

Despite these classifications, sarcomas are still difficult to set apart. Elomina elaborates that such a cancer is further divided by grade assignments where “grade refers to the extent by which the sarcoma resembles its normal bone or soft tissue counterpart.” High-grade sarcomas, tumors that are poorly differentiated and dissimilar to normal tissues, are aggressive and difficult to manage, while low-grade sarcomas are less likely to spread.

Differentiating the unknown

Regardless of grade, sarcomas may be similar to more common cancers called carcinomas. However, they share important differences. What sets sarcomas apart is their path of metastasis—the way they spread. Elomina posits that “carcinomas usually spread through the lymph nodes…sarcomas, on the other hand, usually spread through the blood vessels.”

As sarcoma cells become a direct participant in the body’s regular blood circulation, they spread to other organs easily. “There are a few sarcomas that are also capable of spreading [through] lymphatic fluid,” Sacdalan clarifies. This includes clear cell sarcomas—tumors in the soft tissues of extremities, such as arms and legs.

Why sarcomas travel through these paths is currently unknown; however, researchers assure that causes and differences of sarcoma types are actively being investigated.

Clearing the fog

Current research has identified a few potential risk factors that may predispose someone to sarcomas. Such a cancer may be caused by factors including genetic mutations, which may or may not be hereditary. The cause of these mutations is currently unknown.

Apart from genetic predisposition, both Elomina and Sacdalan state that some sarcomas are also associated with specific conditions such as prolonged inflammation in some areas of the body, exposure to radiation, and abnormalities in bone minerals. For example, radiotherapy may cause DNA damage in cells and may potentially cause mutations leading to cancer. Age may also play a part. Bone sarcomas usually arise in younger patients, while soft tissue sarcomas primarily affect the elderly.

The physical and beyond

With all of this said, a sarcoma’s rapid growth and subtle warning signs make it even more disturbing. In the early stages, most patients may mistake their symptoms as benign. Elomina points out that most sarcoma patients notice growing masses or lumps in their bodies, usually enlarging at a rapid pace.

Some of these lumps that arise under the skin do not cause harm, potentially furthering the patients’ hesitance for medical evaluation. But Elomina expounds that “these reach large sizes and can infiltrate and destroy the surrounding normal tissues.” A breakdown of skeletal muscles and fat in the body may also occur in response to the tumor. This may prompt pain and weakening, or loss of the bones’ function. All in all, “These can be disfiguring and life-threatening,” the oncologist sums up.

Aside from the physical effects of a sarcoma, Elomina mentions that the diagnosis, towering cost of treatment, and potential effects of treatment—such as for loss of limbs—may cause emotional distress to the patients and their families.

Heads up

Fueled by the effects of sarcomas, the investigation of treatment options is more crucial than ever. “The mainstay of treatment of sarcoma is surgery,” Elomina informs. These may range from the removal of the tumor or the entire extremity affected. When this is inadequate due to complications in the sarcoma’s primary location, radiotherapy is another option. Chemotherapy may also be performed before the surgery to reduce the size of the tumor, or after the surgery to clear leftover sarcoma cells.

On the other hand, “some sarcomas are very advanced at presentation and have invaded vital structures, which preclude surgical intervention,” Elomina reports. When these are observed, a combination of chemotherapy and radiation is used.

Sarcomas definitely embody a multitude of complications, ranging from complexities of their forms to unavailability of treatment options. The unknown is troubling, thus research is all the more important. After all, no one can promise a patient that they will get better.

However, the more researchers and doctors understand sarcomas, the better they may be at treating them.  Learning more about how they work, what they do, and what can be done, is just the first step of many. One day, perhaps patients and their families may have a better grasp of what is happening to their bodies, their diagnosis, and their treatments.

By Arianne Joy Melendres

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