Sarcomas: Rare and Complex Cancers

 

Sarcomas: Rare and Complex Cancers

Sarcomas are rare cancers that a general doctor seldom encounters, similar to other uncommon medical conditions. As a result, most doctors have limited experience detecting and treating these unusual diseases. This is why specialized centers, known as “Centers of Excellence,” have been established. These programs within healthcare facilities offer expertise in the diagnosis and treatment of specific conditions.

Experience and Expertise

Experience means having seen a significant number of patients with the same medical condition. Expertise involves continual learning and staying updated on current medical guidelines, new studies, and ongoing clinical trials.

We aim to help you understand what an accurate diagnosis of uncommon cancers like sarcomas entails and who can be involved in a complex treatment plan. We also want to help you decipher the many difficult medical terms you may encounter when faced with a sarcoma diagnosis.

In most cases, doctors must remove abnormal tissue and send it to a pathologist to determine whether their patient has cancer. This can occur with a tiny biopsy using a thin needle, during an endoscopy, or through surgery to remove a portion or all of the tumor. For some cancer types, the surgeon may also remove adjacent lymph nodes so the pathologist can determine whether they contain cancer cells.

A pathologist may also examine cells present in bodily fluids, such as urine, mucus from the lungs, abdominal or chest cavity fluid, the fluid around the brain and spinal cord, cervical smears, or bone marrow.

The Pathology Standard: Histopathology

Histopathology is what most people associate with a pathologist’s day-to-day duties. Tissue or cell specimens are cut into very thin slices, mounted on glass slides, dyed with specific chemical stains, and examined under a microscope. Staining helps identify various types of cells and tissues and provides valuable information on cell pattern and shape and tissue structure. The pathologist looks for tumor cells and checks if they infiltrate surrounding normal tissue. This description may also mention how abnormal the cells appear, known as “grading.” Tumor grade reflects this abnormality: grade 1 tumors have cells that still resemble normal cells, while grade 3 tumors have lost most of their normal cell or tissue appearance.

However, pathology departments specializing in cancer diagnosis must do much more to ensure an accurate diagnosis and appropriate treatment plan.

Use The Very Big Lupe: Electron Microscopy

is used when a greater magnification is required to detect distinctive changes inside the cells. It involves an electron beam rather than a light beam. This technique is used for diagnosis of osteosarcomas, among other.

Colored Cell Characteristics: Immunohistochemistry

allows the pathologist to distinguish between different kinds and subtypes of cancer by recognizing specific molecules within abnormal cells, termed as tumor markers. The presence of such tumor markers, which are detectable by antibodies and specific dyes or fluorescence, may also indicate how aggressive the tumor is and what type of treatment it may respond to. Hormone receptors and the so called HER2 are well-known tumor markers for breast cancer. Moreover, there are several markers that can be utilized to diagnose sarcoma.

Examine Cell by Cell: Flow Cytometry

measures cells in a sample of a patient’s blood, bone marrow, or other tissue. The cells are stained with a fluorescent dye, placed in a fluid, and then passed one at a time through a beam of light. The stained cells react to the beam and show their certain characteristics, such as size, shape, and the presence of tumor (or other) markers on the cell surface.

Genes and Cellular Weak Points: Molecular Profiling

uses a sample of tissue, blood, or other body fluid to check for certain genes, proteins, or other molecules that may be a sign of a disease or can help to distinguish different cancer types. It can also be used to check for changes in a gene or chromosome that may increase a person’s risk of developing cancer. Molecular profiling is also used to find out how well certain treatment may work.

All Chromosomes Normal? — Cytogenetic analysis

counts and examines the chromosomes of cells in a tissue sample for any abnormalities, including damaged, missing, altered, or additional chromosomes. Changes in certain chromosomes can help diagnose cancer, and determine which treatments are most promising.

Unwanted Genetic Rearrangements: FISH (fluorescence in situ hybridization)

is used to look at and count genes or chromosomes in cells and tissues. Laboratory-made pieces of DNA (the chemical material that conveys genetic information) containing fluorescent dyes are added to a sample of a patient’s cells or tissues. When these DNA fragments bind to certain genes or chromosomal regions in the sample, they glow up under a fluorescent microscope. The FISH test detects irregular cancer chromosomes or genes, making it important in accurately diagnosing cancer and selecting the best treatment option.

Imaging — The Body in Pictures

After a sarcoma or any other cancer has been detected, tests are performed to determine whether cancer cells have spread to surrounding tissue or other regions of the body. This technique is known as “staging”. The staging method yields valuable information for treatment planning.

Staging entails creating many “pictures,” or images of body parts. They may be done through a variety of methods, each of which provides a unique picture of the body. The hospital departments involved include radiology and nuclear medicine.

Oldest and Most Frequently Used: X-ray

provide pictures of inside organs or bones to identify diseases or injuries. A special machine emits a little quantity of ionizing radiation. This radiation passes through the body and is recorded on a special device to create the image.

Skeletal Details: Bone Scan

is more sensitive than an x-ray, therefore, it shows any abnormal areas of bone more clearly. It can check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in bone parts containing cancer cells and is detected by a scanner.

A Body, Virtually in Little Slices: CT scan (computerized tomography)

makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly.

Visible Sugar Hunger of Cells: PET scan (positron emission tomography scan)

is a procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and takes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.

A Combinatory Approach: PET-CT scan

combines the pictures from a PET scan and a CT scan done at the same time on the same machine. The pictures from both scans are combined to generate a more detailed picture than either test would make by itself.

Spinning Water: MRI (magnetic resonance imaging)

uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. The easy summary of the underlying physics is, that the techniques of MRI are using the average 70% water of our tissues to create pictures from inside the body with amazing clarity.

Treatment — Many Sarcoma Types, Many Treatment Plans

The numerous types of sarcomas necessitate diverse treatment approaches. Furthermore, strategies for treatment may differ depending on whether the tumor has recurred or spread throughout the body. Experts in sarcoma treatment have to consider every option and make the best choices together with the patient and their family.

If Possible, a Potential Cure: Surgery

is the most common treatment for soft tissue sarcoma. It may be the only treatment needed for small, low-grade tumors, especially in the trunk, arms, or legs. Usually, the tumor is removed along with some normal tissue around it. For tumors of the head, neck, abdomen, and trunk, as little normal tissue as possible is removed.

When it comes to sarcomas of bone tissue, the complete removal of an tumor in an arm or leg may be difficult. Limb-sparing surgery aims to save the use and appearance of the limb is saved. Tissue and bone that are removed may be replaced with a graft using tissue and bone taken from another part of the patient’s body, or with an implant such as artificial bone. In rare cases, an amputation may become necessary to save the patient’s life. However, studies have shown that survival is the same whether the first surgery done is a limb-sparing surgery or an amputation — therefore, second opinion is always advisable when an amputation may be recommended.

Optimizing Surgery Success: Neoadjuvant and Adjuvant Treatment

Radiation therapy or chemotherapy may be given before or after surgery to improve its long-term success.

When given before surgery, radiation therapy or chemotherapy will make the tumor smaller and reduce the amount of tissue that needs to be removed during surgery. Treatment given before surgery is called neoadjuvant therapy.

When given after surgery has removed all visible tumor, radiation therapy or chemotherapy aims to kill remaining cancer cells. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.

X-ray Killing of Tumor Cells: Radiation therapy

uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. The way the radiation therapy is given depends on the type and stage of the cancer being treated. There are two types of radiation therapy:

External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer.

Intensity-modulated radiation therapy (IMRT) is a type of 3-dimensional (3-D) radiation therapy that uses a computer to make pictures of the size and shape of the tumor. Thin beams of radiation of different intensities (strengths) are aimed at the tumor from many angles. This type of external radiation therapy causes less damage to nearby healthy tissue.

Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. Therefore, it is also called brachytherapy (“brachy = short distance).

Toxins against Tumor Cells: Chemotherapy

is a cancer treatment that uses chemicals to inhibit the growth of cancer cells, either by killing them or preventing them from dividing. When chemotherapy is given by mouth or injected into a vein or muscle, the chemicals enter the bloodstream and can reach cancer cells all throughout the body. Chemotherapy also has an effect on normal body cells that require rapid division for constant supply or repair, such as skin cells, hair roots, mucosa, and blood-producing cells in the bone marrow.

A Radioactive Element as a Trojan Horse: Samarium

is a radioactive medication that mimics calcium, which is required when bone cells grow and form new bone mass. Osteosarcoma or Ewing sarcoma tumor cells are extremely active. As a result, Samarium accumulates in bone locations where the tumor grows, killing the malignant cells. The radioactive action only penetrates a few millimeters, sparing healthy bone tissue.

Samarium may be utilized when surgery is not an option, or if osteosarcoma has returned following treatment.

Samarium can potentially destroy blood cells in the bone marrow. As a result, before therapy, stem cells (immature blood cells) are extracted from the patient’s blood or bone marrow and frozen for storage. After the samarium treatment is completed, the stored stem cells are thawed and administered to the patient via infusion. These reinfused stem cells develop and replenish the body’s blood cells.

Precise Identification and Attack: Targeted Therapy

is a treatment that use substances to identify and destroy certain cancer cells. There are different kinds of targeted therapy, including:

Tyrosine kinase inhibitors block (“inhibit”) signals that cancer cells need to grow and divide. Tyrosine kinase inhibitors are used to treat soft tissue sarcoma or GISTs that cannot be removed by surgery. These substances are sometimes given for as long as the tumor does not grow, and serious side effects do not occur.

Kinase inhibitors block a protein needed for cancer cells to divide. They can be used to treat recurrent or metastatic osteosarcoma.

Mammalian target of rapamycin (mTOR) inhibitors block a protein called mTOR, which may keep cancer cells from growing and prevent the growth of new blood vessels that tumors need to grow. mTOR inhibitors are used to treat recurrent osteosarcoma.

Histone methyltransferase inhibitor may help keep cancer cells from growing and are used to treat soft tissue sarcoma.

Immensity’s Do-it-Yourself: Immunotherapy

Immunotherapy is a treatment that uses the patient’s own immune system by boosting, directing, or restoring the body’s natural defenses against cancer.

Immune checkpoint inhibitor therapy is a type of immunotherapy. Some types of immune system cells have certain proteins, called checkpoint proteins, on their surface. These checkpoints help keep immune responses from being too strong, but also from killing cancer cells. When these checkpoints are blocked, the immune system can kill cancer cells better. There are several types of checkpoint inhibitors. So called PD-1 inhibitors that are used to treat progressive and recurrent soft tissue sarcoma.

CTLA-4 inhibitors block the CTLA-4 protein on the surface of immune cells and thereby also help keep the body’s immune responses in check. CTLA-4 inhibitors are used in clinical trials to treat soft tissue sarcomas.

When Standard Therapies Are Limited: Clinical Trial

While some treatments mentioned above are standard (the currently used treatment), some are being tested in clinical trials. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

When Life Quality is Most Important: Supportive Care

The goal of supportive care is to prevent or treat the symptoms of a disease, side effects caused by treatment, and psychological, social, and spiritual problems related to a disease or its treatment.

Supportive care helps improve the quality of life of patients who have a serious or life-threatening disease.

Radiation therapy is sometimes given as supportive care to relieve pain in patients with large tumors that have spread.

You need more information about clinical trials or coping with an advanced cancer condition? Read more in our blog https://theknowhow.ae/ovarian-cancer-coping-with-an-advanced-condition/

TheKnowHow Independent Second Opinion Service

Are you or a loved one suffering from a sarcoma and are unsure about the exact diagnosis or are concerned about your current treatment choices?

TheKnowHow Independent Second Opinion Service is not intended to take you away from your treating doctor, but rather provide an extra level of competence. Get an unbiased assessment from an international expert without having to travel or schedule appointments. Our impartial specialists conduct a record-based assessment of your current health state and all available treatment options, including their advantages and potential hazards.

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