Skip to content

Sarcoma Types

Sarcomas, which are rare malignancies, originate in the supportive tissues of the body, including bone, cartilage, tendons, fat, and muscle. 

There are two primary types: soft tissue sarcomas and bone sarcomas. Treatment approaches and diagnostic procedures vary based on the specific subtype. 

Explore further details and comprehensive information about each type below.


Benign Bone And Soft Tissue Tumors

There are several types of benign bone and soft tissue tumors, each varying based on the specific site and tissue involved. These tumors, which do not metastasize, can develop in various tissues that provide structural support and connectivity throughout the body. Benign tumors can affect bones, fibrous tissues, and the soft tissues that envelop and protect vital organs, including muscles, tendons, and ligaments.

Desmoid tumors are noncancerous growths that occur in the connective tissue, most commonly found in the abdomen, arms, and legs. Also known as aggressive fibromatosis, these tumors can vary significantly in their behavior. While some grow slowly and may not require immediate treatment, others can grow quickly and may need interventions such as surgery, radiation therapy, chemotherapy, or other medications.

Characteristics and Behavior

Although desmoid tumors are not classified as cancers because they do not metastasize, they can be very aggressive. They often invade nearby structures and organs, resembling the behavior of cancerous growths. Due to their aggressive nature, individuals with desmoid tumors are frequently treated by cancer specialists.

Symptoms

Symptoms of desmoid tumors differ based on their location. Generally, desmoid tumors can manifest as a mass or area of swelling, pain, loss of function in the affected area, and cramping and nausea when located in the abdomen. It’s important to consult a doctor if you experience any persistent or worrisome symptoms.

Causes

The exact cause of desmoid tumors is unclear. They develop when a connective tissue cell undergoes changes in its DNA, causing rapid cell multiplication and the formation of a tumor that can invade healthy tissue.

Risk Factors

Several factors may increase the risk of developing desmoid tumors:

  • Young adult age: Most common in adults in their 20s and 30s, rare in children and older adults.
  • Genetic syndromes: Conditions like familial adenomatous polyposis (FAP) significantly increase the risk. FAP, caused by a gene mutation, leads to numerous colon polyps and can be inherited.
  • Pregnancy: Rarely, desmoid tumors may develop during or shortly after pregnancy.
  • Injury: A small number of cases occur in individuals who have recently experienced an injury or undergone surgery.
Treatment Options

The treatment for desmoid tumors varies based on their growth rate and location. Some may require monitoring, while others need surgical removal, radiation therapy, chemotherapy, or targeted drug therapy to manage their growth and symptoms.

Giant cell tumor of bone is a rare, fast-growing noncancerous tumor. It most often develops in adults between the ages of 20 and 40, particularly after skeletal bone growth is complete. These tumors are slightly more common in women and typically grow near a joint at the end of a bone. Common locations include the knee, bones of the arms and legs, and occasionally flat bones such as the breastbone or pelvis.

Characteristics and Behavior

Giant cell tumors are aggressive despite being noncancerous. They often grow rapidly and can cause significant bone destruction. These tumors typically occur near joints, leading to symptoms that affect joint function. Due to their aggressive nature, giant cell tumors require prompt and often intensive treatment to prevent bone damage and preserve function.

Symptoms

Symptoms of giant cell tumors vary depending on their location. They may include a visible bump, bone fracture, fluid buildup in the nearby joint, limited movement in the nearest joint, swelling, and pain at the affected joint. If you experience any persistent or concerning symptoms, it is important to consult a healthcare provider for a diagnosis.

Causes

The exact cause of giant cell tumors is unknown. However, they have been linked to Paget disease of bone, a chronic condition that causes bones to become enlarged and misshapen.

Risk Factors

There are no identified risk factors for giant cell tumor of bone. It typically arises spontaneously in young adults, usually between the ages of 20 and 40.

Treatment Options

The primary goal in treating giant cell tumors is to remove the tumor and prevent bone damage. Treatment options may include:

  • Surgery: To remove the tumor and any damaged bone.
  • Bone Grafting: To repair bone defects after tumor removal.
  • Bone Reconstruction: To restore the structure and function of the affected bone.
  • Physical Therapy: To regain strength and mobility.
  • Radiation Therapy: For tumors that cannot be surgically removed.

In severe cases, amputation may be necessary. Giant cell tumors can recur, so long-term follow-up with a healthcare provider is essential.

Tenosynovial Giant Cell Tumor (TGCT) is a rare, benign tumor that occurs in the joint, tendon sheath, or bursae. In 2013, the World Health Organization reclassified related conditions—Giant Cell Tumor of the Tendon Sheath (GCT-TS), nodular tenosynovitis, pigmented villonodular synovitis (PVNS), and diffuse-type giant cell tumor—under the single entity of TGCT, with two distinct subtypes.

Characteristics and Behavior

TGCT is non-life-threatening but locally aggressive. It often causes significant discomfort and can interfere with joint function. The tumor can be classified as either localized or diffuse, with the latter being more likely to recur after treatment. These tumors are benign but can exhibit characteristics similar to soft tissue cancers, known as sarcomas.

Symptoms

Symptoms of TGCT include joint pain, swelling, stiffness, and reduced range of motion. It is essential to consult a healthcare provider if you experience these symptoms.

Causes

The exact cause of TGCT is unknown, but it involves abnormal cell growth in the synovium, a thin layer of tissue lining the joints and tendon sheaths.

Risk Factors

TGCT can affect people of all ages but is most commonly diagnosed in individuals aged 25-50. There are no specific known risk factors for developing TGCT.

Treatment Options

Treatment for TGCT often involves surgery to remove the tumor. However, the tumor can recur, particularly in the diffuse type. Other treatments may include radiation therapy, targeted drug therapy, and physical therapy to manage symptoms and improve joint function. The treatment plan should be individualized based on the tumor type and patient needs.


Soft Tissue Sarcoma

There are several types of soft tissue sarcoma, depending on the affected site. They can develop within any tissues responsible for supporting, connecting, enveloping, and safeguarding the body’s organs, including nerves, muscle, fat, fibrous tissues, tendons and ligaments, and blood vessels.

Undifferentiated pleomorphic sarcoma (UPS) is a rare cancer that primarily develops in the soft tissues. It typically occurs in the arms or legs and less frequently in the retroperitoneum (area behind the abdominal organs). The name reflects the microscopic appearance of the cancer cells: “undifferentiated” indicates the cells don’t resemble the tissues where they originate, and “pleomorphic” refers to their varied shapes and sizes.

Characteristics and Behavior

UPS is known for its aggressive nature and potential to spread (metastasize) to other parts of the body. It is often detected when it causes noticeable symptoms such as swelling or a lump.

Symptoms

Undifferentiated pleomorphic sarcoma symptoms vary based on the tumor’s location. Symptoms may include a growing lump or swelling, pain, tingling, and numbness if the lump becomes large, and swelling in the hand or foot if an arm or leg is affected. Abdominal tumors may cause pain, loss of appetite, and constipation. Other symptoms can include fever and weight loss.

Causes

The exact cause of UPS is unknown. However, it is believed to result from genetic mutations that lead to abnormal cell growth in soft tissues.

Risk Factors

UPS is most commonly diagnosed in adults aged 50 and older. There are no specific known risk factors, but a history of radiation therapy or certain inherited conditions may increase the risk.

Treatment Options

Treatment for UPS typically involves a combination of surgery, radiation therapy, and chemotherapy. Surgery aims to remove the tumor, while radiation and chemotherapy are used to target any remaining cancer cells and reduce the risk of recurrence.

Leiomyosarcoma (LMS) is a rare type of cancer that originates in the smooth muscle tissue, commonly found in the uterus, stomach, intestines, and blood vessels. It is a malignant tumor that can grow and spread to other parts of the body.

Characteristics and Behavior

LMS is known for its aggressive nature and ability to metastasize. It often presents as a large, painless mass that can affect the function of the organs it involves. 

Symptoms

Symptoms of LMS depend on the tumor’s location and size and may not be present at first. They may include a noticeable lump, pain, swelling, and functional impairment of the affected organ. Abdominal LMS may cause gastrointestinal symptoms like nausea, vomiting, and changes in bowel habits.

Causes

The exact cause of LMS is not well understood, but it involves genetic mutations that lead to abnormal growth of smooth muscle cells.

Risk Factors

There are no well-defined risk factors for LMS. However, it most commonly occurs in middle-aged and older adults and is more frequently diagnosed in women due to its prevalence in the uterus.

Genetic Risk Factors:

  • Li-Fraumeni syndrome: (A rare genetic disorder predisposing individuals to various cancers due to mutations in the TP53 tumor suppressor gene, including fibrosarcoma.)

  • Neurofibromatosis type 1: (A genetic disorder causing tumors to grow on nerves, raising the risk of fibrosarcoma and other cancers.)

  • Nevoid basal cell carcinoma syndrome: (Also known as Gorlin syndrome, it’s a genetic condition leading to multiple basal cell carcinomas and increasing the risk of fibrosarcoma.)

  • Retinoblastoma: (A hereditary condition causing eye cancer and raising the chance of fibrosarcoma and other cancers due to gene mutations.)

  • Tuberous sclerosis: (A genetic disorder causing benign tumors in various organs, potentially increasing the risk of fibrosarcoma.)

  • Werner syndrome: (A rare genetic disorder accelerating aging and raising the risk of several cancers, including fibrosarcoma.)

Treatment Options

Treatment for LMS typically includes surgery to remove the tumor, radiation therapy to target any remaining cancer cells, and chemotherapy to address any potential metastasis. The treatment plan is individualized based on the tumor’s location and stage.

Liposarcoma is a rare type of cancer that originates in fat cells and commonly occurs in the abdomen or limbs, though it can also affect other areas, including vital organs. It is a malignant tumor that can grow and spread to other parts of the body.

Characteristics and Behavior

Liposarcoma can vary in its behavior, from slow-growing to highly aggressive. It often presents as a large, painless mass, which can disrupt the function of nearby organs and tissues.

Symptoms

Often, liposarcoma presents without symptoms, though a visible, initially painless lump may develop under the skin. Clear warning signs include a new or persistently growing lump, painful swelling or numbness around the lump, blood in the stool or black/tarry stool, blood in the vomit, and abdominal pain or cramping.

Causes

The exact cause of liposarcoma is unknown, but it involves genetic mutations that lead to the uncontrolled growth of fat cells.

Risk Factors

Liposarcoma can affect individuals of any age but is most commonly diagnosed in adults aged 50-65. There are no specific known risk factors.

Treatment Options

Treatment for liposarcoma typically involves surgery to remove the tumor, radiation therapy to target residual cancer cells, and chemotherapy to treat any metastasis. The treatment approach is personalized based on the tumor’s type, size, location, and stage.

Rhabdomyosarcoma is a rare type of cancer that forms in soft tissue, specifically in skeletal muscle tissue or sometimes in hollow organs such as the bladder or uterus. It most commonly affects children and adolescents.

Characteristics and Behavior

Rhabdomyosarcoma is aggressive and can spread to other parts of the body. It typically presents as a rapidly growing mass, which may cause functional impairment depending on its location.

Symptoms

The signs and symptoms of rhabdomyosarcoma (RMS) vary based on tumor location. Commonly, RMS presents as a persistent and enlarging lump or swelling, potentially painful, often in the arms or legs. In the eye region, it can cause bulging, drooping eyelids, redness, or infection. In the head and neck, symptoms include painless lumps, facial asymmetry, blurred vision, swelling, breathing problems, and possible discharge or bleeding. Abdominal or pelvic RMS may lead to an enlarged abdomen, pain, vomiting, or constipation. RMS in the bladder or prostate can cause blood in the urine and painful urination, while RMS in the reproductive organs may result in painless lumps or vaginal discharge.

Causes

The exact cause of rhabdomyosarcoma is unknown. It involves genetic mutations that lead to abnormal growth of muscle cells.

Risk Factors

Rhabdomyosarcoma is more common in children and adolescents, with no specific known risk factors. Genetic conditions like Li-Fraumeni syndrome (a rare genetic disorder increasing the risk of various cancers due to gene mutations) and neurofibromatosis type 1 (a genetic disorder causing tumors to grow on nerves) can increase risk.

Treatment Options

Treatment for rhabdomyosarcoma often involves a combination of surgery, chemotherapy, and radiation therapy. The treatment plan is tailored to the tumor’s size, location, and stage, and aims to remove the tumor and prevent recurrence.

Synovial sarcoma is a rare type of cancer that typically occurs near large joints of the arm, neck, or leg, mainly close to the knees. It often presents as a slow-growing lump under the skin, which may or may not be painful.

Characteristics and Behavior

Synovial sarcoma is an aggressive cancer that can spread to other parts of the body. It is known for its slow growth and can be mistaken for other less serious conditions and can take years to diagnose.

Symptoms

Symptoms include a noticeable lump, swelling, and pain near the affected joint. It can also cause reduced range of motion in the affected area.

Causes

The exact cause of synovial sarcoma is unknown, but it involves genetic mutations that lead to abnormal cell growth in the soft tissues around joints.

Risk Factors

Synovial sarcoma can affect people of any age but is most commonly diagnosed in young adults. There are no specific known risk factors.

Treatment Options

Treatment for synovial sarcoma typically involves surgery to remove the tumor, often followed by radiation therapy and chemotherapy to address any remaining cancer cells and reduce the risk of recurrence. The treatment approach is tailored to the tumor’s size, location, and stage.

Angiosarcoma is a rare, aggressive cancer that forms in the lining of blood vessels and lymph vessels. It can occur anywhere in the body but most commonly affects the skin of the head and neck.

Characteristics and Behavior

Angiosarcoma is known for its rapid growth and high potential to spread (metastasize) to other parts of the body. It often presents as a bruised or purplish area on the skin, which can be mistaken for a benign condition.

Symptoms

Symptoms of angiosarcoma include a growing skin lesion that may look like a bruise, a growing lump, swelling, pain, and skin changes over the affected area. If the tumor is internal, symptoms may include pain or swelling in the affected organ.

Causes

The exact cause of angiosarcoma is not well understood. It involves genetic mutations that lead to abnormal growth of cells in the lining of blood or lymph vessels.

Risk Factors

Risk factors for angiosarcoma include previous radiation therapy, chronic lymphedema (swelling caused by a buildup of lymph fluid due to damage or blockage in the lymphatic system), exposure to certain chemicals, and genetic predisposition. It is most commonly diagnosed in older adults.

Treatment Options

Treatment for angiosarcoma often involves a combination of surgery, radiation therapy, and chemotherapy. The goal is to remove the tumor and manage any metastasis. The treatment plan is individualized based on the tumor’s size, location, and stage.

Fibrosarcoma is a rare type of cancer that develops in the fibrous connective tissues of the body, commonly affecting the arms, knees, thighs, and trunk. It is most often diagnosed in adults and can be aggressive, spreading to other parts of the body.

Characteristics and Behavior

Fibrosarcoma typically presents as a firm mass that can grow rapidly. It may invade nearby tissues and metastasize to distant organs, making early diagnosis and treatment crucial.

Symptoms

Symptoms of fibrosarcoma include a noticeable lump, pain, feeling of pins and needles, swelling, weight loss, and limited movement in the affected area.

Causes

The exact cause of fibrosarcoma is unknown. It involves genetic mutations that lead to uncontrolled growth of fibrous tissue cells.

Risk Factors

Genetic Risk Factors:

  • Familial adenomatous polyposis: (A genetic condition characterized by the development of numerous polyps in the colon and rectum, increasing the risk of fibrosarcoma.)

  • Li-Fraumeni syndrome: (A rare genetic disorder predisposing individuals to various cancers due to mutations in the TP53 tumor suppressor gene, including fibrosarcoma.)

  • Neurofibromatosis type 1: (A genetic disorder causing tumors to grow on nerves, raising the risk of fibrosarcoma and other cancers.)

  • Nevoid basal cell carcinoma syndrome: (Also known as Gorlin syndrome, it’s a genetic condition leading to multiple basal cell carcinomas and increasing the risk of fibrosarcoma.)

  • Retinoblastoma: (A hereditary condition causing eye cancer and raising the chance of fibrosarcoma and other cancers due to gene mutations.)

  • Tuberous sclerosis: (A genetic disorder causing benign tumors in various organs, potentially increasing the risk of fibrosarcoma.)

  • Werner syndrome: (A rare genetic disorder accelerating aging and raising the risk of several cancers, including fibrosarcoma.)

Other Risk Factors:

  • Previous radiation therapy: (Exposure to radiation, particularly in therapeutic settings, can increase the risk of developing fibrosarcoma.)

  • Exposure to certain chemicals: (Chemicals like thorium dioxide, vinyl chloride, or arsenic have been linked to an increased risk of fibrosarcoma.)

  • Lymphedema: (Chronic swelling due to lymphatic system dysfunction may raise the risk of developing fibrosarcoma in affected areas.)

Treatment Options

Treatment for fibrosarcoma often involves surgery to remove the tumor, followed by radiation therapy and chemotherapy to address any remaining cancer cells and reduce the risk of recurrence. The treatment plan is personalized based on the tumor’s size, location, and stage.

Malignant Peripheral Nerve Sheath Tumor is a rare and aggressive type of cancer that arises from the protective lining of the nerves. It is most commonly found in the deep tissues of the arms, legs, and trunk.

Characteristics and Behavior

MPNST typically presents as a rapidly growing mass, often associated with pain or neurological symptoms. It has a high potential to metastasize, making early diagnosis and treatment essential.

Symptoms

Symptoms of MPNST include a noticeable lump, pain, weakness, and numbness in the affected area, and sometimes impaired nerve function.

Causes

The exact cause of MPNST is unclear. However, it is often associated with genetic conditions such as neurofibromatosis type 1 (a genetic disorder causing tumors to grow on nerves), which increases the risk.

Risk Factors

The primary risk factor for MPNST is neurofibromatosis type 1 (NF1). Other risk factors include radiation therapy and noncancerous nerve tumors. It can also develop sporadically without any known risk factors.

Treatment Options

Treatment for MPNST generally involves a combination of surgery, radiation therapy, and chemotherapy. The primary goal is to remove the tumor and prevent its spread. The treatment approach is tailored based on the tumor’s size, location, and stage.

GIST is a rare type of cancer that originates in the digestive tract, most commonly in the stomach or small intestine. It arises from special cells known as interstitial cells of Cajal, which are part of the autonomic nervous system.

Characteristics and Behavior

GISTs can vary in behavior, from slow-growing to aggressive. They often present as a mass in the gastrointestinal tract, which can cause bleeding and obstruction.

Symptoms

Symptoms might not be present at first. Symptoms of GIST include abdominal pain, a noticeable lump in the abdomen, gastrointestinal bleeding, fatigue, and vomiting. Symptoms vary based on the tumor’s location and size.

Causes

The exact cause of GIST is unknown, but it involves mutations in certain genes such as KIT or PDGFRA, which lead to uncontrolled cell growth.

Risk Factors

Risk factors for GIST include being over 50 years of age and having genetic mutations. Family history and certain genetic syndromes also increase risk.

Treatment Options

Treatment for GIST typically involves surgery to remove the tumor and targeted therapy with medications like imatinib, which inhibits the growth of cancer cells. The treatment approach is individualized based on the tumor’s size, location, and genetic mutations.

Kaposi sarcoma is a type of cancer where abnormal cells develop in the skin or the mucous membranes lining the gastrointestinal (GI) tract, spanning from the mouth to the anus and including the stomach and intestines. These tumors typically appear as purple patches or nodules on the skin or mucous membranes and can potentially spread to lymph nodes and lungs. This condition is more prevalent in males and in individuals with weakened immune systems

Characteristics and Behavior

Kaposi Sarcoma can vary in presentation, from indolent skin lesions to aggressive forms that spread rapidly, particularly in immunocompromised individuals.

Symptoms

Symptoms include skin lesions, swelling, and potentially respiratory or gastrointestinal issues if internal organs are involved.

Causes

Kaposi Sarcoma is mainly caused by human herpesvirus 8 (HHV-8), often in combination with immunosuppression, such as in HIV/AIDS patients.

Risk Factors

Risk factors include having HIV/AIDS, receiving an organ transplant, or other conditions that suppress the immune system.

Treatment Options

Treatment approaches for Kaposi sarcoma include:

  • Boosting immune system function: Strengthening the immune system is the primary and most effective therapy for Kaposi sarcoma patients, especially those with immune deficiencies.
  • Antiretroviral therapy (a treatment regimen used to manage infections caused by retroviruses, notably HIV): For AIDS patients, antiretroviral therapy used to manage AIDS can also be effective in treating Kaposi sarcoma. Transplant recipients may be advised to adjust immunosuppressant drugs.
  • Local therapies: Topical treatments like injecting chemotherapy directly into lesions, cryosurgery (surgery that involves the use of extreme cold to destroy abnormal tissues) , excisions (removing by cutting out), phototherapy (using ultraviolet light), or local radiation may be recommended for patients with few small lesions.
  • Chemotherapy: If immune deficiency improvements don’t lead to improvement, chemotherapy may be necessary. Intravenous or oral chemotherapy may be used.
  • Immunotherapy: This treatment activates the body’s immune system to fight cancer and is being explored for Kaposi sarcoma. Clinical trials and emerging treatment options should be discussed with a healthcare provider.
  • Surgery: Generally not recommended due to ineffectiveness in curing the disease and the risk of lesion recurrence.

Myxofibrosarcoma (MFS) is a type of soft tissue sarcoma that develops in the cells surrounding the connective tissue. It often appears as a painless, slow-growing mass under the skin, particularly in the arms and legs, but can also occur in other areas of the body.

Characteristics and Behavior

Myxofibrosarcoma tends to be locally aggressive, meaning it can invade nearby tissues and structures. It has a tendency to recur even after surgical removal, and in some cases, it may metastasize to distant organs.

Symptoms

Symptoms may include a palpable lump under the skin, which may or may not be painful, bone pain, unexpected bone fractures, abdominal pain, and unexplained weight loss.

Causes

The exact cause of myxofibrosarcoma is unknown. However, it is believed to develop due to genetic mutations in the connective tissue cells, leading to uncontrolled growth and tumor formation.

Risk Factors

Risk factors for myxofibrosarcoma include previous radiation therapy to the affected area, and certain genetic conditions that predispose individuals to develop soft tissue sarcomas.

Treatment Options

Treatment for myxofibrosarcoma typically involves a combination of surgery, radiation therapy, and chemotherapy. The goal of treatment is to remove the tumor completely, preserve function in the affected area, and prevent recurrence or spread to other parts of the body. Targeted therapy and immunotherapy may also be options in some cases to specifically target cancer cells and enhance the body’s immune response against the tumor.


Bone Sarcoma

There are several types of bone sarcoma, depending on the affected site. Bone sarcomas can affect any bone tissue, including the hard outer layer, the spongy inner layer, and the cartilage that covers bone surfaces.

Osteosarcoma is a type of bone cancer that develops in the cells responsible for forming new bone tissue. It primarily affects the long bones of the arms and legs, although it can occur in any bone in the body.

Characteristics and Behavior

Osteosarcoma is known for its aggressive nature, often growing rapidly and invading surrounding tissues. It tends to occur in teenagers and young adults, although it can also affect older adults and children.

Symptoms

Symptoms of osteosarcoma may include pain and swelling near the affected bone, and pain from a bone that breaks for no reason.

Causes

The exact cause of osteosarcoma is unknown, but it is believed to develop due to genetic mutations that disrupt the normal growth and development of bone cells, leading to the formation of cancerous tumors.

Risk Factors

Risk factors for osteosarcoma include previous radiation therapy, certain genetic conditions such as Li-Fraumeni syndrome (a rare genetic condition that increases the risk of various cancers) and hereditary retinoblastoma (a genetic disorder causing eye cancer), and certain bone diseases such as Paget’s disease of the bone (a bone disorder where abnormal bone remodeling occurs, leading to weakened, enlarged, and deformed bones).

Treatment Options

Treatment for osteosarcoma typically involves a combination of surgery, chemotherapy, and sometimes radiation therapy. The primary goal of treatment is to remove the tumor, preserve limb function and mobility, and prevent recurrence or spread to other parts of the body. Chemotherapy is often used before and after surgery to shrink the tumor and kill any remaining cancer cells. In some cases, targeted therapy and immunotherapy may also be used to specifically target cancer cells and boost the immune system’s response against the tumor.

Ewing Sarcoma is a type of bone cancer that primarily affects children and young adults. It develops in the bones or soft tissue around the bones, often in the pelvis, legs, ribs, arms, or spine.

Characteristics and Behavior

Ewing sarcoma is known for its aggressive nature, growing quickly and potentially spreading to other parts of the body, such as the lungs, bone marrow, or other bones.

Symptoms

Symptoms of Ewing sarcoma may include pain and swelling near the affected bone or site of the tumor. Some patients may also experience fever and weight loss.

Causes

The exact cause of Ewing sarcoma is unknown, but it is believed to develop due to genetic mutations in the cells that form bone or soft tissue.

Risk Factors

Risk factors for Ewing sarcoma include exposure to radiation and certain genetic factors such as Fanconi anemia (a rare genetic disorder that affects the bone marrow’s ability to produce blood cells).

Treatment Options

Treatment for Ewing sarcoma often involves a combination of chemotherapy, surgery to remove the tumor, and sometimes radiation therapy. Targeted therapy and immunotherapy may also be options in certain cases to specifically target cancer cells and boost the immune system’s response against the tumor.

Chondrosarcoma is a type of bone cancer that originates in cartilage cells, the gristly connective tissue from which most bones develop. It primarily affects the femur, arm, pelvis, or knee, though it can also occur in other areas like the ribs. As the second most common primary bone cancer, chondrosarcoma rarely affects individuals under 20, with risk increasing until age 75. Incidence is equal between males and females. 

Characteristics and Behavior

Unlike cancers that spread to bones from other organs, primary bone cancers like chondrosarcoma begin in the bone itself. Chondrosarcoma is characterized by the development of malignant tumors in cartilage cells. These tumors typically grow slowly but can vary in their aggressiveness.

Symptoms

Chondrosarcoma symptoms can vary depending on the tumor’s location. Common signs include a noticeable mass on the bone, pressure sensation around the mass, and increasing pain that’s particularly bothersome at night. This pain typically responds to anti-inflammatory medications like ibuprofen but not necessarily to rest. Additionally, there may be local swelling associated with the tumor.

Causes

The cause of chondrosarcoma is not fully understood, but it may involve genetic or chromosomal factors that increase susceptibility to this type of cancer. Additionally, chondrosarcomas can sometimes develop as a delayed effect of radiation therapy used to treat other cancers.

Risk Factors

Chondrosarcoma often originates from normal cartilage cells or can develop from preexisting benign bone or cartilage tumors. Some benign conditions associated with the development of chondrosarcoma include enchondromas (affecting the hands primarily), multiple exostoses or osteochondromas (multiple growths near growth plate ends), Ollier disease (cluster of enchondromas), and Maffucci syndrome (combination of multiple enchondromas and angiomas).

Treatment Options

Treatment for Ewing sarcoma often involves a combination of chemotherapy, surgery to remove the tumor, and sometimes radiation therapy. Targeted therapy and immunotherapy may also be options in certain cases to specifically target cancer cells and boost the immune system’s response against the tumor.

Chordomas are malignant tumors that can develop anywhere along the spine or at the base of the skull. They most commonly occur in the lower back (sacral area) and the base of the skull, each accounting for approximately one-third to one-half and one-third of cases, respectively. These tumors originate from remnants of the notochord, an embryonic tissue that eventually forms the center of spinal disks.

Characteristics and Behavior

Although chordomas typically grow slowly, they can metastasize and become locally aggressive. Their growth can cause significant problems by putting pressure on or invading critical parts of the brain or spine, leading to pain, nerve issues, or potentially life-threatening complications.

Symptoms

As chordomas grow, they can press on the spine, brain, and nerves, leading to symptoms specific to their location. These symptoms may include pain, tingling, numbness, weakness, lack of bladder or bowel control, sexual dysfunction, vision problems, endocrine issues, and swallowing difficulties. In cases where the chordoma has grown very large, it might be possible to feel a lump.

Causes

The exact cause of cancerous chordomas is unknown. In most cases, doctors do not understand why cancer develops from leftover notochord tissue. Researchers are investigating this question, but finding answers is challenging due to the rarity of chordomas.

Risk Factors

There are no known environmental, dietary, or lifestyle risk factors for chordoma. Most chordomas occur randomly and are not due to inherited genetic traits. However, several genetic factors are associated with chordoma.

Familial Chordoma: In rare cases, multiple family members may be affected by chordoma, indicating a strong genetic predisposition. Some families with familial chordoma have an extra copy of the brachyury gene, though no test currently exists for detecting these extra copies. The National Cancer Institute is conducting a genetics study to identify additional hereditary causes of chordoma.

Tuberous Sclerosis Complex (TSC): Children with TSC, a genetic disease caused by changes in the TSC1 or TSC2 genes, have a higher incidence of chordomas.

Treatment Options

Prompt treatment is essential and involves preoperative planning, surgery (en bloc resection), and postoperative therapy. After surgery, patients are closely monitored in the neurosurgical intensive care unit (NCCU) and later undergo physical and occupational therapy. Radiation therapy may also be necessary to reduce tumor size or prevent spread. 
Due to the risk of regrowth, consistent follow-up is required. Patients typically need an MRI every three months during the first year post-surgery, with the interval potentially increasing over time.