Soft Tissue Sarcoma

Description

Soft tissue sarcomas are a group of solid tumors that can develop anywhere in the body, and originate in soft tissues such as cartilage, fat, muscle, nerves, blood vessels, or deep skin tissues. Although soft tissue sarcomas can occur anywhere in the body, they most commonly occur (about 43%) in the arms and legs.

Epidemiology Data (US & Europe)

Diagnoses of soft tissue sarcoma represent about 0.7% of all new cancer cases in the United States. These tumors can occur at any age, and account for 7-10% of pediatric cancers. It is estimated that about 12,000 new cases (between 3 and 4 cases per every 100,000 people) of adult soft tissue sarcoma will be diagnosed this year in the United States. The rate of new soft tissue sarcoma cases in Europe is similarly low, and is estimated to be between 4 and 5 new cases per every 100,000 adults.

Risk Factors

Risk factors that increase the chance for developing soft tissue sarcoma include exposure to radiation or certain chemicals, genetic predisposition, and for at least one type of soft tissue sarcoma (lymphangiosarcoma), a damaged lymphatic system.

Survival

It is estimated that almost 5,000 patients will die of soft tissue sarcomas in the United States this year. The estimated 5-year survival rate of people with newly diagnosed soft tissue sarcoma is around 50%. For patients with soft tissue sarcomas of stage I, II, or III, without tumors that have spread to the lymph nodes, the 5-year survival rate is 83%. For patients with stage III tumors that have spread to the lymph nodes, the 5-year survival rate is 54%. For patients with soft tissue sarcoma that has spread to more distant parts of the body, the 5-year survival rate is 16%.

Types

There are about 50 different types of soft tissue sarcoma. In adults, the most common types are undifferentiated pleomorphic sarcoma, liposarcoma, and leiomyosarcoma. Rhabdomyosarcoma is the most common soft-tissue sarcoma in childhood.

Classification of Molecular Alterations

Because of the many different types of soft tissue sarcoma, the molecular changes in each tumor can significantly vary. Nevertheless, soft tissue sarcomas can be broadly characterized by the type of genetic and molecular alterations involved in their development. Three common genetic changes include: activation of genes involved in cell growth and proliferation (e.g. KIT gene in gastrointestinal stromal tumors, duplication or deletion of certain genes (e.g. amplification of MDM2 genes in well-differentiated liposarcomas), and rearrangements of DNA (e.g. the reciprocal translocation between chromosome 18 and chromosome X in synovial sarcoma).

Symptoms

Symptoms vary by soft tissue sarcoma type, however, general symptoms include a soft tissue lump that is (1) increasing in size, (2) is greater than 5 cm, (3) extends to deeper tissues, and (4) is painful.

Staging

Progression of soft tissue sarcoma is divided into four stages (I-IV), with stages I and II having sub-stages of IA (tumor size ≤5 cm), IB (>5 cm), IIA (≤5 cm), and IIB (>5 cm). For all stages, the primary tumor may be either just beneath the skin or deeper in the muscle and connective tissue. Staging is identified by the likelihood of tumor growth and spread, with stage I tumors being likely to grow and spread slowly, and stage III tumors, likely to grow and spread quickly. Stages I and II soft tissue sarcomas are localized at the site of primary growth, whereas those of stages III and IV have spread to nearby lymph nodes, and more distant parts of the body, respectively.

Treatment Options

Surgery is the primary treatment for soft tissue sarcoma. Radiation therapy may also be added for high-grade, deep tumors and for select low-grade tumors that are closer to the surface. Chemotherapy may be started before or after surgery. The efficacy of chemotherapeutic agents varies, depending on tumor type.

Ovarian Cancer

Description

Ovarian cancer is a disease in which cancer cells are found in the ovaries. In addition to forming in the ovary, cancers that form in the nearby fallopian tube or in the peritoneum (the membrane that lines the abdominal cavity) and spread to the ovary are often considered ovarian cancer, therefore following the same course of treatment. Most ovarian cancers form after menopause, with over half being found in women of 63 years of age or older.

Epidemiology Data (US & Europe)

The number of new cases of ovarian cancer has been slowly decreasing every year since the mid-1980s. New diagnoses of ovarian cancer make up about 3% of new cancer cases in American women. It is estimated that about 21,000 and 66,000 women in the United States and Europe, respectively, will be diagnosed with ovarian cancer this year, and about 1.3% of women will be diagnosed with ovarian cancer at some point during their lifetime.

Risk Factors

Family history is the single most important risk factor in the development of ovarian cancer. Women with familial cancer syndromes, or with inherited mutations in the genes BRCA1 or BRCA2 are at increased risk. Other risk factors include increased age, being overweight, and the use of estrogen in postmenopausal women.

Survival

More than 14,000 and 43,000 women are estimated to die of ovarian cancer this year in the United States and Europe, respectively, making this disease the fifth leading cause of cancer-related deaths in women. The relative 5-year survival rate for women with ovarian cancer is around 46%. For patients with ovarian tumors of stage I, II, or III, without tumors that have spread to the lymph nodes, the 5-year survival rate is 92%. For patients with stage III tumors that have spread to the nearby lymph nodes, the 5-year survival rate is 73%. For patients with ovarian tumors that have spread to more distant parts of the body, the 5-year survival rate is 28%.

Types

Ovarian cancer can develop from three different cell types: epithelial cells that form the tissue that covers the ovaries, stromal cells that produce hormones or form connective tissue within the ovary, and germ cells, or egg cells. About 90% of ovarian cancers are ovarian epithelial tumors, with the rest being stromal cell or germ cell tumors. Ovarian stromal cell tumors and germ cell tumors have a slightly greater prognosis, with 5-year survival rates of 75% and 90%, respectively.

Classification of Molecular Alterations

Common genetic changes found in ovarian cancer include the inactivation of tumor suppressor genes, which act to prevent abnormal cell growth in normal cells, and the activation of oncogenes, which stimulate the uncontrolled growth of cancer cells. Mutations in the tumor suppressor genes BRCA1, BRCA2, p53, and PTEN are commonly seen in ovarian tumors, as are mutations in the oncogenes BRAF, KRAS, and HER2.

Symptoms

The most common symptoms of ovarian cancer include bloating, pelvic/abdominal pain, trouble eating or feeling full quickly and changes in the urgency or frequency of urination. Although these symptoms are more likely to be noticed if the cancer has spread beyond the ovaries, early-stage cancers can also cause these symptoms.

Staging

Ovarian cancer progression is divided into four stages (I-IV), with numerous sub-stages, depending on the exact locations of the tumor spread. Stage I indicates a cancer that is localized within the ovary (or ovaries) or fallopian tube(s), and has not spread to other tissues. Stage II ovarian cancer is present within one or both ovaries or fallopian tubes, and has spread to other organs within the pelvis. Stage III ovarian cancer may or may not have spread to nearby organs, but is indicated by spread to nearby lymph nodes or to the lining of the abdomen (the peritoneum). In the most advanced stage of ovarian cancer, stage IV, the cancer has spread to the spleen, lungs, liver, or other organs outside of the abdomen.

Treatment Options

Two or more different types of treatment are most commonly used to treat ovarian cancer. Surgery is the primary treatment for ovarian cancer, and is used to both correctly evaluate the cancer and to remove as much of the cancerous material as possible. Other treatments include the use of one or more chemotherapeutic agents, targeted therapy, which unlike traditional chemotherapy, specifically targets cancer cells, and radiation therapy. Additionally, for ovarian stromal tumors, hormone therapy may be used.

Small Cell Lung Cancer

Description

Small cell lung cancer is one of the two main types of lung cancer—non-small cell lung cancer being the other—and is named for the small size of the cancer cells when viewed under a microscope. Because these two types of lung cancer differ both in their outlook (prognosis) and course of treatment, they are generally considered separately. About 10% to 15% of all lung cancers are small cell lung cancers, which often develop near the center of the chest, grow and spread quickly, and have almost always spread to other parts of the body before they are detected. The other 85% to 90% of lung cancers are non-small cell lung cancers, which can be further characterized into three different subtypes: adenocarcinomas, squamous cell carcinomas, and large cell carcinomas. These pages will focus mainly on small cell lung cancer, except where data only exist for lung cancer as a whole.

Epidemiology Data (US & Europe)

Lung cancer (both small cell and non-small cell lung cancer) is the second most common cancer in the United States, representing about 13% of all new cancer cases. It is estimated that about 221,000 and 410,000 people in the United States and Europe, respectively, will be diagnosed with lung cancer this year, and about 6.6% of men and women will be diagnosed with lung cancer at some point during their lifetime.

Risk Factors

Tobacco smoke is the leading cause for the development of small cell lung cancer, and most deaths due to small cell lung cancer are associated with smoking or with exposure to secondhand smoke. Additional risk factors include exposure to radon, air pollution, or other chemicals. Although these environmental factors are the predominant risk factors for small cell lung cancer, genetic factors may also play role in the development of some cancers.

Survival

It is estimated that each year, about 158,000 and 354,000 people die of lung cancer in the United States and Europe, respectively. About 17% of patients survive 5 years or more after being diagnosed with lung cancer. For patients with localized lung cancers that have not spread, the 5-year survival rate is 55%. For patients with tumors that have spread to regional lymph nodes within the chest, the 5-year survival rate is 27%. For patients with tumors that have spread to more distant parts of the body, the 5-year survival rate is about 4%.

Types

There are two types of small cell lung cancer: small cell carcinoma and combined small cell carcinoma. Combined small cell carcinoma is a relatively uncommon tumor in which some cells have characteristics of small cell carcinoma and other cells with characteristics of non-small cell lung cancers. The clinical characteristics of these two cancer types are not significantly different, and the overall survival and course of treatment is similar.

Classification of Molecular Alterations

When comparing tumors, small cell lung cancers can vary greatly in the exact molecular changes that have occurred to transform healthy cells into cancerous cells. Nevertheless, nearly all small cell lung cancers have lost two key genes responsible for preventing uncontrolled cell growth (TP53 and RB1). Other common genetic changes include the deletion of large amounts of DNA from chromosome 3, as well as the abnormal activation of cellular growth and survival processes.

Symptoms

Most lung cancers do not cause any symptoms until they’ve reached an advanced stage and are difficult to cure, but some people with early lung cancer do have symptoms. Common symptoms include: a persistent cough, chest pain, hoarseness, weight loss and loss of appetite, coughing up blood, shortness of breath, feeling tired or weak, lung infections like bronchitis or pneumonia, or new onset of wheezing. Additionally, lung cancer that has spread can cause bone pain, neurologic changes, yellowing of the skin and eyes, or lumps under the surface of the skin.

Staging

Lung cancer staging uses a three-part system, in which the Tumor size, the spread to lymph Nodes, and the spread to other organs (Metastasis) is evaluated. This TNM system is then used to assign an overall stage of 0, I, II, III, or IV to the cancer. It should be noted that some of these stages can be further divided into sub-stages, depending on the tumor’s size, lymph node involvement, and spread to distant sites.

In stage 0, cancer cells are only found in the topmost layers of the air passages, and have not invaded deeper into the lung tissue nor spread to lymph nodes or other sites. Stage I tumors are no larger than 5 cm, and have not spread to lymph nodes or to distant organs, whereas stage II tumors can be larger and may or may not have spread to lymph nodes on the same side as the primary tumor. Stage III tumors can be any size, and generally have spread to lymph nodes on the same side as the primary tumor, as well as potentially having spread to lymph nodes on the opposite side. In stage IV, the tumor has spread to the other lung or to other organs such as the liver, bones, or brain.

Treatment Options

Most patients with small cell lung cancer receive a combination of two chemotherapy drugs as a first-line therapy. Additional radiation therapy may be used simultaneously with the chemotherapy, or after chemotherapy has ended. Surgery is rarely used as the main treatment in small cell lung cancer.

Multiple Myeloma

Description

Multiple myeloma is a cancer of plasma cells—cells of the immune system that produce antibodies and are primarily located in the bone marrow. The out-of-control growth of cancerous plasma cells produces a tumor called an isolated plasmacytoma, and when multiple plasmacytomas are present, a patient is said to have multiple myeloma. Most often these plasmacytomas develop in bone, but it is also possible for plasmacytomas to form in other tissues. In addition to the uncontrolled growth of plasma cells, multiple myeloma is characterized by the production of large amounts of antibodies or fragments of antibodies (a condition called amyloidosis), which can accumulate in distant tissues and damage certain organs.

Epidemiology Data (US & Europe)

Multiple myeloma is a relatively uncommon cancer, with new cases comprising about 1.6% of all new cancer cases in the United States. It is estimated that about 27,000 and 39,000 men and women in the United States and Europe, respectively, will be diagnosed with multiple myeloma this year, and about 0.7% of men and women will be diagnosed with multiple myeloma at some point during their lifetime.

Risk Factors

The most significant risk factor for multiple myeloma is age, as more 96% of cases are diagnosed in patients older than 45 years. Other risk factors include gender (men are more likely to develop multiple myeloma than women), race (people of African ancestry are more than twice as likely than whites to develop multiple myeloma), radiation exposure, family history, being overweight, and having other plasma cell diseases.

Survival

About 11,000 and 24,000 patients in the United States and Europe, respectively, are estimated to die of multiple myeloma this year. The relative 5-year survival rate for patients with newly diagnosed multiple myeloma is about 47%. For patients with earlier stages of multiple myeloma, the 5-year survival rate is 67%, whereas the 5-year survival rate for patients with later stages is 45.4%.

Types

In some patients, symptomatic multiple myeloma can be preceded by asymptomatic myeloma (also called smoldering, or indolent myeloma) or a condition called monoclonal gammopathy of undetermined significance (MGUS). Neither of these conditions are symptomatic, and because they may not progress to symptomatic multiple myeloma, may not require immediate treatment.

Classification of Molecular Alterations

Multiple myeloma is a complex disease in which the genetic changes that result in cancer can vary from patient to patient. Chromosomal translocations, in which a plasma cell’s DNA is reshuffled between chromosomes, account for about half of the primary events that lead to the formation of cancer. Other possible genetic changes can include the duplication or loss of DNA, the deregulation of cellular growth processes, and the loss of mechanisms to repair DNA.

Symptoms

Although some patients with multiple myeloma have no symptoms at all—disease in these patients is discovered after routine blood tests—the most common symptoms of multiple myeloma include pain, weakness, or fractures in bones; reduced red blood cells, white blood cells, and/or platelets; high blood levels of calcium; sudden back pain, numbness, or muscle weakness; confusion, dizziness, or stroke-like symptoms; kidney problems; and infections. Additional symptoms due to the abnormal production of antibody fragments (called amyloidosis) include heart problems, enlarged liver and spleen, enlarged tongue, skin changes, diarrhea, and carpal tunnel syndrome.

Staging

Recently, a new staging system for multiple myeloma has been developed, which determines the stage of the disease based on blood levels of certain protein markers, beta-2-microglobin and albumin. Using this new International Staging System, the progression of multiple myeloma is divided into three stages (I-III). In stage I, patients have beta-2-microglobin less than 3.5 mg/L and albumin greater than or equal to 3.5 gm/dL. In stage II, patients have either beta-2-microglobin greater than 3.5 mg/L but not greater than 5.5 mg/dL with any albumin level, or albumin less than 3.5 g/dL and beta-2-microglobin less than 3.5 mg/L. In stage III, patients can have any level of albumin but have a beta-2-microglobin level of greater than 5.5 mg/dL.

Treatment Options

Treatment options for multiple myeloma include: chemotherapeutic agents, which kill cancer cells; immunomodulating agents, which function by preventing tumor growth by a variety of mechanisms; proteasome inhibitors, which inhibit tumor growth and promote cancer cell death; and radiation therapy, which can directly target multiple myeloma tumors and plasmacytomas. Surgery is rarely used to treat multiple myeloma.

The information contained here is purely for informational purposes and does not, in any event, seek to take the place of a consultation with a medical professional. In the event of doubt or if you have symptoms, you should always consult a medical professional.

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