Bone and Soft Tissue Sarcoma, ALK

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Expand Collapse Bone and Soft Tissue Sarcoma  - General Description This year about 12,000 people in the U.S. will be told by a doctor that they have cancer of the soft tissue. Sarcomas develop more commonly in adults, although certain types of sarcoma are found more typically in children.

Soft tissue sarcomas can form almost anywhere in the body, including cartilage, fat, muscle, fibrous tissue, blood vessels, and other connective or supportive tissues; osteosarcomas develop in bone, liposarcomas form in fat; rhabdomyosarcomas form in muscle; Ewing sarcomas form in bone and soft tissue; Kaposi sarcoma and uterine sarcoma are other types of soft tissue sarcomas. Because there are many types of soft tissue sarcoma, the cell type must be identified before treatment decisions are made. There are ongoing clinical trials using many forms of therapy in specific types of sarcoma.

Source: National Cancer Institute, 2017
This year about 12,000 people in the U.S. will be told by a doctor that they have cancer of the soft tissue. Sarcomas develop more commonly in adults, although certain types of sarcoma are found more typically in children.

Soft tissue sarcomas can form almost anywhere in the body, including cartilage, fat, muscle, fibrous tissue, blood vessels, and other connective or supportive tissues; osteosarcomas develop in bone, liposarcomas form in fat; rhabdomyosarcomas form in muscle; Ewing sarcomas form in bone and soft tissue; Kaposi sarcoma and uterine sarcoma are other types of soft tissue sarcomas. Because there are many types of soft tissue sarcoma, the cell type must be identified before treatment decisions are made. There are ongoing clinical trials using many forms of therapy in specific types of sarcoma.

Source: National Cancer Institute, 2017
This year about 12,000 people in the U.S. will be told by a doctor that they have cancer of the soft tissue. Sarcomas develop more commonly in adults, although certain types of sarcoma are found more typically in children.

Soft tissue sarcomas can form almost anywhere in the body, including cartilage, fat, muscle, fibrous tissue, blood vessels, and other connective or supportive tissues; osteosarcomas develop in bone, liposarcomas form in fat; rhabdomyosarcomas form in muscle; Ewing sarcomas form in bone and soft tissue; Kaposi sarcoma and uterine sarcoma are other types of soft tissue sarcomas. Because there are many types of soft tissue sarcoma, the cell type must be identified before treatment decisions are made. There are ongoing clinical trials using many forms of therapy in specific types of sarcoma.

Source: National Cancer Institute, 2017
This year about 12,000 people in the U.S. will be told by a doctor that they have cancer of the soft tissue. Sarcomas develop more commonly in adults, although certain types of sarcoma are found more typically in children.

Soft tissue sarcomas can form almost anywhere in the body, including cartilage, fat, muscle, fibrous tissue, blood vessels, and other connective or supportive tissues; osteosarcomas develop in bone, liposarcomas form in fat; rhabdomyosarcomas form in muscle; Ewing sarcomas form in bone and soft tissue; Kaposi sarcoma and uterine sarcoma are other types of soft tissue sarcomas. Because there are many types of soft tissue sarcoma, the cell type must be identified before treatment decisions are made. There are ongoing clinical trials using many forms of therapy in specific types of sarcoma.

Source: National Cancer Institute, 2017
Expand Collapse ALK  - General Description
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ALK is a gene that provides the code for making a protein called anaplastic lymphoma kinase. This protein belongs to a family of proteins on the cell surface known as receptor tyrosine kinases (RTKs). RTKs are the first link in a chain that sends signals from the outside of a cell to the parts inside the cell that control different cellular processes, such as cell growth, cell division and cell differentiation. Anaplastic lymphoma kinase is believed to play a key role in brain development and helps regulate the proliferation of nerve cells during early stages of development. In cancer, either due to mutation or rearrangements in the ALK gene, its activity is continuously switched on, which in turn drives the cancer process.

At least 16 mutations in the ALK gene have been found in some patients with neuroblastoma, a cancer that develops in the immature nerve cells (neuroblasts) during childhood. In most cases, each mutation alters the structure of the ALK protein in different ways. These mutations result in the signaling pathway being switched on, increasing the proliferation of immature nerve cells and leading to neuroblastoma. Some of these mutations are called somatic because they are acquired during the course of a person's life and are found only in cells that become cancerous (not inherited from a parent). In some people with neuroblastoma, extra copies (gene amplification) of ALK cause too much protein to be made.

Rearrangements in the ALK gene also serve as an important driver of tumor growth. These rearrangements result in the production of a recombinant protein that is comprised of the front end of one protein fused together with the tyrosine kinase domain of ALK. The fusion partner can be any one of a number of genes, depending on the malignancy. For instance, in approximately 70 to 80% of ALK-positive anaplastic large cell lymphomas (ALCL), ALK is paired with the Nucleophosmin (NPM) gene. In lung cancer, ALK's translocation partner is primarily the EML4 gene. ALK rearrangements have also been described in other tumors including inflammatory myofibroblastic tumors, neural tumors, rhabdomyosarcomas and in some subtypes of breast cancer. Another type of rearrangement, an inversion, is found in a few people with non-small cell lung cancer (NSCLC), the most common type of lung cancer.

Source: Genetics Home Reference
ALK is a gene that provides the code for making a protein called anaplastic lymphoma kinase. This protein belongs to a family of proteins on the cell surface known as receptor tyrosine kinases (RTKs). RTKs are the first link in a chain that sends signals from the outside of a cell to the parts inside the cell that control different cellular processes, such as cell growth, cell division and cell differentiation. Anaplastic lymphoma kinase is believed to play a key role in brain development and helps regulate the proliferation of nerve cells during early stages of development. In cancer, either due to mutation or rearrangements in the ALK gene, its activity is continuously switched on, which in turn drives the cancer process.

At least 16 mutations in the ALK gene have been found in some patients with neuroblastoma, a cancer that develops in the immature nerve cells (neuroblasts) during childhood. In most cases, each mutation alters the structure of the ALK protein in different ways. These mutations result in the signaling pathway being switched on, increasing the proliferation of immature nerve cells and leading to neuroblastoma. Some of these mutations are inherited and some are called somatic because they are acquired during the course of a person's life and are found only in cells that become cancerous (not inherited from a parent). In some people with neuroblastoma, extra copies (gene amplification) of ALK cause too much protein to be made.

Rearrangements in the ALK gene also serve as an important driver of tumor growth. These rearrangements result in the production of a recombinant protein that is comprised of the front end of one protein fused together with the tyrosine kinase domain of ALK. The fusion partner can be any one of a number of genes, depending on the malignancy. For instance, in approximately 70 to 80% of ALK-positive anaplastic large cell lymphomas (ALCL), ALK is paired with the Nucleophosmin (NPM) gene. In lung cancer, ALK's translocation partner is primarily the EML4 gene. ALK rearrangements have also been described in other tumors including inflammatory myofibroblastic tumors, neural tumors, rhabdomyosarcomas and in some subtypes of breast cancer. Another type of rearrangement, an inversion, is found in a few people with non-small cell lung cancer (NSCLC), the most common type of lung cancer.

Source: Genetics Home Reference
Expand Collapse ALK  in Bone and Soft Tissue Sarcoma
Approximately 50% of inflammatory myofibroblastic tumors (IMT) carry an ALK translocation. In these cases, distinct ALK chromosomal rearrangements have been identified with a number of partner genes, including TPM3, TPM4, CLTC, ATIC, CARS, RANB2, SEC31L1 and PPFIBP1.

Approximately 50% of inflammatory myofibroblastic tumors (IMT) carry an ALK translocation. In these cases, distinct ALK chromosomal rearrangements have been identified with a number of partner genes, including TPM3, TPM4, CLTC, ATIC, CARS, RANB2, SEC31L1 and PPFIBP1.

PubMed ID's
20979472, 22664824, 22034911, 21030459, 11406658, 10383129
Expand Collapse No mutation selected
The mutation of a gene provides clinicians with a very detailed look at your cancer. Knowing this information could change the course of your care. To learn how you can find out more about genetic testing please visit http://www.massgeneral.org/cancer/news/faq.aspx or contact the Cancer Center.

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Your Matched Clinical Trials

Trial Matches: (D) - Disease, (G) - Gene
Trial Status: Showing all 10 results Per Page:
Protocol # Title Location Status Match
NCT00585195 A Study Of Oral PF-02341066, A c-Met/Hepatocyte Growth Factor Tyrosine Kinase Inhibitor, In Patients With Advanced Cancer A Study Of Oral PF-02341066, A c-Met/Hepatocyte Growth Factor Tyrosine Kinase Inhibitor, In Patients With Advanced Cancer MGH Open DG
NCT02568267 Basket Study of Entrectinib (RXDX-101) for the Treatment of Patients With Solid Tumors Harboring NTRK 1/2/3 (Trk A/B/C), ROS1, or ALK Gene Rearrangements (Fusions) Basket Study of Entrectinib (RXDX-101) for the Treatment of Patients With Solid Tumors Harboring NTRK 1/2/3 (Trk A/B/C), ROS1, or ALK Gene Rearrangements (Fusions) MGH Open DG
NCT02601950 A Phase II, Multicenter Study of the EZH2 Inhibitor Tazemetostat in Adult Subjects With INI1-Negative Tumors or Relapsed/Refractory Synovial Sarcoma A Phase II, Multicenter Study of the EZH2 Inhibitor Tazemetostat in Adult Subjects With INI1-Negative Tumors or Relapsed/Refractory Synovial Sarcoma MGH Open D
NCT02642016 A Study to Evaluate the Safety and Pharmacokinetics of KTN0158 in Adult Patients With Advanced Solid Tumors A Study to Evaluate the Safety and Pharmacokinetics of KTN0158 in Adult Patients With Advanced Solid Tumors MGH Open D
NCT02611024 Pharmacokinetic Study of PM01183 in Combination With Irinotecan in Patients With Selected Solid Tumors Pharmacokinetic Study of PM01183 in Combination With Irinotecan in Patients With Selected Solid Tumors MGH Open D
NCT01858168 Phase I Study of Olaprib and Temozolomide for Ewings Sarcoma Phase I Study of Olaprib and Temozolomide for Ewings Sarcoma MGH Open D
NCT01659203 Proton or Photon RT for Retroperitoneal Sarcomas Proton or Photon RT for Retroperitoneal Sarcomas MGH Open D
NCT02180867 Radiation Therapy With or Without Combination Chemotherapy or Pazopanib Hydrochloride Before Surgery in Treating Patients With Newly Diagnosed Non-Rhabdomyosarcoma Soft Tissue Sarcomas That Can Be Removed by Surgery Radiation Therapy With or Without Combination Chemotherapy or Pazopanib Hydrochloride Before Surgery in Treating Patients With Newly Diagnosed Non-Rhabdomyosarcoma Soft Tissue Sarcomas That Can Be Removed by Surgery MGH Open D
NCT02576431 Study of LOXO-101 in Subjects With NTRK Fusion Positive Solid Tumors (NAVIGATE) Study of LOXO-101 in Subjects With NTRK Fusion Positive Solid Tumors (NAVIGATE) MGH Open D
NCT02660034 The Safety, Pharmacokinetics and Antitumor Activity of the BGB-A317 in Combination With the BGB-290 in Subjects With Advanced Solid Tumors The Safety, Pharmacokinetics and Antitumor Activity of the BGB-A317 in Combination With the BGB-290 in Subjects With Advanced Solid Tumors MGH Open D
MGH has many open clinical trials for other cancers not shown on the Targeted Cancer Care website. They can be found on the MassGeneral.org clinical trials search page.

Additional clinical trials may be applicable to your search criteria, but they may not be available at MGH. These clinical trials can typically be found by searching the clinicaltrials.gov website.
Trial Status: Showing all 10 results Per Page:

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