Incidence Rate Trend

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          Incidence Rate Trend s                                                                                U . S . Bone Sa rcoma Incidence by Ag e
         Sarcomas are a diverse group of malignant tumors
         that develop from fat, muscles, nerves, joints, blood                                         7-
         vessels, bones, and deep skin tissues . They comprise                                         6-
         about 1 percent of adult and 15 percent of pediatric                                     0
         malignancies. Sarcomas are difficult to differentiate from                                    5-
         other malignancies when they are found within organs ;                                   CL 4-
         thus, they are frequently misdiagnosed and highly                                        0
                                                                                                  r- 3d
         underreported . As a result, although the incidence
         estimates presented here include the best available data,
         they are probably low . Because sarcomas often afflict
         people in the prime of life, the number of years of life                                                                   .

         lost is substantial despite the relatively low incidence . It
                                                                                                        198 3   1987         1991           1995            1 999      200 2
         is estimated that approximately 12,290 Americans will
         be diagnosed with sarcoma and 4,760 will die from the
                                                                                                             U. S . Soft Tissue Sarcoma Incidence by Age
         disease in 2006 .
         Soft tissue sarcoma' and osteosarcoma (bone sarcoma)
         incidence rates have remained relatively constant over
         the past 30 years ; however, soft tissue sarcoma is more                                      s

         deadly due to the lack of detectable symptoms at early                                   ° 5
         disease stages . Soft tissue sarcoma occurs three times
         more frequently in adults than in children . Multiple                                    CL 4-,
         subtypes of osteosarcoma and soft tissue sarcoma exist ;                                    3 -~
         the exact number of Americans with each sarcoma
         subtype is unknown .                                                                          2-i
         Source for incidence data : Surveillance, Epidemiology, and                                                                         . :~ .-
         End Results (SEER) Program and the National Center for Health                                 p =r _ . . . ,I_ ._r , T--v- -_ --- 1 ....r--T . _...---T ,, .._F
         Statistics. Additional statistics and charts are available at http :/`Iseer                     1983 1987 1991 1995 1999 200 3
         'Does not include Kaposi's sarcoma, which is addressed in a
         separate Snapshot .                                                                                                                           Age <20   ~ 20+

         Trends in NCI Funding for                                                                            2005 Sarcoma Research Funding *

         Sarcoma Research
        Funding data for sarcoma has only been collected since
        2005 . In 2005, the National Cancer Institute's (NCI's)
        investment2 in sarcoma research was $35 .9 million 3
        which included $25 .9 million for soft tissue sarcoma
                        .              -
        and $10 .0 million for bone sarcoma research . Bone
                                                                                                                                                                    Sarcom a
        Source : NCI Financial Management Branch Soft Tissue-~~
        hitp :/~`fn~b .caii< Sarcoma

        2The estimated NCI investment is based on funding associate d
        with a broad range of peer-reviewed scientific activities . For
        additional information on research priorities and funding, see
        hitp :.1./-~uu-w.n i h .gov/aboudresearchpriariti es . htm#averview.
        'Does not include Kaposi's sarcoma, which is addressed in a
        separate Snapshot                                                                                                 *The total NCI budget was $4 .88 in fiscal year 2005.

    Sn apsh ots can b e found on line at http :llplanniny .caricer.govldisease;snapshots.shtml,
Examples of NCI Research                                                       NCI Sarcoma
Initiatives Relevant to Sarcom a                                             Res ea rch Portfo li o
    The Community Clini c al On cology Program
    ( CCOP ) and the Minority -Base d Communit y                                                       ,   . .                      . ~

    Clini cal On c olo gy Pro g ram (MB -C COP) are
    comprehensive clinical trial mechanisms that
    disseminate the latest cancer prevention and
    treatment research findings to the community .                              r ' - _ - T, ,
    Twenty-four of the NCI-funded CCOP and MB-
    CCOP groups currently participate in sarcoma
    clinical trials . http ://www .cancer .gov/prevention/                                       5 0
    ccapiaEaout.htm l

    The Sarcoma Progress Review Group ( PRG )
                                                                        Percentage of Total Dollars by Scientif ic Are a
    and the Adolescent and Young Adult Oncology
                                                                                      Fiscal Year 2005
    (AYAO) PRG , two panels of prominent scientists                                                                            ; vant
    and patient advocates, assessed the state of the                                                               i

    science and identified future research priorities
    for sarcoma and other understudied cancers i n                  The Mamma lian Gene Collection provides full-
    adolescents and young adu l ts . http :llplanning               length open reading frame (FL-ORF) clones for
    .caneer .govipdrprgreports/2004sarcoma .pdf and                 many human genes, including those implicated
    EittpJ/pfanning .cancer .gov/disease/AYAO_PRG                   in the development of sarcoma . http ://mgc .nci .nih
    _Report_2 006_FI NAL .pdf                                       .9o v
•   NCI's D rug Deve lop me nt Group ( DD G) supports               The Director 's Challen ge : Toward a Molecular
    the oversight and preclinical/clinical development              Classi fication of Tumors includes two projects
    of therapeutics, including potential agents for the             focused on defining the molecular classification
    treatment of sarcoma . http ://dtp .nci .nih .gov/docs/         of sarcomas based on their genetic and molecular
    dd;tddg'%5Fcurrent . htm l                                      profiles . http ://dc .nci .nih .gov/organization/
•   C lin ical tria ls are actively recruiting patients with        principal l nvestigat.ors/DCabstracts
    sarcoma to test new treatments and treatment                    The Soft Tiss ue Sarcoma Home Pag e, the Bone
    combinations . For example, NCI's Pediatric                     Can cer Hom e Page , the Uterine Sarcoma Home
    Oncology Branch is conducting immunotherapy                     Page, and the Ewing's Famil y of Tumors Home
    trials to test tumor vaccines and stem cell                     Page provide up-to-date information on treatment,
    transplantation for pediatric sarcomas . http :i/tivww          prevention, genetics, screening, and testing for
    .cancer. gavlsearch/c! in icaltrial s                           various types of sarcoma . http ://www .cancer .gov!
• caMOD : NCI 's Can ce r Model s Database allows                   cancerinfoltypestsoft-tissue-sarcoma/ and http ://www
  researchers to submit and retrieve animal models         and http ://www
  of cancer, including several bone and soft tissue
  sarcoma mode l s . fittps ://cancerrrxodels .nci .ni}i .govt      littp ://www,ancerinfo/type,s/ewings /
  camod/login .do

Selected Opportunities for Advancement of Sarcoma Researc h
• Improve basic biologic research and understanding              • Develop new sarcoma-specific animal models,
  of host/patient biology in adolescent and young                  including models of metastatic disease .
  adult non-Kaposi's sarcomas .
                                                                 • Continue to identify and understand the molecules
    Develop comprehensive molecular approaches                     that contribute to sarcoma metastasis (such as the
    to sarcoma detection and treatment .                           gene ezrin) .

                                                                                                                 Last updated September 2006