CANCERS BRIEFLY by hcj

VIEWS: 14 PAGES: 13

									CANCERS BRIEFLY
(all notes taken from Clinical Oncology, Rubin)

BLADDER CANCER
     Sixth most common CA in US
     Ninth leading cause of CA death in US
     38,300 dx in males; 14,900 dx in females in 2000
     median age at dx is 65 yo
     Risk factors include:
           o Smoking
                        50% of cases in men
                        33% of cases in females
           o aromatic amines
           o polycyclic aromatic hydrocarbons
           o some examples: dye, rubber manufacturing, diesel exhaust, dry cleaning, shoe making
           o cholorinated drinking water
           o water contaminated with fertilizers
           o phenacetin
           o cyclophosphamide
           o radiation exposure
           o schistosoma assoc with squamous cell carcinoma (remember the rest are transitional cell)
     Clinical s/sx
           o hematuria is most common
           o urgency or burning on urination
     Diagnostic procedures:
           o TURB
                        Transurethral resection of the bladder, which is essentially a biopsy
           o US
           o Intravenous pyelography
           o CT and MRI
           o Chest x-ray, because of the link to smoking
           o Bone scans in advanced CA, I guess this is because it spreads there?
     Histiopathology
           o Transitional cell carcinoma is by far the most common
           o Rhabdomyosarcomas most common in peds
           o Squam cell is 8%; endemic in Egypt
           o Adeno are less common; occur in urachus (remember, this is the median umbilical fold on
                the post aspect of the ant abdo wall and is where the baby pee pee-ed out of in the womb)
                or trigone of the bladder
     Staging
           o At DX 75% are superficial; 20% are invasive; 5% are metastatic
           o Superficial CA is managed with TURB and follow up with cytoscopy
           o Invasive CA is managed with cystectomy, radiation, and chemo; and any combo of the
                three
                        50% of these peeps die
                        a common surgery is a cystoprostatectomy and also taking out the urethra in a
                         technique termed urinary diversion
           o metastatic CA – median survival time is 1 yr
     Follow up: (some blood work that can monitor state of tumor load)
           o Bladder tumor antigen
           o VEGF
           o Telomerase
           o NMP22 – immunoassay for urinary nuclear matrix protein
            o   “among the best studied predictors of a reduced survival is the overexpression of the
                tumor suppressor gene TP53” (pg 536)
            o   retinoblastoma protein – another tumor suppressor gene that regulates progression
                through the cell cycle

PANCREATIC CANCER
     most common CA of the major digestive glands in the US
     in 2000, 28,300 cases; 28,200 died (only 100 survived!)
     majority occur between 60-80 yo
     Risk factors:
            o Cig smoking
            o High fat, high meat consumption
            o Alcohol consumption
            o Controversial info on coffee< (I’m drinking some as I type this, thank goodness)
            o Diabetes mellitus
            o Previous peptic ulcer surgery
            o Chronic pancreatitis
            o Genetic predisposition
     s/sx:
            o insidious onset
            o abdo or back pn
            o anorexia
            o early satiety
            o wt loss
            o dyspepsia
            o weakness
            o bloating
            o fatigue
            o nausea
            o vomiting
            o new-onset hyperglycemia
            o psychiatric disturbances (depression)
            o migratory thrombophlebitis
            o PE: jaundice, abdo mass, epigastric tenderness, palpable GB, hepatomegaly; however< at
                least 50% of all pts had no physical findings
            o Metastatic signs include: supraclavicular nodes (virchow’s), umbilical nodule (sister
                joseph nodes), peritoneal shelf on rectal exam (blumer’s shelf)
     Dx:
            o Biopsy
            o However, dx is often made indirectly by a biopsy of a metastatic site that reveals a
                histology compatible with pancreatic origin along with
                        Appropriate presentation
                        Mass detected in pancreas on US or CT or MRI or ERCP
                        Laproscopy
                        “helical CT remains the gold standard”
                        serology studies
                                   CA 19-9
                                        o This one is most useful and 85% sensitive
                                   CA 125
                                   CEA
     Histiopathology
            o Most common tumor is a solid adenocarcinoma
             o   50-60% of tumors arise in the head of the gland, the remaining are distributed evenly in
                 the body and the tail
             o tumor size is single most significant factor in prognosis – 2-3 cm is cutoff point
             o extension into the duodenum, stomach, retroperitoneally (remember, the tail goes retro!),
                 portal vv, para-aortic lymph nodes
        Metastasis
             o liver is most common
             o lung, brain, bone
        Treatment
             o Surgery is the only known curative modality
             o Small percentage of pts have temp palliation with chemo; 5-FU, mitocycin C; gemcitabine
             o Some more palliation
                         Biliary enteric bypass
                         Biliary stent
                         Celiac axis block (for pain relief)
                         Radiation for mets

HEPATOCELLULAR CARCINOMA
     15,300 cases in 2000
     however, HCC is one of the most common CA worldwide
     dx at 40 yo or >
     men > women
     geographically, Asia highest risk; then, Africa
     Risk Factors:
          o Hep C and B (sequence that we should all know is: “itis” acute then chronic, cirrhosis,
                then HCC)
          o Cirrhosis, irrespective of etiology
                        80% of HCC’s occur in cirrhotic livers
                        alcohol, esp when combined with smoking
          o aflotoxin
          o a radiocontrast agent, Thorotrast
          o anabolic steroids
          o OCPs
     Dx:
          o AFP!
                        Present in 75% of cases
                        Levels higher than 400 ng/mL indicates CA 95% of time
          o US
          o S/sx:
                        Hepatomegaly, ascites, pn, anorexia, wt loss, malaise, abdo mass, rarely jaundice,
                         unexplained elevated liver enzymes, RUQ pn, portal HTN, esopho varices,
                         splenomegaly
                        Some assoc pathology: hypoglycemia, hypertension, erythrocytosis,
                         hypercalcemia, hypercholesterolemia
          o Helical CT
          o Histiopathology
                        85-95% arise in liver cells (parenchymal, bile duct, etc)
                        the rest from bile duct
                        spread is to LUNG and BONE
     Treatment
          o Surgery is choice treatment (uhhh< when is it not?)
          o Factors to consider include presence of ascites, level of cirrhosis, functionality of the liver,
                etc<the better off a patient is, the more likely they will consider surgery
            o    5 yr survival rate is 30% ( for surgery )
            o    when liver is cirrhotic< they consider doing liver transplants< survival rates range from
                 49-62%
            o    compared with other cancers, CHEMO AND RADIATION ARE OF LIMITED
                 USEFULNESS
            o    some other weird treatments< just so that you have heard them:
                         percutaneous ethanol injections
                         transcatheter arterial chemoembolization
                         hormone therapy
                                    tamoxifen
                                    it has not been of much use, though
                         gene therapy



UTERINE CANCER
     epidemiology
          o 12,800 cases in 2000
          o 4,600 deaths
          o 65,000 cases of carcinoma in situ
          o mean diagnosis at age 52, however, cases range from 17-90 yo
                        so this means<. Screw the recommendations< paps til the day we die, ladies!
          o Squamous cell 85-90 percent; 10% adenocarcinoma
          o Epidermoid (which is squam cell) occurs more often in women who had first intercourse
               at an early age, have had multiple partners, or who have had multiple pregnancies
          o Other risk factors include
                        Hx of chlamydia, gonorrhea, syphilis, herpes, trich
                        Hpv
                        Hx of OCPs
                        DES
          o Etiology:
                        HPV 16, 18, 31, 33, 35, 39
                        16 is most common
                        18 causes more adenocarcinomas
                        when men have penile cancer (ewww) or hpv it increases our chances too
          o screening:
                        Pap, go for the posterior vaginal pool and endocervical too
                        s/sx: anything that smells of an infection, pelvic pn, back pn, urinary s/sx
                                  LBP suggests the poss of periaortic lymph node involvement with
                                   extension into the lumbosacral roots, or hydronephrosis
                        look for local spread by doing a bimanual
          o DX:
                        Colposcopy
                        Conization
                        Fractional curettage may be done
                        PE: supraclavicular nodes, abdo, liver, rectal exam, in particular
                        Chest X-ray
                        Intravenous pyelogram (a CT is a substitute)
     DES assoc with clear cell carcinomas
          o There may be benign changes as well, such as “cock’s comb,” adenosis, and transverse
               ridges
     Small cell carcinomas
          o Aggressive
     TREATMENTS
            o   Cervical intraepithelial neoplasia is usually treated with cryotherapy, LEEP, or laser
                therapy
            o   Surgery is limited to cancers in stage I and IIA
            o   The risk of myph node mets in lesions that have less than 3 mm of invasion is less than 1%
            o   These pts are treated with extrafascial hysterectomy without pelvic lymphademopathy
            o   Depending on childbearing status, sparing of organs is considered
            o   Radiation
                        Cure rates
                                  I: 85%
                                  II: 65%
                                  III: 45%
                                  IV: 25%
                        Most commonly, pts are treated with a combo of external and intracavitary
                         radiation
                        They are also inserting interstitial implants for radiation too
            o   Chemo:
                        Chemo is mainly reserved for advanced or disseminated disease and generally
                         takes the form of combination protocols including cisplastin

NONMELANOMA SKIN CANCER
    Most common and most preventable
    900,000 to 1.2 mil a year
    1200 deaths per year
    premalignant lesions include:
          o actinic keratoses
          o epithelial hyperplasia
          o leukoplakia
          o nevi
          o burn scars
    malignant lesions include:
          o basal cell carcinoma
          o bowen’s dz (intraepithelial carcinoma)
          o squamous cell carcinoma
          o keratoacanthoma (acute epithelial cancer)
          o melanoma
          o merkel cell carcinoma
          o connective tissue tumors
          o mycosis fungoides
          o malig lymphomas
          o KS
          o Secondary mets
    Risk factors:
          o Fair skin
          o Hx of sun exposure, esp blistering during childhood
          o Males > females
          o Burn CA occurs equally in blacks and whites
    Etiology
          o UVB light
                       Mechanism is DNA damage AND immune inhibition towards tumor in skin
          o Genetics
                       Xeroderma pigmentosum: an autosomal recessive defect in DNA repair
                       Basal cell nevus syndrome: autosomal dominant, assoc with multiple BCCs
                       Albinism: autosomal recessive
                     Epidermodysplasia verruciformis: autosomal recessive assc with widespread
                      HPV, leading to the development of warts that degen to SCCs
         o   Chemical exposure
                     Arsenic assoc with SCC
                     Chimney sweeps get scrotal cancer (bummer)
                     Cig smoking
         o   Immunosuppression
                     Remember that the skin is not only the biggest organ of elimination, but also of
                      protection (ie immunologic); without it, we’d be toast
                     Think about organ transplants as well as HIV
         o   Chronic inflammation or irritation
                     SCCs in burn scars (these guys are way more aggressive than regular SCCs)
                     Think about syphilis, lupus, granulomas, chronic ulcers
   DX
         o    Excisional biopsy
                      Diagnostic and therapeutic
                      Entire lesion is removed along with 0.5-2 cm of margin
        o Incisional biopsy
   Now for the list<
        o BCC
                      Most common
                      Most occur on the neck and head, nealy always on hair-bearing skin
                      Four types: nodular ulcerative (45-60%) (these are the ones that we are familiar
                       with), superficial (red, scaly macules), sclerosing (flat fibrotic, white-yellow
                       macules that become firm and depressed as they enlarge), pigmented (look like
                       MM)
                      Occur when basal cells fail to develop into keratinocytes
                      They burrow deep and may cause marked deformity (rodent ulcers)
        o SCC
                      Keratinocytes that invade the dermal/epidermal junction
                      May develop conical mounds of keratin called cutaneous horns
        o Bowen’s dz
                      Superficial, velvety, red-brown nodule or plaque
                      Mildly pruritic
                      Indolent and slow growing, however, they degen into invasive SCC
        o Keratoacanthoma
                      Domed papules with a central keratotic plug
                      Capable of rapid growth , considerable soft tissue destruction
                      May be assoc with SCC, but may also be benign and self-healing
        o Merkel cell
                      70-80 yo
                      neuroectodermal derivation
                      high rate of local recurrence and frequent nodal and distant mets
                      firm, pink-red nodules with an intact epidermis
        o mycosis fungoides
                      cutaneous t-cell lymphoma
                      also called Sezary syndrome
        o KS
                      Most common in elderly males of Mediterranean origin
                      You know that is is assoc with HIV
        o Mets
                      These guys are usually firm nodules with an intact epidermis
   Staging: just so you know, staging excludes carcinoma of the eyelid, vulva, and penis
       Treatment
            o Cure rate is around 90%
            o Surgery
                          Surgery: excision is the txt of choice for lesions < 3 cm
            o Some other treatments include: curettage with electrodesiccation, mohs’ surgery
                 (microscopically controlled so that the tumor is accurately mapped and meticulously
                 excised), cryotherapy, lasers
            o Radiation:
                          Large deep cancers are often irradiated
                          May have an advantage over surgery for small lesions on lip, eyelid, ear, nose,
                           lesions on cartilage or bone, multiple tumors, tumors with lymph node mets
                          The incidence of secondary radiogenic skin tumors with an area previously
                           irridated for skin CA is about 3.5%...and it takes an average of 13 years to
                           develop
            o Chemo:
                          Topical 5-FU is used most often for actinic keratoses
            o Systemic retinoids have been shown to induce regression but not cure
            o Alpha interferon for BCC and SCC
       Results:
            o BCC has an overall higher cure rate than SCC
            o SCC mets more often to regional lymph nodes
            o Recurrence rates
                          BCC: 5-10%
                          SCC: 25-30%
       Prevention: the book talks about this, but it’s so elementary that I can’t type it< I just can’t
            o The book, however, says that “diets low in fat have been reported to decrease the risk of
                 nonmelanoma skin cancer” yeah!

HODGKIN’S LYMPHOMA
    Epidemiology:
          o HD accounts for approx 12% of all lymphomas (malig ones)
          o 7400 diagnosed annually
          o age related peaks: in young adults and then in older adults
          o pts with congenital immunodeficiency have an increased risk
    Etiology:
          o If there is one thing we learned in med school, may it be the REED-STERNBERG CELL
               (they are also called lacunar cells, because the cytoplasm retracts from the nucleus on
               preparation leaving moons around the nucleus<)
                       The RS cell = a germinal center B-cell that has lost its ability to express Ig
          o We don’t know what causes it but here are the allopathic community’s thoughts<
          o Pts have a defective cellular immunity< hmmm?
          o There is some kind of relation to EBV< currently they call it a “cofactor”
    DX:
          o UNIFOCAL IN ORIGIN
          o PAINLESS LYMPH NODE ENLARGEMENT
          o s/sx
                       1/3 have constitutional s/sx such as fever, malaise, night sweats, wt loss
                       cervical and supraclavicular node involvement in 60-80% of pts
                       3-10% present with isolated subdiaphragm involvement, usually with inguinal
                        lymphadenopathy, or less commonly, an abdo mass
          o Biopsy of the node
          o Some imaging includes: chest X-ray, chest CT scan, abdo and pelvic CT scan, MRI +
               gadolinium, bipedal lyphangiogram (LAG) is for retroperitoneal lymph node assessment
           o     LABS:
                          CBC
                          Leukocytosis, normocytic normochromic anemia, and eosinophilia may occur
                          Alk phos may indicate liver or bone involvement
                          Hypergammaglobulinemia (hypo is seen in more advanced cases)
           o Bone marrow biopsy to assess involvement
      Classification:
           o There are four subtypes:
                          Nodular sclerosis: 75%
                          Lymphocytic predominance: 5%
                          Mixed cellularity: 15-20%
                          Lymphocyte depletion: the rest..
                                    Rare and aggressive
                          From class: seeing leukocytes is a good sign, fever and weight loss is ominous
      Treatment:
           o Surgery not really used except for dx
           o Radiation therapy:
                          Cure rate: (10 year) is 70-80%
                          Rate from freedom from relapse is 70-80%
                          They use an ass-load of radiation so complications abound – like pneumonitis,
                           pericarditis, menstrual dysfunction, aspermia, clinical hypothyroid (they say it’s
                           rare at 5-15%...what?) and subclinical compensatory hypothyroid (30-60%)
                          Secondary tumors from radiation – risk is 6 fold
           o Chemo:
                          MOPP is used
                          ABVD as well
           o A combo approach is most widely used
           o Bone marrow transplant is used for pts with advanced dz
           o Relapse: occurs most often within four years
      PEDIATRIC HD
           o He didn’t talk about this at all in class. The book has some interesting things to say< SO
                 this is what I think is most important.
                          Way higher male: female ratio
                          Most common in adolescence
                          Role of EBV is well established
                          They are way more careful with radiation and use lower doses because of pesky
                           sequellae like profound musculoskeletal retardation, shortened sitting height,
                           decreased mandibular growth, decreased mm development
                          So the preferred txt is low dose radiation and chemo (MOPP is most common)
                          The younger the age, the better the prognosis

NON-HODGKIN’S LYMPHOMA
    55,000 dx in 2000
    26,000 died
    peak incidence is in > 50 yo
    men slightly > women
    Etiology:
          o Association with viral infections:
                       HTLV-1
                       HIV
          o Translocation of the oncogene c-myc in BURKITT’S LYMPHOMA
          o Exposure to herbicides
    Different types include:
             o   B CELL
                         These include burkitt’s and non burkitt’s type
                         10% of lymphoblastic lymphomas total
                         MALT are of b cell origin
             o   T CELL
                         Almost all have the enzyme terminal deoxynucleotidyl transferase, a marker for
                          apoptosis
                         90% of lymphoblastic lymphomas
                         Remember mycosis fungoides
       Growth fractions = can equal 100% in some lymphomas (scary)
       DX:
            o Noncontiguous spread
            o Same s/sx as HD
            o Must be done from biopsy
            o Aspiration of bone marrow is required in all cases b/c of the frequent presence of bone
                 marrow involvement
            o CBC
            o Chest x ray, bone scan, gallium whole body
       TXT:
            o “chemo is the mainstay of treatment for the majority of patients with NHL because the
                 majority of pts present as systemic dz”
            o the most common drugs used in the management of the NHL include single alkylating
                 agents and CVP for the low grade lymphomas, CHOP for the diffuse large cell ones, and
                 leukemia-like regimens for lymphoblastic and burkitt’s lymphoma
       Results:
            o For low grade stage III or IV, survival rates are poor 20-40% at 10 years
            o For intermediate grades, 5 year survival rates are 40-55%
            o Aggressive high grade lymphomas are relatively rare in adults
            o Staging is way less important at predicting prognosis (vs HD) than histiological type<
                 check out graph on page 325 for staging vs histo as predictors< but all are a steady
                 decline down to around 20-40 % survival at 15 years out

MULTIPLE MYELOMA
      2000 dx in 2000
      mean age is 62 yo
      etiology:
            o high dose radiation from atomic bomb exposure<nice
            o genetic susceptibility
            o c-myc translocation
            o IL-6 acts as a paracrine growth factor as well as an inhibitor of apoptosis of tumor cells
      DX:
            o s/sx:
                        bone pn accompanied by anemia
                        hypercalcemia
                        renal insufficiency
                        bence-jones proteinuria
                        pt are at an increased risk for bacterial infection
            o osteolytic lesions
            o elevated serum or urine M-protein
            o marrow plasmocytosis
            o monoclonal serum gammopathy
      Txt:
            o Radiation:
                           It’s used to palliate and to txt solitary bone tumors or isolated extramedullary
                            sites
              o    Chemo:
                             Survival goes from 3-4 years max with chemo to 1 year (median) survival
                              without chem.
                             Melphalan is the most commonly used alkylating agent
                             Interferon
                                       The two txt above improve response rates but do not improve survival
                                        time
                             VAD
                             VBCMP
              o    Bone marrow transplant
                             It did prolong survival time by a couple of years
              o    All in all, txts can prolong life span to about 4 years

LUNG CANCER
     15% for men and 13% for women: total cancer types
     over all 5 year survival is lower than 14%
     in 2000, 164,100 new cases
     156,900 deaths per year
     1,037,000 mil new cases a year worldwide
     epidemiology:
           o average age of onset 60 yo
           o rising incidence rate of women in the 1990s
           o ¼ of cases in non smokers seems to be due to second hand smoke
                        higher nitrosamine levels and smaller particles make it worse
     etiology
           o tobacco smoke
           o asbestos
                        mesotheliomas
                        bronchogenic carcinoma
           o atmospheric pollution
           o metals
           o chemical products
           o oncogenes
                        kras and myc
           o tumor suppressor genes
                        RB
                        Tp53
                        CDKN2
     Detection:
           o “a change in pulmonary habits is the most significant sign of lung cancer”
           o S/sx
                        Some of the less common include SVC obstruction, shoulder or arm pn with
                         brachial plexus involvement, recurrent pleural effusions and pneumonitis,
                         cardiac arrhythmia or failure, hoarseness (remember that recurrent laryngeal nn
                         paralysis)
                        PARANEOPLASTIC SYNDROMES
                                   METABOLIC
                                        o Cushing’s
                                        o Hypercalcemia
                                        o Excessive ADH
                                        o Carcinoid syndrome
                               NEUROMUSCULAR
                                   o Peripheral neuritis
                                   o Cortical or cerebellar degen
                                   o myopathy
                               DERMATOLOGIC
                                   o Acanthosis nigricans
                                   o dermatomyositis
                               SKELETAL
                                   o Hypertrophic osteoarthropathy, including clubbing of the
                                      fingers
                               VASCULAR
                                   o Migratory thrombophlebitis
                                   o Nonbacterial verrucous endocarditis
                               HEMATOLOGIC
                                   o Anemia
                                   o DIC
   Dx:
          o Xray
          o CT
          o MRI
          o Fiberbronchoscopy
          o Brush biopsy under videoscopy
          o Cytology studies include sputum examinations
          o Bronchial washings: these are not as good as cytology studies
          o Percutaneous needle biopsy
                    They admit a risk of seeding along the needle tract
        o Tumor markers
                    CEA is the gold standard for non small cell CA
                              It’s concentration correlates to tumor extent
                    CYFRA for squamous cell carcinoma
                    NSE (neuron specific enolase) for small cell CA
        o FOUR MAJOR HISTIOLOGIC TYPES
                    SCC
                              Most common in the world
                              Least lethal
                    Adenocarcinoma
                              Most common in the US
                              Tends to be located more in the periphery but it mets widely to the
                               other lung, liver, bone, kidney, and CNS
                    Large cell anaplastic
                              Mets like adeno does
                    Small cell
                              Tends to be disseminated at dx
                              Aggressive/rapidly growing
   Treatment
        o Surgery
                     For localized non small cell CA, surgery is the txt of choice (stages I and II)
                    at DX, 1/3 of pts are inoperable
        o immunotherapy
                    BCG, a strain of mycobacterium was used but now studies show inefficacy
                    Same thing with interferon
        o Radiation
                    Palliative
                      Survival rates (5y) are 4-9% (impressive)
             o   Chemo
                      For NSCLC, alkylating agents have a detrimental effect on survival while
                       regimens containing cisplastin have a modest benefit (we’re talking single
                       percentage points here)
                      For SCLC, chemo has been the main therapeutic modality for the last 15 years
       Results
            o For Non Small Cell, overall survival rates (5y) are 5-10%
            o For Small Cell, median survival rates are at about 1 year

OVARIAN CANCER
     In 2000, 23,100 diagnosed
     14,000 deaths per year
     most malignant female cancer
     >70% of cancers present at stage III or IV
     avg. age is 65 yo
     most are epithelial, although in young women the majority are of germ cell origin
     etiology
           o risks:
                        multiparity
                        OCP
                        Breast feeding
                        Early first pregnancy
                        Previous hysterectomy
                        Talcum powder
                        Asbestos
                        Genetic in 10%
                                  Inheritable CA seems to affect women 10-20 years earlier than sporadic
                                   cancer
                                  Autosomal dominant with penetrance
                                  Lynch II syndrome
                                  There is some link to BRCA 1 (and a possibility of link to 2)
     DX:
           o US, transvaginally
           o Colonoscopy, to r/o colon cancer (I can see why, with the s/sx)
           o Color flow Doppler (to assess ovarian vascular changes)
           o CA-125, better for response to txt and not for dx
           o S/sx: remember that most women have none until it has mets everywhere
                        Pelvic pn
                        Bloating
                        Irregular bowel habits
                        Nausea
                        Dyspnea
                        Early satiety
                        Abdo distention
                        Adnexal mass is the most common sign, esp in post menopausal women
     Histopathology
           o 80-90% are epithelial in origin
           o clear cell are a type of epithelial assoc with DES
           o 10% are germ cell
     Treatment
           o Surgery is optimal, but so few pts have early stage CA at dx
             o   They debulk the tumors as much as possible as well as cryoreductive surgery with the
                 same goal in mind
             o   Radiation therapy, in trials, has really shown no benefit
             o   Chemo
                        Cisplastin has been shown to be the most active agent
                        Studies really don’t show much hope in chemo either

SOFT TISSUE SARCOMA
      FROM MESENCHYMAL NOT EPITHELIAL TISSUE
      Pts with soft tissue masses should undergo biopsy always!
      Epidemiology
            o In 2000, 8100 arose from soft tissue, 2500 from bones and joints
      Etiology
            o Genetic
                         Assoc with familial polyposis
                         Retinoblastoma
                         Neurofibromatosis type 1
                         Li-fraumeni syndrome
            o Chemical and radiation exposure
            o “an interesting feature of soft tissue sarcomas is that some of these tumors display stable
                chromosomal translocations”
      DX:
            o Pts most often present with a painless mass
            o Frequently pts recall a hx of a traumatic event to the area
            o Sarcomas grow by local extension, infiltrate adjacent tissue, and extend along tissue
                planes; they rarely traverse major fascial planes or bones
            o LUNG is the first site of mets (>50%) (exception is in liposarcomas)
      TXT:
            o Surgery is considered the primary treatment
                         Amputation
            o Radiation is rarely used alone, but almost always used with surgery
            o Brachytherapy
                         Inserting catheters in the pt that will be filled with radiation
            o Chemo is used for mets and inoperable tumors
      Results:
            o “overall, txt for soft tissue sarcomas with limb-sparing techniques can now achieve
                excellent local control rates when they are combined with adjunctive radiation (>90), but
                the 25-50% distant failure rate requires the use of a systemic therapy.”
            o With radiation, there is complication with wound healing (esp with pre op radiation)
            o Chemo reduces mets but has little effect on survival rates in the end

								
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