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    OBJECTIVE.

    Adrenocortical Carcinoma (ACC)

     Rare  Aggressive tumor arising from the

    adrenal cortex with a large mass.

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    Adrenocortical carcinoma (ACC)

      detected at earlier stage

      typical clinical, biochemical, and imaging for rapid diagnosisand therapy

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    COC!"#$O

      C% and &R$ ' determining the extent of local anddistant tumor spread

      C% and &R$ ' guide surgery and further patientmanagement.

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    ACCs account for only .*.+ of all cancers or -*+ patients per - million population per year.

      children years old (fungsional)  individuals in their fourth and fifth decades of

    life(nonfungsional)

       /emale 0 &an

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    &ost ACCs are sporadic1 however, they alsocan be associated with several complexgeneticsyndromes.

      !i2/raumeni Cancer #yndrome

      Carney Complex

      3ec4with25iedemann #yndrome

      /amilial Adenomatous 6olyposis Coli

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    ACCs are  commonly in children than in adults. 

    variety of hormones androgens, cortisol,estrogens, and aldosterone.  Cushing syndrome

    ›  (/ig - )

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    /ig. -78+2year2old woman with cortisol2secreting right adrenocortical carcinomaresulting in Cushing syndrome.

    A, "nenhanced C% scan shows large low2attenuation suprarenal mass(arrowheads), with internal areas of highattenuation (arrows) consistent withhemorrhage.

    3, Axial %-2weighted &R image showshigh signal intensity (arrows) within

    right adrenal mass (arrowheads)consistent with hemorrhage.

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    patients present with a large massand symptoms related to mass efect :

     

    abdominal or ank pain  palpable mass  Metastatic to the regional and

    paraaortic lymph nodes, lung, liver,

    and bone

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    Separating benign rom malignant

    adrenal cortical neoplasms there aremacroscopic and microscopic criteria thatavor malignancy.

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    Macroscopic Criteria

      tumor wet weight !"" g  tumor with a grossly lobulated cut

    surace  #ecrotic areas  Calci$cation  %emorrhage in the tumor

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    Microscopic Criteria

      architectural disarray  mitotic rate,  marked nuclear pleomorphism  nuclear atypia 

    hyperchromasia  capsular invasion  venous or sinusoidal invasion.

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      large

      %omogeneous  &egion o low attenuation correspond to

    necrosis pathologically '(ig. ) * 

    tumors + cm necrosis

    CT

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      (ig. )/!0year0old woman withadrenocortical carcinoma.

    1, 2ortal venous phase C3 scanshows large heterogeneouslyenhancing let suprarenal massthat displaces let kidneyineriorly. &egions ononenhancing tissue 'arrows*are consistent with necrosis.

    4, 15ial 3)0weighted M& imageshows high signal intensity'arrows* consistent withnecrosis.

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    •Heterogeneous•0n T1- Isointense or slightlyhypointense

    •On T2- hyperintense

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    (ig. /)"0year0old woman withadrenocortical carcinoma.

    1 and 4, 15ial '1* and sagittal'4* contrast enhanced imagesshow large right suprarenal mass

    that is displacing inerior venacava medially and right kidneyineriorly. 3umor contains ecks ocoarse calci$cation 'black arrows,4* and macroscopic at '6)" %7*'white arrows* centrally.

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    (ig. 8!)0year0oldman with metastaticadrenocortical carcinoma.

    2ortal venous phaseC3 scan shows large,

    irregular right suprarenalmass ' black arrow * withenhancing tumorthrombus'arrowheads *e5tending into right renalvein and inerior venacava resulting in luminal

    $lling deect. 1t least twometastatic deposits 'whitearrows* are shown withinad9acent liver.

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    (ig. !+"0year0oldwoman withadrenocortical

    carcinoma '1CC*.

    1 and 4, 15ial 3)0weighted '1* andcoronal M&

    venography '4*images showlargeheterogeneouslet0sided 1CC'asterisk* with tumorthrombus e5tending

    into adrenal vein'arrow, 4* and ineriorvena cava 'arrow, 1*.

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    Fig. 6—50-year-old woman with right-sided adrenocortical carcinoma (ACC).Dedicated adrenal CT with washot stdies shows !.5-cm right s"rarenal mass.

     A# $nenhanced CT image shows that lesion (arrow) has attenation o% !& '$ and

    there%ore reires %rther characteriation. * and C# CT images o+tained 60

    seconds (*) and ,5 mintes (C) a%ter administration o% contrast material show

    lesion (arrow) has attenation /ales o% 5 and 1 '$# res"ecti/ely. These

    attenation /ales reslt in a+solte "ercentage washot o% 12 at ,5 mintes#ma3ing lesion indeterminate +y CT washot criteria. 4ass was con%irmed to +e

     ACC on histology.

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    Fig. —1-year-old man with right-sided adrenocortical carcinoma (ACC).

     A and *# Chemical shi%t imaging shows large irreglar right-sided lesion (arrow)

    that does not show signal loss +etween in-"hase (A) and ot-o%-"hase (*)

    images. Tmor was con%irmed to +e ACC on histology

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      1denoma  2heochromocytoma 

    ymphoma

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    );< o patients

    =malignant epithelial

    ung

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    (ig. >!)0year0old man with metastaticadrenocortical carcinoma '1CC*.

    16C, 15ial diagnostic contrast0enhanced C3image '1* and a5ial '4* and coronal 'C*

    used 2?3@C3 images show large rightadrenal mass 'arrow* with avid (ABuptake and multiple metabolically activehepatic metastases 'arrowheads*.

     A *

    C

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      #onspeci$c

      sot0tissue masses

      cystic changes with or withoutcalci$cation.

    nection

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     A#

    16-year-oldman who

    "resented with hy"ertension and

    %lshing. Arterial "hase CT image

    shows large heterogeneos

    enhancing le%t s"rarenal mass#

    which also showed intense

    metaiodo+enylganidine "ta3e (not

    shown). 4ass was con%irmed to +e

    "heochromocytoma a%ter srgery.

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    4, );0year0old woman whopresented with let ankpain and palpitations.2ortal venous phase C3image shows large letsuprarenal mass with

    regions o necrosis'arrows* seen centrally.Mass was con$rmed to becollision or compositetumor on pathologice5amination andcontained both

    pheochromocytoma andganglioneuroma tissue.

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    C, 8)0year0old man who

    presented withreractoryhypertension. 2ortalvenous phase imagethrough upperabdomen shows

    lobulated in$ltrativelet suprarenal mass.Mass was con$rmedto be benignganglioneuroma onpathologice5amination

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      Surgery

      Chemotherapy

      &adiotherapy

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      stage D 1CC have a median survival o /months.

      patients with stage and tumors have anappro5imately +!< !0year survival

      patients with stage and D disease have 8"<and E"< !0year survival.

      patients undergoing apparent completeresection, /!6>!< will develop recurrent ormetastatic disease.

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    Fig. ,0—5-year-old man with recrrent

    disease in right adrenalectomy +ed. 7rgical

    cli"s (arrows) are engl%ed +y a+normal

    enhancing so%t tisse in right adrenalectomy

    +ed. n%iltrati/e recrrent tmor (arrowheads)in/ades li/er and in%erior /ena ca/a.

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     3he imaging appearances o 1CC arediverse because o the variable presenceo necrosis, hemorrhage, calci$cation, and

    intracellular lipid content. 1s illustrated,other diseases can simulate 1CC, andamiliarity with both typical and atypicalappearances on cross0sectional imaging

    taken in con9unction with clinicalinormation helps to suggest the accuratediagnosis and appropriate treatment.

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    8