Absite Review Series.ppt - SUNY Downstate Medical … · ABSITE Review Series: Series: Cystosarcoma...

ABSITE Review ABSITE Review ABSITE Review ABSITE Review Series: Series: Series: Series:

CystosarcomaCystosarcomaCystosarcomaCystosarcomaPhyllodesPhyllodes and and yy

DCISDCISAudrey C. Durrant, M.DAudrey C. Durrant, M.Dy ,y ,

December 5, 2008December 5, 2008

www.downstatesurgery.org

Cystosarcoma PhyllodesCystosarcoma PhyllodesCystosarcoma PhyllodesCystosarcoma Phyllodes

Johannes Muller 1838 first to coin Johannes Muller 1838 first to coin cystosarcoma phyllodescystosarcoma phyllodesJJ y p yy p yDenoting cystic appearance and leafDenoting cystic appearance and leaf--like growth patterns of large tumorlike growth patterns of large tumor

Most common nonMost common non--epithelial neoplasm of the breast epithelial neoplasm of the breast Accounts for 0.5Accounts for 0.5--1% all breast carcinomas1% all breast carcinomasExclusive disease of womenExclusive disease of womenCan occur women all ages Can occur women all ages –– includes adolescents and elderlyincludes adolescents and elderlyMajority in 4Majority in 4thth decade of lifedecade of life



Stromal tumors well circumscribedStromal tumors well circumscribedTypically quite large Typically quite large –– mean diameter 4mean diameter 4--5 cm5 cmPresent as painless massPresent as painless mass

SolitarySolitaryyyDiscreteDiscretemobilemobile

Mammographically indistinguishable from fibroadenomasMammographically indistinguishable from fibroadenomasg p y gg p y gMajority are benign Majority are benign 1010--40% take malignant course40% take malignant courseDecision to perform excision biopsy based on : Decision to perform excision biopsy based on : Decision to perform excision biopsy based on : Decision to perform excision biopsy based on :

Large tumor sizeLarge tumor sizeHistory rapid growthHistory rapid growthPatient agePatient agePatient agePatient age



Malignancy based on Malignancy based on g yg yHigh mitotic rate (>10 mitotic figures per 10 HPF)High mitotic rate (>10 mitotic figures per 10 HPF)Infiltrative bordersInfiltrative bordersAreas of stromal overgrowthAreas of stromal overgrowthAreas of stromal overgrowthAreas of stromal overgrowth

Stromal overgrowth most impt predictor of Stromal overgrowth most impt predictor of aggressive behaviouraggressive behaviourTend to recur regardless of malignancyTend to recur regardless of malignancyDepending on series Depending on series –– local recurrencelocal recurrence

55 15% for benign tumors15% for benign tumors55--15% for benign tumors15% for benign tumors2020--30% for malignant tumors30% for malignant tumors


Cystosarcoma Phyllodes Cystosarcoma Phyllodes TreatmentTreatment

Surgical therapy aimed at complete excision with at least 1cm Surgical therapy aimed at complete excision with at least 1cm g py pg py pmarginsmarginsEither wide local excision or simple mastectomyEither wide local excision or simple mastectomyGeiser et al,Geiser et al, at University of Oklahoma Health Science Center, at University of Oklahoma Health Science Center, Tulsa, reviewed all cases of phyllodes treated at their institution Tulsa, reviewed all cases of phyllodes treated at their institution over 24 yrs and found an increased recurrence of all tumors over 24 yrs and found an increased recurrence of all tumors over 24 yrs and found an increased recurrence of all tumors, over 24 yrs and found an increased recurrence of all tumors, benign or malignant, when margins were positivebenign or malignant, when margins were positiveStrongest indicator of local recurrence is a positive marginStrongest indicator of local recurrence is a positive marging p gg p g


Cystosacroma PhyllodesCystosacroma PhyllodesCystosacroma PhyllodesCystosacroma Phyllodes

Local recurrence salvageable with total mastectomyLocal recurrence salvageable with total mastectomy

For all phyllodes tumors, regardless of size, reported rates distant For all phyllodes tumors, regardless of size, reported rates distant metastasis for malignant tumors 25metastasis for malignant tumors 25--40%40%

axillary nodal metastases in phyllodes tumors is <1% axillary nodal metastases in phyllodes tumors is <1% -- No need for ALNDNo need for ALND

Metastases when they occur are most common to:Metastases when they occur are most common to:yyLung (most common)Lung (most common)BoneBoneMediastinumMediastinum

No role for radiation therapy, chemotherapy or hormonal therapy has No role for radiation therapy, chemotherapy or hormonal therapy has been established for this diseasebeen established for this disease


Ductal Carcinoma in Ductal Carcinoma in Situ (DCIS)Situ (DCIS)

Incidence DCIS increasing since introduction Incidence DCIS increasing since introduction Incidence DCIS increasing since introduction Incidence DCIS increasing since introduction screening mammogram c.f. older series when DCIS screening mammogram c.f. older series when DCIS remained undetected until it became palpableremained undetected until it became palpableDCIS now accounts for approximately 20% of all DCIS now accounts for approximately 20% of all newly diagnosed breast cancersnewly diagnosed breast cancersMedian age reported for patients with DCIS 47Median age reported for patients with DCIS 47--63 yrs63 yrsRelative rates invasive ductal carcinoma remain Relative rates invasive ductal carcinoma remain relatively constantrelatively constant


DCISDCISPathologyPathology

DCIS represents DCIS represents h t h t heterogeneous group heterogeneous group histiologic changes of histiologic changes of varying malignant potentialvarying malignant potentialAll h t i d b All h t i d b All characterized by All characterized by proliferation of malignant proliferation of malignant epithelial cells within epithelial cells within breast ducts without breast ducts without breast ducts without breast ducts without invasion through basement invasion through basement membranemembraneMay be associated May be associated May be associated May be associated inflammatory reaction, inflammatory reaction, stromal response or stromal response or lymphoid infiltrationlymphoid infiltrationy py p



Generally classified as 1 of 5 subtypes:Generally classified as 1 of 5 subtypes:y ypy ypComedoComedoSolidSolidCribiformCribiformMicropapillaryMicropapillarypapillarypapillarypapillarypapillary

Based on differences architectural pattern cancer Based on differences architectural pattern cancer cells and nuclear featurescells and nuclear featuresDCIS broadly characterized as comedo and non DCIS broadly characterized as comedo and non comedocomedo



N d ll b h l k l N d ll b h l k l Noncomedo: all subtypes that lack central Noncomedo: all subtypes that lack central necrotic cellular debrisnecrotic cellular debrisComedo necrosis correlates with increased Comedo necrosis correlates with increased risk of local recurrence and invasionrisk of local recurrence and invasionNuclear features used to classify lesions Nuclear features used to classify lesions as low, intermediate or high gradeas low, intermediate or high grade, g g, g g


DCISDCISNatural HistoryNatural History

DCIS is a precursor of invasive breast cancerDCIS is a precursor of invasive breast cancerDCIS is a precursor of invasive breast cancerDCIS is a precursor of invasive breast cancer

N t ll DCIS ill b b t N t ll DCIS ill b b t Not all DCIS will become breast cancerNot all DCIS will become breast cancer

However, most women with lowHowever, most women with low--grade DCIS grade DCIS left untreated will eventually develop invasive left untreated will eventually develop invasive y py pbreast cancer at the same sitebreast cancer at the same site


DCIS DCIS PresentationPresentation

Usually found by presence Usually found by presence y y py y pmicrocalcifications on microcalcifications on screening mammographyscreening mammographyC l ifi ti th t t C l ifi ti th t t Calcifications that represent Calcifications that represent DCIS usually linear or DCIS usually linear or branching patternbranching patternMay present as palpable May present as palpable mass, mammographic mass mass, mammographic mass or bloody nipple dischargeor bloody nipple dischargeor bloody nipple dischargeor bloody nipple discharge


DCISDCISDiagnosisDiagnosisDiagnosisDiagnosis

Stereotatic biopsyStereotatic biopsyM di d bi b i d b M di d bi b i d b Mammogram directed biopsy obtained by Mammogram directed biopsy obtained by radiologistradiologist

Core needle biopsyCore needle biopsyRadiologist or surgeon use hollow needle to Radiologist or surgeon use hollow needle to remove tissue sample with palpable massremove tissue sample with palpable mass

Surgical biopsySurgical biopsy (wide local excision or (wide local excision or lumpectomy)lumpectomy)

If core needle or stereotactic biopsy show If core needle or stereotactic biopsy show areas atypical hyperplasia or confirm DCIS or areas atypical hyperplasia or confirm DCIS or inconclusive in face highly suspicious inconclusive in face highly suspicious mammogrammammogramPerformed with assistance or perPerformed with assistance or per--operative operative needle localization of mammographic needle localization of mammographic abnormalityabnormalityBx should aim for negative margins Bx should aim for negative margins –– approx 1 approx 1 cmcmcmcm


Mastectomy vs Breast Mastectomy vs Breast Conservation SurgeryConservation Surgery

In past DCIS treated with MRMIn past DCIS treated with MRMIn past DCIS treated with MRMIn past DCIS treated with MRMRationale was based on high incidence of Rationale was based on high incidence of multifocality and multicentricitymultifocality and multicentricityy yy yCurrent recommendations advocate BCSCurrent recommendations advocate BCSLumpectomy followed by radiation therapy most Lumpectomy followed by radiation therapy most Lumpectomy followed by radiation therapy most Lumpectomy followed by radiation therapy most common treatment for DCIScommon treatment for DCISRandomized trials have indicated that the addition Randomized trials have indicated that the addition of tamoxifen decreased contralateral breast cancer of tamoxifen decreased contralateral breast cancer and ipsilateral recurrence in patients with invasive and ipsilateral recurrence in patients with invasive cancercancer


Mastectomy vs BCSMastectomy vs BCSMastectomy vs BCSMastectomy vs BCS

Mastectomy indicated Mastectomy indicated Mastectomy indicated Mastectomy indicated Diffuse malignant appearing calcificationsDiffuse malignant appearing calcificationsPersistent positive margins after attempted BCSPersistent positive margins after attempted BCSPersistent positive margins after attempted BCSPersistent positive margins after attempted BCSLarge (i.e. >3cm) high grade DCISLarge (i.e. >3cm) high grade DCISP ti t fP ti t fPatient preferencePatient preferenceRadiation contraindicatedRadiation contraindicated


DCISDCISOutcomesOutcomes

In general overall survival rate excellentIn general overall survival rate excellentIn general overall survival rate excellentIn general overall survival rate excellentWeng et al. reported overall survival rate of Weng et al. reported overall survival rate of 97% at 8 yrs97% at 8 yrs97% at 8 yrs97% at 8 yrsLocal recurrence rates in this series were 25% Local recurrence rates in this series were 25% for pts with DCS treated with BCS onlyfor pts with DCS treated with BCS onlyfor pts with DCS treated with BCS onlyfor pts with DCS treated with BCS only

13% for BCS and radiation13% for BCS and radiation4% for those treated with mastectomy4% for those treated with mastectomyyy

About 50% pts with recurrences after DCIS About 50% pts with recurrences after DCIS have invasive tumorshave invasive tumors


SurveillanceSurveillanceSurveillanceSurveillance

Following BCS: post surgical mammogram should be Following BCS: post surgical mammogram should be Following BCS: post surgical mammogram should be Following BCS: post surgical mammogram should be obtained to screen for residual microobtained to screen for residual micro--calcificationscalcificationsMammogram should be obtained 4Mammogram should be obtained 4--6 months after 6 months after ggcompletion of radiation therapy to establish new completion of radiation therapy to establish new baselinebaselineF/U of pts after BCS and radiation should include F/U of pts after BCS and radiation should include twice yearly PE and annual mammogram for first 5 twice yearly PE and annual mammogram for first 5 yearsyearsAnnual PE and mammogram thereafterAnnual PE and mammogram thereafter


Surveillance (cont’d)Surveillance (cont’d)Surveillance (cont d)Surveillance (cont d)

Both pts treated with BCS and mastectomy Both pts treated with BCS and mastectomy Both pts treated with BCS and mastectomy Both pts treated with BCS and mastectomy should be monitored closely for new primary should be monitored closely for new primary cancers in the contralateral breastcancers in the contralateral breastcancers in the contralateral breastcancers in the contralateral breastRisk that new primary cancer will develop in Risk that new primary cancer will develop in contralateral breast after treatment for DCIS contralateral breast after treatment for DCIS contralateral breast after treatment for DCIS contralateral breast after treatment for DCIS is 2is 2--5 times the risk of development of a first 5 times the risk of development of a first primary breast cancerprimary breast cancerprimary breast cancerprimary breast cancer


QuestionsQuestionsQuestionsQuestions

11 Phyllodes tumors:Phyllodes tumors:1.1. Phyllodes tumors:Phyllodes tumors:a)a) Present in postmenopausal womenPresent in postmenopausal womenb)b) Are often malignantAre often malignantb)b) Are often malignantAre often malignantc)c) Require mastectomy because of their high Require mastectomy because of their high

recurrence raterecurrence raterecurrence raterecurrence rated)d) Tend to recurTend to recur)) Are responsive to hormonal manipulationAre responsive to hormonal manipulatione)e) Are responsive to hormonal manipulationAre responsive to hormonal manipulation



22 All of the following is true about Cystosarcoma All of the following is true about Cystosarcoma 2.2. All of the following is true about Cystosarcoma All of the following is true about Cystosarcoma phyllodes of the breast phyllodes of the breast exceptexcept::

a)a) Is the most common primary breast sarcomaIs the most common primary breast sarcomaa)a) Is the most common primary breast sarcomaIs the most common primary breast sarcomab)b) Treatment is local excision with margin of normal tissueTreatment is local excision with margin of normal tissue)) Only 10% are malignantOnly 10% are malignantc)c) Only 10% are malignantOnly 10% are malignant

d)d) Lymph node dissection is not indicatedLymph node dissection is not indicatedI th l ti t th i i I th l ti t th i i e)e) In the male patient the prognosis is worseIn the male patient the prognosis is worse



3.3. Which of the following statements is incorrect Which of the following statements is incorrect ggregarding ductal carcinoma in situ?regarding ductal carcinoma in situ?

a)a) BreastBreast--conservation therapy should be done for conservation therapy should be done for localized disease, particularly non comedo varietylocalized disease, particularly non comedo variety, p y y, p y y

b)b) Axillary lymph node dissection is not necessaryAxillary lymph node dissection is not necessaryc)c) The most common clinical presentation is a The most common clinical presentation is a

palpable mass palpable mass palpable mass palpable mass d)d) After breastAfter breast--conserving surgery, radiotherapy is conserving surgery, radiotherapy is

administered in tangential fields to the whole administered in tangential fields to the whole breastbreast

e)e) There is no role for chemotherapy in the treatment There is no role for chemotherapy in the treatment of DCISof DCIS



4.4. Which of the following treatments is best for a 40 Which of the following treatments is best for a 40 4.4. Which of the following treatments is best for a 40 Which of the following treatments is best for a 40 yr old woman with extensive microcalifications yr old woman with extensive microcalifications involving the entire upper aspect of the right involving the entire upper aspect of the right breast and biopsy that show a comedo pattern of breast and biopsy that show a comedo pattern of DCIS?DCIS?

a)a) Local excision aloneLocal excision aloneb)b) Irradiation aloneIrradiation alone)) Local excision plus irradiationLocal excision plus irradiationc)c) Local excision plus irradiationLocal excision plus irradiation

d)d) Right total mastectomyRight total mastectomye)e) Right total mastectomy followed by irradiationRight total mastectomy followed by irradiatione)e) Right total mastectomy followed by irradiationRight total mastectomy followed by irradiation


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