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Case 2 - iap-yhdistysavain-fi-bin.directo.fi · Case 2 Risto Pirinen, MD, PhD Dept of Clinical...

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11/28/07 11/28/07 1 Case 2 Case 2 Risto Pirinen, MD, PhD Risto Pirinen, MD, PhD Dept of Clinical Pathology Dept of Clinical Pathology Kuopio University hospital Kuopio University hospital Kuopio, Finland Kuopio, Finland
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11/28/0711/28/07 11

Case 2Case 2

Risto Pirinen, MD, PhDRisto Pirinen, MD, PhD

Dept of Clinical PathologyDept of Clinical Pathology

Kuopio University hospitalKuopio University hospital

Kuopio, FinlandKuopio, Finland

Patient historyPatient history

!! 79 year old woman, cleaner79 year old woman, cleaner

!! Rheumatoid arthritis, high blood pressure,Rheumatoid arthritis, high blood pressure,osteoporosisosteoporosis

!! Family:Family:–– 1 sister died because of lung cancer, another1 sister died because of lung cancer, another

because of lymhomabecause of lymhoma

!! 10/2005 -> abdominal pain, fewer,10/2005 -> abdominal pain, fewer,inflammation parameters inflammation parameters !!!!

Clinical dataClinical data

!! Gastroscopy & colonoscopy: Gastroscopy & colonoscopy: ØØ

!! Mammography: Mammography: Ø, Thorax rtg: Ø, no pleuralØ, Thorax rtg: Ø, no pleuraleffusioneffusion

!! Gynecological examination & UÄ: Gynecological examination & UÄ: Ø, no anyØ, no anysigns of ovarial tumourssigns of ovarial tumours

!! Abdominal CT: ØAbdominal CT: Ø

!! Diverticulitis ?Diverticulitis ?

Clinical data (2)Clinical data (2)

!! 12/05: prolonged symptoms 12/05: prolonged symptoms !!

!! Controlled abdominal CT : ascites Controlled abdominal CT : ascites !!,,suspicion of peritoneal carcinosissuspicion of peritoneal carcinosis

!! Unknown primary tumour ->Unknown primary tumour ->–– Diagnostic laparoscopy and biopsiesDiagnostic laparoscopy and biopsies

LaparoscopyLaparoscopy

!! Diffuse peritoneal carcinosisDiffuse peritoneal carcinosis

!! Tumour nodules in the mesenterium of theTumour nodules in the mesenterium of thesmall intestinesmall intestine

!! Abundant tumoural infiltration in the omentalAbundant tumoural infiltration in the omentalplateplate

!! AdhesionsAdhesions

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Diagnosis ?Diagnosis ?

LMW CK

CK 5/6

CK 7

CALRETININ

CA 19-9 AB-PAS

CEA BER-EP4

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Diagnosis ?Diagnosis ?

SummarySummary

!! Infiltrative tumour,Infiltrative tumour,epitheliod morphologyepitheliod morphology

!! Small papillary andSmall papillary andtubular structurestubular structures

!! Nuclear pleomorphism,Nuclear pleomorphism,multi-nucleated cellsmulti-nucleated cells

!! NecrosisNecrosis

!! no cell-associatedno cell-associatedmucinsmucins

!! Immunohistochemistry:Immunohistochemistry:

–– Positive:Positive:!! High/low mol weightHigh/low mol weight

cytokeratins, CK 5/6,cytokeratins, CK 5/6,CK 7, CK 19, calretinin,CK 7, CK 19, calretinin,vimentinvimentin

–– Negative:Negative:!! CK20, Melan-A, PLAP,CK20, Melan-A, PLAP,

CD15, CEA, CA19-9,CD15, CEA, CA19-9,Ber-EP4Ber-EP4

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Primary malignant mesotheliomaPrimary malignant mesotheliomaof the abdominal cavityof the abdominal cavity

Follow-upFollow-up

!! Patient died 2/2006, symptomatic treatmentPatient died 2/2006, symptomatic treatment

!! Forensic examination because of assumedForensic examination because of assumedasbestos exposureasbestos exposure

Forensic examination and autopsy:Forensic examination and autopsy:

–– Large tumoural mass in abdominal cavityLarge tumoural mass in abdominal cavity

!! Surrounding the large- and small intestine,Surrounding the large- and small intestine,liver and spleenliver and spleen

!! No infiltration to the other organs, orNo infiltration to the other organs, ormetastatic spreadmetastatic spread

!! No pleural plaques or tumoursNo pleural plaques or tumours

–– PneumoniaPneumonia

–– A lung sample for asbestos analysisA lung sample for asbestos analysis

Histopathological findings fromHistopathological findings fromautopsyautopsy

!! Abdominal cavity: epithelioid mesotheliomaAbdominal cavity: epithelioid mesothelioma

!! Lung:Lung:

–– Severe pneumoniaSevere pneumonia

–– fibrosis, no honeycombingfibrosis, no honeycombing

–– Asbestos bodies Asbestos bodies ~ ~ 2-10 / HPF2-10 / HPF

!! Other organs: Other organs: ØØ

!! No any signs of metastatic diseaseNo any signs of metastatic disease

Additional information:Additional information:

–– Patient lived in Patient lived in ””PaakkilaPaakkila””, a little village in, a little village inEastern Finland (at the age 6-14y)Eastern Finland (at the age 6-14y)

!! Asbestos mining 1918-1975Asbestos mining 1918-1975

!! The apartment was 0.5 km from the mine, inThe apartment was 0.5 km from the mine, inwhich a mother of the patient was workingwhich a mother of the patient was working

–– No working history in mineNo working history in mine

–– From y. 1962 as a cleaner and work manager,From y. 1962 as a cleaner and work manager,occupational exposure from the pipework isoccupational exposure from the pipework ispossible (boiler rooms etc.), but is not enough topossible (boiler rooms etc.), but is not enough toexplain the count of antophyllite accumulationexplain the count of antophyllite accumulation

–– A latency period from the exposure in childhoodA latency period from the exposure in childhood!! 32 milj asbestos / g, anthopfyllite32 milj asbestos / g, anthopfyllite

11/28/0711/28/07 2222

Case 1Case 1

Risto Pirinen, MD, PhDRisto Pirinen, MD, PhDDept of Clinical PathologyDept of Clinical PathologyKuopio University HospitalKuopio University Hospital

Kuopio, FinlandKuopio, Finland

Patient historyPatient history

!! 70 year old woman70 year old woman

!! DyspnoeaDyspnoea

!! Abundant right-sided hemorrhagic pleural effusionAbundant right-sided hemorrhagic pleural effusion–– Cytology: papa class 1-2Cytology: papa class 1-2

!! Thorax CT: diffuse tumoural nodules in the pleuraThorax CT: diffuse tumoural nodules in the pleura–– Largest tumors in the right side of the heart, lateralLargest tumors in the right side of the heart, lateral

pleura and around hilar regionspleura and around hilar regions

–– Mesothelioma vs. metastatic disease ?Mesothelioma vs. metastatic disease ?

!! Thoracoscopic biopsyThoracoscopic biopsy

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Diagnosis ?Diagnosis ?

EMA

PANCYTOKERATIN

CD99

CALPONIN

CALRET

ImmunohistochemistryImmunohistochemistry

!! Positive:Positive:–– PancytokeratinPancytokeratin

–– EMAEMA

–– CK7CK7

–– CalretiniiniCalretiniini

–– CD99CD99

–– CalponinCalponin

–– VimentinVimentin

–– Ki-67 (70 %)Ki-67 (70 %)

!! Negative:Negative:–– CK20CK20

–– SynaptophysinSynaptophysin

–– ChromograninChromogranin

–– HMB-45, Melan-AHMB-45, Melan-A

–– Actin, desminActin, desmin

–– Bcl-2Bcl-2

*

* Epithelial component

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Primary diagnosisPrimary diagnosis

Synovial sarcoma ?Synovial sarcoma ?

vs.vs.

Biphasic malignant mesothelioma ?Biphasic malignant mesothelioma ?

Additional investigationsAdditional investigations

!! FISH to detect the spesific translocationFISH to detect the spesific translocation(X;18) noted in the synovial sarcoma: (X;18) noted in the synovial sarcoma: ØØ

!! Additional immunohistochemistry:Additional immunohistochemistry:

–– Ber-EP4: +/- (epithelial component showedBer-EP4: +/- (epithelial component showedfocal positivity)focal positivity)

–– CK 5/6: +, a weak positivity was noted inCK 5/6: +, a weak positivity was noted inepithelial componentepithelial component

BER-EP4Ber-EP4

CK 5/6

Results, finalResults, final

!! PositivePositive::–– PancytokeratinPancytokeratin

–– EMAEMA

–– CK7CK7

–– CK 5/6 (+/-)CK 5/6 (+/-)

–– CalretininCalretinin

–– CD99CD99

–– CalponinCalponin

–– VimentinVimentin

–– Ki-67 (70 %)Ki-67 (70 %)

!! Negative:Negative:–– CK20CK20

–– CEACEA

–– SynaptophysinSynaptophysin

–– ChromograninChromogranin

–– HMB-45, Melan-AHMB-45, Melan-A

–– Actin, desminActin, desmin

–– Ber-EP4 (+/-)Ber-EP4 (+/-)

–– Bcl-2Bcl-2

*

* Epithelial component

No spesific translocation t(X;18) (SYT/SSX)

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Final diagnosisFinal diagnosis

Biphasic malignantBiphasic malignantmesotheliomamesothelioma

ConclusionsConclusions

!! Differential diagnosis between BMM and BSS mayDifferential diagnosis between BMM and BSS maybe difficultbe difficult–– Similarity of histological growth patterns and cellSimilarity of histological growth patterns and cell

morphologymorphology

!! Immunohistochemistry is helpful, but not alwaysImmunohistochemistry is helpful, but not alwaysconclusiveconclusive

!! The specific translocation t(x;18) is noted in >90 %The specific translocation t(x;18) is noted in >90 %of the cases of SSof the cases of SS

!! Final diagnosis should be made by combining theFinal diagnosis should be made by combining theclinical, radiological and morphological findings.clinical, radiological and morphological findings.


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