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Kasus 2 73 år. Biopsi fra cervix. Øvrig sykehistorie ukjent

Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

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Page 1: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Kasus 2

73 år. Biopsi fra cervix. Øvrig sykehistorie ukjent

Page 2: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 3: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 4: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 5: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 6: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 7: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 8: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 9: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 10: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 11: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 12: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 13: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 14: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 15: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 16: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 17: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 18: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 19: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Basalt Carcinom

Page 20: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Basalt Carcinom

• Sjelden

• Eldre kvinner

• Meget god prognose– Adenoid basalt epiteliom?

– I ”ren” form

– Blandingssvulster

• Ingen klinisk tumor

• Oppdages tilfeldig

• CIN

• HPV 16/33

Page 21: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Basalt Carcinom

• Lobulær arkitektur

• Små, velavgrensede epiteliale øyer

• Basaloide celler– Mørke, runde/ovale kjerner

– Lite cytoplasma

– Perifer pallisadering

• Få mitoser, lite atypi

Page 22: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Basalt Carcinom

• Lobulær arkitektur

• Små, velavgrensede epiteliale øyer

• Basaloide celler– Mørke, runde/ovale kjerner

– Lite cytoplasma

– Perifer pallisadering

• Få mitoser, lite atypi

Page 23: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Basalt Carcinom

• Kjerteldifferensiering

Page 24: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Basalt Carcinom

• Kjerteldifferensiering

• Plateepiteldifferensiering– Eventuelt CIN123

– Fortsatt velavgrenset

– Tynn brem av basaloide celler

Page 25: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Basalt Carcinom

• Kjerteldifferensiering

• Plateepiteldifferensiering– Eventuelt CIN123

– Fortsatt velavgrenset

– Tynn brem av basaloide celler

• Immunhistokjemi– P16+

– P63+

Page 26: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Basalt Carcinom

• Vevsreaksjon?

• Cribriform vekst?

• Høygradige kjerner?

• Mange mitoser?

• Nekrose?

• Etc…

• Differensialdiagnostikk!!

Page 27: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Differensialdiagnoser

• Adenoid basal hyperplasi– Overfladisk (<1 mm)

– Horisontal utbredelse

Page 28: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Differensialdiagnoser

• Adenoid basal hyperplasi– Overfladisk (<1 mm)

– Horisontal utbredelse

• Adenoid cystisk carcinom– Solid variant !

– Høygradig malign

Page 29: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Differensialdiagnoser

• Adenoid basal hyperplasi– Overfladisk (<1 mm)

– Horisontal utbredelse

• Adenoid cystisk carcinom– Solid variant !

– Høygradig malign

• Basaloid plateepitelcarcinom– Høygradig malign

Page 30: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Differensialdiagnoser

• Adenoid basal hyperplasi– Overfladisk (<1 mm)

– Horisontal utbredelse

• Adenoid cystisk carcinom– Solid variant !

– Høygradig malign

• Basaloid plateepitelcarcinom– Høygradig malign

• Adenoskvamøst carcinom– Plate- og adenocarcinom

– Begge komponenter oftest høygradig maligne

Page 31: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

• Adenoid basalt carcinom 10

• Adenoid basalt epiteliom 1

• Plateepitelcarcinom 6

• Basaloid plateepitelcarcinom 3

• Lite differensiert carcinom 1

• Basalcellecarcinom 1

• Basoskvamøst carcinom 1

• Infiltrerende tumor, basalcellecarcinomlignende 1

Innsendte diagnoser

Page 32: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Neste kasus…

Page 33: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Kasus 3

76 år. Cervixbiopsi viste lite differensiert carcinom. Radikal hysterektomimed lymfeknutetoilette ble gjennomført. Snitt fra uterus.

Page 34: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 35: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 36: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 37: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 38: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 39: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 40: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 41: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 42: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 43: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 44: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 45: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

PAS

Page 46: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

SMA

Page 47: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

PAN-CK

Page 48: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

P16

Page 49: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma
Page 50: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Cystisk Carcinom

Page 51: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Cystisk Carcinom

• Sjelden

• Eldre kvinner

• ”Non-caucasians”

• Mucinøs ovarialtumor

• Aggressiv

– Ofte fjernmetastaser

– Høy mortalitet

• Tumor, blødning, etc…

• HR-HPV

• Tilsvarende tumor i spyttkjertel…?

Page 52: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Cystisk Carcinom

• WHO Classification of Tumours of Female ReproductiveOrgans -2014:– ”Definition: A tumour that resembles adenoid cystic carcinoma of the

salivary gland”

• WHO Classification of Tumours, Head and Neck Tumours –2005:– ”Definition: ACC is a basaloid tumour consisting of epithelial and

myoepithelial cells in variable morphologic configurations, includingtubular, cribriform and solid patterns. It has a relentless clinical courseand usually a fatal outcome”

Page 53: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Cystisk Carcinom

• WHO Classification of Tumours of Female ReproductiveOrgans -2014:– ”Definition: A tumour that resembles adenoid cystic carcinoma of the

salivary gland”

• WHO Classification of Tumours, Head and Neck Tumours –2005:– ”Definition: ACC is a basaloid tumour consisting of epithelial and

myoepithelial cells in variable morphologic configurations, includingtubular, cribriform and solid patterns. It has a relentless clinical courseand usually a fatal outcome”

Page 54: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Cystisk Carcinom

• Basaloide celler– Mørke, runde/ovale kjerner

– Lite cytoplasma

– Perifer pallisadering

Page 55: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Cystisk Carcinom

• Basaloide celler– Mørke, runde/ovale kjerner

– Lite cytoplasma

– Perifer pallisadering

• Lett/moderat atypi– Grovere enn ABC

Page 56: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Cystisk Carcinom

• Basaloide celler– Mørke, runde/ovale kjerner

– Lite cytoplasma

– Perifer pallisadering

• Lett/moderat atypi– Grovere enn ABC

• Mitoser

Page 57: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Cystisk Carcinom

• WHO Classification of Tumours of Female ReproductiveOrgans -2014:– ”Definition: A tumour that resembles adenoid cystic carcinoma of the

salivary gland”

• WHO Classification of Tumours, Head and Neck Tumours –2005:– ”Definition: ACC is a basaloid tumour consisting of epithelial and

myoepithelial cells in variable morphologic configurations, includingtubular, cribriform and solid patterns. It has a relentless clinical courseand usually a fatal outcome”

Page 58: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Cystisk Carcinom

• WHO Classification of Tumours of Female ReproductiveOrgans -2014:– ”Definition: A tumour that resembles adenoid cystic carcinoma of the

salivary gland”

• WHO Classification of Tumours, Head and Neck Tumours –2005:– ”Definition: ACC is a basaloid tumour consisting of epithelial and

myoepithelial cells in variable morphologic configurations, includingtubular, cribriform and solid patterns. It has a relentless clinical courseand usually a fatal outcome”

Page 59: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Cystisk Carcinom

• Cribriformt– Karakteristisk

– PAS+ materiale i lumen

• Tubulært

• Solid

Page 60: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Cystisk Carcinom

• Cribriformt– Karakteristisk

– PAS+ materiale i lumen

• Tubulært

• Solid

Page 61: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Cystisk Carcinom

• Cribriformt– Karakteristisk

– PAS+ materiale i lumen

• Tubulært

• Solid

Page 62: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Cystisk Carcinom

• Cribriformt– Karakteristisk

– PAS+ materiale i lumen

• Tubulært

• Solid

• Nekrose

• Stromal respons

Page 63: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Cystisk Carcinom

• WHO Classification of Tumours of Female ReproductiveOrgans -2014:– ”Definition: A tumour that resembles adenoid cystic carcinoma of the

salivary gland”

• WHO Classification of Tumours, Head and Neck Tumours –2005:– ”Definition: ACC is a basaloid tumour consisting of epithelial and

myoepithelial cells in variable morphologic configurations, includingtubular, cribriform and solid patterns. It has a relentless clinical courseand usually a fatal outcome”

Page 64: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Cystisk Carcinom

• WHO Classification of Tumours of Female ReproductiveOrgans -2014:– ”Definition: A tumour that resembles adenoid cystic carcinoma of the

salivary gland”

• WHO Classification of Tumours, Head and Neck Tumours –2005:– ”Definition: ACC is a basaloid tumour consisting of epithelial and

myoepithelial cells in variable morphologic configurations, includingtubular, cribriform and solid patterns. It has a relentless clinical courseand usually a fatal outcome”

Page 65: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Cystisk Carcinom

• «Påvist lungemetastaser etter ett år og cytostatikabehandlet. Død to år senere»

Page 66: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Cystisk Carcinom

• WHO Classification of Tumours of Female ReproductiveOrgans -2014:– ”Definition: A tumour that resembles adenoid cystic carcinoma of the

salivary gland”

• WHO Classification of Tumours, Head and Neck Tumours –2005:– ”Definition: ACC is a basaloid tumour consisting of epithelial and

myoepithelial cells in variable morphologic configurations, includingtubular, cribriform and solid patterns. It has a relentless clinical courseand usually a fatal outcome”

Page 67: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Cystisk Carcinom

• WHO Classification of Tumours of Female ReproductiveOrgans -2014:– ”Definition: A tumour that resembles adenoid cystic carcinoma of the

salivary gland”

• WHO Classification of Tumours, Head and Neck Tumours –2005:– ”Definition: ACC is a basaloid tumour consisting of epithelial and

myoepithelial cells in variable morphologic configurations, includingtubular, cribriform and solid patterns. It has a relentless clinical courseand usually a fatal outcome”

Page 68: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Cystisk Carcinom

Calponin

SMACK7

SMA

Page 69: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Cystisk Carcinom

SMA

Page 70: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Cystisk Carcinom• ”Myoepithelial cells are absent by light microscopy, but ultrastructural

examination in some cases have shown evidence of myoepithelial

differentiation” - Atlas of Gynecological Surgical Pathology: Expert consult (Clement, Young) – 2014

• ”However, unlike ACC of other sites, ACC contain few myoepithelial cellsdetected by electron microscopy or S100 and actin immunohistochemicalstains” - Blaustein`s Pathology of the Female Genital Tract (Kurman) – 2014

• ”ACC of the cervix lacks myoepithelial cells”

• ”Actin and S100 (…) are negative in most ACC of the cervix unlike ACC in other locations” - AFIP Atlas of Tumor Pathology, vol 13, series 4 (Kurman) – 2010

• ”Immunoperoxidase (…) may be used to characterize this tumor, includingmyoepithelial markers” - Biopsy Interpretation of the Uterine Cervix and Corpus (Malpica, Euscher)-2015

Page 71: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Cystisk Carcinom

• WHO Classification of Tumours of Female ReproductiveOrgans -2014:– ”Definition: A tumour that resembles adenoid cystic carcinoma of the

salivary gland”

• …videre:– ”There may be two tumours demonstrating this morphological

pattern, one being a true ACC and the other being an ACC-like basaloidsquamous cell carcinoma”

– ”Immunohistochemistry (…) can be used to distinguish these tumours, including myoepithelial markers”

Page 72: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Adenoid Cystisk Carcinom

• Immunhistokjemi/spesialanalyser– CK, CK7

– p16

– PAS

– Myoepiteliale markører (?)

• SMA, S100, Calponin, p63

Page 73: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Differensialdiagnoser

• Adenoid basalt carcinom– Eldre kvinner

– Basaloid

– Ofte kjerteldifferensiering

Page 74: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Differensialdiagnoser

• Adenoid basalt carcinom– Eldre kvinner

– Basaloid

– Ofte kjerteldifferensiering

Page 75: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Differensialdiagnoser

• Adenoid basalt carcinom– Eldre kvinner

– Basaloid

– Ofte kjerteldifferensiering

– Tilfeldig funn

– Ikke cribriform

– Ikke PAS+ hyalint materiale

– «Mangler» malignitetskriterier

Page 77: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Differensialdiagnoser

• Andre carcinomer– Endometrioid

– «Usual» adenocarcinom

– Mesonefrisk carcinom• Eosinofilt luminalt materiale

– Småcellet carcinom• Nevroendokrine markører

– Basaloid plateepitelcarcinom• ACC-lik fenotype (WHO)

Page 78: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Differensialdiagnoser

• Andre carcinomer– Endometrioid

– «Usual» adenocarcinom

– Mesonefrisk carcinom• Eosinofilt luminalt materiale

– Småcellet carcinom• Nevroendokrine markører

– Basaloid plateepitelcarcinom• ACC-lik fenotype (WHO)

Page 79: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Differensialdiagnoser

• Andre carcinomer– Endometrioid

– «Usual» adenocarcinom

– Mesonefrisk carcinom• Eosinofilt luminalt materiale

– Småcellet carcinom• Nevroendokrine markører

– Basaloid plateepitelcarcinom• ACC-lik fenotype (WHO)

Page 80: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

• Adenoid cystisk carcinom 18

• ACC eller «ACC-like basaloid squamous carcinoma» 1

• Lavt differensiert adenocarcinom 1

• Småcellet plateepitelcarcinom 1

• Basaloid carcinom 1

• Lavt differensiert carcinom 1

• Carcinom med blandet differensiering, cystisk/basaloid 1

Innsendte diagnoser

Page 81: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Neste kasus…

Page 82: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Kasus 8

41 år. Øvrig sykehistorie ukjent. Biopsi fra cervix.

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Desmin

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MyoD1

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Embryonalt Rhabdomyosarcom / Sarcoma Botryoides

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Embryonalt Rhabdomyosarcom / Sarcoma Botryoides

• RMS som komponent– Carcinosarcom/MMMT– Adenosarcom– Heterologt element

• Embryonalt RMS– Vulva og Vagina

• < 5år

– Cervix• Barn og unge kvinner• Rimelig god prognose• Ofte lokal eksisjon + CT/RT

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Embryonalt Rhabdomyosarcom / Sarcoma Botryoides

Dehner et al - 2011

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Embryonalt Rhabdomyosarcom / Sarcoma Botryoides

Dehner et al - 2011

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Embryonalt Rhabdomyosarcom / Sarcoma Botryoides

Dehner et al - 2011

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1/14 recidiv

Embryonalt Rhabdomyosarcom / Sarcoma Botryoides

Dehner et al - 2011

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• Sarcoma botryoides– Botrys (gr) – drueklase

– Cambium layer

Embryonalt Rhabdomyosarcom / Sarcoma Botryoides

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• Sarcoma botryoides– Botrys (gr) – drueklase

– Cambium layer

Embryonalt Rhabdomyosarcom / Sarcoma Botryoides

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• Sarcoma botryoides– Botrys (gr) – drueklase

– Cambium layer

Embryonalt Rhabdomyosarcom / Sarcoma Botryoides

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• Sarcoma botryoides– Botrys (gr) – drueklase

– Cambium layer

Embryonalt Rhabdomyosarcom / Sarcoma Botryoides

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• Sarcoma botryoides– Botrys (gr) – drueklase

– Cambium layer

• ”Non-botryoid” ERMS– Ikke cambium layer

Embryonalt Rhabdomyosarcom / Sarcoma Botryoides

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• Udifferensierte celler– Små, ovale, hyperkromate kjerner– Lite cytoplasma– 2-12 mitoser/10HPF– Dette er IKKE lymfocytter!

• Myxoid / Ødem• Rhabdomyoblaster

– Større, avlange– Rikelig eosinofilt cytoplasma– +/- tverrstriping– Oftest (ikke alltid) til stede

• Brusk• Ofte bevarte kjertler !

Embryonalt Rhabdomyosarcom / Sarcoma Botryoides

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• Udifferensierte celler– Små, ovale, hyperkromate kjerner– Lite cytoplasma– 2-12 mitoser/10HPF– Dette er IKKE lymfocytter!

• Myxoid / Ødem• Rhabdomyoblaster

– Større, avlange– Rikelig eosinofilt cytoplasma– +/- tverrstriping– Oftest (ikke alltid) til stede

• Brusk• Ofte bevarte kjertler !

Embryonalt Rhabdomyosarcom / Sarcoma Botryoides

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• Udifferensierte celler– Små, ovale, hyperkromate kjerner– Lite cytoplasma– 2-12 mitoser/10HPF– Dette er IKKE lymfocytter!

• Myxoid / Ødem• Rhabdomyoblaster

– Større, avlange– Rikelig eosinofilt cytoplasma– +/- tverrstriping– Oftest (ikke alltid) til stede

• Brusk• Ofte bevarte kjertler !

Embryonalt Rhabdomyosarcom / Sarcoma Botryoides

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• Udifferensierte celler– Små, ovale, hyperkromate kjerner– Lite cytoplasma– 2-12 mitoser/10HPF– Dette er IKKE lymfocytter!

• Myxoid / Ødem• Rhabdomyoblaster

– Større, avlange– Rikelig eosinofilt cytoplasma– +/- tverrstriping– Oftest (ikke alltid) til stede

• Brusk• Ofte bevarte kjertler !

Embryonalt Rhabdomyosarcom / Sarcoma Botryoides

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• Udifferensierte celler– Små, ovale, hyperkromate kjerner– Lite cytoplasma– 2-12 mitoser/10HPF– Dette er IKKE lymfocytter!

• Myxoid / Ødem• Rhabdomyoblaster

– Større, avlange– Rikelig eosinofilt cytoplasma– +/- tverrstriping– Oftest (ikke alltid) til stede

• Brusk• Ofte bevarte kjertler !

Embryonalt Rhabdomyosarcom / Sarcoma Botryoides

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Embryonalt Rhabdomyosarcom / Sarcoma Botryoides

• Desmin, MSA– Rhabdo OG leio

– Moden muskulatur

– Høyt og lavt differensierte

– Ikke udifferensierte

– Uspesifikk

• Myogenin, MyoD1– Myogenic nuclear regulatory proteins

– Tidlig i rhabdomyogenesen

– Lavt- og udifferensierte

– Rhabdomyosarcom

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Differensialdiagnoser

• Adenosarcom– Epidemiologi

• Unge voksne kvinner

– Makroskopisk• Polypp

– Mikroskopisk• Benignt epitel + Malignt stroma

• Periglandulær hypercellularitet

• Varierende grad av atypi

• Heterologe elementer

– Brusk, fett, muskulatur, etc

• Sarcomatous overgrowth

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Differensialdiagnoser

• Adenosarcom– Epidemiologi

• Unge voksne kvinner

– Makroskopisk• Polypp

– Mikroskopisk• Benignt epitel + Malignt stroma

• Periglandulær hypercellularitet

• Varierende grad av atypi

• Heterologe elementer

– Brusk, fett, muskulatur, etc

• Sarcomatous overgrowth

– Men…• Fibrøs polypp, ikke «botryoid»

• «Leaf like», «phyllodes like»

• Ikke cambium layer

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• Embryonalt rhabdomyosarcom/sarcoma botryoides 9

• Rhabdomyosarcom 7

• Sarcom 3

• Småcellet carcinom? Adenosarcom? 1

• Infiltrerende småcellet carcinom 1

• Embryonalt carcinom 1

• Embryonalt. Usikker. 1

• Leiomyosarcom 1

Innsendte diagnoser

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Neste kasus…

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Kasus 10

Uterusamputert 39 år gammel, fjernet begge adnex 51 år gammel. Nå vaginalblødning. Snitt fra cervixstump.

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PAN-CK

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P16

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PAX5

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Mesonefrisk carcinom

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Mesonefrisk carcinom

• «Extremely rare»– Cirka 30 beskrevet

• ”Alle” aldre (34-72 år)

• 25% mortalitet

• Cervix >> corpus, vagina

• Klinisk tumor, blødning

• Ikke HPV-relatert

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Mesonefrisk carcinom

• «Extremely rare»– Cirka 30 beskrevet

• ”Alle” aldre (34-72 år)

• 25% mortalitet

• Cervix >> corpus, vagina

• Klinisk tumor, blødning

• Ikke HPV-relatert

• Mesonefric/Wolffianremnant…

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• Caspar Friedrich Wolff– 1735-94

– «Theoria Generationis», Univ i Halle, 1759

– Epigenesis

Preformasjonisme

Homunculus

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• Caspar Friedrich Wolff– 1735-94

– «Theoria Generationis», Univ i Halle, 1759

– Epigenesis

Preformasjonisme

Homunculus

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• Caspar Friedrich Wolff– 1735-94

– «Theoria Generationis», Univ i Halle, 1759

– Epigenesis

– «One of the founders of embryology»

– Wolffske gang (Ductus Mesonephricus)

Preformasjonisme

Homunculus

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• Caspar Friedrich Wolff– 1735-94

– «Theoria Generationis», Univ i Halle, 1759

– Epigenesis

– «One of the founders of embryology»

– Wolffske gang (Ductus Mesonephricus)

Fra Müllerske gang Fra Wolffske gang

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• Caspar Friedrich Wolff– 1735-94

– «Theoria Generationis», Univ i Halle, 1759

– Epigenesis

– «One of the founders of embryology»

– Wolffske gang (Ductus Mesonephricus)

• Mesonefriske-/Wolffske rester– Ovariehilus

– Ligamentum latum

– Lateralt/posterolateralt i cervix• 22% av voksne kvinner

– Lateralt/posterolateralt i vagina

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• Caspar Friedrich Wolff– 1735-94

– «Theoria Generationis», Univ i Halle, 1759

– Epigenesis

– «One of the founders of embryology»

– Wolffske gang (Ductus Mesonephricus)

• Mesonefriske-/Wolffske rester– Tilfeldig funn

– «Åpenbar» benign histologi

– Tubulære strukturer

• Ofte eosinofilt, PAS+ innhold

• Ofte omkring sentral gang

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Mesonefrisk carcinom

• Ofte mesonefrisk rest

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Mesonefrisk carcinom

• Ofte mesonefrisk rest

• Infiltrasjon/atypi/mitoser

• Lite/ingen vevsreaksjon

• Heterogenitet– 6 vekstmønstre

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Mesonefrisk carcinom

• Ofte mesonefrisk rest

• Infiltrasjon/atypi/mitoser

• Lite/ingen vevsreaksjon

• Heterogenitet– 6 vekstmønstre

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Mesonefrisk carcinom

• Tubulært– Eosinofilt hyalint materiale

• Duktalt– «Endometrioid-lignende»

• Retiformt

• ”Sex-cord like”

• Solid

• Sarcomatoid– Heterologe elementer?

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Mesonefrisk carcinom

• Tubulært– Eosinofilt hyalint materiale

• Duktalt– «Endometrioid-lignende»

• Retiformt

• ”Sex-cord like”

• Solid

• Sarcomatoid– Heterologe elementer?

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Mesonefrisk carcinom

• Tubulært– Eosinofilt hyalint materiale

• Duktalt– «Endometrioid-lignende»

• Retiformt

• ”Sex-cord like”

• Solid

• Sarcomatoid– Heterologe elementer?

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Mesonefrisk carcinom

• Tubulært– Eosinofilt hyalint materiale

• Duktalt– «Endometrioid-lignende»

• Retiformt

• ”Sex-cord like”

• Solid

• Sarcomatoid– Heterologe elementer?

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Differensialdiagnoser

• Mesonefrisk hyperplasi– Lobulær/diffus/ductal

– Subklinisk, «incidental finding»

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Differensialdiagnoser

• Mesonefrisk hyperplasi– Lobulær/diffus/ductal

– Subklinisk, «incidental finding»

– “Its distinction from mesonephric remnants is arbitrary and based on the appearance of the lobules, which are larger, more loosely organized and more irregularly shaped” (Robboy's Pathology of the Female Reproductive Tract, 2nd ed)

– “The distinction from mesonephric carcinoma is based on the disorderly invasion, back-to-back crowding, and cytologic atypia usually present in carcinoma” (Zaino, Modern pathology – 2000)

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Differensialdiagnoser

• Andre carcinomer

– «Usual» adenocarcinoma

– Endometrioid

– Adenoid cystisk carcinom• PAS+ materiale

– Serøst• Retiformt mønster

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Differensialdiagnoser

• Andre carcinomer

– «Usual» adenocarcinoma

– Endometrioid

– Adenoid cystisk carcinom• PAS+ materiale

– Serøst• Retiformt mønster

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Differensialdiagnoser

• Andre carcinomer

– «Usual» adenocarcinoma

– Endometrioid

– Adenoid cystisk carcinom• PAS+ materiale

– Serøst• Retiformt mønster

• Carcinosarcom• Heterogenitet

• Heterologe element

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Differensialdiagnoser

Residency Program - Case of the Month February 2010 Final Diagnosis - Presented by Mary Tomic, M.D. UC Davis Health system Sacramento Ca USA

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Differensialdiagnoser

Residency Program - Case of the Month February 2010 Final Diagnosis - Presented by Mary Tomic, M.D. UC Davis Health system Sacramento Ca USA

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Differensialdiagnoser

Residency Program - Case of the Month February 2010 Final Diagnosis - Presented by Mary Tomic, M.D. UC Davis Health system Sacramento Ca USA

P16PAX5

NegPos

PosNeg

Pos/NegNeg

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• Mesonefrisk carcinom 13

• Adenocarcinom 4

• Endometrioid carcinom 2

• Serøst carcinom 2

• Bifasisk tumor, carcinosarcom 1

• Nevroendokrint carcinom 1

• Lavt differensiert nevroendokrin tumor 1

Innsendte diagnoser

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Neste kasus…

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Kasus 13

68 år. Snitt fra cervixutskrap

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p16 neg ?

P53..?

Endometrium?

Adneks?

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Serøst carcinom

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Serøst carcinom

• Low grade serous carcinoma– Ovarium og peritoneum

• Serous borderline tumor (SBT)• ”invasive, distinctive patterns, low-

grade malignant cytological atypia”• P53wt, P16-• Ca 50 år

• High grade serous carcinoma– Tube, ovarium og peritoneum

• Serous intraep tubar carcinoma (STIC)• ”papillary, glandular and solid

patterns, high grade nuclear atypia”• Mutert P53, P16+• Ca 63 år

• Serous carcinoma– Endometrium

• Serous endometr intraep carcinoma• ”complex papillary or glandular,

marked nuclear pleomorphism”• Mutert P53, P16+• ”Postmenopausal women”

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Serøst carcinom

• Low grade serous carcinoma– Ovarium og peritoneum

• Serous borderline tumor (SBT)• ”invasive, distinctive patterns, low-

grade malignant cytological atypia”• P53wt, P16-• Ca 50 år

• High grade serous carcinoma– Tube, ovarium og peritoneum

• Serous intraep tubar carcinoma (STIC)• ”papillary, glandular and solid

patterns, high grade nuclear atypia”• Mutert P53, P16+• Ca 63 år

• Serous carcinoma– Endometrium

• Serous endometr intraep carcinoma• ”complex papillary or glandular,

marked nuclear pleomorphism”• Mutert P53, P16+• ”Postmenopausal women”

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Serøst carcinom

• Low grade serous carcinoma– Ovarium og peritoneum

• Serous borderline tumor (SBT)• ”invasive, distinctive patterns, low-

grade malignant cytological atypia”• P53wt, P16-• Ca 50 år

• High grade serous carcinoma– Tube, ovarium og peritoneum

• Serous intraep tubar carcinoma (STIC)• ”papillary, glandular and solid

patterns, high grade nuclear atypia”• Mutert P53, P16+• Ca 63 år

• Serous carcinoma– Endometrium

• Serous endometr intraep carcinoma• ”complex papillary or glandular,

marked nuclear pleomorphism”• Mutert P53, P16+• ”Postmenopausal women”

• Serous carcinoma– Cervix

• ?• ”rare, identical histological

appearance to endometrial or adnexal serous carcinoma”

• ?• ?

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Serøst carcinom

Ueda et al-2012

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Serøst carcinom

Ueda et al-2012

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Serøst carcinom

• 17 pasienter og recidiv

Zhou et al 1998

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Serøst carcinom

• 17 pasienter og recidiv

• Klinisk stadium

• Tumors størrelse

• Infiltrasjonsdybde

• Lymfeknutemetastase

Zhou et al 1998

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Serøst carcinom

• Bimodal aldersfordeling– <45 år (12stk), 3/10 recidiv

– >65 år (5stk), 4/5 recidiv

Zhou et al 1998

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Serøst carcinom

• Bimodal aldersfordeling– <45 år (12stk), 3/10 recidiv

– >65 år (5stk), 4/5 recidiv

Zhou et al 1998

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Serøst carcinom

• Bimodal aldersfordeling– <45 år (12stk), 3/10 recidiv

– >65 år (5stk), 4/5 recidiv

• p53 status– ”positive” 5/12 (??)

– ”most cells positive” 2/12 (?)• 4/5 recidiv

– ”negative” 7/12• 2/7 recidiv

Zhou et al 1998

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Serøst carcinom

• Bimodal aldersfordeling– <45 år (12stk), 3/10 recidiv

– >65 år (5stk), 4/5 recidiv

• p53 status– ”positive” 5/12 (??)

– ”most cells positive” 2/12 (?)• 4/5 recidiv

– ”negative” 7/12• 2/7 recidiv

Zhou et al 1998

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Serøst carcinom

• Bimodal aldersfordeling– <45 år (12stk), 3/10 recidiv

– >65 år (5stk), 4/5 recidiv

• p53 status– ”positive” 5/12 (??)

– ”most cells positive” 2/12 (?)• 4/5 recidiv

– ”negative” 7/12• 2/7 recidiv

• To ulike tumortyper?

Zhou et al 1998

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Serøst carcinom

Ueda et al-2012

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Serøst carcinom

Ueda et al-2012

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NOFECH-MOZES 2006

n=10

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NOFECH-MOZES 2006

n=10

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Serøst carcinom

• Primære serøse carcinom i cervix er sjeldne!

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Serøst carcinom

• Primære serøse carcinom i cervix er sjeldne!

• Finnes de?

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Serøst carcinom

• Primære serøse carcinom i cervix er sjeldne!

• Finnes de?

• Metastase fra HGSC i adneks?

• Direkte innvekst fra serøst carcinom i endometrium?

• Annen type primært adenocarcinom i cervixmed morfologi som HGSC?

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Serøst carcinom

• Primære serøse carcinom i cervix er sjeldne!

• Finnes de?

• Metastase fra HGSC i adneks?

• Direkte innvekst fra serøst carcinom i endometrium?

• Annen type primært adenocarcinom i cervixmed morfologi som HGSC?

• Primært serøst carcinom i cervix?

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Innsendte diagnoser

• Serøst carcinom 22

• Endometrioid carcinom 1

• Endocervicalt adenocarcinom, villoglandulær type 1

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Neste kasus…

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Case 19

63 år gammel kvinne med tumor i uterus. Utskrap.

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Primært myelosarcom

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Primært myelosarcom

• Ekstramedullær proliferasjon av umodne myeloide celler– Primært- uten leukemi– Sekundært - med leukemi

• Sjelden

• Alle aldre

• Utvikler ”alltid” AML

• Dårlig prognose

• Tidlig behandling KAN hjelpe

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Primært myelosarcom

• Ekstramedullær proliferasjon av umodne myeloide celler– Primært- uten leukemi– Sekundært - med leukemi

• Sjelden

• Alle aldre

• Utvikler ”alltid” AML

• Dårlig prognose

• Tidlig behandling KAN hjelpe

• Histologi– Diffus proliferasjon– Runde/rundovale kjerner– Lett-moderat atypi– Mitoser– Oftest monotont– Eventuelt differensiering

• CD68, MPO, CD117, CD99, CD34, TdT, CD56, CD30, etc…

• Differensialdiagnostikk– Husk at det finnes!

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Hematopoietisk stamcelle

Myeloid stamcelleLymfoid stamcelle

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Hematopoietisk stamcelle

Myeloid stamcelleLymfoid stamcelle

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Hematopoietisk stamcelle

Myeloid stamcelleLymfoid stamcelle

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Hematopoietisk stamcelle

Myeloid stamcelleLymfoid stamcelle

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Primært myelosarcom

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Primært myelosarcom

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Primært myelosarcom

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Primært myelosarcom

• Ekstramedullær proliferasjon av umodne myeloide celler– Primært- uten leukemi– Sekundert - med leukemi

• Sjelden

• Alle aldre

• Utvikler ”alltid” AML

• Dårlig prognose

• Tidlig behandling KAN hjelpe

• Histologi– Diffus proliferasjon– Runde/rundovale kjerner– Lett-moderat atypi– Mitoser– Oftest monotont– Eventuelt differensiering

• CD68, MPO, CD117, CD99, CD34, TdT, CD56, CD30, etc…

• Differensialdiagnostikk– Husk at det finnes!

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Primært myelosarcom

• Ekstramedullær proliferasjon av umodne myeloide celler– Primært- uten leukemi– Sekundert - med leukemi

• Sjelden

• Alle aldre

• Utvikler ”alltid” AML

• Dårlig prognose

• Tidlig behandling KAN hjelpe

• Histologi– Diffus proliferasjon– Runde/rundovale kjerner– Lett-moderat atypi– Mitoser– Oftest monotont– Eventuelt differensiering

• Differensialdiagnostikk– Inflammasjon– Lymfom– Carcinom (udiff, småcellet, …)– Sarcomer (ESS, UUS, …)– Etc etc

– CD68, MPO, CD117, CD99, CD34, TdT, CD56, CD30, etc…

– Husk at det finnes!

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Primært myelosarcom

• Ekstramedullær proliferasjon av umodne myeloide celler– Primært- uten leukemi– Sekundert - med leukemi

• Sjelden

• Alle aldre

• Utvikler ”alltid” AML

• Dårlig prognose

• Tidlig behandling KAN hjelpe

• Histologi– Diffus proliferasjon– Runde/rundovale kjerner– Lett-moderat atypi– Mitoser– Oftest monotont– Eventuelt differensiering

• Differensialdiagnostikk– Inflammasjon– Lymfom– Carcinom (udiff, småcellet, …)– Sarcomer (ESS, UUS, …)– Etc etc

– CD68, MPO, CD117, CD99, CD34, TdT, CD56, CD30, etc…

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Auodin et al - 2003

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Primært myelosarcom

• Ekstramedullær proliferasjon av umodne myeloide celler– Primært- uten leukemi– Sekundert - med leukemi

• Sjelden

• Alle aldre

• Utvikler ”alltid” AML

• Dårlig prognose

• Tidlig behandling KAN hjelpe

• Histologi– Diffus proliferasjon– Runde/rundovale kjerner– Lett-moderat atypi– Mitoser– Oftest monotont– Eventuelt differensiering

• Differensialdiagnostikk– Inflammasjon– Lymfom– Carcinom (udiff, småcellet, …)– Sarcomer (ESS, UUS, …)– Etc etc

– CD68, MPO, CD117, CD99, CD34, TdT, CD56, CD30, etc…

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Primært myelosarcom

• Ekstramedullær proliferasjon av umodne myeloide celler– Primært- uten leukemi– Sekundert - med leukemi

• Sjelden

• Alle aldre

• Utvikler ”alltid” AML

• Dårlig prognose

• Tidlig behandling KAN hjelpe

• Histologi– Diffus proliferasjon– Runde/rundovale kjerner– Lett-moderat atypi– Mitoser– Oftest monotont– Eventuelt differensiering

• Differensialdiagnostikk– Inflammasjon– Lymfom– Carcinom (udiff, småcellet, …)– Sarcomer (ESS, UUS, …)– Etc etc

– CD68, MPO, CD117, CD99, CD34, TdT, CD56, CD30, etc…

– HUSK AT DET FINNES !!

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Yamauchi & Yasuda (2002):74 pasienter, myelosarcom

Harris &Scully(1984):27 pasienter , lymfom eller myelosarcom i uterus/vagina

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Yamauchi & Yasuda (2002):74 pasienter, myelosarcom

Harris &Scully(1984):27 pasienter , lymfom eller myelosarcom i uterus/vagina

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Yamauchi & Yasuda (2002):74 pasienter, myelosarcom

Harris &Scully(1984):27 pasienter , lymfom eller myelosarcom i uterus/vagina

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Yamauchi & Yasuda (2002):74 pasienter, myelosarcom

Harris &Scully(1984):27 pasienter , lymfom eller myelosarcom i uterus/vagina

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Yamauchi & Yasuda (2002):74 pasienter, myelosarcom

Harris &Scully(1984):27 pasienter , lymfom eller myelosarcom i uterus/vagina

NB! HUSK AT DET FINNES…

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Innsendte diagnoser

• Myelosarcom 2

• Sarcom 4• Endometriestromasarcom 3• Neuroendokrin tumor 1

• Udifferensiert malign tumor 2• Spolformet malign tumor 3• Udifferensiert carcinom 2

• Udifferensiert carcinom? Småcellet carcinom? Epitelioid leiomyosarcom? Endometriestromasarcom? Sarcom? Lymfom? Melanom? 5

• Leiomyom 1• Carcinosarcom (myelosarcom) 1

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Neste kasus…

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Kasus 40

67 år. Utskrap fra corpus uteri.

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Espinosa et al, 2015

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MammaglobinGCDFP-15

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Metastase til uterus

fra lobulært carcinom i mamma

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Metastase til uterus

• Sjelden– Abrams 1950

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Metastase til uterus

• Sjelden– Abrams 1950

• 5,9% uterus

• 0,3% cervix

– Sjeldnere i dag?

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Metastase til uterus

• Sjelden– Abrams 1950

• 5,9% uterus

• 0,3% cervix

– Sjeldnere i dag?

• Ofte kjent grunnsykdom– ”enkel” diagnostikk

• Primær manifestasjon– Ofte vanskelig

• Klinikk ”som gyn primærtx”

• Histologi ”som gyn primærtx”

• Sjelden; tenker ikke på det…

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• Genitalt origo, adneks

Metastase til uterusMenczer et al 2010:Cancer ovarii, stg II-IV, hysterektomert, n=554

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• Genitalt origo, adneks– Uterusaffeksjon 52%

Metastase til uterusMenczer et al 2010:Cancer ovarii, stg II-IV, hysterektomert, n=554

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• Genitalt origo, adneks– Uterusaffeksjon 52%

– 40% økt risiko ved HGSC

Metastase til uterusMenczer et al 2010:Cancer ovarii, stg II-IV, hysterektomert, n=554

Page 272: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

• Genitalt origo, adneks– Uterusaffeksjon 52%

– 40% økt risiko ved HGSC

– Mikroskopisk 38%

– Makroskopisk 14%

Metastase til uterusMenczer et al 2010:Cancer ovarii, stg II-IV, hysterektomert, n=554

Page 273: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

• Genitalt origo, adneks– Uterusaffeksjon 52%

– 40% økt risiko ved HGSC

– Mikroskopisk 38%

– Makroskopisk 14%

– «Ikke endometrium» 37% («70%»)

Metastase til uterusMenczer et al 2010:Cancer ovarii, stg II-IV, hysterektomert, n=554

Page 274: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

• Genitalt origo, adneks– Uterusaffeksjon 52%

– 40% økt risiko ved HGSC

– Mikroskopisk 38%

– Makroskopisk 14%

– «Ikke endometrium» 37% («70%»)

– Kun endometrium 3,4% («6,5%»)

– Endometrium mm 8,6% («16%»)

Metastase til uterusMenczer et al 2010:Cancer ovarii, stg II-IV, hysterektomert, n=554

Page 275: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

• Genitalt origo, adneks– Uterusaffeksjon 52%

– 40% økt risiko ved HGSC

– Mikroskopisk 38%

– Makroskopisk 14%

– «Ikke endometrium» 37% («70%»)

– Kun endometrium 3,4% («6,5%»)

– Endometrium mm 8,6% («16%»)

– Altså; carcinom i myometrium og serosa men ikke endometrium

…suspekt!

Metastase til uterusMenczer et al 2010:Cancer ovarii, stg II-IV, hysterektomert, n=554

Page 276: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

• Genitalt origo, adneks– Uterusaffeksjon 52%

– 40% økt risiko ved HGSC

– Mikroskopisk 38%

– Makroskopisk 14%

– «Ikke endometrium» 37% («70%»)

– Kun endometrium 3,4% («6,5%»)

– Endometrium mm 8,6% («16%»)

– Altså; carcinom i myometrium og serosa men ikke endometrium

…suspekt!

• Genitalt origo, annet– Metastase

– Direkte innvekst

Metastase til uterusMenczer et al 2010:Cancer ovarii, stg II-IV, hysterektomert, n=554

Page 277: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

• Extragenitalt origo

Metastase til uterus

Kumar et al 1982 : 63 kvinner med extragenital cancer og metastase til uterus

Page 278: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

• Extragenitalt origo– Mamma 43%

– Colon 18%

– Ventrikkel 11%

– Pancreas 11%

Metastase til uterus

Kumar et al 1982 : 63 kvinner med extragenital cancer og metastase til uterus

Page 279: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

• Extragenitalt origo– Mamma 43%

– Colon 18%

– Ventrikkel 11%

– Pancreas 11%

– Kun myometrium 64%

– Endo- og myometrium 33%

– Kun endometrium 4%

Metastase til uterus

Kumar et al 1982 : 63 kvinner med extragenital cancer og metastase til uterus

Page 280: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

• Extragenitalt origo– Mamma 43%

– Colon 18%

– Ventrikkel 11%

– Pancreas 11%

– Kun myometrium 64%

– Endo- og myometrium 33%

– Kun endometrium 4%

– Altså; carcinom i myometrium men ikke endometrium…suspekt!

Metastase til uterus

Kumar et al 1982 : 63 kvinner med extragenital cancer og metastase til uterus

Page 281: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Metastase til uterus

• Carcinom kun i serosa og/eller myometrium?

• Dersom endometrium/-cervix– Bevarte benigne kjertler?

– In-situ lesjoner?

• Spesifikk ”uterus-fremmed” morfologì?– Single files

– Dirty necrosis

– Signetringceller

– Andre

• Sykehistorie !!!

• Immunhistokjemi…

• Immunhistokjemi…

Page 286: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

Innsendte diagnoser

• Metastase fra lobulært carcinom 9

• Metastase fra malignt melanom 3• Endometriepolypp med metastase 2• Signetringcellecarcinom. Metastase? 2• Metastase fra adenocarcinom 1• Metastase? 1

• Endometriepolypp med infiltrasjon av carcinom 1• Infiltrerende carcinom 1• Klarcellet carcinom 1• Atypisk leiomyom? Metastase fra ventrikkelcarcinom? 1• Malign tumor 1• Pecom 1

Page 287: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

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Page 288: Kasus 2 - Legeforeningen · 2019-03-14 · • WHO Classification of Tumours of Female Reproductive Organs -2014: – ”Definition: A tumour that resembles adenoid cystic carcinoma

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