( )
PEComa/LAM UTROSCT IMT BCOR Adenomyoma Mullerian adenosarcoma
FFPE
PEC (perivascular epithelioid cell) proliferation PEComa:, LAM (lymphangioleiomyomatosis):,
PEComa (perivascular epithelioid cell tumor),tongue-like infiltration PEC key finding SMA, desmin, caldesmon ) HMB45, Melan-A, MiTF ) Benign, uncertain malignant potential, malignant, GYN, mtor,
PEComa (perivascular epithelioid cell tumor) leio??
PEComa (perivascular epithelioid cell tumor)
PEComa (perivascular epithelioid cell tumor)
PEComa (perivascular epithelioid cell tumor)
HMB45
82F
82F
SMA
HMB-45
HMB-45
PEComa (perivascular epithelioid cell tumor) GYN by Schoolmaster et al.) Benign/uncertain malignant potential Malignant 4 5cm High-grade atypia Mitoses > 1/50HPF Necrosis LVI Bennet threshold aggressive behavior PEComa evidence
PEComa (perivascular epithelioid cell tumor) TFE3: 2 3 IHC, fusion Folpe et al. AJSP, 2005 Schoolmaster et al. AJSP, 2014 Bennet et al. AJSP, 2018 RAD51B fusion fusion, Bennet et al. AJSP, 2018
LAM (Lymphangioleiomyomatosis) LAM LAM LAM, PEC Hayashi et al. AJSP, 2011
47F
Uterine tumor resembling ovarian sex cord-stromal tumors (UTROSCT) resemble UTROSCT, endometrial stromal tumor with sex cord-like elements (ESTSCLE) IHC Inhibin, calretinin, SF-1, FOXL2 sex cord, FOXL2 Dicer-1 granulosa cell tumor Sertoli cell tumor Irving et al. Mod Pathol, 2006 de Leval et al. AJSP 2010 Chiang et al. AJSP, 2015 Croce et al. Int J Gyn Pathol, 2016
Inhibin
Calretinin
Uterine tumor resembling ovarian sex cord-stromal tumors (UTROSCT) JAZF1 ESTSCLE PHF1 UTROSCT low-grade ESS UTROSCT NCOA2/3 (ESR1-NCOA3, ESR1-NCOA2, GREB1-NCOA2) Staats et al. AJSP, 2009 Chiang et al. AJSP. 2011 Dickson et al. AJSP 2019 GREB1-CTNNB1 UTROSCT 1 Croce et al. Genes Chromosomes and Cancer, 2019 UTROSCT ESTSCLE
In amatory myofibroblastic tumor (IMT) Myxoid DD ALK myxoid STUMP leiomyosarcoma IMT ) ALK-positivity diagnostic Desmin, SMA, CD10, ER, PgR variably positive,, Rabban et al. AJSP 2005 Shintaku et al. Pathol Int, 2006 Parra-Herran et al. AJSP, 2015 Parra-Herran et al. AJSP, 2016 Deverearux et al. AJSP 2019 Mohammad et al. AJSP 2019
In amatory myofibroblastic tumor (IMT) myxoid leio?
In amatory myofibroblastic tumor (IMT)
In amatory myofibroblastic tumor (IMT)
In amatory myofibroblastic tumor (IMT)
ALK
ZC3H7B-BCOR Spindle cell Myxoid stroma mild to moderate Mitosis: CD10, Cyclin D1: + ER, PgR: variable LG-ESS BCOR Panagopoulos et al. Genes Chromosomes and Cancer, 2013 Hoang et al. AJSP, 2017 Lewis et al. Mod Pathol, 2018
Hoang et al. AJSP, 201
Hoang et al. AJSP, 201
Hoang et al. AJSP, 2017
Hoang et al. AJSP, 2017
Hoang et al. AJSP, 2017
ZC3H7B-BCOR Spindle cell Myxoid stroma mild to moderate Mitosis: CD10, Cyclin D1: + ER, PgR: variable LG-ESS BCOR BCOR internal tandem duplication high-grade ESS undifferentiated sarcoma 2 ( 24 ) spindle cell epithelioid/round cell Mitosis: CD10: negative or focal + Cyclin D1: + Panagopoulos et al. Genes Chromosomes and Cancer, 2013 Hoang et al. AJSP, 2017 Lewis et al. Mod Pathol, 2018 Marino-Enriquez et al. AJSP 2018 High-grade ESS,
JAZF1/SUZ12 YWHAE/NUTM2A JAZF1/PHF1 YWHAE/NUTM2B EPC1-PHF1 EPC1-SUZ12 EPC1-BCOR EPC2-PHF1 ALK ZC3H7B-BCOR BRD8-PHF1 MEAF6-PHF1 TPM3-NTRK1 LMNA-NTRK1 TPR-NTRK1 RBPMS-NTRK3 GREB1-NCOA2 ESR1-NCOA2 ESR1-NCOA3 MBTD1-CXorf67 EWSR1-FLI1 EWSR1-WT1 ASPSCR1-TFE3 GREB1-CTNNB1 BCOR internal tandem duplication,
biphasic lesion Adenomyoma Mullerian adenosarcoma
Adenomyoma, endocervical type endometrioid type Polypoid endometrioid type adenomyoma nodular adenomyosis
,,
,, Adenomyoma adenomyosis,,,,
Adenomyoma 21 adenomyoma MED12 (c. 131G>A, p.g44d) 2 Fumarate hydratase (FH) Herediary leiomyomatosis and renal cell cancre (HLRCC) syndrome Heikkinen et al. Human Reprod Open, 2018
Adenomyomatous polyp Polypoid adenomyoma,adenomyoma adenomyomatous polyp
52F
Adenomyomatous polyp Polypoid adenomyoma,adenomyoma adenomyomatous polyp adenomyomatous polyp 84, CD10,Desmin, MED12 Strickland et al. Human Pathol, 2019 Adenomyomatous polyp endometrial polyp )
APAM endometrial carcinoma 1
66F
Atypical polypoid adenomyoma (APAM) Squamous morule,, endometrioid carcinoma APAM endometrioid carcinoma
34F
Atypical polypoid adenomyoma (APAM) β-catenin Nemejcava et al. AJSP, 2015 mtor PTEN APAM localized form of atypical hyperplasia
Atypical polypoid adenomyoma (APAM),low grade,
(Low grade) Mullerian adenosarcoma, key.,,,. Sarcomatous overgrowth, Sarcomatous overgrowth,, Mullerian adenosarcoma
59F
(Low grade) Mullerian adenosarcoma 28% MDM2/CDK4 72% PIK3CA/AKT/PTEN p53 sarcomatous overgrowth Howitt et al. J of Pathol, 2015 19, DICER1, FGFR2, KMT2C, NCOA )
(Low grade) Mullerian adenosarcoma
Variant adenosarcoma (tentative) ( ) biphasic tumor MED12, MED12, Variant adenosarcoma (MED12-mutated adenosarcoma-like tumor. Yuan et al. Hitopathology, 2017 polypoid,1 fibrous to somewhat myoid appearance ) adenomyosis Adenomyosis low grade Mullerian adenosarcoma
Yuan et al. Hitopathology, 2017
High grade Mullerian adenosarcoma Hodgson et al. AJSP, 2017 adenosarcoma, 9 Sarcomatous overgrowth 7 (78%) TP53 Sarcomatous overgrowth,high grade sarcoma, p53 IHC
p53
Adenosarcoma ( ) spindle cell,
Biphasic lesion Adenomyoma Adenomyomatous polyp APAM Adenosarcoma
,,