Da Anatomia Patológica para a Clínica -...

Carcinoma de Células Renais

Da Anatomia Patológica para a Clínica - 2para a Clínica - 2

Dr. Pedro OliveiraHospital da Luz Lisboa, Portugal

Carcinoma de céls. renais com translocação da família MiT

2

Carcinoma de células renais:

Da Anatomia Patológica para a Clínica

família MiT

MiT Family Translocation-Associated Renal Cell Carcinoma

Arch Pathol Lab Med—Vol 139, October 2015



A Contemporary Update With Emphasis on Morphologic, Immunophenotypic, and Molecular Mimics

Martin J. Magers, MD; Aaron M. Udager, MD, PhD; Rohit Mehra, MD



Xp11 (TFE3)40% dos casos pediátricos de CCR e < 5% dos

do adulto Associação a quimioterapia prévia

Suspeita em H&E: céls. grandes epitelióides de citoplasma claro ou eosinófilo, alto-grau citoplasma claro ou eosinófilo, alto-grau nuclear, de disposição papilar ou em ninhos sólidos com corpos psamomatosos

Diagnóstico: PAX8, TFE3 IHQ/FISH

Melhor prognóstico no grupo pediátrico; nos adultos igual ao CCR células claras

via molecular do MET



t(6:11) (TFE3)

Raro - grupo pediátrico e adultos

Associação a quimioterapia prévia

Suspeita em H&E: padrão bifásico com Suspeita em H&E: padrão bifásico com células epitelióides grandes e uma população secundária de céls. pequenas, eosinófilas dispostas em rosetas

Diagnóstico: PAX8, TFEB IHQ/FISH

Curso indolente/Excelente prognóstico

via molecular do MET

Carcinoma de céls. renais deficiente em SDH 3



(Succinato desidrogenase)

Succinate dehydrogenase-deficient renal cell carcinoma: detailed characterization of 11 tumors defining a unique subtype of renal cell carcinomaSean R Williamson , John N Eble , Mahul B Amin , Nilesh S Gupta , Steven C Smith , Lynette

M o d e r n P a t h o l o g y (2015) 28, 80–94

& 2015 USCAP, Inc All rights reserved 0893-3952/15 $32.00

Carcinoma de células renais:Da Anatomia Patológica para a Clínica

Sean R Williamson1, John N Eble2, Mahul B Amin3, Nilesh S Gupta1, Steven C Smith3, Lynette M Sholl4, Rodolfo Montironi5, Michelle S Hirsch4 and Jason L Hornick4

1Department of Pathology and Laboratory Medicine, Henry Ford Health System, Detroit, MI, USA; 2Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN, USA; 3Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, USA; 4Department of Pathology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA and 5Institute of Pathological Anatomy and Histopathology, School of Medicine, Polytechnic University of the Marche Region (Ancona), United Hospitals, Ancona, Italy

Muito Raro

Mutações germinais na SDH causam um risco

em vida de 14% para desenvolvimento CCR

Associação frequente a paragangliomas, GISTs,

adenomas hipófise, feocromocitomas


adenomas hipófise, feocromocitomas

Adultos jovens

Suspeita em H&E: sólido, oncocitóide, vacúolos intracitoplasmáticos, núcleos de baixo-grau; raramente alto-grau ou necrose.

Diagnóstico: IHQ: SDHB(-), CD117 (+), PAX-8 (+)

Prognóstico variável (30% metastizam)

Succinate Dehydrogenase (SDH)-deficient Renal Carcinoma: A Morphologically Distinct

EntityA Clinicopathologic Series of 36 Tumors From 27 Patients

Am J Surg Pathol ●Volume 38, Number 12, December 2014

ORIGINAL ARTICLE


A Clinicopathologic Series of 36 Tumors From 27 Patients

Anthony J. Gill, MD, FRCPA,*wz Ondrej Hes, MD,y Thomas Papathomas, MD,8

Monika Sˇedivcova´, MSc,y Puay Hoon Tan, MD,z Abbas Agaimy, MD,#

Per Arne Andresen, MSc,** Andrew Kedziora, FRCPA,w Adele Clarkson, BSc,*w Christopher W. Toon,

FRCPA,wzww Loretta Sioson, BSc,wz Nicole Watson,w

Angela Chou, FRCPA,wzz Julie Paik, MBBS,*w Roderick J. Clifton-Bligh, PhD, FRACP,zyy Bruce G.

Robinson, MD, FRCPA,zyy Diana E. Benn, PhD,zyy Kirsten Hills, FRCPA,88 Fiona Maclean,

FRCPA,zz Nicolasine D. Niemeijer, MD,##

Ljiljana Vlatkovic, MD,*** Arndt Hartmann, MD,# Eleonora P.M. Corssmit, MD,## Geert J.L.H. van

Leenders, MD,PhD,8 Christopher Przybycin, MD,www

Jesse K. McKenney, MD,www Cristina Magi-Galluzzi, MD,PhD,www Asli Yilmaz, MD,zzz Darryl

Yu, MD,zzz Katherine D. Nicoll, FRCPA,yyy Jim L. Yong, FRCPA,yyy

Mathilde Sibony, MD, PhD,888 Evgeny Yakirevich, MD, DSc,zzz Stewart Fleming, MD, FRCPATH,###

Chung W. Chow, FRCPA,**** Markku Miettinen, MD,wwww

Michal Michal, MD,y and Kiril Trpkov, MDzzz

Carcinoma céls. renais associados ao ALK (quinase do Linfoma anaplásico)

4



do Linfoma anaplásico)

Renal cell carcinoma with novel VCL–ALK fusion: new representative of ALK-associated tumor spectrum

Modern Pa tho logy (2011) 24, 430–442

& 2011 USCAP, Inc. All rights reserved 0893-3952/11 $32.00



Larisa V Debelenko1, Susana C Raimondi1, Najat Daw2, Bangalore R Shivakumar1, Dali Huang3, Marilu Nelson4 and Julia A Bridge3,4,5

1Department of Pathology, St Jude Children’s Research Hospital, Memphis, TN, USA; 2Department of Oncology, St Jude

Children’s Research Hospital, Memphis, TN, USA; 3Department of Pathology and Microbiology, University of Nebraska

Medical Center, Omaha, NE, USA; 4Department of Pediatrics/Meyer Munroe Institute, University of Nebraska Medical

Center, Omaha, NE, USA and 5Department

of Orthopedic Surgery, University of Nebraska Medical Center, Omaha, NE, USA

ALK alterations in adult renal cell carcinoma: frequency, clinicopathologic features and outcome in a large series of consecutively treated patients

Modern Pa tho logy (2012) 25, 1516–1525& 2012 USCAP, Inc. All rights reserved 0893-3952/12 $32.00


treated patients

William R Sukov1, Jennelle C Hodge1, Christine M Lohse2, Monica K Akre1, Bradley C Leibovich3, R Houston Thompson3 and John C Cheville1

1Department of Laboratory Medicine & Pathology, Mayo Clinic, Rochester, MN, USA; 2Division of Biomedical

Statistics & Informatics, Mayo Clinic, Rochester, MN, USA and 3Department of Urology, Mayo Clinic, Rochester,

MN, USA

Muito Raro - crianças/adultos

No grupo pediátrico associado a Anemia Falciforme

Suspeita em H&E: papilar/sólido e



Suspeita em H&E: papilar/sólido e oncocitico

Diagnóstico: ALK IHQ/FISH

Mau prognóstico

crizotinib (Xalkori®) e ceritinib (Zykadia®)

Carcinoma céls. renais associados à 5



associados à Leiomiomatose hereditária

Short Communication

American Journal of Pathology, Vol. 159, No. 3, September 2001

Copyright © American Society for Investigative Pathology



Familial Cutaneous Leiomyomatosis Is a Two-Hit

Condition Associated with Renal Cell Cancer of

Characteristic Histopathology

Inherited susceptibility to uterine leiomyomas and

renal cell cancer

PNAS | March 13, 2001 | vol. 98 | no. 6 | 3387–3392


renal cell cancerVirpi Launonen*†, Outi Vierimaa†‡, Maija Kiuru*†, Jorma Isola§, Stina Roth*, Eero Pukkala¶, Pertti Sistonen" , Riitta Herva**, and Lauri A.

Aaltonen*††

*Department of Medical Genetics, Haartman Institute, University of Helsinki, P. O. Box 21, FIN-00014, Helsinki, Finland; ‡Department of Clinical Genetics, Oulu University

Hospital, Kajaanintie 50, FIN-90220, Oulu, Finland; §Institute of Medical Technology, University of Tampere, FIN-33014, Tampere, Finland;¶Finnish Cancer Registry, Institute for Statistical and Epidemiological Cancer Research, Finnish Cancer Registry, FIN-00170, Helsinki, Finland;"Finnish Red Cross Blood Transfusion Service, Kivihaantie 7, FIN-00310, Helsinki, Finland; and **Department of Pathology, Oulu University Hospital, Kajaanintie 52, FIN-90220,

Oulu, Finland

Autossómico dominante/Fumarato

Mulheres < 30 anos (50% dos casos)

Leiomiomas cutâneos e uterinos

Suspeita em H&E: papilar de alto grau;


Suspeita em H&E: papilar de alto grau;

nucléolo proeminente com halo

perinucleolar

Diagnóstico: clínica+H&E+molecular

Mau prognóstico

REV IEW

NATURE CLINICAL PRACTICE UROLOGY SUDARSHAN ETAL. FEBRUARY 2007 VOL 4 NO 2


REV IEW

www.nature.com/clinicalpractice/uro

Mechanisms of Disease: hereditary leiomyomatosis and renal cell cancer—a distinct form of hereditary kidney cancerSunil Sudarshan, Peter A Pinto, Len Neckers and W Marston Linehan*

VEGF

TGFα GLUT1

PDGF

HIFHPH

O2

HPH

O2

HIF OH VHL complex

HIF HIF OH VHL complex

HIF Degradation: nomoxia

VHL: pseudohypoxia


PDGF

EPO

HPH

O2

HIF

HPH

HIF OH HLRCC: pseudohypoxia

Fumarate Malate

Carcinoma céls. renais tubulo-cístico

6



renais tubulo-cístico

Tubulocystic Carcinoma of the Kidney

Am J Surg Pathol ● Volume 33, Number 3, March 2009

ORIGINAL ARTICLE



Tubulocystic Carcinoma of the Kidney

Clinicopathologic Analysis of 31 Cases of a Distinctive Rare Subtype of Renal Cell Carcinoma

Mahul B. Amin, MD,* Gregory T. MacLennan, MD,w Ruta Gupta, MD,* David Grignon, MD,z Francois

Paraf, MD, PhD,y Annick Vieillefond, MD,J Gladell P. Paner, MD,*

Mark Stovsky, MD, MBA, FACS,w Andrew N. Young, MD, PhD,z John R. Srigley, MD,#

and John C. Cheville, MD **

Muito Raro; 60 anos

Aspecto esponjoso; bem circunscrito

H&E: cistos e túbulos com epitélio plano a

“hobnail”; baixo-grau nuclear



“hobnail”; baixo-grau nuclear

Diagnóstico: H&E Prognóstico favorável

10% progridem e podem desdiferenciar (relação com HLRCC?)

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