RENAL CELL CARCINOMA IN CHILDREN AND ADOLESCENTS A RETROSPECTIVE - - PowerPoint PPT Presentation

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renal cell carcinoma in children and adolescents
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RENAL CELL CARCINOMA IN CHILDREN AND ADOLESCENTS A RETROSPECTIVE - - PowerPoint PPT Presentation

Dec 24, 2022 161 likes •331 views

RENAL CELL CARCINOMA IN CHILDREN AND ADOLESCENTS A RETROSPECTIVE ANALYSIS OF A COOPERATIVE EUROPEAN COHORT Thomas Denize 3rd year resident @anotherpathres Pathology department, Pr Aurore Coulomb MD PhD APHP.6 Armand Trousseau Hospital, Paris,

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RENAL CELL CARCINOMA IN CHILDREN AND ADOLESCENTS

A RETROSPECTIVE ANALYSIS OF A COOPERATIVE EUROPEAN COHORT

Thomas Denize 3rd year resident @anotherpathres Pathology department, Pr Aurore Coulomb MD PhD APHP.6 Armand Trousseau Hospital, Paris, France T eam: Simona Massa, Christian Vokuhl, Alexander Valent, Adele T esti, Linda Dainese, Lucia Militti, Nathalie Rioux- Leclerq Estelle Thebaud, Rhoikos Furtwaengler, Kathy Pritchard-Jones, Norbert Graf, Filippo Spreafico, Arnauld Verschuur, Gordan Vujanic, Paola Collini

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INTRODUCTION – RENAL CELL CARCINOMA

2016 WHO Classification: 16 subtypes + 6 emerging entities

2 – 6 % of renal malignancies in children

Variable prognosis depending on the subtype

Microphtalmia-associatedTranscription Factor translocated carcinoma

Renal cell carcinoma in children, adolescents and young adults: A national cancer database study. Akhavan A and al. Pediatric Urology 2015

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MATERIEL AND METHODS

4 countries: France, Germany, Italy, United Kingdom

RTSG/SIOP pathologist panel

More than 40 centers

Centralized review of all cases

Reference: 2016 WHO classification

French/Italian cases: unified wide IHC panel

CK7, AMACR, CAIX, TFE3, Vimentin, CD117, CK19, HMWCK, P63, INI1, HMB45, MelanA, ALK, SDHB, FH 

TFE3 FISH on all cases

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DEMOGRAPHICS

162 children: 87 males, 75 females

166 tumors

Median age: 11 years old (9m-18y)

Mean size: 6.4cm

Mediorenal 22% Upper pole 23% Lower pole 34% Whole kidney 17% upper 2/3 4%

TUMOR LOCALIZATION

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HISTOTYPE REPARTITION

MiTF 41% Others 59%

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TFE3 RCC

N = 62

Girls > boys (SR 1.6)

Mean age 9.8y, median 11y

66% N+;

FO: late relapses, 4 known M+

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MITF-TRANSLICATION RCC

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IHC

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AMACR 94% CAIX 47% HMB45 18% Vim 57%

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BEWARE OF TFEB

N = 6

Can look like anything

Perform FISH whenever TFE3 is negative

No amplification in our series MelanA

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OTHER SUBTYPES

Unclassified Papillary chromophobe CCRCC, adult- type collecting duct carcinoma SDHB deficient ALK translocated associated with neuroblastoma FH deficient NON-MITF TUMORS

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FOCUS ON: HEREDITARY LEIOMYOMATOSIS AND RENAL CELL CANCER SYNDROME, OR FH-DEFICIENT RCC

New entity 2016 WHO classification

Cutaneous and uterine leiomyomas + RCC → genetic counseling

Loss of FH IHC

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FH-DEFICIENT RCC

Male, 14y

17cm left renal mass

N+

Death 17m after diagnosis → M+ (lung, bone)

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FH DEFICIENT RCC

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FH DEFICIENT RCC

FH FH AMACR CK7

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CONCLUSION

MiTF translocated RCC is the main subtype of pediatric RCC (41%)

Non MiTF translocated RCC: heterogenous (9 subtypes), similar to adult RCC

Accurate diagnosis requieres a large IHC panel including FH and SDHB

TFE3 FISH should be performed on all pediatric RCC

Ongoing molecular analysis for better characterization of the MiTF translocated RCC