Impa c t of dia g nosis with Pa nc re a tic Ne uroe ndoc rine T - - PowerPoint PPT Presentation

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Oct 08, 2023 6 likes •166 views

14 th Inte rna tiona l VHL Me dic a l Re se a rc h Symposium Impa c t of dia g nosis with Pa nc re a tic Ne uroe ndoc rine T umors on Morta lity in Pa tie nts with Von Hippe l- L inda u Dise a se L ia t Arnon, MSc , Re ut Ha lpe rin, MD

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SLIDE 1

Impa c t of dia g nosis with Pa nc re a tic Ne uroe ndoc rine T umors on Morta lity in Pa tie nts with Von Hippe l- L inda u Dise a se

L ia t Arnon, MSc , Re ut Ha lpe rin, MD PhD, Amit T irosh, MD VHL Clinic a l Ca re Ce nte r, Ne uroe ndoc rine T umors Se rvic e , Division of E ndoc rinolog y, Dia be te s a nd Me ta bolism, She ba Me dic a l c e nte r, T e l Ha shome r, Isra e l 14th Inte rna tiona l VHL Me dic a l Re se a rc h Symposium

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SLIDE 2
  • Orig ina te from ne uroe ndoc rine c e lls
  • T

he inc ide nc e of NE Tha s inc re a se d

  • Most PNE

Ta re spora dic

  • T

he y c a n a lso de ve lop a s pa rt of inhe rite d syndrome s, suc h a s von Hippe l- L inda u (VHL ).

L ung NE T Pa nc re a tic NE T Ga stro inte stina l NE T

VHL related PNET

Sporadic PNET

VHL related PNET

Sporadic PNET

OR

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SLIDE 3

Ga rc ia -Ca rb o ne ro e t al., 2016, Bla nsfie ld e t al., 2007, K ra uss e t a l., 2018, I g a ra shi e t a l., 2014

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SLIDE 4
  • Pre va le nc e : 8-17%
  • Me dia n a g e a t onse t: 34y (14-55)
  • Surve illa nc e - Size (g e notype )
  • Ma na g e me nt is ma inly surg ic a l

Bla nsfie ld e t al., 2007, I g a ra shi e t al., 2014, L

  • nse r e t al., 2003, T

iro sh e t al., 2017, Krauss e t al., 2018

68Ga-DOTATATE PET/CT (somatostatin receptor-based imaging)

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SLIDE 5
  • L

e a ding c o ntrib uto rs fo r VHLmo rb idity a nd mo rta lity:

 Ce ntra l ne rvo us syste m he ma ng io b la sto ma (HB)  Cle a r c e ll re na l c e ll c a rc ino ma (RCC)

  • Wha t is the impa c t of dia g nosis with PNE

T

  • n the ove ra ll morta lity in

pa tie nts with VHL ?

L

  • nse re t al., 2003
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SLIDE 6
  • Re trospe c tive study, b a se d o n the Surve illanc e , E

pide mio lo g y and E nd Re sults (SE E R) da ta b a se .

  • Pa tie nts:

Dia g no se d with RCC, PNE T , HB a nd/ o r PPGL

  • VHL

dia g nosis:

  • Ana lysis:

Univa ria te a na lysis with surviva l a na lysis b y K a pla n-Me ie r c urve s, Multiva ria b le a na lysis using the Co x pro po rtio na l re g re ssio n.

Bind e rup, M. L . M. e t al. (2017)

 “I

nte rna tio na l Crite ria ”

 “Da nish Crite ria ”

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SLIDE 7

p va lue Spora dic VHL

16,174 170 n <0.001 63.9 (11.3) 46.6 (17.0) Me a n a g e (SD) <0.001 15,620 164 RCC <0.001 419 91 PNE T <0.001 75 83 HB <0.001 60 7 PPGL

SD, sta nd a rd d e via tio n; RCC, re na l c e ll c a rc ino ma ; PNET , pa nc re a tic ne uro e nd o c rine tumo r; HB, he ma ng io b la sto ma ; PPGL , phe o c hro mo c yto ma / pa ra g a nglio ma

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SLIDE 8

p va lue Spora dic VHL

n=419 n=91 <0.001 60.2±13.0 54.0±14.0 Ag e a t dia g no sis me a n (SD) 0.8 229 (54.7) 48 (52.7) Ma le se x n(%) 0.08 217 (57.9) 32 (45.7) Me ta sta tic PNE T n(%) 0.007 Dia me te r n(%) 27 (9.2) 10 (16.9) <10 mm 114 (38.6) 31 (52.5) 11-29 mm 154 (52.2) 18 (30.5) >30 mm

SD, sta nd a rd d e via tio n; PNET , pa nc re a tic ne uro e nd o c rine tumo r;

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SLIDE 9
  • Pa tie nts

with VHL

  • re la te d

PNET ha d lo we r ACM risk c o mpa re to pa tie nts with spo ra dic PNE T

  • Hig he r ACM risk in pa tie nts with VHLa nd PNE

Tvs. tho se witho ut PNE T .

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SLIDE 10

All PNE T Spora dic PNE T VHL

  • re la te d PNE

T

A tre nd fo r hig he r risk fo r ACM wa s fo und in pa tie nts ha rb o ring PNE T with dia me te r ≥30 vs. <30 mm (p=0.06 a nd p=0.1 in pa tie nts with spo ra dic a nd VHL

  • re la te d PNE

T , re spe c tive ly).

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SLIDE 11
  • Simila r tre nd o f hig he r ACM risk with

hig he r g ra de s in VHL

  • re la te d a nd

spo ra dic PNE T

Gra de Sta g e

  • Hig he r

ACM risk in pa tie nts with spo ra dic a dva nc e d PNET , with a simila r tre nd a mo ng pa tie nts with a dva nc e d VHL

  • re la te d PNE

T .

However, is PNET diagnosis an independent risk factor for ACM in VHL?

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SLIDE 12
  • Dia g no sis with PNE

T wa s no t a sso c ia te d with inc re a se d fo r ACM (HR 0.42, 95% CI 0.12-1.5, p=0.18).

  • An inc re a se d risk fo r ACM wa s

de te c te d a mo ng pa tie nts with me ta sta tic PNE T (HR 3.71, 95% CI1.4-9.8, p=0.008).

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SLIDE 13

Doe s PNE T dia g nosis a ffe c t ACM in pa tie nts with VHL ?

  • Ha s no inde pe nde nt impa c t o n ACM in

pa tie nts with VHL .

  • Co mple x e ffe c t o f multiple VHL
  • re la te d

tumo rs o n ACM.

  • Hig he r ACM risk in pa tie nts with

a dva nc e d PNE T

  • Impo rta nc e o f surve illa nc e fo r e a rly

de te c tio n o f hig h-risk PNE T

. L

  • c a lize d PNE

T Adva nc e d PNE T Dia me te r Gra de Sta g e VHL

  • re la te d PNE

T spo ra dic PNE T .

Do pa tie nts with spora dic a nd VHL re la te d PNE T diffe r in ACM risk?

ACM

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SLIDE 14
  • Unde re stima tio n o f VHL

po pula tio n (SE E R da ta b a se )

  • So me o f the va ria b le s ha ve missing va lue s
  • No da ta o n VHLg e no type
  • No lo ng itudina l da ta o n tumo r size dyna mic s
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SLIDE 15
  • PNE

T surve illa nc e in VHL ma y a ffe c t ACM b y re duc ing the ra te o f me ta sta tic

dise a se .

  • T

he impa c t of e a rly de te c tion of hig h- risk PNE

To n ACM sho uld b e inve stig a te d in

future studie s.

  • Ca re g ive r- pa tie nt re la tions ma y a ssist in time ly e xe c utio n o f the VHL

A g uide line s fo r o ptima l sc re e ning .

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SLIDE 16

14th Inte rna tiona l VHL Me dic a l Re se a rc h Symposium L ia t.a rnon@she ba .he a lth.g ov.il