Rachel Riechelm and Álvaro Lopes de Oliveira discuss paragangliomas and pheochromocytoma – Tumor Board Tuesday
Tumor Board Tuesday shared on X:
“No Tumor Board Tuesday this past week, so we’re bringing you a special edition of case wrap-up.
Rachel Riechelm and Álvaro Lopes de Oliveira discuss paragangliomas and pheochromocytoma:
– Functional vs. non-functional
– Markers & imaging to inform treatment
– Treatment sequencing – a huge unknown!
Tumor Board Tuesday Case Discussion:
3 major subtypes of paraganglioma:
– VHL (belzutifan in the future?)
– Wnt (low TMB)
– Kinase-driven (RET rearranged)
For now, clinical decision-making relies on imaging (MIBG, DOTATATE) and aggressiveness.
Grab your free CME here.
Takeaways:
– Approximately 50% of PPGLs = germline mutation
– SDHB mutation = more aggressive disease; treatment implications
– Radioligand, TKIs, chemo – sequencing unclear; treatment determined by molecular markers, disease biology
Check out the full discussion here.
Rachel Riechelm discusses initial workup:
— 24h metanephrines (is it a pheo/functional tumor?)
Imaging:
-MIBG scan (norepi)
-DOTATATE (somatostain r)
-FDG PET
!! ALL 3 scans highly preferred !!
Role of next-gen sequencing in PPGL?
– Germline testing for all patients
– SDHB mutation = common; implications for family, more aggressive disease
Tumor NGS may have increased value in the future; actionable mutations rare, but could change with new treatments (VHL)
How do we treat? Treatment sequencing still a question:
– MIBG+ = MIBG therapy (only approved treatment for pheo)
– DOTATATE/Ga PET+ = PRRT with lu177-somatostatin
– Aggressive disease = cape/tem
Also can use TKIs vs. chemo. If imaging mixed, choose treatment based on the most avid scan.
Clinical trials in PPGL/pheochromocytoma are key!
Join us next week as Maria M. Hincapie and afarkkila take us through the ins and outs of PARPi therapy in ovarian cancer. SPECIAL TIME: TBT will start at 1 PM EST on May 7th! See you there!”
Source: Tumor Board Tuesday/X
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