Johnathan Ebben: 3 major subtypes of paraganglioma
Johnathan Ebben, Oncology Fellow at the University of Wisconsin-Madison, shared on X/Twitter:
“Tumor Board Tuesday Case
3 major subtypes of paraganglioma
- VHL (belzutifan in the future?)
- Wnt (low TMB)
- Kinase driven (RET rearranged)
For now, clinical decision making= imaging (MIBG, DOTATATE) and aggressiveness.
Grab your free CME here.
Take home:
- ~50% PPGLs= germline mut
- SDHB mut= more aggressive disease; treatment implications
- Radioligand, TKIs, chemo—sequencing unclear; treatment determined by molecular markers, disease biology
Check out the full discussion here.
Rachel Riechelmann 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 generation sequencing in PPGL?
— GERMLINE TESTING for all patients —
- SDHBm= common; implications for family, more aggressive disease
Tumor NGS may have higher value in future; actionable mutations rare, but could change with new treatment (VHL).
How do we treat? Treatment sequencing still a ?
- MIBG+ = MIBG therapy (only approved treatment for pheochromocytoma)
- DOTATATE/Ga PET+= PRRT with lu177-somatostatin
- Aggressive dz= cape/tem
Also can use TKIs vs chemotherapy. If imaging mixed, choose treatment based on most avid scan.
Clinical trials in PPGL/pheochromocytoma are key!
Join us next week as María Mercedes Hincapié and Anniina Färkkilä 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: Johnathan Ebben/X
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