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