Pheochromocytoma

Section editor
	Unfilled If you are interested in this role, please contact us at [email protected].

3 regimens on this page 5 variants on this page

Guidelines

Given the rapid change in evidence in many areas of hematology/oncology, readers are encouraged to consider any guideline published 5+ years ago to be for historical purposes, only.

ESMO-EURACAN

• 2020: Fassnacht et al. Adrenocortical carcinomas and malignant phaeochromocytomas: ESMO–EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up PubMed

NANETS

• 2021: Fishbein et al. The North American Neuroendocrine Tumor Society Consensus Guidelines for Surveillance and Management of Metastatic and/or Unresectable Pheochromocytoma and Paraganglioma PubMed

NCCN

• NCCN does not currently have guidelines at this granular level; please see NCCN Guidelines - Neuroendocrine and Adrenal Tumors.

All lines of therapy

Cyclophosphamide, Dacarbazine, Vincristine

CVD: Cyclophosphamide, Vincristine, Dacarbazine

Regimen

Study	Evidence
Averbuch et al. 1988	Pilot, fewer than 20 pts

Chemotherapy

• Cyclophosphamide (Cytoxan) 750 mg/m² IV once on day 1
• Dacarbazine (DTIC) 600 mg/m² IV once per day on days 1 & 2
• Vincristine (Oncovin) 1.4 mg/m² IV once on day 1

21-day cycles

References

1. Averbuch SD, Steakley CS, Young RC, Gelmann EP, Goldstein DS, Stull R, Keiser HR. Malignant pheochromocytoma: effective treatment with a combination of cyclophosphamide, vincristine, and dacarbazine. Ann Intern Med. 1988 Aug 15;109(4):267-73. link to original article contains dosing details in abstract PubMed

Iobenguane I 131 monotherapy

Regimen variant #1

Study	Evidence
Rose et al. 2003b	Pilot, fewer than 20 pts

Note: Patients underwent stem cell harvest prior to treatment in case autologous stem cell infusion was needed.

Radiotherapy

• Iobenguane I 131 (Azedra) 12 to 18 mCi/kg (maximum single dose of 850 mCi) IV over 2 hours once on day 1

Supportive therapy

• Intravenous fluids started 12 hours before 131I-MIBG administration.
• Potassium iodide (KI) 6 mg/kg PO once at least 2 hours prior to 131I-MIBG, then potassium iodide (KI) 0.88 mg/kg PO Q4H x 7 days, then potassium iodide (KI) 1 mg/kg PO (frequency not specified) "for 45 days after the infusion."
• Potassium perchlorate 8 mg/kg once at least 2 hours prior to 131I-MIBG, then potassium perchlorate 2 mg/kg PO every 6 hours x 5 days.

"Repeat 131I-MIBG treatments were administered on a case-by-case basis in an effort to improve the overall response"

Regimen variant #2

Study	Evidence
Krempf et al. 1991	Pilot, fewer than 20 pts

Radiotherapy

• Iobenguane I 131 (Azedra) 740 megabequerel/mg every 3 months

References

1. Krempf M, Lumbroso J, Mornex R, Brendel AJ, Wemeau JL, Delisle MJ, Aubert B, Carpentier P, Fleury-Goyon MC, Gibold C, Guyot M, Lahneche B, Marchandise X, Schlumberger M, Charbonnel B, Chatal JF. Use of m-[131I]iodobenzylguanidine in the treatment of malignant pheochromocytoma. J Clin Endocrinol Metab. 1991 Feb;72(2):455-61. link to original article contains dosing details in abstract PubMed
2. Rose B, Matthay KK, Price D, Huberty J, Klencke B, Norton JA, Fitzgerald PA. High-dose 131I-metaiodobenzylguanidine therapy for 12 patients with malignant pheochromocytoma. Cancer. 2003 Jul 15;98(2):239-48. link to original article contains dosing details in manuscript PubMed