Pheochromocytoma
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3 regimens on this page
5 variants on this page
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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
- 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 | Dates of enrollment | Evidence |
|---|---|---|
| Pryma et al. 2018 (MIP-IB12B) | 2009-06 to 2016-02 | Phase 2 (RT) |
Note: This was the study cited by the FDA in their approval.
Radiotherapy
- Iobenguane I 131 (Azedra) by the following weight-based criteria:
- More than 62.5 kg: 18.5 GBq IV over 30 minutes once on day 1
- 62.5 kg or less: 0.296 GBq/kg IV over 30 minutes once on day 1
90-day cycle for up to 2 cycles
Regimen variant #2
| 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 #3
| Study | Evidence |
|---|---|
| Krempf et al. 1991 | Pilot, fewer than 20 pts |
Radiotherapy
- Iobenguane I 131 (Azedra) 740 megabequerel/mg every 3 months
References
- 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
- 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
- MIP-IB12B: Pryma DA, Chin BB, Noto RB, Dillon JS, Perkins S, Solnes L, Kostakoglu L, Serafini AN, Pampaloni MH, Jensen J, Armor T, Lin T, White T, Stambler N, Apfel S, DiPippo VA, Mahmood S, Wong V, Jimenez C. Efficacy and Safety of High-Specific-Activity 131I-MIBG Therapy in Patients with Advanced Pheochromocytoma or Paraganglioma. J Nucl Med. 2019 May;60(5):623-630. Epub 2018 Oct 5. link to original article link to PMC article PubMed NCT00874614
Subsequent lines of therapy
Sunitinib monotherapy
Regimen
| Study | Dates of enrollment | Evidence | Comparator | Comparative Efficacy |
|---|---|---|---|---|
| Baudin et al. 2024 (FIRSTMAPPP) | 2011-12-01 to 2019-01-31 | Randomized Phase 2 (E-esc) | Placebo | Superior PFS12 (primary endpoint) PFS12: 36% vs 19% |
References
- FIRSTMAPPP: Baudin E, Goichot B, Berruti A, Hadoux J, Moalla S, Laboureau S, Nölting S, de la Fouchardière C, Kienitz T, Deutschbein T, Zovato S, Amar L, Haissaguerre M, Timmers H, Niccoli P, Faggiano A, Angokai M, Lamartina L, Luca F, Cosentini D, Hahner S, Beuschlein F, Attard M, Texier M, Fassnacht M; ENDOCAN-COMETE; ENSAT Networks. Sunitinib for metastatic progressive phaeochromocytomas and paragangliomas: results from FIRSTMAPPP, an academic, multicentre, international, randomised, placebo-controlled, double-blind, phase 2 trial. Lancet. 2024 Mar 16;403(10431):1061-1070. Epub 2024 Feb 22. link to original article contains dosing details in abstract PubMed NCT01371201