Pheochromocytoma
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2 regimens on this page
3 variants on this page
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Guidelines
ESMO-EURACAN
- 2020: Fassnacht et al. Adrenocortical carcinomas and malignant phaeochromocytomas: ESMO–EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up
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
NCCN
All lines of therapy
Cyclophosphamide, Dacarbazine, Vincristine
CVD: Cyclophosphamide, Vincristine, Dacarbazine
Regimen
| Study | Evidence |
|---|---|
| Averbuch et al. 1988 | Pilot, <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 | Evidence |
|---|---|
| Rose et al. 2003b | Pilot, <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, <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