Pheochromocytoma

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2 regimens on this page
4 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

NANETS

NCCN

All lines of therapy

Cyclophosphamide, Dacarbazine, Vincristine

CVD: Cyclophosphamide, Vincristine, Dacarbazine

Regimen

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

Chemotherapy

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 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

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

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
  3. 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