CC BY-NC-ND 4.0 · Indian J Med Paediatr Oncol 2022; 43(02): 208-215
DOI: 10.1055/s-0041-1735600
Case Report with Review of Literature

Metastatic Extra-Adrenal Pheochromocytoma with Single Kidney and Renal Compromise: A Case Report of Excellent Response, Tolerability, and Outcome to a Modified Regimen of 131I-mIBG, and Decision-Making between 131I-mIBG Therapy and PRRT

Sarvesh Loharkar
1   Radiation Medicine Centre, Bhabha Atomic Research Centre, Tata Memorial Hospital Annexe, Mumbai, Maharashtra, India
2   Homi Bhabha National Institute, Mumbai, Maharashtra, India
Sandip Basu
1   Radiation Medicine Centre, Bhabha Atomic Research Centre, Tata Memorial Hospital Annexe, Mumbai, Maharashtra, India
2   Homi Bhabha National Institute, Mumbai, Maharashtra, India
› Author Affiliations
Financial Support and Sponsorship Nil.


Determining the choice and the goal is key element for decision-making of a systemic radionuclide therapy. They should be clearly defined in deciding and individualizing the dose and regimen. For iodine-131 metaiodobenzylguanidine (131I-mIBG) therapy, the important considerations during dose fractionation include disease burden, tumor biology, functional symptoms, and associated comorbidities, all of which are important determinants for the intent and course of treatment. Herein, we present the case of a 67-year-old elderly female with highly functional metastatic recurrent extra-adrenal pheochromocytoma (presenting 42 years after the primary surgery and 32 years following excision of pararenal recurrence) with multiple comorbidities including single kidney and borderline renal compromise, treated successfully with a relatively lower dose of 131I-mIBG (cumulative dose of 22.2 GBq in four cycles with a mean dose of 5.7 GBq per therapy cycle). The excellent tumor burden reduction, hormonal tumor marker response, and most importantly asymptomatic status could be achieved with the administered dose. On follow-up, none of the pretherapeutic parameters (including renal function) showed any further derangement compared with the baseline during next 24 months following the treatment. All cycles were well tolerated with only reversible hematological toxicity that normalized without any active intervention. The report is intended to provide some guidance for future therapeutic regimens.

Declaration of Patient Consent

The authors certify that they have obtained all appropriate patient consent forms.

Publication History

Received: 17 July 2020

Accepted: 25 December 2020

Article published online:
02 February 2022

© 2022. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (

Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India

  • References

  • 1 Chrisoulidou A, Kaltsas G, Ilias I, Grossman AB. The diagnosis and management of malignant phaeochromocytoma and paraganglioma. Endocr Relat Cancer 2007; 14 (03) 569-585
  • 2 Adjallé R, Plouin PF, Pacak K, Lehnert H. Treatment of malignant pheochromocytoma. Horm Metab Res 2009; 41 (09) 687-696
  • 3 Kundu S, Kand P, Basu S. Comparative evaluation of iodine-131 metaiodobenzylguanidine and 18-fluorodeoxyglucose positron emission tomography in assessing neural crest tumors: will they play a complementary role?. South Asian J Cancer 2017; 6 (01) 31-34
  • 4 Tan TH, Hussein Z, Saad FF, Shuaib IL. Diagnostic performance of (68)Ga-DOTATATE PET/CT, (18)F-FDG PET/CT and (131)I-MIBG scintigraphy in mapping metastatic pheochromocytoma and paraganglioma. Nucl Med Mol Imaging 2015; 49 (02) 143-151
  • 5 Sisson JC, Shapiro B, Beierwaltes WH. et al. Radiopharmaceutical treatment of malignant pheochromocytoma. J Nucl Med 1984; 25 (02) 197-206
  • 6 Hoefnagel CA. Radionuclide therapy revisited. Eur J Nucl Med 1991; 18 (06) 408-431
  • 7 Fitzgerald PA, Goldsby RE, Huberty JP. et al. Malignant pheochromocytomas and paragangliomas: a phase II study of therapy with high-dose 131I-metaiodobenzylguanidine (131I-MIBG). Ann N Y Acad Sci 2006; 1073: 465-490
  • 8 Wafelman AR, Hoefnagel CA, Maes RA, Beijnen JH. Radioiodinated metaiodobenzylguanidine: a review of its biodistribution and pharmacokinetics, drug interactions, cytotoxicity and dosimetry. Eur J Nucl Med 1994; 21 (06) 545-559
  • 9 Basu S, Nair N. Stable disease and improved health-related quality of life (HRQoL) following fractionated low dose 131I-metaiodobenzylguanidine (MIBG) therapy in metastatic paediatric paraganglioma: observation on false “reverse” discordance during pre-therapy work up and its implication for patient selection for high dose targeted therapy. Br J Radiol 2006; 79 (944) e53-e58
  • 10 Rachh SH, Abhyankar S, Basu S. [131I]Metaiodobenzylguanidine therapy in neural crest tumors: varying outcome in different histopathologies. Nucl Med Commun 2011; 32 (12) 1201-1210
  • 11 Matthay KK, Weiss B, Villablanca JG. et al. Dose escalation study of no-carrier-added 131I-metaiodobenzylguanidine for relapsed or refractory neuroblastoma: new approaches to neuroblastoma therapy consortium trial. J Nucl Med 2012; 53 (07) 1155-1163
  • 12 Tobes MC, Fig LM, Carey J, Geatti O, Sisson JC, Shapiro B. Alterations of iodine-131 MIBG biodistribution in an anephric patient: comparison to normal and impaired renal function. J Nucl Med 1989; 30 (09) 1476-1482
  • 13 Boltze C, Mundschenk J, Unger N. et al. Expression profile of the telomeric complex discriminates between benign and malignant pheochromocytoma. J Clin Endocrinol Metab 2003; 88 (09) 4280-4286
  • 14 Kapur S, Iqbal AN, Levin MB. A case of malignant metastatic pheochromocytoma after eight years of primary diagnosis. World J Oncol 2014; 5 (01) 33-40
  • 15 van Hulsteijn LT, Niemeijer ND, Dekkers OM, Corssmit EP. (131)I-MIBG therapy for malignant paraganglioma and phaeochromocytoma: systematic review and meta-analysis. Clin Endocrinol (Oxf) 2014; 80 (04) 487-501
  • 16 Kaltsas GA, Mukherjee JJ, Foley R, Britton KE. Treatment of metastatic pheochromocytoma and paraganglioma with 131I-meta-iodobenzylguanidine (MIBG). Endocrinologist 2001; 13: 321-333
  • 17 Troncone L, Rufini V. 131I-MIBG therapy of neural crest tumours (review). Anticancer Res 1997; 17 (3B): 1823-1831
  • 18 Gedik GK, Hoefnagel CA, Bais E, Olmos RA. 131I-MIBG therapy in metastatic phaeochromocytoma and paraganglioma. Eur J Nucl Med Mol Imaging 2008; 35 (04) 725-733
  • 19 Rose B, Matthay KK, Price D. et al. High-dose 131I-metaiodobenzylguanidine therapy for 12 patients with malignant pheochromocytoma. Cancer 2003; 98 (02) 239-248
  • 20 Basu S, Abhyankar A, Jatale P. The current place and indications of 131I-metaiodobenzylguanidine therapy in the era of peptide receptor radionuclide therapy: determinants to consider for evolving the best practice and envisioning a personalized approach. Nucl Med Commun 2015; 36 (01) 1-7
  • 21 Wafelman AR, Nortier YL, Rosing H. et al. Renal excretion of meta-iodobenzylguanidine after therapeutic doses in cancer patients and its relation to dose and creatinine clearance. Nucl Med Commun 1995; 16 (09) 767-772
  • 22 Basu S. Dose fractionation in 131I-metaiodobenzylguanidine (MIBG) therapy: should the tumour biology and intent of therapy be the guide?. Eur J Nucl Med Mol Imaging 2010; 37 (09) 1798-1799