Retrospective study of patients with metastatic/progressive pheochromocytoma (PCC) and paraganglioma (PGL) treated with (131) I MIBG in our hospital during 20152018 period.
Methods: There are no established criteria for establishing PCC/PGL as malignant apart from de presence of metastases at diagnosis. Radionuclide therapy (131) I-metaiodobenzylguanidine (MIBG) is frequently used in this patients when surgery is not possible. Indication of MIBG treatment was made in the Neuroendocrine Tumor Multidisciplinary Team meeting. After a (123) I-MIBG to assess tracer uptake standard 200 mCi (131) I-MIBG dose was administered in each patient until disease stabilization and then follow up was made.
|Paciente A||Paciente B||Paciente C|
|Diagnosis||PGL stage IV||PCC stage IV||PCC stage IV|
|Metastases at diagnosis||liver, bone, peritoneum, mesenteric lymph nodes||Lung||Diaphragm, liver, periadrenalectomy tissue, para aortic lymph node|
|Clinically functioning tumor||Mild hypertension Flushing||Bad controlled hypertension||Bad controlled hypertension|
|Other treatments before I-MIBG||Surgery 2009-2013 Temodal+Capecitabine 2015 (neutropenia)||Surgery 1980||Surgery 2004|
|Treatment cycles||4 (Jan 2016-March 2017)||4 (Sept 2015-Jan 2017)||4 (Nov 2013-2014)+4 (Feb 2018-August 2018)|
|Before treatment Plasma Normetanephrine (N<180 pg/ml) Urine Normetanephirne (N<444 ug/24h)||January 2016 -Plasma Normetanephrine 199 pg/ml -Urine Normetanephrine 1122 ug/24||September 2015 -Plasma Normetanephrine 300 pg/ml -Urine Normetanephrine 1192ug/24 h||2013 -Plasma Normetanephrine 788 (<444)|
|After last MIBG treatment (hormonal response)||October 2018 -Plasma Normetanephrine 158 pg/ml -Urine Normetanephrine 614 ug/24 h||November 2018 -Plasma Normetanephrine 2 213 pg/ml -Urine Normetanephrine 631 ug/24 h||-|
|Clinical Response to treatment||Less frequent and intense hot flashes Well controlled hypertension||Well controlled hypertension||Better control of hypertension|
|Radiological response (RECIST) to treatment||Stable disease||Stable disease||Progression liver metastases in 2014 Stable disease 2018|
|Metabolic response to treatment||Stabilization or mild response in liver and mesentery. Metabolic response in bone||Stabilization or partial response (lung)||Partial metabolic response in liver Stabilization para aortic lymph node|
|Free Progression Survival||+36 months||+40 months||+45 months|
|Cumulative activity (MBq)||800 mCi||800 mCi||1550 mCi|
|Haematological toxicity||None||Neutropenia grade2||None|
Conclusion: Therapy with (131) I-MIBG is a safe therapeutical option in patients with metastatic PCC/PGL, leading to easier control of hypertension, and mild improvement or at least stabilization of disease progression without major side effects in our 4 year centre experience.
18 - 21 May 2019
European Society of Endocrinology