Adrenal disease

Clinical Profile, Outcomes, and Predictors of Malignant Pheochromocytoma and Paraganglioma: Insights From a Single‐Center Cohort

Clin Endocrinol (Oxf)Published Retrospective cohort study

Authors' conclusion

Malignant PPGLs had a variable clinical course and were amenable to multimodal therapeutic strategies with a favorable outcome in about 67% of the patients. Although mPPGL had larger tumor diameter compared to non-mPPGL, no particular size cut-off could accurately predict metastases. Adjuvant 131I MIBG therapy is a useful treatment option in resource limited settings.

Study context

Population
Patients with PPGL managed at a single centre over 11 years (2013–2024)
Participants
218 patients
Exposure
Malignant PPGL vs non-metastatic PPGL
  • Malignant pheochromocytoma and paraganglioma (mPPGL)n = 36
  • non-mPPGLn = 182reference
Outcome
Tumor size, mPPGL vs non-mPPGL
Methodological detailssingle-centre
  • Single-centre

Main finding

As reported in the abstract

mPPGL

8.8

± 5.2

non-mPPGL

6.3

± 3.3

P = 0.001

Observational data: associations, not effects.

Further findings

Clinical profile of mPPGL

Median age at diagnosis of metastases
39.5 years (range 10-62)
Synchronous metastases
21 (58.3%)
Metachronous metastases
15 (41.7%)
Median time to metachronous metastases from primary diagnosis
76 months (range 13-270 months)

Detection of metastases by imaging

18FDG PET-CT
100% (4/4)
68 Ga DOTATATE PET-CT
100% (4/4)
CT/MRI
97.2% (35/36)
131I MIBG scan
79.3% (23/29)

Treatment and outcomes of mPPGL

Surgery as primary treatment
78%
131I MIBG therapy
19 (52.8%)
Died due to metastatic disease
11
Died within a year of diagnosis of metastases
9
Median survival at last follow-up
31.5 months (range 3-96)

Original publication

Clinical Profile, Outcomes, and Predictors of Malignant Pheochromocytoma and Paraganglioma: Insights From a Single‐Center Cohort. Clin Endocrinol (Oxf). Published 30 July 2025. doi:10.1111/cen.70007

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