Metastatic Pheochromocytoma and Paraganglioma: Somatostatin Receptor 2 Expression, Genetics, and Therapeutic Responses
Authors' conclusion
SSTR2 expression was independently associated with SDHB/SDHx mutations and metastatic disease. We confirm a high disease control rate of somatostatin receptor-based therapies in metastatic PPGLs.
Study context
- Population
- Patients with pheochromocytomas and paragangliomas (PPGLs) in the ENSAT registry at 6 specialized Endocrine Tumor Centers in Germany, The Netherlands and Switzerland; Immunohistochemistry (IHC) for SDHB and SSTR2 in patients with available tumor tissue
- Participants
- 202 patients
- Outcome
- Association of SSTR2 IHC positivity with genetic and clinical-pathological features of PPGLs
Methodological detailsmulticentre, 6 specialized Endocrine Tumor Centers in Germany, The Netherlands and Switzerland, ENSAT registry
- Multicentre
- 6 specialized Endocrine Tumor Centers in Germany, The Netherlands and Switzerland
- ENSAT registry
Main finding
As reported in the abstract
- SSTR2 positive
- 50%
- SSTR2 positivity significantly associated with SDHB- and SDHx-related PPGLs; strongest SSTR2 staining intensity in SDHB-related PPGLs
- P = .01
- SSTR2 expression significantly associated with metastatic disease independent of SDHB/SDHx mutation status
- P < .001
Observational data: associations, not effects.
Further findings
Disease control rate with first-line SSTR-based radionuclide therapy in metastatic PPGLs
- Disease control rate (n = 22, n = 11 SDHx)
- 67%
Disease control rate with first-line "cold" somatostatin analogs in metastatic PPGLs
- Disease control rate (n = 6, n = 3 SDHx)
- 100%
Original publication
Metastatic Pheochromocytoma and Paraganglioma: Somatostatin Receptor 2 Expression, Genetics, and Therapeutic Responses. J Clin Endocrinol Metab. Published 22 March 2023. doi:10.
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