ArticleThe Journal of clinical endocrinology and metabolism2023
Metastatic Pheochromocytoma and Paraganglioma: Somatostatin Receptor 2 Expression, Genetics, and Therapeutic Responses.
Article in The Journal of clinical endocrinology and metabolism, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 35 papers.
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Who cites it
35 citing papers in PubMed, 43 citations in OpenAlex.
- Evaluation of [¹⁸F]AlF-NOTA-octreotide PET/CT in routine clinical use: a retrospective analysis in 288 neuroendocrine tumor patients.European journal of nuclear medicine and molecular imaging · 2026Article
- [European journal of nuclear medicine and molecular imaging · 2026Article
- Systemic management of adrenal malignancies: adrenocortical carcinoma and pheochromocytoma/paraganglioma.Endocrine connections · 2026Article
- Functional profiling of somatostatin receptors identifies somatostatin receptor subtype 2 as a vulnerability in Succinate Dehydrogenase SDHB-deficient pheochromocytomas and paragangliomas.Molecular biomedicine · 2026Article
- [European journal of nuclear medicine and molecular imaging · 2026Article
- Spinal decompression and radionuclide therapy for an unresectable FGF23 transmitted tumor causing cervical myelopathy: a case report and literature review.Frontiers in oncology · 2026Article
- Update on Systemic Therapies for Metastatic/Unresectable Pheochromocytomas and Paragangliomas and Future Directions.Cancers · 2025Review
- [European journal of nuclear medicine and molecular imaging · 2025Article
- New Insights into the Clinical Characterization of SDHAF2-related Familial Paraganglioma Syndrome.The Journal of clinical endocrinology and metabolism · 2025Article
- Potentially fatal crisis afterEndocrine journal · 2025Review
- The Perioperative Biochemical and Clinical Considerations of Pheochromocytoma Management.International journal of molecular sciences · 2025Review
- Familial Paraganglioma Presenting With Acute Coronary Syndrome and Coronary Vasospasm.JCEM case reports · 2025Article
- The Biochemical-Imaging Connection: Urinary Noradrenaline and Fluorodeoxyglucose-Positron Emission Tomography in Unresectable or Metastatic Pheochromocytomas and Paragangliomas.Diagnostics (Basel, Switzerland) · 2025Article
- Clinical, genetic, radiological characteristics and management of mediastinal paragangliomas: a literature review and case series.Endocrine-related cancer · 2025Review
- Clinicopathological and genomic analysis of pediatric pheochromocytoma and sympathetic paraganglioma.Endocrine journal · 2025Article
- [Germline mutations define the prognosis and therapy of pheochromocytomas and paragangliomas].Magyar onkologia · 2025Review
- Pheochromocytomas and Paragangliomas-Current Management.Cancers · 2025Review
- Ga-68 DOTATATE PET imaging in the management of head and neck paragangliomas: a potential game changer.Frontiers in oncology · 2025Article
- Bone metastases in pheochromocytomas and paragangliomas: a narrative review of presentation, diagnosis and management.Frontiers in endocrinology · 2025Review
- SDHB-Associated Pheochromocytomas: What is Their Clinical Behavior?Annals of surgical oncology · 2024Article
Corrections and comments
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Authors and funding
33 authors at 13 institutions in 7 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
contextPheochromocytomas and paragangliomas (PPGLs) with pathogenic mutations in the succinate dehydrogenase subunit B (SDHB) are associated with a high metastatic risk. Somatostatin receptor 2 (SSTR2)-dependent imaging is the most sensitive imaging modality for SDHB-related PPGLs, suggesting that SSTR2 expression is a significant cell surface therapeutic biomarker of such tumors.
objectiveExploration of the relationship between SSTR2 immunoreactivity and SDHB immunoreactivity, mutational status, and clinical behavior of PPGLs. Evaluation of SSTR-based therapies in metastatic PPGLs.
methodsRetrospective analysis of a multicenter cohort of PPGLs at 6 specialized Endocrine Tumor Centers in Germany, The Netherlands, and Switzerland. Patients with PPGLs participating in the ENSAT registry were included. Clinical data were extracted from medical records, and immunohistochemistry (IHC) for SDHB and SSTR2 was performed in patients with available tumor tissue. Immunoreactivity of SSTR2 was investigated using Volante scores. The main outcome measure was the association of SSTR2 IHC positivity with genetic and clinical-pathological features of PPGLs.
resultsOf 202 patients with PPGLs, 50% were SSTR2 positive. SSTR2 positivity was significantly associated with SDHB- and SDHx-related PPGLs, with the strongest SSTR2 staining intensity in SDHB-related PPGLs (P = .01). Moreover, SSTR2 expression was significantly associated with metastatic disease independent of SDHB/SDHx mutation status (P < .001). In metastatic PPGLs, the disease control rate with first-line SSTR-based radionuclide therapy was 67% (n = 22, n = 11 SDHx), and with first-line "cold" somatostatin analogs 100% (n = 6, n = 3 SDHx).
conclusionSSTR2 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.
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