ReviewCurrent treatment options in oncology2020
Emerging Treatments for Advanced/Metastatic Pheochromocytoma and Paraganglioma.
Review in Current treatment options in oncology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 38 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
38 citing papers in PubMed, 74 citations in OpenAlex.
- Safety and dosimetry of [European journal of nuclear medicine and molecular imaging · 2025Trial
- BUB1 as a candidate non-oncogene addiction vulnerability in metastatic phaeochromocytoma/paraganglioma.Scientific reports · 2026Article
- Update on Systemic Therapies for Metastatic/Unresectable Pheochromocytomas and Paragangliomas and Future Directions.Cancers · 2025Review
- TheInternational journal of molecular sciences · 2025Article
- The Role of Artificial Intelligence in Advancing Theranostics Dosimetry for Cancer Therapy: a Review.Nuclear medicine and molecular imaging · 2025Review
- A Rare Case of Bilateral Pheochromocytomas: Diagnostic and Surgical Challenges in a 38-Year-Old Female.Cureus · 2025Article
- Familial and early recurrent pheochromocytoma in a child with a novel in-frame duplication variant ofClinical pediatric endocrinology : case reports and clinical investigations : official journal of the Japanese Society for Pediatric Endocrinology · 2024Article
- Screening and treatment of endocrine hypertension focusing on adrenal gland disorders: a narrative review.Journal of Yeungnam medical science · 2024Article
- Therapeutic Strategy for Functional Metastatic Malignant Paraganglioma: A Case Report and Review of the Literature.Cureus · 2024Article
- Management of Abdominal Paraganglioma: A Single Center's Experience.Medicina (Kaunas, Lithuania) · 2024Article
- The Management of Phaeochromocytomas and Paragangliomas in the Era of Precision Medicine: Where Are We Now? Evidence-Based Systemic Treatment Options and Future Cluster Oriented Perspectives.Pharmaceuticals (Basel, Switzerland) · 2024Review
- Rare and aggressive metastatic pheochromocytoma recurrence in a patient with MEN 2A syndrome.BMJ case reports · 2024Article
- Intraoperative radiofrequency ablation for unresectable abdominal paraganglioma: a case report.Frontiers in endocrinology · 2024Article
- A comprehensive characterisation of phaeochromocytoma and paraganglioma tumours through histone protein profiling, DNA methylation and transcriptomic analysis genome wide.Clinical epigenetics · 2023Article
- Research in the genetics of pheochromocytoma and paraganglioma: a bibliometric analysis from 2002 to 2022.Clinical and experimental medicine · 2023Article
- Metastatic paraganglioma.BMJ case reports · 2023Article
- The connection between tricarboxylic acid cycle enzyme mutations and pseudohypoxic signaling in pheochromocytoma and paraganglioma.Frontiers in endocrinology · 2023Review
- Tumour microenvironment in pheochromocytoma and paraganglioma.Frontiers in endocrinology · 2023Review
- New Biology of Pheochromocytoma and Paraganglioma.Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists · 2022Review
- Locally invasive recurrence or metastasis of pheochromocytoma into the liver?-clinicopathological challenges.World journal of surgical oncology · 2022Article
Corrections and comments
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
opinion statementThe incidence of metastatic pheochromocytoma (PHEO) and paraganglioma (PGL) may occur in as many as 35% of patients particularly with PGL and even more frequently in those with specific mutations. Biochemical, morphological, and molecular markers have been investigated for use in the distinction of benign from malignant PHEO/PGL. PHEO/PGL metastasizes via hematogenous or lymphatic routes and shows differences based on mutational status. The most common sites of involvement in patients that have an SDHB mutation are the bone (78%), lungs (45%), lymph nodes (36%), and liver (35%). In patients with sporadic PHEO/PGL, the most common sites of metastasis are the bones (64%), lungs (47%), lymph nodes (36%), and liver (32%). Metastases may be present at presentation or may occur later. Metastases to the liver and lungs are associated with a shorter survival. Overall, the estimated 5-year survival rates are between 34 and 74%. Currently, treatments for metastatic PHEO/PGL are essentially palliative. Surgery is potentially curative; however, tumor dissemination limits the chance for a curative resection. When surgical intervention is not amenable, the therapeutic options include radiolabeled MIBG (Azedra®-iobenguane 131 was recently FDA-approved for patients > 12 years and older with iobenguane scan positive) or systemic chemotherapy with cyclophosphamide, vincristine, and dacarbazine (CVD) with an overall objective response rate (ORR) of less than 40%; however, it is not clear if the administration of CVD impacts overall survival, as nearly all patients develop progressive and ultimately fatal disease. Other treatment modalities under investigation include cytoreductive techniques, novel radiopharmaceuticals, chemotherapy, radiotherapy, immunotherapy, and experimental therapies. Here we are discussing emerging treatment for advanced/metastatic PHEO/PGL.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.