Phaeochromocytomas and sympathetic paragangliomas.
B-J Petri1, C H J van Eijck, W W de Herder
1Department of Pathology, Erasmus MC-University Medical Centre, Rotterdam, The Netherlands.
The British Journal of Surgery
|November 18, 2009
Summary
Genetic testing for phaeochromocytomas (PCCs) and sympathetic paragangliomas (sPGLs) should be routine. Early identification of familial cancer syndromes improves patient and relative outcomes through standardized screening and tailored surgical approaches.
Area of Science:
- Endocrinology
- Oncology
- Genetics
Background:
- 24% of phaeochromocytomas (PCCs) and sympathetic paragangliomas (sPGLs) are linked to familial cancer syndromes.
- Key syndromes include MEN2, VHL, NF1, and PCC-paraganglioma syndrome.
- Early identification is crucial for patients and their families.
Purpose of the Study:
- To review the literature on PCC, sPGL, and associated hereditary syndromes.
- To provide guidance on diagnosis and management of these conditions.
Main Methods:
- Literature review of PubMed, Ovid, and Embase databases until 2009.
- Focused on articles concerning PCC, sPGL, hereditary syndromes, and treatment strategies.
Main Results:
- Hereditary syndromes are a significant factor in PCC and sPGL cases.
- Surgical resection is the primary treatment for PCC and sPGL.
- Management of bilateral or multiple tumors requires careful consideration.
Conclusions:
- Routine genetic testing for PCC and sPGL is recommended.
- Referral to a clinical geneticist is advised for affected individuals.
- Standardized screening protocols for mutation carriers and cortex-sparing adrenalectomy are preferred treatments.
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