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Annals of Surgical Oncology
Published in: Annals of Surgical Oncology 5/2013

01-05-2013 | Endocrine Tumors

Doing Away with the Rule of 10 %

Authors: Sarah C. Oltmann, MD, Herbert Chen, MD

Published in: Annals of Surgical Oncology | Issue 5/2013

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Excerpt

Pheochromocytomas (PCC) and paragangliomas (PGL) are rare tumors that require definitive surgical resection for management. Depending on the location of the tumor, the patient may present to any number of surgical specialists, including endocrine surgery, urology, surgical oncology, thoracic surgery, otolaryngology, and general surgery. Traditionally, PCC has been associated with the so-called Rule of 10 %, but over the last 10–15 years, this rule has been challenged as more and more data have accrued demonstrating much higher rates of germ line mutations.1‐3 …
Literature
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Chen H, Sippel RS, O’Doriso MS, Vinik AL, Lloyd RV, Pacak K. The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer. Pancreas. 2010;39:775–83.PubMedCrossRef
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Hensen EF, Bayley JP. Recent advances in the dentics of SDH-related paraganglioma and pheochromocytoma. Fam Cancer. 2011;10:355–63.PubMedCrossRef
3.
Pęczkowska M, Cascon A, Prejbisz A, et al. Extra-adrenal and adrenal pheochromocytomas associated with a germline SDHC mutation. Nat Clin Pract Endocrinol Metab. 2008;4:111–5.PubMedCrossRef
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Fishbein L, Merrill S, Fraker DL, Cohen DL, Nathanson KL. Inherited mutations in pheochromocytoma and paraganglioma: why all patients should be offered genetic testing. Ann Surg Oncol. In press.
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Pęczkowska M, Erlic S, Hoffmann MM, et al. Impact of screening kindreds for SDHD p.Cys11X as a common mutation associated with paraganglioma syndrome type 1. J Clin Endocrinol Metab. 2008;93:4818–25.PubMedCrossRef
Metadata
Title
Doing Away with the Rule of 10 %
Authors
Sarah C. Oltmann, MD
Herbert Chen, MD
Publication date
01-05-2013
Publisher
Springer-Verlag
Published in
Annals of Surgical Oncology / Issue 5/2013
Print ISSN: 1068-9265
Electronic ISSN: 1534-4681
DOI
https://doi.org/10.1245/s10434-013-2944-3

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