Objective: The aim was to formulate clinical practice guidelines for pheochromocytoma and paraganglioma (PPGL). Participants: The Task Force included a chair selected by the Endocrine Society Clinical Guidelines Subcommittee (CGS), seven experts in the field, and a methodologist. The authors received no corporate funding or remuneration. Evidence: This evidence-based guideline was developed using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system to describe both the strength of recommendations and the quality of evidence. The Task Force reviewed primary evidence and commissioned two additional systematic reviews. Consensus Process: One group meeting, several conference calls, and e-mail communications enabled consensus. Committees and members of the Endocrine Society, European Society of Endocrinology, and Americal Association for Clinical Chemistry reviewed drafts of the guidelines. Conclusions: The Task Force recommends that initial biochemical testing for PPGLs shou...

/pdf/pheochromocytoma-and-paraganglioma-an-endocrine-society-4ij7m1t7fo.pdf

Pheochromocytoma and paraganglioma: An endocrine society clinical practice guideline

https://febs.onlinelibrary.wiley.com/doi/pdf/10.1016/j.molonc.2008.04.001

Imaging and cancer: A review

Imaging of neuroendocrine tumors.

Imaging of Neuroendocrine Tumors

Context: Pheochromocytomas and paragangliomas (PHEO/PGL) are neuroendocrine tumors that arise from sympathetic and parasympathetic paraganglia. Diagnosed rarely during childhood, PHEO/PGL are nonetheless important clinical entities, particularly given our evolving understanding of their pathophysiology. Evidence Acquisition: We identified articles through the U.S. National Library of Medicine by using the search terms pheochromocytoma and paraganglioma. Results were narrowed to manuscripts that included children and studies related to the genetics of PHEO/PGL. Web-based resources for genetic disorders were also used. For all articles, we performed subsequent reference searches and verification of source data. Evidence Synthesis: Up to 20% of PHEO/PGL are diagnosed in children. Most are functional tumors, and clinical presentation includes symptoms related to catecholamine hypersecretion and/or tumor mass effect. Increasingly, PHEO/PGL are identified during presymptomatic screening in children with genetic...

/pdf/a-current-review-of-the-etiology-diagnosis-and-treatment-of-4a2wxcxvwo.pdf

A current review of the etiology, diagnosis, and treatment of pediatric pheochromocytoma and paraganglioma.

Abstract. Scott Berglund A, Lennart Hulthén U, Manhem P, Thorsson O, Wollmer P, Törnquist C (Malmö University Hospital, Malmö, Sweden). Metaiodobenzylguanidine scintigraphy and computed tomography in clinical practice. Primary and secondary evaluation for localization of phaeochromocytomas. J Intern Med 2001; 249: 247–251.

Metaiodobenzylguanidine (MIBG) scintigraphy and computed tomography (CT) in clinical practice. Primary and secondary evaluation for localization of phaeochromocytomas: MIBG AND CT IN CLINICAL PRACTICE

OBJECTIVE: To determine the diagnostic value of metaiodobenzylguanidine (MIBG) scintigraphy compared with computed tomography (CT) for the localization of phaeochromocytomas in clinical practice. DESIGN: Retrospective comparison between MIBG scintigrams and CT for localization of phaeochromocytomas in all patients successively examined with MIBG scintigraphy in Malmo from 1984 until January 1997. SETTING: Malmo University Hospital, Sweden. SUBJECTS: Sixty-four patients with clinically suspected phaeochromocytomas. MAIN OUTCOME MEASURES: MIBG scintigrams and CTs classified as positive or negative based on original interpretations (primary evaluation) and in a secondary evaluation by one blinded examiner are assessed through histological confirmation or clinical rule out of phaeochromocytomas. RESULTS: Twenty-five patients had surgically removed phaeochromocytomas. The remaining 39 patients had no proof of phaeochromocytomas. In the secondary evaluation, sensitivity for MIBG scintigraphy was 88% (22/25) and for CT was 100% (25/25). The specificity for MIBG scintigraphy was 89% (35/39) but only 50% for CT (18/36). Two out of a total of six extra-adrenal tumours were amongst the false-negative MIBG scintigrams. CONCLUSIONS: MIBG scintigraphy for the localization of phaeochromocytomas is superior to CT as far as specificity, whereas CT has a higher sensitivity. After biochemical diagnosis, CT will detect most phaeochromocytomas. MIBG scintigraphy can be of value in patients who show inconclusive results with biochemical testing and CT. (Less)

Metaiodobenzylguanidine (MIBG) scintigraphy and computed tomography (CT) in clinical practice. Primary and secondary evaluation for localization of phaeochromocytomas

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Metaiodobenzylguanidine (MIBG) scintigraphy and computed tomography (CT) in clinical practice. Primary and secondary evaluation for localization of phaeochromocytomas: MIBG AND CT IN CLINICAL PRACTICE

Metaiodobenzylguanidine (MIBG) scintigraphy and computed tomography (CT) in clinical practice. Primary and secondary evaluation for localization of phaeochromocytomas