Over-diagnosis of potential malignant behavior in MEN 2A-associated pheochromocytomas using the PASS and GAPP algorithms

Langenbeck's Archives of Surgery
Adam StenmanCarl Christofer Juhlin

Abstract

Pheochromocytomas (PCCs) exhibit malignant potential, but current histological modalities for the proper detection of aggressive behavior are debated. The two most widespread algorithms are the "Pheochromocytoma of the Adrenal Gland Scaled Score" (PASS) and the "Grading System for Adrenal Pheochromocytoma and Paraganglioma" (GAPP), both which mostly rely on histological parameters to identify PCC patients at risk of disseminated disease. Since the algorithms are derived from studies using predominantly sporadic PCCs, little is known whether the PASS or GAPP scores can predict malignant potential in hereditary cases. PASS and GAPP were applied on 41 PCCs; 13 PCCs were diagnosed in ten multiple endocrine neoplasia type 2A (MEN 2A) patients carrying established germline RET proto-oncogene mutations, as well as 28 assumed sporadic PCCs. Six out of thirteen MEN 2A tumors (46%) exhibited PASS scores ≥ 4, indicative of a potential for aggressive behavior. In addition, 7/13 tumors (54%) exhibited GAPP scores ≥ 3, indicative of a "moderately differentiated type" with risk of future recurrence. All MEN 2A PCCs with an elevated PASS score also displayed an elevated GAPP score. In contrast, 4/28 (14%) sporadic PCCs demonstrated PASS scores...Continue Reading

References

Jan 16, 2009·The American Journal of Surgical Pathology·David WuVânia Nosé
Nov 2, 2011·Endocrine-related Cancer·Jenny WelanderOliver Gimm
Nov 10, 2011·Langenbeck's Archives of Surgery·Pierre de WaillyJean Louis Kraimps
Feb 14, 2014·Endocrine-related Cancer·Noriko KimuraMitsuhide Naruse
Apr 2, 2014·Nature Reviews. Endocrinology·Graeme Eisenhofer, Arthur S Tischler
Apr 4, 2014·The Journal of Clinical Endocrinology and Metabolism·Jenny WelanderPeter Söderkvist
Dec 20, 2016·PloS One·Kyong Young KimSeong Yeon Kim

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