title: The characterization of pheochromocytoma and its impact on overall survival in multiple endocrine neoplasia type 2.
abstract: BACKGROUND
  Pheochromocytoma (PHEO) occurs in 50% of patients with multiple endocrine neoplasia type 2 (MEN2). It is unknown if the presence of PHEO is associated with more aggressive medullary thyroid cancer (MTC).
  
  
  OBJECTIVE
  To present our experience with MEN2 PHEO and evaluate whether PHEO impacts MTC overall survival in patients with RET codon 634 mutations.
  
  
  METHODS
  We performed a retrospective chart review of MEN2 patients at MD Anderson Cancer Center from 1960 through 2012.
  
  
  METHODS
  The study group comprised 85 patients (group 1) with MEN2-associated PHEO. Of these, 59 patients (subgroup 1) with RET codon 634 mutations were compared to 48 patients (group 2) with RET codon 634 mutations, but without MEN2-associated PHEO.
  
  
  METHODS
  Of 85 patients with MEN2 and PHEO, 70 had MEN2A and 15 had MEN2B. Median age at PHEO diagnosis was 32 years. The initial manifestation of MEN2 was MTC in 60% of patients, synchronous MTC and PHEO in 34%, and PHEO in 6% of patients. Of patients, 72% had bilateral PHEO, and most tumors were synchronous (82%). Subgroup analysis of MEN2 patients with and without PHEO, who were carriers of RET codon 634, the most common mutation with PHEO, showed no significant differences in the stage of MTC at initial diagnosis. The median follow-up time for patients with PHEO was 249 months and without PHEO was 67 months (P < .01). Survival analyses among RET 634 carriers did not show shorter survival for patients with PHEO. The median survival time for patients with PHEO was 499 months and without PHEO was 444 months (P < .05).
  
  
  CONCLUSIONS
  PHEO in MEN2 patients are usually bilateral and unlikely to be metastatic. Subgroup analysis of patients with RET 634 mutations with and without PHEO showed that PHEO was not associated with a more advanced stage of MTC at diagnosis or a shorter survival.
authors.affiliation: MD, AssociateDepartment of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, 1400 Pressler Street Unit 1461, Houston, TX 77030. cjimenez@mdanderson.org.
authors.forename: Sonali
authors.initials: S
authors.lastname: Thosani
authors.forename: Montserrat
authors.initials: M
authors.lastname: Ayala-Ramirez
authors.forename: Lynn
authors.initials: L
authors.lastname: Palmer
authors.forename: Mimi I
authors.initials: MI
authors.lastname: Hu
authors.forename: Thereasa
authors.initials: T
authors.lastname: Rich
authors.forename: Robert F
authors.initials: RF
authors.lastname: Gagel
authors.forename: Gilbert
authors.initials: G
authors.lastname: Cote
authors.forename: Steven G
authors.initials: SG
authors.lastname: Waguespack
authors.forename: Mouhammed Amir
authors.initials: MA
authors.lastname: Habra
authors.forename: Camilo
authors.initials: C
authors.lastname: Jimenez
mesh_terms: D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000235:genetics / Q000401:mortality*; D000328:Adult; D017668:Age of Onset; D018278:Carcinoma, Neuroendocrine; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D020022:Genetic Predisposition to Disease / Q000453:epidemiology / Q000235:genetics; D018095:Germ-Line Mutation; D006801:Humans; D008297:Male; D008875:Middle Aged; D018813:Multiple Endocrine Neoplasia Type 2a / Q000235:genetics / Q000401:mortality*; D020125:Mutation, Missense; D010673:Pheochromocytoma / Q000235:genetics / Q000401:mortality*; D016016:Proportional Hazards Models; D051096:Proto-Oncogene Proteins c-ret / Q000235:genetics*; D012189:Retrospective Studies; D013964:Thyroid Neoplasms / Q000235:genetics / Q000401:mortality*; D055815:Young Adult
chemical_list: D051096:Proto-Oncogene Proteins c-ret; C099282:RET protein, human
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