PMID- 28458907
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20220310
IS  - 2052-0573 (Print)
IS  - 2052-0573 (Electronic)
IS  - 2052-0573 (Linking)
VI  - 2017
DP  - 2017
TI  - Hypersecretion of ACTH and PRL from pituitary adenoma in MEN1, adequately managed 
      by medical therapy.
LID - 10.1530/EDM-17-0027 [doi]
LID - 17-0027
AB  - SUMMARY: A 54-year-old man had gastrinoma, parathyroid hyperplasia and pituitary 
      tumor. His family history indicated that he might have multiple endocrine 
      neoplasia type 1 (MEN1). MEN1 gene analysis revealed a heterozygous germline 
      mutation (Gly156Arg). Therefore, we diagnosed him with MEN1. Endocrinological 
      tests revealed that his serum prolactin (PRL) and plasma adrenocorticotropic 
      hormone (ACTH) levels were elevated to 1699 ng/mL and 125 pg/mL respectively. 
      Immunohistochemical analysis of the resected pancreatic tumors revealed that the 
      tumors did not express ACTH. Overnight 0.5 and 8 mg dexamethasone suppression 
      tests indicated that his pituitary tumor was a PRL-ACTH-producing plurihormonal 
      tumor. Before transsphenoidal surgery, cabergoline was initiated. Despite no 
      decrease in the volume of the pituitary tumor, PRL and ACTH levels decreased to 
      37.8 ng/mL and 57.6 pg/mL respectively. Owing to the emergence of metastatic 
      gastrinoma in the liver, octreotide was initiated. After that, PRL and ACTH 
      levels further decreased to 5.1 ng/mL and 19.7 pg/mL respectively. He died from 
      liver dysfunction, and an autopsy of the pituitary tumor was performed. In the 
      autopsy study, histopathological and immunohistochemical (IHC) analysis showed 
      that the tumor was single adenoma and the cells were positive for ACTH, growth 
      hormone (GH), luteinizing hormone (LH) and PRL. RT-PCR analysis showed that the 
      tumor expressed mRNA encoding all anterior pituitary hormones, pituitary 
      transcription factor excluding estrogen receptor (ER) beta, somatostatin receptor 
      (SSTR) 2, SSTR5 and dopamine receptor D (D2R). PRL-ACTH-producing tumor is a very 
      rare type of pituitary tumor, and treatment with cabergoline and octreotide may 
      be useful for controlling hormone levels secreted from a plurihormonal pituitary 
      adenoma, as seen in this case of MEN1. LEARNING POINTS: Although plurihormonal 
      pituitary adenomas were reported to be more frequent in patients with MEN1 than 
      in those without, the combination of PRL and ACTH is rare.RT-PCR analysis showed 
      that the pituitary tumor expressed various pituitary transcription factors and 
      IHC analysis revealed that the tumor was positive for PRL, ACTH, GH and 
      LH.Generally, the effectiveness of dopamine agonist and somatostatin analog in 
      corticotroph adenomas is low; however, if the plurihormonal pituitary adenoma 
      producing ACTH expresses SSTR2, SSTR5 and D2R, medical therapy for the pituitary 
      adenoma may be effective.
FAU - Uraki, Shinsuke
AU  - Uraki S
AD  - The 1st Department of Internal Medicine, Wakayama Medical University, 
      WakayamaJapan.
FAU - Ariyasu, Hiroyuki
AU  - Ariyasu H
AD  - The 1st Department of Internal Medicine, Wakayama Medical University, 
      WakayamaJapan.
FAU - Doi, Asako
AU  - Doi A
AD  - The 1st Department of Internal Medicine, Wakayama Medical University, 
      WakayamaJapan.
FAU - Furuta, Hiroto
AU  - Furuta H
AD  - The 1st Department of Internal Medicine, Wakayama Medical University, 
      WakayamaJapan.
FAU - Nishi, Masahiro
AU  - Nishi M
AD  - The 1st Department of Internal Medicine, Wakayama Medical University, 
      WakayamaJapan.
FAU - Usui, Takeshi
AU  - Usui T
AD  - Department of Medical Genetics, Shizuoka General Hospital, Shizuoka CityJapan.
FAU - Yamaue, Hiroki
AU  - Yamaue H
AD  - The 2nd Department of Surgery, Wakayama Medical University, WakayamaJapan.
FAU - Akamizu, Takashi
AU  - Akamizu T
AD  - The 1st Department of Internal Medicine, Wakayama Medical University, 
      WakayamaJapan.
LA  - eng
PT  - Journal Article
DEP - 20170406
PL  - England
TA  - Endocrinol Diabetes Metab Case Rep
JT  - Endocrinology, diabetes & metabolism case reports
JID - 101618943
PMC - PMC5404709
EDAT- 2017/05/02 06:00
MHDA- 2017/05/02 06:01
PMCR- 2017/04/06
CRDT- 2017/05/02 06:00
PHST- 2017/03/06 00:00 [received]
PHST- 2017/03/17 00:00 [accepted]
PHST- 2017/05/02 06:00 [entrez]
PHST- 2017/05/02 06:00 [pubmed]
PHST- 2017/05/02 06:01 [medline]
PHST- 2017/04/06 00:00 [pmc-release]
AID - EDM170027 [pii]
AID - 10.1530/EDM-17-0027 [doi]
PST - epublish
SO  - Endocrinol Diabetes Metab Case Rep. 2017 Apr 6;2017:17-0027. doi: 
      10.1530/EDM-17-0027. eCollection 2017.