PMID- 31555208
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20200225
IS  - 1664-2392 (Print)
IS  - 1664-2392 (Electronic)
IS  - 1664-2392 (Linking)
VI  - 10
DP  - 2019
TI  - Giant Prolactinoma Causing Hydrocephalus and Intracranial Hypertension as First 
      Manifestations of Multiple Endocrine Neoplasia Type 1.
PG  - 582
LID - 10.3389/fendo.2019.00582 [doi]
LID - 582
AB  - Context: Overall, giant prolactinomas are rare tumors (4%), especially those 
      larger than 60 mm (1%). Despite the predominance of macroadenoma documented in 
      multiple endocrine neoplasia type 1 (MEN1)-related prolactinoma, only three giant 
      prolactinoma cases were described so far (size > 40 mm and prolactin > 1,000 
      ng/mL). None of them was larger than 60 mm or presented hydrocephalus or 
      intracranial hypertension (ICH) as initial manifestation of MEN1. Case 
      Description: A 21-years-old man presented with ICH as the first clinical 
      manifestation of MEN1. He harbored a MEN1 germline mutation but refused periodic 
      vigilance after normal hormonal screening at age 14 years. During investigation, 
      magnetic resonance imaging (MRI) of the skull showed an expansive 
      sellar/parasellar lesion (75 x 44 x 36 mm) with moderate to severe supratentorial 
      obstructive hydrocephalus and an extremely high serum prolactin (PRL) of 10,800 
      ng/mL, without combined hypersecretion of other pituitary hormones. He was 
      diagnosed with giant prolactinoma, and cabergoline was initiated. The patient 
      evolved with early improvement of clinical complaints for hydrocephalus and ICH 
      and PRL reached normal values (11 ng/mL) in association with significant tumoral 
      shrinkage after 18 months on cabergoline. After 2 months of cabergoline, 
      cerebrospinal fluid leakage was diagnosed and corrective surgery was provided. 
      The mean dose of cabergoline was 3 mg/week throughout treatment. Conclusion: We 
      reported the first case with hydrocephalus and ICH as the initial clinical 
      manifestation of a giant prolactinoma in MEN1. From our knowledge, this is the 
      largest MEN1-related prolactinoma reported so far. Notably, all four MEN1-related 
      giant prolactinomas cases reported were younger than 21 years strengthening the 
      importance to routine MEN1 genetic testing for prolactinoma in this age group. 
      Also, they all had initial effective response with dopamine agonist ensuring this 
      drug as first-line treatment for MEN1-related giant prolactinoma. However, the 
      scarce number of treated patients and progression of cabergoline resistance in 
      two of them suggest strict surveillance.
FAU - Dantas, Naiara C B
AU  - Dantas NCB
AD  - Walter Cantidio University Hospital, Federal University of Ceara, Fortaleza, 
      Brazil.
FAU - Soares, Carlos E L
AU  - Soares CEL
AD  - Faculty of Medicine, Drug Research and Development Center (NPDM), Federal 
      University of Ceara (UFC), Fortaleza, Brazil.
FAU - Martins, Manoel R A
AU  - Martins MRA
AD  - Walter Cantidio University Hospital, Federal University of Ceara, Fortaleza, 
      Brazil.
FAU - Lourenco, Delmar M Jr
AU  - Lourenco DM Jr
AD  - Endocrine Genetics Unit (LIM-25), Endocrinology Division, Hospital das Clinicas, 
      School of Medicine, University of Sao Paulo, Sao Paulo, Brazil.
AD  - Endocrine Oncology Division, Institute of Cancer of the State of Sao Paulo, Sao 
      Paulo, Brazil.
FAU - Quidute, Ana R P
AU  - Quidute ARP
AD  - Walter Cantidio University Hospital, Federal University of Ceara, Fortaleza, 
      Brazil.
AD  - Faculty of Medicine, Drug Research and Development Center (NPDM), Federal 
      University of Ceara (UFC), Fortaleza, Brazil.
LA  - eng
PT  - Case Reports
DEP - 20190828
PL  - Switzerland
TA  - Front Endocrinol (Lausanne)
JT  - Frontiers in endocrinology
JID - 101555782
PMC - PMC6722186
OTO - NOTNLM
OT  - dopaminergic agonist
OT  - giant prolactinoma
OT  - intracranial hypertension
OT  - multiple endocrine neoplasia type 1
OT  - obstructive hydrocephalus
OT  - pituitary adenoma
EDAT- 2019/09/27 06:00
MHDA- 2019/09/27 06:01
PMCR- 2019/01/01
CRDT- 2019/09/27 06:00
PHST- 2019/02/07 00:00 [received]
PHST- 2019/08/09 00:00 [accepted]
PHST- 2019/09/27 06:00 [entrez]
PHST- 2019/09/27 06:00 [pubmed]
PHST- 2019/09/27 06:01 [medline]
PHST- 2019/01/01 00:00 [pmc-release]
AID - 10.3389/fendo.2019.00582 [doi]
PST - epublish
SO  - Front Endocrinol (Lausanne). 2019 Aug 28;10:582. doi: 10.3389/fendo.2019.00582. 
      eCollection 2019.