PMID- 30319541
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20191120
IS  - 1664-2392 (Print)
IS  - 1664-2392 (Electronic)
IS  - 1664-2392 (Linking)
VI  - 9
DP  - 2018
TI  - Total and Subtotal Parathyroidectomy in Young Patients With Multiple Endocrine 
      Neoplasia Type 1-Related Primary Hyperparathyroidism: Potential Post-surgical 
      Benefits and Complications.
PG  - 558
LID - 10.3389/fendo.2018.00558 [doi]
LID - 558
AB  - Background: The choice of surgical treatment for patients with Multiple Endocrine 
      Neoplasia type 1 (MEN1)-related primary hyperparathyroidism (PHPT) remains 
      controversial and it has not been specifically addressed in young patients. 
      Methods: This is a retrospective case series study. The study includes the 
      surgical data and the follow-up of 38 patients younger than 30 years of age, all 
      diagnosed with MEN1, collected and followed-up between 1991 and 2017 at the 
      Regional Referral Center for Inherited Endocrine Tumors of the Tuscany Region, 
      and operated by parathyroidectomy. Genetic and/or clinical MEN1 diagnosis was 
      made before surgery in all patients. Subtotal (9/38 patients) or total 
      parathyroidectomy with auto-transplantation (28/38 patients) were performed in 
      all patients but one, in whom a single mediastinal adenoma was excised from the 
      aorto-pulmonary window. All patients but one, who was operated in 2017, had a 
      post-operatory follow-up of at least 12 months. Results: Total parathyroidectomy 
      (TPTX), with auto-transplantation, was the most frequently adopted operation both 
      as primary (20/38 patients) and secondary (8/38 patients) surgery, followed by 
      subtotal parathyroidectomy (SPTX; 9/38 patients) and limited parathyroidectomy 
      (1/38 patient). At follow-up, lasting a mean of 11.8 +/- 6.6 years (range 0-23 
      years), no persistent PHPT was observed. PHPT recurred in 4/28 TPTX (14%) and in 
      2/9 SPTX (22%). Permanent hypoparathyroidism showed no statistically significant 
      difference between the procedures (2/9 in SPTX and 5/28 in TPTX). Conclusions: 
      Data from this retrospective study showed the efficacy of TPTX for the treatment 
      of MEN1-PHPT, also in adolescent and young patients, showing, in our series, no 
      risk of PHPT permanence and a longer disease-free period and, subsequently, the 
      possibility to postpone re-intervention with respect to both limited PTX and 
      SPTX. The risk of permanent hypoparathyroidism in TPTX was comparable to STPX, 
      and could be mitigated over the years.
FAU - Tonelli, Francesco
AU  - Tonelli F
AD  - Department of Surgery and Translational Medicine, University of Florence, 
      Florence, Italy.
FAU - Marini, Francesca
AU  - Marini F
AD  - Department of Surgery and Translational Medicine, University of Florence, 
      Florence, Italy.
FAU - Giusti, Francesca
AU  - Giusti F
AD  - Department of Surgery and Translational Medicine, University of Florence, 
      Florence, Italy.
FAU - Brandi, Maria Luisa
AU  - Brandi ML
AD  - Department of Surgery and Translational Medicine, University of Florence, 
      Florence, Italy.
LA  - eng
PT  - Journal Article
DEP - 20180924
PL  - Switzerland
TA  - Front Endocrinol (Lausanne)
JT  - Frontiers in endocrinology
JID - 101555782
PMC - PMC6165877
OTO - NOTNLM
OT  - multiple endocrine neoplasia type 1
OT  - parathyroid adenoma
OT  - parathyroidectomy
OT  - primary hyperparathyroidism
OT  - young MEN1 patients
EDAT- 2018/10/16 06:00
MHDA- 2018/10/16 06:01
PMCR- 2018/01/01
CRDT- 2018/10/16 06:00
PHST- 2018/06/26 00:00 [received]
PHST- 2018/09/03 00:00 [accepted]
PHST- 2018/10/16 06:00 [entrez]
PHST- 2018/10/16 06:00 [pubmed]
PHST- 2018/10/16 06:01 [medline]
PHST- 2018/01/01 00:00 [pmc-release]
AID - 10.3389/fendo.2018.00558 [doi]
PST - epublish
SO  - Front Endocrinol (Lausanne). 2018 Sep 24;9:558. doi: 10.3389/fendo.2018.00558. 
      eCollection 2018.