PMID- 24726110 OWN - NLM STAT- MEDLINE DCOM- 20141219 LR - 20191210 IS - 1531-5037 (Electronic) IS - 0022-3468 (Linking) VI - 49 IP - 4 DP - 2014 Apr TI - Preoperative multiple endocrine neoplasia type 1 diagnosis improves the surgical outcomes of pediatric patients with primary hyperparathyroidism. PG - 546-50 LID - S0022-3468(13)00927-5 [pii] LID - 10.1016/j.jpedsurg.2013.11.059 [doi] AB - BACKGROUND: Primary hyperparathyroidism (PHPT) is uncommon in children. The surgical management of PHPT in children has evolved over the past two decades. METHODS: A retrospective study of patients who underwent parathyroidectomy for PHPT diagnosed at age < 18 years and managed at a tertiary referral center for endocrine and familial disorders. RESULTS: Thirty-eight patients met eligibility criteria (1981-2012). Median age at PHPT diagnosis was 15 years. Two-thirds of patients were symptomatic (68%, n=26), most commonly from nephrolithiasis. Twenty-six (68%) patients underwent a standard cervical exploration while 32% underwent a focused unilateral parathyroidectomy. Multiple endocrine neoplasia type 1 (MEN1) was diagnosed preoperatively in 22/26 patients. Patients with a preoperative diagnosis of MEN1 were more likely to undergo a complete initial operation (>/= 3 gland parathyroidectomy with transcervical thymectomy, 13/22, 59% vs. 0/4, 0%; P=0.03) and less likely to have recurrent disease (10/22, 45% vs. 3/4, 75%; P<0.001) during follow up than patients diagnosed postoperatively. CONCLUSIONS: Children with PHPT should raise suspicion for MEN1. Preoperative MEN1 evaluation helped guide the extent of initial parathyroidectomy and was associated with lower rates of recurrence in sporadic and familial PHPT in pediatric patients. Management should occur at a high volume center with experienced clinicians and genetic counseling services. CI - Copyright (c) 2014 Elsevier Inc. All rights reserved. FAU - Romero Arenas, Minerva A AU - Romero Arenas MA AD - The University of Texas MD Anderson Cancer Center, Houston, TX, USA. FAU - Morris, Lilah F AU - Morris LF AD - The University of Texas MD Anderson Cancer Center, Houston, TX, USA. FAU - Rich, Thereasa A AU - Rich TA AD - The University of Texas MD Anderson Cancer Center, Houston, TX, USA. FAU - Cote, Gilbert J AU - Cote GJ AD - The University of Texas MD Anderson Cancer Center, Houston, TX, USA. FAU - Grubbs, Elizabeth G AU - Grubbs EG AD - The University of Texas MD Anderson Cancer Center, Houston, TX, USA. FAU - Waguespack, Steven G AU - Waguespack SG AD - The University of Texas MD Anderson Cancer Center, Houston, TX, USA. FAU - Perrier, Nancy D AU - Perrier ND AD - The University of Texas MD Anderson Cancer Center, Houston, TX, USA. Electronic address: NPerrier@mdanderson.org. LA - eng PT - Evaluation Study PT - Journal Article DEP - 20131127 PL - United States TA - J Pediatr Surg JT - Journal of pediatric surgery JID - 0052631 SB - IM MH - Adolescent MH - Child MH - Female MH - Follow-Up Studies MH - Humans MH - Hyperparathyroidism, Primary/etiology/*surgery MH - Male MH - Multiple Endocrine Neoplasia Type 1/complications/*diagnosis MH - Neoplasm Recurrence, Local MH - *Parathyroidectomy/methods MH - Retrospective Studies MH - Thymectomy MH - Treatment Outcome OTO - NOTNLM OT - Children OT - Genetic testing OT - Hereditary disorders OT - Hypercalcemia OT - Multiple endocrine neoplasia OT - Primary hyperparathyroidism EDAT- 2014/04/15 06:00 MHDA- 2014/12/20 06:00 CRDT- 2014/04/15 06:00 PHST- 2013/09/11 00:00 [received] PHST- 2013/11/15 00:00 [revised] PHST- 2013/11/19 00:00 [accepted] PHST- 2014/04/15 06:00 [entrez] PHST- 2014/04/15 06:00 [pubmed] PHST- 2014/12/20 06:00 [medline] AID - S0022-3468(13)00927-5 [pii] AID - 10.1016/j.jpedsurg.2013.11.059 [doi] PST - ppublish SO - J Pediatr Surg. 2014 Apr;49(4):546-50. doi: 10.1016/j.jpedsurg.2013.11.059. Epub 2013 Nov 27.