Haiyun Wang1, Run Wang1, Tianhao Hu1, Zixun Wang1, Yuzhou Duan1, Sheng Han2, Anhua Wu3. 1. Department of Neurosurgery, Heping District, The First Hospital of China Medical University, Nanjing Street 155, Shenyang, 110001, China. 2. Department of Neurosurgery, Heping District, The First Hospital of China Medical University, Nanjing Street 155, Shenyang, 110001, China. hansheng2001_x@aliyun.com. 3. Department of Neurosurgery, Heping District, The First Hospital of China Medical University, Nanjing Street 155, Shenyang, 110001, China. wuanhua@yahoo.com.
Abstract
PURPOSE: Nonfunctional pituitary adenomas (NFPAs) in pediatric and adolescent age are extremely rare. This study aimed to report a series of 14 pediatric and adolescent NFPAs to assist in its clinical management. METHODS: A total of 14 consecutive patients pathologically diagnosed with NFPAs (age ≤ 20 years) were retrospectively examined, and the clinical data were analyzed. RESULTS: NFPA is uncommon in pediatric and adolescent patients (0.4%). The most common clinical symptoms were a headache (6/14, 42.9%) and visual loss (4/14, 28.6%). Ten patients (71.4%) had preoperative hypopituitarism. All patients were diagnosed with macroadenoma including 8 (57.1%) invasive tumors, and the average tumor diameter was 2.8 cm. All patients underwent transsphenoidal surgery, and a near-total resection was achieved in nine (64.3%) patients. Postoperative visual acuity improved in three patients (75%). The results of immunohistochemistry showed 6 silent plurihormonal adenomas (42.9%), 3 null cell adenomas (21.4%), 3 silent lactotroph adenomas (21.4%), 1 silent gonadotroph adenoma (7.1%) and 1 silent corticotroph adenoma (7.1%). The mean follow-up was 54.8 months, and five patients had tumor recurrence. Tumors with Ki-67 ≧ 2% (28.6%) showed higher recurrence rate than those with lower index (P = 0.001). Two patients received secondary surgery and radiation for recurrent tumors suffered from panhypopituitarism. CONCLUSION: Pediatric and adolescent NFPA is clinically rare, and shows potential invasiveness. The silent plurihormonal adenoma is the most frequent phenotype. Transsphenoidal surgery is as safe and effective as in adults. However, individualized care and teamwork of neurosurgeons, pediatricians, endocrinologists, and radiation oncologists are important, especially for recurrent diseases.
PURPOSE:Nonfunctional pituitary adenomas (NFPAs) in pediatric and adolescent age are extremely rare. This study aimed to report a series of 14 pediatric and adolescent NFPAs to assist in its clinical management. METHODS: A total of 14 consecutive patients pathologically diagnosed with NFPAs (age ≤ 20 years) were retrospectively examined, and the clinical data were analyzed. RESULTS: NFPA is uncommon in pediatric and adolescent patients (0.4%). The most common clinical symptoms were a headache (6/14, 42.9%) and visual loss (4/14, 28.6%). Ten patients (71.4%) had preoperative hypopituitarism. All patients were diagnosed with macroadenoma including 8 (57.1%) invasive tumors, and the average tumor diameter was 2.8 cm. All patients underwent transsphenoidal surgery, and a near-total resection was achieved in nine (64.3%) patients. Postoperative visual acuity improved in three patients (75%). The results of immunohistochemistry showed 6 silent plurihormonal adenomas (42.9%), 3 null cell adenomas (21.4%), 3 silent lactotroph adenomas (21.4%), 1 silent gonadotroph adenoma (7.1%) and 1 silent corticotroph adenoma (7.1%). The mean follow-up was 54.8 months, and five patients had tumor recurrence. Tumors with Ki-67 ≧ 2% (28.6%) showed higher recurrence rate than those with lower index (P = 0.001). Two patients received secondary surgery and radiation for recurrent tumors suffered from panhypopituitarism. CONCLUSION: Pediatric and adolescent NFPA is clinically rare, and shows potential invasiveness. The silent plurihormonal adenoma is the most frequent phenotype. Transsphenoidal surgery is as safe and effective as in adults. However, individualized care and teamwork of neurosurgeons, pediatricians, endocrinologists, and radiation oncologists are important, especially for recurrent diseases.
Entities:
Keywords:
Nonfunctional pituitary adenomas; Pediatric and adolescent; Silent plurihormonal adenoma; Transsphenoidal surgery
Authors: T Feola; R Sa Pirchio; G Puliani; R Pofi; M Crocco; V Sada; F Sesti; F Verdecchia; D Gianfrilli; M Appetecchia; N Di Iorgi; M L Jaffrain-Rea; R Pivonello; A M Isidori; A B Grossman; E Sbardella Journal: J Endocrinol Invest Date: 2022-08-24 Impact factor: 5.467