Partington M D, Davis D H, Laws E R, Scheithauer B W
Department of Neurological Surgery, Mayo Clinic, Rochester, Minnesota.
J Neurosurg. 1994 Feb;80(2):209-16. doi: 10.3171/jns.1994.80.2.0209.
Pituitary adenomas are uncommon in childhood. Between 1975 and 1988, 36 patients underwent transsphenoidal resection at the Mayo Clinic before their 17th birthday. Fifteen (41.7%) had prolactin (PRL)-secreting tumors, 16 (44.4%) had adrenocorticotropic hormone (ACTH)-secreting tumors (including two patients with Nelson-Salassa syndrome), and three (8.3%) had tumors secreting growth hormone (GH); two patients (5.6%) had clinically nonfunctioning tumors. Of the 36 patients, 21 (58.3%) were girls, and girls predominated in every group except that with ACTH-secreting tumors, where they accounted for only 37.5% of the patients. The average age at the time of surgery was 14.7 +/- 2.2 years, the youngest patient being 7.3 years old. The ACTH-secreting tumors were associated with an earlier onset of symptoms than the other tumor types: six (37.5%) of 16 patients became symptomatic before 10 years of age, compared with two (10.0%) of the 20 other patients. Presenting symptoms primarily reflected endocrine dysfunction, with neurological presentation (visual field deficits) occurring in only two patients. There were no deaths in this patient group. Significant operative morbidity included steroid-induced psychosis in one patient and diabetes insipidus in three, which resolved in two. Plurihormonal tumors, broadly defined as tumors producing more than one hormone, were common, comprising five (36%) of 14 PRL-secreting tumors, all three GH-secreting tumors, and one of two clinically nonfunctioning tumors, or 25.7% overall. Macroadenomas accounted for 89% of plurihormonal tumors. Long-term follow-up review (median > 5 years) revealed good control of PRL-secreting tumors (although five of 15 patients had received postoperative radiotherapy), contrasted with a 25% late recurrence rate for ACTH-secreting tumors, which had an 80% initial remission rate. The majority of patients had good endocrine function, including reproductive function. It is concluded that: 1) although pediatric pituitary adenomas occur primarily in adolescence, Cushing's disease is found at any age; 2) transsphenoidal surgery is feasible and safe in this age group; 3) plurihormonal tumors occur more frequently in the pediatric age group than in adults; and 4) long-term control rates in PRL- and ACTH-secreting tumors are probably similar to those seen in adults.
垂体腺瘤在儿童时期并不常见。1975年至1988年间，36例患者在梅奥诊所17岁生日前接受了经蝶窦切除术。15例（41.7%）患有分泌催乳素（PRL）的肿瘤，16例（44.4%）患有分泌促肾上腺皮质激素（ACTH）的肿瘤（包括2例尼尔森-萨拉萨综合征患者），3例（8.3%）患有分泌生长激素（GH）的肿瘤；2例（5.6%）患有临床无功能肿瘤。36例患者中，21例（58.3%）为女孩，除分泌ACTH的肿瘤组外，女孩在其他各肿瘤组中均占多数，在分泌ACTH的肿瘤组中女孩仅占患者总数的37.5%。手术时的平均年龄为14.7±2.2岁，最年轻的患者为7.3岁。分泌ACTH的肿瘤比其他类型的肿瘤症状出现得更早：16例患者中有6例（37.5%）在10岁前出现症状，而其他20例患者中只有2例（10.0%）在10岁前出现症状。主要症状反映内分泌功能障碍，仅2例患者出现神经系统症状（视野缺损）。该患者组无死亡病例。显著的手术并发症包括1例患者出现类固醇诱导的精神病和3例患者出现尿崩症，其中2例尿崩症患者病情缓解。多激素肿瘤（广义上定义为分泌一种以上激素的肿瘤）很常见，在14例分泌PRL的肿瘤中有5例（36%），3例分泌GH的肿瘤全部为多激素肿瘤，2例临床无功能肿瘤中有1例为多激素肿瘤，总体占25.7%。大腺瘤占多激素肿瘤的89%。长期随访复查（中位随访时间>5年）显示，分泌PRL的肿瘤得到了良好控制（尽管15例患者中有5例接受了术后放疗），相比之下，分泌ACTH的肿瘤晚期复发率为25%，其初始缓解率为80%。大多数患者内分泌功能良好，包括生殖功能。结论如下：1）尽管儿童垂体腺瘤主要发生在青春期，但库欣病在任何年龄均可发现；2）经蝶窦手术在该年龄组可行且安全；3）多激素肿瘤在儿童年龄组比成人更常见；4）分泌PRL和ACTH的肿瘤的长期控制率可能与成人相似。
