Pereira Bernardo Dias, Raimundo Luísa, Mete Ozgur, Oliveira Ana, Portugal Jorge, Asa Sylvia L
Serviço de Endocrinologia e Diabetes, piso 8, Hospital Garcia de Orta, E.P.E, 2801-951, Almada-Setúbal, Portugal.
Department of Pathology, University Health Network, 200 Elizabeth Street, 11th Floor, Toronto, ON, Canada.
Endocr Pathol. 2016 Mar;27(1):25-33. doi: 10.1007/s12022-015-9395-2.
Thyrotropin (TSH)-secreting pituitary adenomas are exceedingly rare at the pediatric age and no cases of co-secretion with other pituitary hormones in these tumors have been described in this age range. We present a case of a monomorphous plurihormonal pituitary adenoma that co-secreted TSH and GH in a pediatric patient. A 13-year-old male presented with increasing height velocity (17.75 cm/year, 9.55SD), weight loss, and visual impairment. Initial biochemical evaluations revealed secondary hyperthyroidism. A giant pituitary tumor compressing the surrounding structures was detected by magnetic resonance, and a transsphenoidal surgery was initially performed. Pathological examinations revealed an atypical, monomorphous plurihormonal Pit-1 lineage tumor with mixed features of silent subtype 3 adenoma and acidophil stem cell adenoma. In the postoperative period, secondary hyperthyroidism recurred with high levels of both GH and IGF1. In addition, due to tumor re-growth, a multimodality treatment plan was undertaken including surgery, somatostatin analogs, and radiotherapy. We report the first pediatric case of a plurihormonal TSH- and GH-secreting pituitary adenoma, further expanding the clinical manifestations of pediatric pituitary tumors. Comprehensive pathological evaluation and close follow-up surveillance are crucial to the prompt delivery of the best therapeutic options in the context of this particularly aggressive pituitary tumor.
促甲状腺激素（TSH）分泌型垂体腺瘤在儿童期极为罕见，且在该年龄范围内尚未有此类肿瘤与其他垂体激素共同分泌的病例报道。我们报告一例小儿患者的单形态多激素垂体腺瘤，该肿瘤同时分泌TSH和生长激素（GH）。一名13岁男性患者出现身高增长速度加快（17.75厘米/年，高于正常均值9.55标准差）、体重减轻和视力损害。初步生化检查显示继发性甲状腺功能亢进。磁共振检查发现一个巨大的垂体肿瘤压迫周围结构，最初进行了经蝶窦手术。病理检查显示为非典型的、单形态多激素Pit-1谱系肿瘤，具有沉默亚型3腺瘤和嗜酸性干细胞腺瘤的混合特征。术后，继发性甲状腺功能亢进复发，GH和胰岛素样生长因子1（IGF1）水平均升高。此外，由于肿瘤复发，采取了包括手术、生长抑素类似物和放疗在内的多模式治疗方案。我们报告了首例小儿多激素分泌TSH和GH的垂体腺瘤病例，进一步扩展了小儿垂体肿瘤的临床表现。对于这种具有特别侵袭性的垂体肿瘤，全面的病理评估和密切的随访监测对于及时提供最佳治疗选择至关重要。
