Department of Neurosurgery, Brain Research Institute, University of Niigata, 1 Asahimachi-dori Chuo-ku, Niigata, 951-8585, Japan.
Pituitary. 2012 Mar;15(1):110-5. doi: 10.1007/s11102-010-0275-y.
In this report, an extremely rare case of pediatric thyrotropin-secreting pituitary macroadenoma (TSHoma) is described. An 8-year-old boy, complaining of unsteady gait, was suspected of endocrinopathy because of emaciation and muscle weakness of the legs. Endocrinological work-up established a diagnosis of hyperthyroidism due to syndrome of inappropriate secretion of TSH. Magnetic resonance imaging showed a pituitary macroadenoma with suprasellar and sphenoidal extension without cavernous sinus invasion. He underwent an endoscopic endonasal transsphenoidal adenomectory due to the diagnosis of TSHoma. The adenoma was soft and it was totally removed. Histopathological staining confirmed diagnosis of TSHoma. Postoperative evaluation revealed a subnormal level of TSH (from 13-21 to 0.03 micro U/ml), normalization of alpha-subunit (from 10.0 to 0.09 ng/ml), and as a result, hypothyroidism. The boy left the hospital with oral levothyroxine that continued until 12 months of discharge. The present 8-year-old case is the youngest case to the best of our knowledge based on a bibliographical search. Reasons for endocrinological remission following adenomectomy are (1) correct diagnosis without delay: lack of cavernous sinus invasion, (2) soft and non-fibrous adenoma tissue, and (3) endoscopic technique with wide vision and illumination: safe even for a 8-year-old child. Early recognition/detection and pituitary-conserving adenomectomy can cure TSHoma and avoid long-term medical therapy and/or irradiation, which contribute to the best interests of patients with TSHoma.
在本报告中,描述了一例极其罕见的儿童促甲状腺素分泌垂体大腺瘤(TSHoma)。一名 8 岁男孩因消瘦和腿部肌无力而出现运动不稳,被怀疑患有内分泌疾病。内分泌检查确立了由于促甲状腺素不适当分泌而导致的甲状腺功能亢进症的诊断。磁共振成像显示垂体大腺瘤伴鞍上和蝶窦延伸,但无海绵窦侵犯。由于诊断为 TSHoma,他接受了内镜经鼻蝶窦垂体腺瘤切除术。该腺瘤质地柔软,完全切除。组织病理学染色证实了 TSHoma 的诊断。术后评估显示 TSH 水平低于正常值(从 13-21 降至 0.03 微 U/ml),α-亚单位正常(从 10.0 降至 0.09ng/ml),结果导致甲状腺功能减退。男孩出院时口服左甲状腺素,持续至出院后 12 个月。根据文献检索,目前的 8 岁病例是我们所知的最年轻的病例。腺瘤切除后内分泌缓解的原因是(1)及时正确诊断:无海绵窦侵犯,(2)腺瘤组织柔软且非纤维性,以及(3)内镜技术具有广阔的视野和照明:即使对于 8 岁儿童也很安全。早期识别/检测和保留垂体的腺瘤切除术可以治愈 TSHoma,并避免长期药物治疗和/或放疗,这符合 TSHoma 患者的最佳利益。