Giri Dinesh, Roncaroli Federico, Sinha Ajay, Didi Mohammed, Senniappan Senthil
Department of Paediatric Endocrinology, Alder Hey Children's NHS Foundation Trust, LiverpoolUK.
Department of Histopathology, Alder Hey Children's Hospital, LiverpoolUK.
Endocrinol Diabetes Metab Case Rep. 2017 Mar 31;2017. doi: 10.1530/EDM-16-0153. eCollection 2017.
Corticotroph adenomas are extremely rare in children and adolescents. We present a 15-year-old boy who was investigated for delayed puberty (A1P2G1, bilateral testicular volumes of 3 mL each). There was no clinical or laboratory evidence suggestive of chronic illness, and the initial clinical impression was constitutional delay in puberty. Subsequently, MRI scan of the brain revealed the presence of a mixed cystic and solid pituitary lesion slightly displacing the optic chiasma. The lesion was removed by transphenoidal surgery and the biopsy confirmed the lesion to be pituitary adenoma. Furthermore, the adenoma cells also had Crooke's hyaline changes and were intensely positive for ACTH. However there was no clinical/biochemical evidence of ACTH excess. There was a spontaneous pubertal progression twelve months after the surgery (A2P4G4, with bilateral testicular volume of 8 mL). Crooke's cell adenoma is an extremely rare and aggressive variant of corticotroph adenoma that can uncommonly present as a silent corticotroph adenoma in adults. We report for the first time Crooke's cell adenoma in an adolescent boy presenting with delayed puberty.
Constitutional delay of growth and puberty (CDGP) is a diagnosis of exclusion; hence a systematic and careful review should be undertaken while assessing boys with delayed puberty.Crooke's cell adenomas are a group of corticotroph adenomas that can rarely present in childhood and adolescence with delayed puberty.Crooke's cell adenomas can be clinically silent but are potentially aggressive tumours that require careful monitoring.
促肾上腺皮质激素腺瘤在儿童和青少年中极为罕见。我们报告一名15岁男孩,因青春期发育延迟接受检查(A1P2G1,双侧睾丸体积均为3毫升)。没有临床或实验室证据提示存在慢性疾病,最初的临床印象是体质性青春期发育延迟。随后,脑部MRI扫描显示存在一个混合性囊性和实性垂体病变,轻微压迫视交叉。该病变通过经蝶窦手术切除,活检证实为垂体腺瘤。此外,腺瘤细胞还出现了克鲁克透明变性,促肾上腺皮质激素染色呈强阳性。然而,没有促肾上腺皮质激素过多的临床/生化证据。术后12个月出现自发性青春期进展(A2P4G4,双侧睾丸体积为8毫升)。克鲁克细胞腺瘤是促肾上腺皮质激素腺瘤中一种极为罕见且具有侵袭性的变体,在成人中可罕见地表现为无功能性促肾上腺皮质激素腺瘤。我们首次报告了一名青春期发育延迟的青少年男孩患有克鲁克细胞腺瘤。
生长和青春期体质性延迟(CDGP)是一种排除性诊断;因此,在评估青春期发育延迟的男孩时应进行系统而仔细的检查。克鲁克细胞腺瘤是促肾上腺皮质激素腺瘤的一种,在儿童和青少年中很少表现为青春期发育延迟。克鲁克细胞腺瘤在临床上可能无症状,但属于潜在侵袭性肿瘤,需要仔细监测。