Amat Madramany A, Gastaldo Simeón E, Revert Ventura A, Escobar Hoyos L A, Riesgo Suárez P
Servicio de Pediatría, Hospital Universitario de la Ribera, Alzira, Valencia, España.
Servicio de Pediatría, Hospital Universitario de la Ribera, Alzira, Valencia, España.
An Pediatr (Barc). 2015 Jan;82(1):e108-12. doi: 10.1016/j.anpedi.2013.12.019. Epub 2014 Mar 14.
A case is presented of a 10-year old boy who had a hypothalamic-pituitary axis disorder. He initially presented with diabetes insipidus that progressed to panhypopituitarism. A hidden hypothalamic lesion should be suspected in all these cases, and should be followed up. New lesions were found in the pituitary stem three years later. Although tumor markers were negative, there was an increase in size, and a biopsy was performed. The histopathology reported a Lymphocytic Hypophysitis. There were increases in the tumor markers during the follow-up, thus a second biopsy was performed, with the diagnosis of Germinoma. Lymphocytic Hypophysitis is an uncommon diagnosis in children. Few cases have been reported, and in some cases, they were later diagnosed with Germinoma. We believe this case highlights the importance of the follow-up of children with Central Diabetes Insipidus with a normal MRI, as well as not taking the diagnosis of Lymphocytic Hypophysitis/lymphocytic Infundibular neurohypophysitis as definitive, as it is a rare diagnosis at this age, and could mask a Germinoma, as recorded in some cases.
本文报告一例10岁男孩患有下丘脑 - 垂体轴疾病。他最初表现为尿崩症，随后发展为全垂体功能减退。所有这些病例均应怀疑存在隐匿性下丘脑病变，并应进行随访。三年后在垂体柄发现了新病变。尽管肿瘤标志物为阴性，但病变大小增加，于是进行了活检。组织病理学报告为淋巴细胞性垂体炎。随访期间肿瘤标志物升高，因此进行了第二次活检，诊断为生殖细胞瘤。淋巴细胞性垂体炎在儿童中是一种罕见的诊断。报道的病例很少，在某些情况下，后来被诊断为生殖细胞瘤。我们认为该病例凸显了对MRI正常的中枢性尿崩症儿童进行随访的重要性，以及不要将淋巴细胞性垂体炎/淋巴细胞性漏斗神经垂体炎的诊断视为定论，因为这在这个年龄段是罕见诊断，并且可能像某些病例中所记录的那样掩盖生殖细胞瘤。
