Gagliardi Filippo, Losa Marco, Boari Nicola, Franzin Alberto, Pozzobon Gabriella, Weber Giovanna, Mortini Pietro
Department of Neurosurgery, San Raffaele Scientific Institute, Vita-Salute University, Milan, Italy.
Childs Nerv Syst. 2013 Jun;29(6):1031-4. doi: 10.1007/s00381-013-2062-3.
Granular cell tumors (GCT) of the neurohypophysis are rare, solitary, nodular-shaped lesions, mostly presenting in the adult age with a female predilection. They rarely grow to a sufficient size to cause mass effect related symptoms and they may be found in most cases incidentally at autopsy of older patients. Few cases of symptomatic GCT of the neurohypophysis have been reported in the literature and only one of these in a pediatric patient in the first decade of life, who presented with central precocious puberty.
We report the case of a 11-year-old boy with a large suprasellar GCT of the neurohypophysis, complaining severe headache and pituitary insufficiency. Before our referral, the child was operated at another insitution through a pterional approach for tumor biopsy and underwent chemotherapy because of the misleading diagnosis of glioma.
The patient was operated on by a fronto-orbito-zygomatic approach with subtotal tumor resection. At last follow-up examination, a partial hypopituitarism was detected. The quality of life with replacement therapy was excellent. Fractionated radiotherapy on tumor remnant was advised.
The reported case is exceptional because the tumor developed in a male pediatric patient, causing clinical symptoms related to intracranial hypertension and unusual endocrinological features. GCT has to be considered in the differential diagnosis of suprasellar masses, to avoid misleading interpretation and consequent wrong therapeutic management.
神经垂体颗粒细胞瘤（GCT）罕见，为孤立性结节状病变，多发生于成年人，女性多见。它们很少生长到足以引起占位效应相关症状的大小，多数情况下是在老年患者尸检时偶然发现。文献中报道的有症状的神经垂体GCT病例很少，其中仅有1例发生在10岁前的儿科患者，表现为中枢性性早熟。
我们报告1例11岁男孩，患有巨大的鞍上神经垂体GCT，主诉严重头痛和垂体功能不全。在我们接诊之前，该患儿在另一家机构通过翼点入路进行了肿瘤活检手术，并因误诊为胶质瘤而接受了化疗。
患者通过额眶颧入路接受手术，肿瘤次全切除。在最后一次随访检查时，发现部分垂体功能减退。替代治疗后的生活质量良好。建议对肿瘤残余进行分次放疗。
报道的该病例很特殊，因为肿瘤发生在男性儿科患者中，引起了与颅内高压相关的临床症状和不寻常的内分泌特征。在鞍上肿块的鉴别诊断中必须考虑GCT，以避免误导性的解读和随之而来的错误治疗管理。
