Teti Claudia, Castelletti Lara, Allegretti Luca, Talco Miryam, Zona Gianluigi, Minuto Francesco, Boschetti Mara, Ferone Diego
Endocrinology Unit, Department of Internal Medicine and Medical Specialties, Center of Excellence for Biomedical Research, University of Genoa, Viale Benedetto XV 6, 16132, Genoa, Italy.
Pituitary. 2015 Oct;18(5):592-7. doi: 10.1007/s11102-014-0612-7.
Pituicytoma is a rare tumor arising from the neurohypophysis or pars intermedia of the adenohypophysis.
A 36 year old male came to our observation presenting polydipsia, polyuria, polyphagia, decreased libido and altered sleep-wake rhythm. The biochemical tests showed hypotonic urine, mild hyperprolactinemia, hypogonadotropic hypogonadism, central hypothyroidism. Magnetic resonance revealed an expansive lesion of the suprasellar region (slightly isointense on T1, hyperintense on T2, impregnating contrast medium), that was partially removed by trans-cranial neurosurgical approach. The histopathological diagnosis was pituicytoma. After surgery, in addition to endocrine disorders, the patient presented severe neurological sequelae and hyperthermia, likely due to damage of the hypothalamus, followed by a progressive metabolic syndrome. The residual tumor was monitored by MRI, and, due to the early gradual increase in volume, was treated by stereotactic radiosurgery.
DISCUSSION/CONCLUSIONS: Pituicytomas are often difficult to distinguish from other hypothalamic or pituitary lesions. However, their identification would be preferable in a pre-operative setting in order to optimize the work-up and to initiate a proactive management of the expected complications.
垂体细胞瘤是一种起源于神经垂体或腺垂体中间部的罕见肿瘤。
一名36岁男性前来就诊,表现为烦渴、多尿、多食、性欲减退及睡眠 - 觉醒节律改变。生化检查显示低渗尿、轻度高泌乳素血症、低促性腺激素性性腺功能减退、中枢性甲状腺功能减退。磁共振成像显示鞍上区有一占位性病变(T1加权像上略等信号,T2加权像上高信号,强化造影剂),经颅神经外科手术部分切除。组织病理学诊断为垂体细胞瘤。术后,除内分泌紊乱外,患者出现严重神经后遗症和高热,可能是由于下丘脑受损,随后出现进行性代谢综合征。通过磁共振成像监测残留肿瘤,由于其体积早期逐渐增大,采用立体定向放射外科治疗。
讨论/结论:垂体细胞瘤常难以与其他下丘脑或垂体病变区分。然而,在术前明确诊断更可取,以便优化检查并对预期并发症进行积极管理。