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孤立性垂体柄增粗儿童中肿瘤性疾病的预测因素。

Predictors of neoplastic disease in children with isolated pituitary stalk thickening.

机构信息

Department of Pediatric Oncology, Dana-Farber Cancer Institute, Boston Children's Hospital, Boston, MA, USA.

出版信息

Pediatr Blood Cancer. 2013 Oct;60(10):1630-5. doi: 10.1002/pbc.24577. Epub 2013 May 14.

Abstract

BACKGROUND

The significance of pituitary stalk thickening (PST) on magnetic resonance imaging (MRI) is often unclear. We evaluated presenting symptoms, MRI findings, clinical course, and outcome predictors of patients with PST.

PROCEDURE

We used a computerized search of the medical record from 1995 to 2008 to identify patients with PST without pituitary mass on MRI. Baseline and follow-up MRIs were reviewed in a blinded fashion. Relevant clinical data were abstracted.

RESULTS

69 patients with reported PST and adequate imaging for review were identified; 42 met study criteria. Median age at first abnormal MRI was 13.6 years (range: 0.8-19.7); 43% were male. Median follow-up was 3.4 years (range 0-12.8). Patients with diabetes insipidus (DI) were significantly more likely to have a neoplastic process than those without (P = 0.0008). Of 16 patients with DI, 8 (50%) had a neoplastic process, including germ cell tumor (n = 4), Langerhans cell histiocytosis (n = 3), and lymphoma (n = 1). Among patients with DI, 7 (44%) also developed anterior pituitary hormone dysfunction (APD), either at presentation or on pre-biopsy follow-up, including 6/8 patients with stalk neoplasm and only 1/8 patients with non-neoplastic PST (P = 0.04). Twenty-six patients presented without DI; none was found to have neoplasm of the stalk except one patient with craniopharyngioma. Progression of PST on follow-up imaging was significantly associated with a subsequent neoplastic diagnosis (P = 0.04).

CONCLUSION

Patients with PST without DI are unlikely to have a neoplastic process. Among patients with DI, APD or progressive stalk increase over time are predictive of neoplasia.

摘要

背景

磁共振成像(MRI)上垂体柄增粗(PST)的意义常常不清楚。我们评估了 PST 患者的临床表现、MRI 结果、临床病程和预后预测因素。

方法

我们使用计算机检索 1995 年至 2008 年的病历,以确定 MRI 上无垂体肿块的 PST 患者。对基线和随访 MRI 进行盲法评估。提取相关临床资料。

结果

共确定了 69 例有报告的 PST 且有足够影像学复查资料的患者;其中 42 例符合研究标准。首次异常 MRI 时的中位年龄为 13.6 岁(范围:0.8-19.7);43%为男性。中位随访时间为 3.4 年(范围 0-12.8)。有尿崩症(DI)的患者比无 DI 的患者更有可能存在肿瘤性疾病(P=0.0008)。在 16 例有 DI 的患者中,8 例(50%)存在肿瘤性疾病,包括生殖细胞瘤(n=4)、朗格汉斯细胞组织细胞增生症(n=3)和淋巴瘤(n=1)。在有 DI 的患者中,7 例(44%)还出现了前垂体激素功能障碍(APD),无论是在首发时还是在活检前随访时,包括 6/8 例存在垂体柄肿瘤的患者和仅 1/8 例存在非肿瘤性 PST 的患者(P=0.04)。26 例患者首发时无 DI;除了 1 例颅咽管瘤患者外,均未发现垂体柄肿瘤。随访时 PST 的进展与随后的肿瘤诊断显著相关(P=0.04)。

结论

无 DI 的 PST 患者不太可能存在肿瘤性疾病。在有 DI 的患者中,APD 或随着时间的推移 PST 逐渐增粗提示存在肿瘤。

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