Department of Neurology, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
Department of Neuro-Oncology, MD Anderson Cancer Center, Houston, Texas, USA.
Eur J Endocrinol. 2019 Oct;181(4):397-407. doi: 10.1530/EJE-18-0795.
Pituitary carcinoma (PC) is an aggressive neuroendocrine tumor diagnosed when a pituitary adenoma (PA) becomes metastatic. PCs are typically resistant to therapy and develop multiple recurrences despite surgery, radiotherapy and chemotherapy. Recently, treatment with temozolomide (TMZ) has shown promising results, although the lack of prospective trials limits assessment of benefit.
We describe a single-center multidisciplinary experience in managing PC patients over a 22-year period and review previously published PC series.
Seventeen patients were identified. Median age at PC diagnosis was 44 years (range 16-82 years), and the median time from PA to PC transformation was 5 years (range 1-29 years). Median follow-up time was 28 months. Most PCs were hormone-positive (n = 12): ACTH (n = 5), PRL (n = 4), LH/FSH (n = 2) and GH (n = 1). All patients underwent at least one resection and at least one course of radiation after PC diagnosis. Immunohistochemistry showed high Ki-67 labeling index (>3%) in 10/15 cases. Eight patients (47%) had only central nervous system (CNS) metastases; six (35%) had combined CNS and systemic metastases. The most commonly used chemotherapy was TMZ, and TMZ-based therapy was associated with the longest PFS in 12 (71%) cases, as well as the longest period from PC diagnosis to first progression (median 30 months). The 2, 3 and 5-year survival rate of the entire cohort was 71, 59 and 35%, respectively. All patients surviving >5 years had been treated with TMZ-based therapy.
PC management benefits from multidisciplinary care and multimodality therapy. TMZ-based regimens were associated with high survival rates and long disease control.
垂体癌（PC）是一种侵袭性神经内分泌肿瘤，当垂体腺瘤（PA）发生转移时即可诊断为 PC。尽管进行了手术、放疗和化疗，但 PC 通常对治疗具有抗性，并且会多次复发。最近，替莫唑胺（TMZ）的治疗显示出了有希望的结果，尽管缺乏前瞻性试验限制了对获益的评估。
我们描述了一个 22 年期间在单中心多学科管理 PC 患者的经验，并回顾了以前发表的 PC 系列。
确定了 17 名患者。PC 诊断时的中位年龄为 44 岁（范围 16-82 岁），从 PA 到 PC 转化的中位时间为 5 年（范围 1-29 年）。中位随访时间为 28 个月。大多数 PC 是激素阳性（n = 12）：ACTH（n = 5），PRL（n = 4），LH/FSH（n = 2）和 GH（n = 1）。所有患者在 PC 诊断后至少进行了一次切除和至少一次放疗。免疫组化显示 15 例中有 10 例 Ki-67 标记指数较高（>3％）。8 名患者（47％）仅有中枢神经系统（CNS）转移；6 名（35％）有 CNS 和全身转移。最常使用的化疗是 TMZ，在 12 例（71％）病例中，TMZ 为基础的治疗与最长的无进展生存期（中位数 30 个月）相关，与从 PC 诊断到首次进展的时间最长（中位数 30 个月）相关。整个队列的 2、3 和 5 年生存率分别为 71％、59％和 35％。所有存活超过 5 年的患者均接受了 TMZ 为基础的治疗。
PC 的管理受益于多学科护理和多模式治疗。基于 TMZ 的方案与高生存率和长期疾病控制相关。
