Raleigh David R, Solomon David A, Lloyd Shane A, Lazar Ann, Garcia Michael A, Sneed Penny K, Clarke Jennifer L, McDermott Michael W, Berger Mitchel S, Tihan Tarik, Haas-Kogan Daphne A
Department of Radiation Oncology, University of California San Francisco, San Francisco, California (D.R.R., S.A.L., A.L., M.A.G., P.K.S., D.A.H.-K.); Division of Neuropathology, Department of Pathology, University of California San Francisco, San Francisco, California (D.A.S., T.T.); Department of Neurology, University of California San Francisco, San Francisco, California (J.L.C.); Department of Neurologic Surgery, University of California San Francisco, San Francisco, California (J.L.C., M.W.M., M.S.B.).
Department of Radiation Oncology, University of California San Francisco, San Francisco, California (D.R.R., S.A.L., A.L., M.A.G., P.K.S., D.A.H.-K.); Division of Neuropathology, Department of Pathology, University of California San Francisco, San Francisco, California (D.A.S., T.T.); Department of Neurology, University of California San Francisco, San Francisco, California (J.L.C.); Department of Neurologic Surgery, University of California San Francisco, San Francisco, California (J.L.C., M.W.M., M.S.B.)
Neuro Oncol. 2017 Jan;19(1):78-88. doi: 10.1093/neuonc/now105. Epub 2016 Jun 9.
Pineal parenchymal tumors (PPTs) are rare neoplasms of the central nervous system, and data concerning clinical outcomes are limited. The purpose of this study was to define the clinical behavior of PPT according to current histopathologic criteria and identify prognostic factors to guide therapeutic decisions.
Seventy-five patients treated for PPT at a single institution between 1992 and 2015 were retrospectively identified. Forty-five resection specimens were available and re-reviewed. Freedom from progression (FFP) and overall survival (OS) were estimated using the Kaplan-Meier method and compared using log-rank tests.
Median follow-up was 4.1 years. All patients initially underwent surgery; 78% of patients with PPT of intermediate differentiation (PPTID) and all patients with pineoblastoma received adjuvant therapy. Pathologic re-review refined classification in 27% of cases, with the majority of these being adult patients with pineal tumors originally classified as pineoblastomas that more accurately resembled PPTID based on the 2007 WHO classification.
Our histologic review also identified that PPTIDs can be classified into small-cell and large-cell morphologic subtypes, which have distinct clinical outcomes. Tumor grade, extent of resection, and neuraxis spread were prognostic for FFP. PPTID subtype, extent of resection, and neuraxis spread were prognostic for OS. Genetic analysis of a pineoblastoma case identified somatic mutations of DICER1, ARID1A, and KDM5C genes.
PPTIDs can be classified into 1 of 2 novel morphologic subtypes that are associated with distinct clinical outcomes. Tumor grade, neuraxis spread, and extent of resection also influence outcome for patients with PPT.
松果体实质肿瘤（PPTs）是中枢神经系统的罕见肿瘤，有关临床结局的数据有限。本研究的目的是根据当前的组织病理学标准确定PPT的临床行为，并确定预后因素以指导治疗决策。
回顾性确定了1992年至2015年间在单一机构接受PPT治疗的75例患者。有45份切除标本可供重新审查。采用Kaplan-Meier法估计无进展生存期（FFP）和总生存期（OS），并使用对数秩检验进行比较。
中位随访时间为4.1年。所有患者最初均接受了手术；78%的中分化松果体实质肿瘤（PPTID）患者和所有松果体母细胞瘤患者接受了辅助治疗。病理重新审查使27%的病例分类得到完善，其中大多数是成年松果体肿瘤患者，最初被分类为松果体母细胞瘤，但根据2007年世界卫生组织分类更准确地类似于PPTID。
我们的组织学审查还发现，PPTID可分为小细胞和大细胞形态学亚型，它们具有不同的临床结局。肿瘤分级、切除范围和神经轴扩散是FFP的预后因素。PPTID亚型、切除范围和神经轴扩散是OS的预后因素。对一例松果体母细胞瘤病例的基因分析确定了DICER1、ARID1A和KDM5C基因的体细胞突变。
PPTID可分为两种新的形态学亚型之一，它们与不同的临床结局相关。肿瘤分级、神经轴扩散和切除范围也会影响PPT患者的结局。
