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Orbital Primary Solitary Fibrous Tumor: A Proposed Recurrence Risk Prediction Model Based on 92 Cases

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机构: [1]Department of Pathology, Beijing Tongren Hospital, Capital Medical University, Beijing, China. [2]Department of Radiology, Beijing Tongren Hospital, Capital Medical University, Beijing, China. [3]Beijing Tongren Eye Center, Beijing Ophthalmology and Visual Science Key Lab, Beijing Tongren Hospital, Capital Medical University, Beijing, China.
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关键词: orbit SFT dominant cell morphological type risk assessment NAB2-STAT6

摘要:
Orbital primary solitary fibrous tumors (OPSFTs) are rare. To further characterize the clinical and pathological features of OPSFTs, 92 cases of OPSFT were analyzed to develop a risk prediction model. OPSFTs were equally distributed between males (n = 45) and females (n = 47) with a mean patient age of 40.8 years (median 39 years; range 5-70 years) at initial diagnosis. The mean tumor size was 2.79 cm (median 2.5 cm). Microscopically, the tumor cells were irregularly arranged in spindle, ovoid, or round shapes with varying amounts of collagen and branching blood vessels. Immunohistochemical staining showed positive STAT6 nuclear expression in all cases, loss of CD34 expression in seven cases, and a mean Ki-67 label index of 5.25% (range 1%-30%). All patients were initially surgically resected and had a median follow-up of 99 months: 33 patients recurred, 6 of whom presented with multiple recurrences and 1 with distant metastases. A predictive model for the risk of recurrence based on tumor size, mitosis, Ki-67 label index, and dominant constituent cell (DCC) was developed based on our results. In conclusion, OPSFTs are rare but can be reliably diagnosed based on characteristic morphological features and STAT6 immunohistochemistry. The rate of local recurrence of orbital tumors tends to be higher than the rate of distant metastases, which can be predicted by a risk stratification model specific to orbital tumors. Long-term clinical follow-up is recommended as advanced disease is common.Copyright © 2023. Published by Elsevier Inc.

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出版当年[2022]版:
大类 | 3 区 医学
小类 | 2 区 病理学
最新[2023]版:
大类 | 2 区 医学
小类 | 2 区 病理学
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出版当年[2021]版:
Q2 PATHOLOGY
最新[2023]版:
Q2 PATHOLOGY

影响因子: 最新[2023版] 最新五年平均 出版当年[2021版] 出版当年五年平均 出版前一年[2020版] 出版后一年[2022版]

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第一作者机构: [1]Department of Pathology, Beijing Tongren Hospital, Capital Medical University, Beijing, China.
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通讯机构: [1]Department of Pathology, Beijing Tongren Hospital, Capital Medical University, Beijing, China. [*1]Department of Pathology, Beijing Tongren Hospital, Capital Medical University.Beijing Key Laboratory of Head and Neck Molecular Diagnostic Pathology, Beijing 100730, China
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