Fairchild Andrew, McCall Chad M, Oyekunle Taofik, Niedzwiecki Donna, Champ Colin, McKinney Matthew, Kelsey Chris R
Department of Radiation Oncology Duke University Medical Center, Durham, NC.
Department of Pathology, Duke University Medical Center, Durham, NC.
Clin Lymphoma Myeloma Leuk. 2021 May;21(5):e464-e469. doi: 10.1016/j.clml.2020.12.015. Epub 2020 Dec 30.
Diagnosing primary mediastinal (thymic) large B-cell lymphoma (PMBCL) is challenging because it is a clinicopathologic entity that shares characteristics with other lymphomas and lacks pathognomonic features. We sought to investigate the fidelity between a working diagnosis of PMBCL at our institution and the clinicopathologic criteria established within the 2017 World Health Organization (WHO) classification.
Medical records and archived tissue of patients treated for stage I-II PMBCL from 1998 to 2018 were retrospectively reviewed for clinical and pathologic conformity with current WHO criteria. Disease was characterized as definitely PMBCL if all of the following were present: anterior mediastinal mass with or without lymph node involvement, no extranodal disease, B-cell antigen expression, Epstein-Barr virus negativity, and at least one supportive feature: female gender under age 40, bulky primary tumor, CD30 weakly positive, compartmentalizing alveolar fibrosis, lack of surface immunoglobulin expression, and MUM1 or CD23 positivity. Disease without supportive features or other pathologic findings more suggestive of other entities was characterized as equivocal for PMBCL. Lack of an anterior mediastinal mass, presence of distant lymph node involvement or extranodal disease, lack of B-cell antigen expression, or Epstein-Barr virus positivity were characterized as definitely not PMBCL. Clinical management and outcomes were also assessed.
Of 63 patients treated for presumed stage I-II PMBCL, 58 (92%) met the criteria for PMBCL. The most common reason for a discordant diagnosis was lack of an anterior mediastinal mass (n = 3). Two additional patients were characterized as having disease equivocal for PMBCL. In retrospect, one patient most likely had a mediastinal gray zone lymphoma due to CD15 positivity and another diffuse large B cell, not otherwise specified, at pathologic review. Five-year progression-free and overall survival were 67% (95% confidence interval, 54-77) and 81% (95% confidence interval, 68-89), respectively, for all patients.
Despite the complexity of the clinicopathologic criteria of PMBCL, most patients (92%) who were treated for stage I-II PMBCL at our institution appear to have been accurately diagnosed.
诊断原发性纵隔（胸腺）大B细胞淋巴瘤（PMBCL）具有挑战性，因为它是一种临床病理实体，与其他淋巴瘤有共同特征且缺乏特异性特征。我们试图研究我院PMBCL工作诊断与2017年世界卫生组织（WHO）分类中确立的临床病理标准之间的符合程度。
回顾性分析1998年至2018年接受I-II期PMBCL治疗患者的病历和存档组织，以评估其临床和病理与当前WHO标准的符合情况。若符合以下所有条件，则疾病被明确诊断为PMBCL：有或无淋巴结受累的前纵隔肿块、无结外疾病、B细胞抗原表达、爱泼斯坦-巴尔病毒阴性，以及至少一项支持特征：40岁以下女性、原发性肿瘤体积大、CD30弱阳性、分隔性肺泡纤维化、缺乏表面免疫球蛋白表达、MUM1或CD23阳性。无支持特征或其他更提示其他实体的病理表现的疾病被归类为PMBCL诊断不明确。无前纵隔肿块、存在远处淋巴结受累或结外疾病、缺乏B细胞抗原表达或爱泼斯坦-巴尔病毒阳性被归类为肯定不是PMBCL。还评估了临床管理和结局。
在63例接受I-II期PMBCL治疗的患者中，58例（92%）符合PMBCL标准。诊断不一致的最常见原因是无前纵隔肿块（n = 3）。另外2例患者被归类为PMBCL诊断不明确。回顾性分析，1例患者因CD15阳性最可能患有纵隔灰色地带淋巴瘤，另1例在病理检查时为弥漫性大B细胞淋巴瘤，未另行分类。所有患者的5年无进展生存率和总生存率分别为分别为67%（95%置信区间，54-77）和81%（95%置信区间，68-89）。
尽管PMBCL的临床病理标准复杂，但在我院接受I-II期PMBCL治疗的大多数患者（92%）似乎得到了准确诊断。
