Koh Jiwon, Jeon Yoon Kyung
Department of Pathology, Seoul National University Hospital, Seoul, Korea.
Department of Pathology, Seoul National University College of Medicine, Seoul, Korea.
J Pathol Transl Med. 2020 May;54(3):253-257. doi: 10.4132/jptm.2019.12.17. Epub 2020 Feb 5.
Follicular lymphoma (FL) with hyaline-vascular Castleman disease (FL-HVCD)-like features is a rare morphologic variant, with fewer than 20 cases in the literature. Herein, we report a case of FL-HVCD in a 37-year-old female who presented with isolated neck lymph node enlargement. The excised lymph node showed features reminiscent of HVCD, including regressed germinal centers (GCs) surrounded by onion skin-like mantle zones, lollipop lesions composed of hyalinized blood vessels penetrating into regressed GCs, and hyalinized interfollicular stroma. In addition, focal areas of abnormally conglomerated GCs composed of homogeneous, small centrocytes with strong BCL2, CD10, and BCL6 expression were observed, indicating partial involvement of the FL. Several other lymphoid follicles showed features of in situ follicular neoplasia. Based on the observations, a diagnosis of FL-HVCD was made. Although FLHVCD is very rare, the possibility of this variant should be considered in cases resembling CD. Identification of abnormal, neoplastic follicles and ancillary immunostaining are helpful for proper diagnosis.
具有透明血管型Castleman病(FL-HVCD)样特征的滤泡性淋巴瘤是一种罕见的形态学变异型,文献报道不足20例。在此,我们报告一例37岁女性的FL-HVCD,该患者表现为孤立性颈部淋巴结肿大。切除的淋巴结显示出类似HVCD的特征,包括退化的生发中心(GCs)被洋葱皮样套区包围、由透明化血管穿透退化的GCs组成的棒棒糖样病变以及透明化的滤泡间基质。此外,观察到由均匀的小中心细胞组成的异常聚集的GCs灶,这些细胞BCL2、CD10和BCL6表达强烈,提示滤泡性淋巴瘤部分受累。其他几个淋巴滤泡显示原位滤泡肿瘤的特征。基于这些观察结果,做出了FL-HVCD的诊断。尽管FL-HVCD非常罕见,但在类似Castleman病的病例中应考虑这种变异型的可能性。识别异常的肿瘤性滤泡和辅助免疫染色有助于正确诊断。