A rare case of primary testicular follicular lymphoma in a pediatric patient.

IF 0.6 4区医学 Q4 HEMATOLOGY

Journal of Hematopathology Pub Date : 2025-01-25 DOI:10.1007/s12308-025-00619-9

Amanda J Nguyen, Rebecca L King, David S Viswanatha, Jess F Peterson, Nina Rahimi, Cody J Artymiuk, Ellen D McPhail

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引用次数: 0

Abstract

Testicular follicular lymphoma (TFL) is an exceedingly rare lymphoma that typically occurs in young male patients and is now recognized as a distinct diagnostic entity in the International Consensus Classification. TFL shows some clinicopathologic and genetic overlap with pediatric-type follicular lymphoma (PTFL). We report a case of TFL occurring in an otherwise healthy 4-year-old boy who presented with painless scrotal swelling. Orchiectomy revealed a 1.5-cm left testicular mass. Histologic sections showed a dense lymphoid infiltrate with nodular/follicular architecture growing between the seminiferous tubules. The infiltrate was composed of CD20/PAX5-positive B-cells that coexpressed germinal center markers (CD10, BCL6, MEF2B); they were negative for BCL2. No BCL2 or BCL6 rearrangements and no TNFRSF14 deletion were detected by FISH. Chromosomal microarray analysis detected copy-neutral loss of heterozygosity (CN-LOH) at 1p36.33-p36.32 (region of TNFRSF14). Next-generation sequencing detected variants in GNA13, RHOA, and TNFRSF14. In conclusion, this case shows the classic clinical, pathologic, and genetic features of TFL and highlights the similarities to PTFL and the importance of distinguishing this entity from other subtypes of FL. Patients with TFL typically respond favorably to orchiectomy and chemotherapy and have excellent clinical outcomes.

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一例罕见的小儿原发性睾丸滤泡性淋巴瘤。

睾丸滤泡性淋巴瘤（TFL）是一种非常罕见的淋巴瘤，通常发生在年轻男性患者中，现在在国际共识分类中被认为是一种独特的诊断实体。TFL与儿科型滤泡性淋巴瘤（PTFL）有一些临床病理和遗传学上的重叠。我们报告一例TFL发生在一个健康的4岁男孩谁提出无痛阴囊肿胀。睾丸切除术显示左侧睾丸肿块1.5 cm。组织学切片显示精小管之间有密集的淋巴浸润及结节/滤泡结构。浸润细胞由共表达生发中心标志物（CD10、BCL6、MEF2B）的CD20/ pax5阳性b细胞组成；BCL2呈阴性。FISH未检测到BCL2或BCL6重排，TNFRSF14缺失。染色体微阵列分析在1p36.33-p36.32 （TNFRSF14区域）检测到拷贝中性杂合性缺失（CN-LOH）。下一代测序检测到GNA13、RHOA和TNFRSF14的变异。总之，该病例显示了TFL的典型临床、病理和遗传特征，并突出了与PTFL的相似性以及将其与其他亚型FL区分开来的重要性。TFL患者通常对睾丸切除术和化疗反应良好，临床结果良好。

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来源期刊

Journal of Hematopathology HEMATOLOGYPATHOLOGY-PATHOLOGY

CiteScore

0.80

自引率

0.00%

发文量

期刊介绍： The Journal of Hematopathology aims at providing pathologists with a special interest in hematopathology with all the information needed to perform modern pathology in evaluating lymphoid tissues and bone marrow. To this end the journal publishes reviews, editorials, comments, original papers, guidelines and protocols, papers on ancillary techniques, and occasional case reports in the fields of the pathology, molecular biology, and clinical features of diseases of the hematopoietic system. The journal is the unique reference point for all pathologists with an interest in hematopathology. Molecular biologists involved in the expanding field of molecular diagnostics and research on lymphomas and leukemia benefit from the journal, too. Furthermore, the journal is of major interest for hematologists dealing with patients suffering from lymphomas, leukemias, and other diseases. The journal is unique in its true international character. Especially in the field of hematopathology it is clear that there are huge geographical variations in incidence of diseases. This is not only locally relevant, but due to globalization, relevant for all those involved in the management of patients.