Clinicopathological analysis of adrenal intravascular large B-cell lymphoma

Jiaxin LIN; Ran WEI; Ruohong SHUI; Hongfen LU; Xiaoqiu LI; Baohua YU

doi:10.19401/j.cnki.1007-3639.2024.11.004

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Clinicopathological analysis of adrenal intravascular large B-cell lymphoma

Article | 更新时间：2025-12-31

- Clinicopathological analysis of adrenal intravascular large B-cell lymphoma
- China Oncology Vol. 34, Issue 11, Pages: 1020-1027(2024)
- 作者机构：
  
  复旦大学附属肿瘤医院病理科，复旦大学上海医学院肿瘤学系，上海 200032
- 作者简介：
- 基金信息：
- DOI：10.19401/j.cnki.1007-3639.2024.11.004
  CLC： R733.4
- Received：11 July 2024，
  
  Revised：2024-11-07，
  
  Published：30 November 2024
- 稿件说明：
移动端阅览
Jiaxin LIN, Ran WEI, Ruohong SHUI, et al. Clinicopathological analysis of adrenal intravascular large B-cell lymphoma[J]. China Oncology, 2024, 34(11): 1020-1027.
DOI：

Jiaxin LIN, Ran WEI, Ruohong SHUI, et al. Clinicopathological analysis of adrenal intravascular large B-cell lymphoma[J]. China Oncology, 2024, 34(11): 1020-1027. DOI： 10.19401/j.cnki.1007-3639.2024.11.004.

摘要

背景与目的：

原发性肾上腺血管内大B细胞淋巴瘤（intravascular large B-cell lymphoma，IVLBCL）是一种罕见的高侵袭性淋巴瘤，目前对其仍缺乏充分认识。本研究旨在探讨肾上腺IVLBCL的临床病理学特征、分子遗传学特征及预后。

方法：

收集复旦大学附属肿瘤医院病理科2012—2023年诊断的肾上腺IVLBCL病例，回顾性分析其临床及组织病理学特征、免疫表型、治疗和预后，并采用靶向二代测序（next-generation sequencing，NGS）技术探讨其分子遗传学特点。本研究已通过复旦大学附属肿瘤医院伦理委员会审查（伦理编号：050432-4-2307E）。

结果：

5例肾上腺IVLBCL患者均为男性，中位年龄为52岁（年龄范围50~82岁）。2例因低热而就诊，1例表现为腹痛，1例为体检发现，1例未获得相关信息。外周血检查示2例有血清乳酸脱氢酶升高，2例出现肾上腺功能异常。影像学检查显示，肾上腺增大或占位性病变伴氟代脱氧葡萄糖（fluorodeoxyglucose，FDG）摄取增高；其中4例累及双侧肾上腺，1例累及右侧肾上腺。显微镜下可见异型大淋巴细胞局限于血管腔内，部分病例血管腔内见纤维素性坏死。免疫组织化学染色显示，所有（5/5）病例的肿瘤细胞CD20阳性，Ki-67增殖指数均较高（

80%），80%（4/5）病例呈非生发中心B细胞样（non-germinal-center B-cell-like，non-GCB）表型，100%（4/4）呈MYC/BCL2双表达；血管内皮细胞标志物染色显示，肿瘤细胞绝大多数位于血管内。3例患者获得治疗和随访信息，其中1例仅接受手术切除的患者确诊后5个月死亡，1例经手术切除后接受R-CHOP方案治疗的患者获得完全缓解，1例穿刺活检确诊后接受R-CHOP方案治疗的患者获得部分缓解；1年总生存率为66.7%，总生存期为5~87个月。1例行NGS检测，结果显示，

MYD

88 V217F、

53、

CDH

1、

ARID

、

MSH

3、

MLH

3、

PTPRK

、

22和

FLCN

等基因错义突变。

结论：

肾上腺IVLBCL发病率低，好发于中老年男性，本组病例以non-GCB为主且常伴MYC/BCL2双表达，并存在

MYD

88非L265P突变。肾上腺IVLBCL由于其临床症状多样化且缺乏特异性导致早期确诊困难，积累更多病例深入了解其临床病理学特征及分子遗传学特征，有助于早期诊断、及时治疗并改善患者预后，为深入认识疾病发生、发展机制、探索治疗靶点提供理论基础。

Abstract

Background and purpose:

Primary adrenal intravascular large B-cell lymphoma (IVLBCL) is rare and highly aggressive. Unfortunately

comprehensive and sufficient understanding of the disease is lacking. This study investigated the clinicopathological and molecular genetic characteristics of adrenal IVLBCL.

Methods:

Adrenal IVLBCL cases diagnosed from 2012 to 2023 were collected from Department of Pathology

Fudan University Shanghai Cancer Center. The clinical and histopathological features

immunophenotype

treatment and prognosis were analyzed. The molecular genetic characteristics were detected usin

g next-generation sequencing (NGS). This study was approved by the Ethics Committee of Fudan University Shanghai Cancer Center (Ethics number: 050432-4-2307E).

Results:

All of the 5 patients were male

with median age 52 years (ranged 50-82 years). Two cases had low-grade fever

1 case had abdominal pain

1 case was found by physical examination

and the information of the remaining one was unknown. Peripheral blood test showed elevated serum lactate dehydrogenase in 2 cases and adrenal dysfunction in 2 cases. On initial diagnosis

imaging tests displayed adrenal enlargement or masses with increased fluorodeoxyglucose (FDG) uptake. Bilateral adrenal glands were involved in 4 cases and only the right adrenal gland was involved in the other case. Morphologically

large atypical lymphocytes were confined to blood vessels

and fibrinous necrosis was observed in some cases. Immunohistochemical study revealed that CD20 was positive in all cases. Ki-67 proliferation index was high

all above 80%. 80% (4/5) of the cases were of non-germinal-center B-cell-like (non-GCB) phenotype

100% (4/4) of the cases had MYC/BCL2 double expression. Endothelial cell markers staining indicated that most of the tumor cells were confined within the blood vessels in all cases. Follow-up data were available in 3 patients. One patient who underwent only surgical resection died 5 months after diagnosis

one achieved complete remission after surgery plus R-CHOP

and the other diagnosed by biopsy achieved a partial remission after R-CHOP. The 1-year overall survival rate was 66.7%

and overall survival was 5-87 months. NGS testing in 1 case showed missense mutations in

MYD

88 V217F

CDH

ARID

MSH

MLH

PTPRK

22 and

FLCN

Conclusion:

Adrenal IVLBCL is rare and tends to occur in the middle-aged and elderly male. The majority of our patient

s were non-GCB phenotype

often accompanied by MYC/BCL2 double expression

and

MYD

88 non-L265P mutation was detected. Early diagnosis of adrenal IVLBCL is difficult due to its diverse clinical symptoms and lack of specificity. It is of great importance to accumulate more cases and further understand the clinicopathological and molecular genetic characteristics of this rare disease

which might not only help with early diagnosis

timely treatment and improvement of prognosis

but also provide a theoretical basis for further understanding the pathogenesis and development of the disease and exploring therapeutic targets.

关键词

Keywords

references

LIU Z Y , ZHANG Y L , ZHU Y C , et al. Prognosis of intravascular large B cell lymphoma (IVLBCL): analysis of 182 patients from global case series [J ] . Cancer Manag Res , 2020 , 12 : 10531 - 10540 . DOI: 10.2147/CMAR.S267825 http://doi.org/10.2147/CMAR.S267825

WHO Classification of Tumours Editorial Board . Haematolymphoid tumours (5th ed) [M ] . Lyon (France) : International Agency for Research on Cancer , 2024 .

HANS C P , WEISENBURGER D D , GREINER T C , et al. Confirmation of the molecular classification of diffuse large B-cell lymphoma by immunohistochemistry using a tissue microarray [J ] . Blood , 2004 , 103 ( 1 ): 275 - 282 . DOI: 10.1182/blood-2003-05-1545 http://doi.org/10.1182/blood-2003-05-1545

WAWIRE J , SAYED S , MOLOO Z , et al. Diffuse large B-cell lymphoma in Kenya: MYC, BCL2, and the cell of origin [J ] . J Glob Oncol , 2019 , 5 : 1 - 8 . DOI: 10.1200/JGO.18.00203 http://doi.org/10.1200/JGO.18.00203

CAMPUZANO-ZULUAGA G , CIOFFI-LAVINA M , LOSSOS I S , et al. Frequency and extent of CD30 expression in diffuse large B-cell lymphoma and its relation to clinical and biologic factors: a retrospective study of 167 cases [J ] . Leuk Lymphoma , 2013 , 54 ( 11 ): 2405 - 2411 .

SWERDLOW S H , CAMPO E , HARRIS N L , et al. World Health Organization classification of tumors of haematopoietic and lymphoid tissues [M ] . Lyon : International Agency for Research on Cancer , 2008 .

MURASE T , YAMAGUCHI M , SUZUKI R , et al. Intravascular large B-cell lymphoma (IVLBCL): a clinicopathologic study of 96 cases with special reference to the immunophenotypic heterogeneity of CD5 [J ] . Blood , 2007 , 109 ( 2 ): 478 - 485 . DOI: 10.1182/blood-2006-01-021253 http://doi.org/10.1182/blood-2006-01-021253

中华医学会血液学分会淋巴细胞疾病学组 , 中国临床肿瘤学会CSCO淋巴瘤专家委员会 . 血管内大B细胞淋巴瘤诊治中国专家共识(2023年版) [J ] . 中华血液学杂志 , 2023 , 44 ( 3 ): 177 - 181 .

Lymphoid Disease Group , Chinese Society of Hematology , Chinese Medical Association ; Lymphoma Expert Committee of Chinese Society of Clinical Oncology CSCO . Chinese expert consensus on the diagnosis and management of intravascular large B cell lymphoma (2023) [J ] . Chin J Hematol , 2023 , 44 ( 3 ): 177 - 181 .

MATSUE K , ABE Y , NARITA K , et al. Diagnosis of intravascular large B cell lymphoma: novel insights into clinicopathological features from 42 patients at a single institution over 20years [J ] . Br J Haematol , 2019 , 187 ( 3 ): 328 - 336 .

KIRIAKOPOULOS A , LINOS D . Intravascular B-large cell lymphoma: an unexpected diagnosis of an incidental adrenal mass [J ] . J Surg Case Rep , 2019 , 2019 ( 2 ): rjz048 .

FUKUSHIMA A , OKADA Y , TANIKAWA T , et al. Primary bilateral adrenal intravascular large B-cell lymphoma associated with adrenal failure [J ] . Intern Med , 2003 , 42 ( 7 ): 609 - 614 .

YU Y , GOVINDARAJAN R . Intravascular large B-cell lymphoma presenting as an isolated cauda equina-conus medullaris syndrome-a case report [J ] . J Spinal Cord Med , 2020 , 43 ( 4 ): 556 - 559 .

TAKAHASHI Y , IIDA K , HINO Y , et al. Silent intravascular lymphoma initially manifesting as a unilateral adrenal incidentaloma [J ] . Case Rep Med , 2012 , 2012 : 849285 .

LI W , LIN W , MA C , et al. A case of intravascular large B-cell lymphoma in the left adrenal and another tumor in the right adrenal detected by (18)F-FDG PET/CT [J ] . Hell J Nucl Med , 2016 , 19 ( 1 ): 57 - 59 . DOI: 10.1967/s002449910342 http://doi.org/10.1967/s002449910342

CUI J , LIU Q , CHENG Y X , et al. An intravascular large B-cell lymphoma with a t(3;14)(q27;q32) translocation [J ] . J Clin Pathol , 2014 , 67 ( 3 ): 279 - 281 . DOI: 10.1136/jclinpath-2013-201980 http://doi.org/10.1136/jclinpath-2013-201980

SRIVATSA S , SHARMA J , LOGANI S . Intravascular lymphoma: an unusual diagnostic outcome of an incidentally detected adrenal mass [J ] . Endocr Pract , 2008 , 14 ( 7 ): 884 - 888 . DOI: 10.4158/EP.14.7.884 http://doi.org/10.4158/EP.14.7.884

VENIZELOS I , TAMIOLAKIS D , PETRAKIS G . High grade primary adrenal intravascular large B-cell lymphoma manifesting as Addison disease [J ] . Rev Esp Enferm Dig , 2007 , 99 ( 8 ): 471 - 474 .

ASKARIAN F , XU D S . Adrenal enlargement and insufficiency: a common presentation of intravascular large B-cell lymphoma [J ] . Am J Hematol , 2006 , 81 ( 6 ): 411 - 413 .

COLAVOLPE C , EBBO M , TROUSSE D , et al. FDG-PET/CT is a pivotal imaging modality to diagnose rare intravascular large B-cell lymphoma: case report and review of literature [J ] . Hematol Oncol , 2015 , 33 ( 2 ): 99 - 109 . DOI: 10.1002/hon.2140 http://doi.org/10.1002/hon.2140

SCHRADER A M R , JANSEN P M , WILLEMZE R , et al. High prevalence of MYD 88 and CD 79 B mutations in intravascular large B-cell lymphoma [J ] . Blood , 2018 , 131 ( 18 ): 2086 - 2089 .

BOONSAKAN P , IAMSUMANG W , CHANTRATHAMMACHART P , et al. Prognostic value of concurrent expression of C-MYC and BCL2 in intravascular large B-cell lymphoma: a 10-year retrospective study [J ] . Biomed Res Int , 2020 , 2020 : 1350820 .

YEGAPPAN S , COUPLAND R , ARBER D A , et al. Angiotropic lymphoma: an immunophenotypically and clinically heterogeneous lymphoma [J ] . Mod Pathol , 2001 , 14 ( 11 ): 1147 - 1156 .

YAMAGUCHI M , SETO M , OKAMOTO M , et al. De novo CD5 + diffuse large B-cell lymphoma: a clinicopathologic study of 109 patients [J ] . Blood , 2002 , 99 ( 3 ): 815 - 821 .

GUPTA G K , JAFFE E S , PITTALUGA S . A study of PD-L1 expression in intravascular large B cell lymphoma: correlation with clinical and pathological features [J ] . Histopathology , 2019 , 75 ( 2 ): 282 - 286 . DOI: 10.1111/his.13870 http://doi.org/10.1111/his.13870

SUEHARA Y , SAKATA-YANAGIMOTO M , HATTORI K , et al. Liquid biopsy for the identification of intravascular large B-cell lymphoma [J ] . Haematologica , 2018 , 103 ( 6 ): e241 -e244.

SHIMADA K , YOSHIDA K , SUZUKI Y , et al. Frequent genetic alterations in immune checkpoint-related genes in intravascular large B-cell lymphoma [J ] . Blood , 2021 , 137 ( 11 ): 1491 - 1502 . DOI: 10.1182/blood.2020007245 http://doi.org/10.1182/blood.2020007245

WILSON W H , YOUNG R M , SCHMITZ R , et al. Targeting B cell receptor signaling with ibrutinib in diffuse large B cell lymphoma [J ] . Nat Med , 2015 , 21 ( 8 ): 922 - 926 . DOI: 10.1038/nm.3884 http://doi.org/10.1038/nm.3884

XIE J L , SHEN X , SHI Q , et al. Clinical significance of MYD 88 non-L265P mutations in diffuse large B-cell lymphoma [J ] . Hematol Oncol , 2022 , 40 ( 5 ): 885 - 893 .

JIANG S Y , QIN Y , GUI L , et al. Genomic alterations and MYD 88MUT variant mapping in patients with diffuse large B-cell lymphoma and response to ibrutinib [J ] . Target Oncol , 2020 , 15 ( 2 ): 221 - 230 .

ZENZ T , KREUZ M, FUGE , et al. TP 53 mutation and survival in aggressive B cell lymphoma [J ] . Int J Cancer , 2017 , 141 ( 7 ): 1381 - 1388 . DOI: 10.1002/ijc.30838 http://doi.org/10.1002/ijc.30838

JACOBS G , HELLMIG S , HUSE K , et al. Polymorphisms in the 3’-untra nslated region of the CDH 1 gene are a risk factor for primary gastric diffuse large B-cell lymphoma [J ] . Haematologica , 2011 , 96 ( 7 ): 987 - 995 .

CARRERAS J , IKOMA H , KIKUTI Y Y , et al. Mutational, immune microenvironment, and clinicopathological profiles of diffuse large B-cell lymphoma and follicular lymphoma with BCL6 rearrangement [J ] . Virchows Arch , 2024 , 484 ( 4 ): 657 - 676 .

DE MIRANDA N F , PENG R J , GEORGIOU K , et al. DNA repair genes are selectively mutated in diffuse large B cell lymphomas [J ] . J Exp Med , 2013 , 210 ( 9 ): 1729 - 1742 .

ZHANG Y , JIA C W , WANG W , et al. The interim analysis from a prospective single-center phase 2 study of zanubrutinib plus R-CHOP in treat-Naïve intravascular large B cell lymphoma [J ] . Blood , 2021 , 138 ( Supplement 1 ): 3563 .

RAJYAGURU D J , BHASKAR C , BORGERT A J , et al. Intravascular large B-cell lymphoma in the United States (US): a population-based study using Surveillance, Epidemiology, and End Results program and National Cancer Database [J ] . Leuk Lymphoma , 2017 , 58 ( 9 ): 1 - 9 .

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