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髌骨原发性大B细胞淋巴瘤伴早期全身进展:一例报告

Primary large B-cell lymphoma of the patella with early systemic progression: A case report.

作者信息

Ben Rejeb Sarra, Ben Romdhane Majdi, Sghaier Majdi, Charfi Mehdi

机构信息

Pathology department, Security Forces Hospital, Marsa, Tunisia.

Orthopedic department, Security Forces Hospital, Marsa, Tunisia.

出版信息

Int J Surg Case Rep. 2025 Jun;131:111344. doi: 10.1016/j.ijscr.2025.111344. Epub 2025 Apr 23.

Abstract

INTRODUCTION

Primary bone lymphoma (PBL) of the patella is an extremely rare condition, with fewer than ten documented cases. We herein described a unique case of primary diffuse large B-cell lymphoma (DLBCL) of the patella, highlighting its diagnostic challenges, therapeutic considerations, and unexpectedly rapid systemic progression, contributing valuable insights to the understanding of this uncommon entity.

CASE PRESENTATION

A 68-year-old woman with a history of papillary thyroid carcinoma reported six months of worsening right knee pain, swelling, and reduced mobility. Imaging showed a lytic patellar lesion with cortical damage and soft tissue involvement. A biopsy confirmed non-germinal center B-cell-like DLBCL, identified by positive CD45, CD20, PAX5, MUM1, and BCL2 markers, and negative CD10, BCL6, and pan-cytokeratin results. PET/CT and bone marrow biopsy confirmed the disease was localized (stage IE). She received R-CHOP chemotherapy, but six months later, PET/CT revealed widespread progression to lymph nodes, adrenals, bones, breasts, and subcutaneous tissues. A breast biopsy matched the original DLBCL. Salvage chemotherapy with the R-ICE regimen (rituximab, ifosfamide, carboplatin, etoposide) was initiated. However, the patient's clinical condition rapidly deteriorated, and she opted for palliative care.

CLINICAL DISCUSSION

Patellar PBL is challenging to diagnose due to its vague symptoms and similarity to metastases or other bone tumors, particularly in patients with prior cancer. Biopsy and immunohistochemistry were essential for confirmation. The rapid systemic spread, potentially tied to the non-GCB subtype, indicates an aggressive disease course, uncommon for early-stage PBL.

CONCLUSION

This case contributes to the sparse literature on patellar PBL, stressing the importance of biopsy for diagnosis and the need for close monitoring due to the risk of swift progression, even in localized disease. Further study of prognostic factors is warranted.

摘要

引言

髌骨原发性骨淋巴瘤(PBL)极为罕见,文献记载病例不足十例。我们在此描述了一例独特的髌骨原发性弥漫性大B细胞淋巴瘤(DLBCL),强调了其诊断挑战、治疗考量以及出乎意料的快速全身进展,为理解这一罕见实体提供了有价值的见解。

病例介绍

一名有乳头状甲状腺癌病史的68岁女性报告右膝疼痛、肿胀且活动能力下降六个月。影像学检查显示髌骨有溶骨性病变,伴有皮质破坏和软组织受累。活检证实为非生发中心B细胞样DLBCL,通过CD45、CD20、PAX5、MUM1和BCL2标记物阳性以及CD10、BCL6和全细胞角蛋白结果阴性得以确诊。PET/CT和骨髓活检证实疾病局限(IE期)。她接受了R-CHOP化疗,但六个月后,PET/CT显示疾病广泛进展至淋巴结、肾上腺、骨骼、乳房和皮下组织。乳房活检结果与原DLBCL相符。开始采用R-ICE方案(利妥昔单抗、异环磷酰胺、卡铂、依托泊苷)进行挽救性化疗。然而,患者的临床状况迅速恶化,她选择了姑息治疗。

临床讨论

由于症状模糊且与转移瘤或其他骨肿瘤相似,尤其是在有既往癌症病史的患者中,髌骨PBL的诊断具有挑战性。活检和免疫组织化学对于确诊至关重要。快速的全身扩散,可能与非生发中心B细胞亚型有关,表明疾病进程具有侵袭性,这在早期PBL中并不常见。

结论

本病例丰富了关于髌骨PBL的稀少文献,强调了活检对于诊断的重要性以及即使在局限性疾病中由于快速进展风险而进行密切监测的必要性。有必要进一步研究预后因素。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6572/12051503/878a8ccc1c03/gr1.jpg

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