# Rare Case of Primary Bone Lymphoma

**Authors:** Madeline L Fudala, Imad Karam, Aye M Thida, Imran Khan, Rachelle Hamadi, Natasha Macapagal-Brown, Raavi Gupta, Mohan Preet

PMC · DOI: 10.7759/cureus.84415 · Cureus · 2025-05-19

## TL;DR

This case report describes an elderly woman with primary bone lymphoma who also had skin lesions and lymph node involvement, making diagnosis challenging.

## Contribution

This is the first documented case of primary bone lymphoma with concurrent cutaneous and lymph node involvement.

## Key findings

- The patient was diagnosed with primary bone lymphoma after imaging and biopsy revealed large lymphoid cells with specific markers.
- The case highlights the diagnostic difficulty of PBL due to nonspecific clinical presentation and variable criteria.
- The patient's condition was classified as high-intermediate risk based on NCCN-IPI criteria due to age and extranodal disease.

## Abstract

Primary bone lymphoma (PBL) is a rare entity defined as a lymphoid neoplasm of bone that occurs in the bone without involvement of lymph nodes or other extranodal sites. In this report, we present a case of an elderly female with PBL who presented with regional lymphadenopathy and cutaneous lesions mimicking the primary cutaneous diffuse B-cell lymphoma (PCDBCL), leg type.

This review describes the case of a 73-year-old female with primary bone lymphoma who presented with cutaneous lesions and regional lymphadenopathy. She was initially diagnosed with Paget’s disease of bone but ultimately presented to the emergency room with a pathologic fracture of the left tibia and painful overlying cutaneous lesions. Imaging studies, including X-ray and computed tomography (CT) scans, along with a tibial biopsy, revealed findings consistent with PBL, characterized by large lymphoid cells that were positive for CD10, CD20, and Bcl6, with a high Ki-67 index. The diagnosis was confirmed despite the atypical cutaneous involvement. Due to her age, low-performance status, and extranodal disease, the patient was classified as high-intermediate risk according to the National Comprehensive Cancer Network International Prognostic Index (NCCN-IPI) criteria. The presence of extranodal involvement also placed her at intermediate risk for central nervous system (CNS) disease.

The clinical presentation of PBLs is often nonspecific, and diagnostic criteria noted in the literature vary, making for a difficult diagnosis in clinical practice. To our knowledge, this patient is the first documented case of PBL with concurrent cutaneous manifestations and regional lymph node involvement.

This case report and literature review investigate the possibility of PBLs presenting with lesions extending beyond the initial area of bony involvement.

## Linked entities

- **Proteins:** MME (membrane metalloendopeptidase), MS4A1 (membrane spanning 4-domains A1), BCL6 (BCL6 transcription repressor), Mki67 (antigen identified by monoclonal antibody Ki 67)
- **Diseases:** primary bone lymphoma (MONDO:0017814), Paget’s disease of bone (MONDO:0005382), central nervous system disease (MONDO:0002602)

## Full-text entities

- **Genes:** KRT20 (keratin 20) [NCBI Gene 54474] {aka CD20, CK-20, CK20, K20, KRT21}, BCL6 (BCL6 transcription repressor) [NCBI Gene 604] {aka BCL5, BCL6A, LAZ3, ZBTB27, ZNF51}, MME (membrane metalloendopeptidase) [NCBI Gene 4311] {aka CALLA, CD10, CMT2T, NEP, SCA43, SFE}
- **Diseases:** PCDBCL (MESH:D016393), central nervous system (CNS) disease (MESH:D002493), lymphoid neoplasm of bone (MESH:D001859), cutaneous lesions (MESH:D009059), lymphadenopathy (MESH:D008206), primary (MESH:D010538), PBL (MESH:D008223)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

3 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12176316/full.md

## References

14 references — full list in the complete paper: https://tomesphere.com/paper/PMC12176316/full.md

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Source: https://tomesphere.com/paper/PMC12176316