Journal of Orthopaedics Trauma Surgery and Related Research

Journal of Orthopaedics Trauma Surgery and Related Research

An Official Journal of Polish Society of Orthopaedics and Traumatology

e-ISSN: 2449-9145

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Thomas Jefferson University, 4201 Henry Avenue, Philadelphia, United States, Email:
*Correspondence: KAYLEE JOHNSON, Thomas Jefferson University, 4201 Henry Avenue, Philadelphia, United States, Email:

Received: 12-May-2024, Manuscript No. jotsrr-24-139228; Editor assigned: 15-May-2024, Pre QC No. jotsrr-24-139228(PQ); Accepted Date: Jun 20, 2024 ; Reviewed: 22-May-2024 QC No. jotsrr-24-139228(Q); Revised: 15-Jun-2024, Manuscript No. jotsrr-24-139228(R); Published: 25-Jun-2024

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Primary Bone Lymphoma (PBL) is a rare form of malignant lymphoma that primarily affects the skeletal system or various osseous sites. It differs from secondary lesions caused by disseminated lymphoma. PBL is defined as lymphoma localized to a single bone without distant spread for at least six months from initial diagnosis. It may involve regional lymph nodes and, rarely, manifests as multifocal lesions in more than one bone. PBL accounts for a small percentage of extra-nodal lymphomas and bone tumors, with a peak incidence in adults aged 40-60. It predominantly consists of diffuse large B cell lymphoma and can be categorized based on involvement and extent of disease. Diagnosis can be challenging due to factors like limited tissue samples and histological complexity. Common symptoms include localized bone pain, swelling, fractures, and neurological deficits. Laboratory tests may show elevated serum lactate dehydrogenase levels. Treatment and prognosis vary depending on factors such as subtype, staging, and patient characteristics. Morphologically, PBL typically presents as diffuse infiltration of large atypical lymphoid cells with variable cellular composition. It may also include rare variants such as Burkitt's lymphoma or Hodgkin's lymphoma. Understanding the clinical, histological, and molecular characteristics of PBL is crucial for accurate diagnosis and management


Primary Bone Lymphoma (PBL); Multifocal lesions; Malignant lymphoma


In contrast to secondary lesions resulting from disseminated lymphoma, Primary Bone Lymphoma (PBL) is an infrequently observed manifestation of malignant lymphoma. It typically originates within the skeletal system or various osseous sites. PBL is characterized by lymphoma confined to a singular bone, without distant dissemination for at least six months following initial diagnosis. Additionally, PBL is defined as lymphoma arising within an osseous site with no evidence of disease at other sites for a minimum of six months. While PBL may involve regional lymph nodes, multifocal presentation, where lymphoma affects two or more bones, is rare and often accompanied by a lack of distant neoplastic dissemination for the same duration. This type of lymphoma is often associated with soft tissue swelling and represents approximately 40% of all bone tumors, with hematological neoplasms like plasma cell myeloma or lymphoma constituting a significant proportion. However, discerning PBL accurately can be challenging due to factors such as obtaining quantifiable tissue samples and the presence of focal fibrosis, miniature cells, and crush artifacts.

Primary Bone Lymphoma accounts for less than 5% of extra-nodal lymphomas, less than 5% of various bone tumors, and less than 1% of non-Hodgkin’s lymphoma. It predominantly affects adults, with the highest incidence observed between the fifth and sixth decades of life, though cases can occur at any age. However, it is exceptionally rare in pediatric populations under ten years old. There is a slight male predominance in PBL, with a male-to-female ratio of 1.5:1.

The majority, approximately 80%, of primary bone lymphomas are classified as diffuse large B cell lymphoma. These lymphomas can be categorized into four groups based on their presentation and extent of involvement: Group 1 comprises solitary bone lymphomas, Group 2 consists of multifocal bony lesions, Group 3 involves distant lymph nodes, and Group 4 demonstrates lymphoma associated with visceral disease. In contrast, secondary involvement of bone by lymphoma is more commonly observed and typically signifies stage IV disease.

Staging of primary bone lymphoma is often conducted using the Ann Arbor staging system for non-Hodgkin’s lymphoma. While primary bone lymphoma can arise in various sites such as the bone marrow, axial skeleton, vertebral column, and femur, its frequent involvement of bony metaphysis suggests progressive disease when observed in other areas like the diaphysis or epiphysis. The exact etiology of primary bone lymphoma is unclear, but contributory factors such as osteoclast activating factor may play a role.

Significant tumor dissemination and disease relapse are dependent on the homing properties of lymphoma cells. Clonal rearrangement of neoplastic lymphocytes, as evidenced by immunoglobulin gene rearrangements such as BCL2, BCL6, or MYC, aids in determining cellular phenotype. While primary bone lymphoma primarily manifests with localized bone pain, palpable swelling, pathological fractures, and neurological symptoms due to spinal cord compression, systemic symptoms such as fever, night sweats, and weight loss are less common.

Relapse of primary bone lymphoma can involve various sites, including different bones, regional lymph nodes, adjacent soft tissue, pulmonary parenchyma, bone marrow, or the Central Nervous System (CNS). Cytological examination typically reveals a combination of centroblasts and immunoblasts, with enlarged, bizarre cells sometimes observed. Peripheral blood films may rarely depict neoplastic lymphoma cells, while centroblasts typically present as enlarged, pleomorphic cells with abundant cytoplasm, lobulated nuclei, and prominent nucleoli.

Tissue samples obtained for diagnosis are often limited to avoid complications from pathological fractures. Gross examination of primary bone lymphoma often reveals a characteristic "fish flesh" appearance, with extra-osseous tumor extension and an indistinct tumor perimeter observed. Microscopically, diffuse large B cell lymphoma is commonly identified, with consistent cellular morphology across diverse sites.

Primary bone lymphoma may also present with variants such as Burkitt’s lymphoma, lymphoblastic lymphoma, follicular lymphoma, low-grade B cell lymphoma, various T cell lymphomas, and Hodgkin’s lymphoma. Diffuse Large B Cell Lymphoma (DLBCL) is a frequent subtype of primary bone lymphoma, often involving the bony pelvis and femur. Neoplastic emergence in adults typically occurs in regions of persistent red marrow.

Primary Bone Lymphoma represents a rare manifestation of malignant lymphoma, primarily affecting the skeletal system or various osseous sites. Its accurate diagnosis and classification are essential for appropriate management and prognostication. While primary bone lymphoma presents unique challenges due to its rarity and diverse clinical presentations, advancements in diagnostic techniques and understanding of its molecular pathogenesis continue to improve patient outcomes.


In conclusion, Primary Bone Lymphoma (PBL) poses a challenging clinical scenario due to its rarity, varied clinical presentations, and diagnostic complexities. Despite its infrequent occurrence, PBL represents a significant manifestation of malignant lymphoma, often necessitating prompt and accurate diagnosis for optimal patient management.

Throughout this discussion, we have explored the distinctive features of PBL, including its predilection for the skeletal system and diverse osseous sites. We have elucidated the criteria for defining PBL, emphasizing its confinement to a singular bone or osseous site without distant dissemination for a specific duration. Moreover, we have highlighted the rarity of multifocal presentation, underscoring its diagnostic significance when observed.

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