Primary malignant lymphoma of bone (PLB) is a rare type of non-Hodgkin lymphoma that primarily originates within the bone tissue. Unlike secondary lymphomas, which are metastases from a different site, PLB arises directly within the skeletal system, often affecting the long bones, pelvis, and vertebrae. It typically presents as a solitary bone lesion, which can lead to symptoms such as localized pain, swelling, and pathological fractures due to the weakening of the bone structure. The most commonly involved histological subtype is diffuse large B-cell lymphoma, but other variants, including follicular lymphoma and anaplastic large cell lymphoma, may also be encountered. Diagnosis is challenging and often necessitates a combination of imaging studies, such as X-rays, MRI, or CT scans to delineate the extent of the lesion, as well as a biopsy to confirm the histological type of the lymphoma. The imaging characteristics may include a lytic bone lesion with a possible associated soft tissue mass, which can mimic other bone pathologies like infections or metastatic disease. Treatment typically involves a multidisciplinary approach, including chemotherapy, radiation therapy, and, in some cases, surgical intervention. Systemic chemotherapy is the cornerstone of treatment and is effective in managing both localized and advanced disease. The addition of radiotherapy particularly benefits patients with localized forms of PLB, especially in cases where there is residual disease post-chemotherapy or when the patient is not a suitable candidate for surgery. Generally, the prognosis for patients with PLB varies greatly depending on factors such as the patient's overall health, age, disease stage at diagnosis, and the specific histological characteristics of the lymphoma. Advanced disease or the presence of other comorbid conditions can negatively impact outcomes. However, with appropriate management, many patients experience significant remission and improvement in quality of life. Ongoing research is focused on understanding the pathophysiology of PLB, which remains less well-defined compared to lymphomas affecting lymph nodes or other soft tissues. The rarity of primary malignant lymphoma of bone poses challenges for accumulating sufficient clinical data to establish standardized treatment protocols and prognostic criteria. As such, awareness among clinicians is essential for prompt diagnosis and effective management, while ongoing studies aim to optimize therapeutic strategies and improve patient outcomes in this unique and complex malignancy. In conclusion, PLB requires a comprehensive approach to diagnosis and treatment, and as more understanding of its biology emerges, tailored therapies may be developed that enhance survival rates and minimize treatment-related morbidity.
