Primary Bone Lymphoma: A Case Series and Review of Literature

Abstract

Primary bone lymphoma (PBL) is a subtype of lymphoma that exclusively affects skeletal tissue. Despite the relatively common involvement of skeletal structures as a manifestation of non-Hodgkin’s lymphoma (NHL), primary and exclusive involvement of the skeletal system is rare. The prevalence of PBL is estimated to be 3–7% amongst primary bone tumors and less than 2% amongst all lymphomas in adults. However, the definition of primary bone lymphoma has been inconsistent over time. Within our institution, we identified four cases of primary bone lymphoma based on diagnostic criteria formed from the general consensus of multiple organizations, including the World Health Organization (WHO) and International Extranodal Lymphoma Study Group (IELSG). Here, we discuss the distinct characteristics amongst these cases in addition to performing a systematic review of current literature regarding this lymphoproliferative entity.

Primary bone lymphoma of the ilium successfully treated without radiation

“…Local soft tissue extension, as seen in our case, may be seen on imaging and is associated with poor prognosis 10 . Bone biopsy is also recommended in order to characterize pathology but must be balanced with the risk of pathological fracture as in our patient 2 …”

“…10 Bone biopsy is also recommended in order to characterize pathology but must be balanced with the risk of pathological fracture as in our patient. 2 Given the rare incidence of PBL, management has been largely driven by retrospective data from case reports and case series. The majority of PBL cases are due to diffuse large B-cell lymphoma (DLBCL), so treatment has typically involved CHOP-based chemotherapy, with R-CHOP demonstrating a higher 3-year progression-free survival (88%) compared to CHOP (52%).…”

“…14 Particular attention to tumor location should be made as radiation therapy to bones with high marrow concentrations, such as the pelvis in our patient, can lead to myelotoxicity and myelosuppression. 2 Here, we describe our unique experience with treating a patient with an untypeable PBL using chemotherapy alone who achieved a durable complete response five years after treatment. Although rare, PBL can present in the ambulatory setting disguised as common musculoskeletal symptoms which may or may not be accompanied by constitutional symptoms.…”

“…The diagnosis of PBL can be challenging as the initial musculoskeletal symptoms can be nonspecific. Laboratory results in PBL are typically unremarkable and relatively unhelpful in diagnosis 2 . Additionally, it can be difficult to differentiate PBL from other primary bone tumors such as osteosarcoma, chondrosarcoma, or Ewing's sarcoma based on radiographic imaging alone.…”

“…Primary bone lymphoma (PBL) is a rare malignancy that comprises less than 5% of all bone malignancies and about 1%-2% of all lymphomas. 1,2 First identified by Oberling in 1928, PBL is defined by the World Health Organization as lymphoma of a single or multiple skeletal sites that may or may not have regional lymph node involvement. 3 Median age range at the time of diagnosis is between 45 and 60 years old, with a slight male preponderance.…”