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Primary Lymphoma of Bone

Primary lymphoma of bone (PLB) is a rare type of cancer starting in bone, it accounts for about 7% of all primary bone tumours. Lymphoma usually starts in the lymph nodes and lymph glands (part of the immune system). PLB, however, starts in the bone. This is distinct from lymphoma which started in the lymph notes and then spread to the bones (bone metastases). Nearly all PLBs are classed as Non-Hodgkin's lymphoma (NHL), but in rare cases they can be Hodgkin's Lymphoma. The peak age of people diagnosed with PLB is in the 50-60 yr age group, the disease is slightly more common in men than in women. Symptoms are usually bone pain and sometimes swelling. The majority of people with PLB are diagnosed with a single localised tumour. PLB has higher survival rates compared to other types of non-Hodgkin's lymphoma.

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Non-Hodgkin's Lymphoma
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    Recent Research Publications

    Kennedy G, Weir P, Johnston K, Elder P
    Surgical Management of Primary Bone Lymphoma of the Hip: A Case Report and Review of the Literature.
    Case Rep Orthop. 2019; 2019:3174768 [PubMed] Free Access to Full Article Related Publications
    Introduction: Primary bone lymphoma (PBL) is a rare bone malignancy which may present with atraumatic pain, swelling, or pathological fracture. Whilst the femur is the most commonly affected site, any bone may be involved. PBL should be distinguished from other bone lesions to determine clinical management.
    Case Report: We report the case of an 89-year-old gentleman who presented to the local emergency department with atraumatic hip pain and inability to weight-bear. Multimodal imaging showed evidence of a tumor involving the proximal femur and adjacent acetabulum with an associated pathological intertrochanteric fracture. Biopsy specimens demonstrated this to be PBL of the diffuse large B-cell subtype. No other disease foci or nodal involvement was identified. The patient underwent proximal femoral replacement and acetabular reconstruction prior to commencing R-Mini-CHOP chemotherapy, during which time he has been permitted to fully weight-bear.
    Conclusion: To our knowledge, this is the first reported case of a patient having PBL with both femoral and acetabular involvements. Due to its infrequent occurrence, evidence remains limited to advise therapeutic guidelines. Our practice concurs with literature suggesting that surgery be reserved for cases of pathological fracture. However, the merits of undergoing surgical fixation prior to chemoradiation treatment have been considered.

    Ben Ayed C, Laabidi S, Said N, et al.
    Primary bone lymphoma: tunisian multicentric retrospective study about 32 cases.
    Tunis Med. 2018; 96(5):269-272 [PubMed] Related Publications
    AIM: To report the epidemiologic, clinical features, treatment modalities and prognosis of primary bone lymphomas (PBL) within a retrospective Tunisian series.
    METHODS: We collected cases of histologically confirmed PBL in 3 medical oncology departments from northern and central Tunisia andwe analyzed their characteristics.
    RESULTS: From January 1990 to July 2014, we collected 32 patients with histologically proven PBL, having a median age of 53 years. They affected mainly the long bones and diagnosed at early stages. 91% of the PBL were large cell B lymphoma. All patients received CHOP or CHOP-likeChemotherapy (CT), associated to Rituximab in the last 9 cases, with 14/32 patients received loco-regional radiotherapy and one patient had a resection-reconstruction surgery. We observed 90% of objective responses after primary CT.With a median follow-up of 38.5 months (1 to 192), the 5-year overall survival OS rate was 63%.18 patients relapsed and 14 remain alive in complete remission.
    CONCLUSION: PBL remains a rare disease lately diagnosed in Tunisia. However, most of the patients had early stages tumors. Furthermore, the efficacy of CT and introduction of Rituximab leads to a high rate of complete/objective responses, improving the survival rate.

    Shen G, Su M, Liu B, Kuang A
    PET/CT Imaging for Solitary Primary Bone Lymphoma of Thoracic Vertebra.
    Clin Nucl Med. 2018; 43(11):857-859 [PubMed] Related Publications
    Primary bone lymphoma without classic nodal lesions or advanced disease may be mistaken for other osseous diseases, especially when involving a single bone site. In this patient, a series of imaging modalities including MRI, bone scan, and F-FDG PET/CT revealed only 1 solitary osseous lytic lesion with abnormal uptake in the third thoracic vertebra, suggesting a primary skeletal malignancy. The subsequent histopathologic examination confirmed the diagnosis of diffuse large B-cell lymphoma. After chemotherapy, he achieved an excellent clinical and metabolic response.

    Hernandez E, Rowan R, Randall M
    A Case of Extranodal Metastasis of Primary Bone Lymphoma in the Lower Extremity.
    J Am Podiatr Med Assoc. 2018; 108(1):52-57 [PubMed] Related Publications
    Primary bone lymphoma is a rare disease, accounting for less than 5% of all extranodal lymphoma. Although the femur is cited as being the most common site, primary bone lymphoma is rare, accounting for less than 1% of all non-Hodgkin's lymphoma. Herein we present a case of diffuse B-cell-type malignant lymphoma manifested as a large soft-tissue mass of the leg, via metastasis of primary non-Hodgkin's lymphoma of the femur, which went untreated. We highlight the advantages of various imaging modalities used throughout the process of diagnosis and treatment because accurate and early diagnosis are essential. This case gives us a unique opportunity to witness the rapid progression of metastasis and an atypical location.

    Galati V, Wortmann F, Stang FH, et al.
    A Rare Manifestation of Primary Bone Lymphoma: Solitary Diffuse Large B-Cell Lymphoma of the Little Finger.
    J Hand Surg Am. 2018; 43(8):779.e1-779.e4 [PubMed] Related Publications
    Solitary primary non-Hodgkin bone lymphoma of the hand is a rare entity with only 3 cases reported in the literature. We report the case of a 77-year-old patient with isolated large B-cell bone lymphoma of the proximal phalanx of the little finger without rheumatoid arthritis or methotrexate treatment. The patient was treated with digital amputation and at 6 months' follow-up showed no relapse or dissemination of the disease.

    Xu Y, Li J, Ouyang J, et al.
    Prognostic relevance of protein expression, clinical factors, and
    Oncotarget. 2017; 8(39):65609-65619 [PubMed] Free Access to Full Article Related Publications
    Primary bone lymphomas (PBLs) are composed of malignant lymphoid cells presenting in osseous sites, without supra-regional lymph node or extranodal involvement. We systematically characterized the immunophenotype and the myeloid differentiation factor 88 (

    Annibali O, Fabbriciani G, Tafuri M, et al.
    Primary bone lymphoma of the talus: a challenging diagnosis.
    Tumori. 2017; 103(Suppl. 1):e62-e65 [PubMed] Related Publications
    PURPOSE: Diagnostic challenges are common in clinical practice and diagnostic or classification criteria for musculoskeletal conditions cannot overshadow clinical skills.
    METHODS: We present the case of a young man who complained of prolonged articular pain and mild swelling of the right ankle in the absence of other remarkable data. Apparently fulfilling the Budapest diagnostic criteria for complex regional pain syndrome, the patient was treated accordingly, but the pain increased over time. Then the patient underwent an additional diagnostic workup including synovial and bone biopsies in 2 separate occasions with the second one demonstrating diffuse lymphoid infiltrate compatible with lymphoma.
    RESULTS: The conclusive diagnosis of primary diffuse large B-cell lymphoma of the talus was made and adequate treatment initiated.
    CONCLUSIONS: The diagnostic difficulties as well as the importance of a multidisciplinary approach for complex cases are highlighted in this report.

    Li X, Xu-Monette ZY, Yi S, et al.
    Primary Bone Lymphoma Exhibits a Favorable Prognosis and Distinct Gene Expression Signatures Resembling Diffuse Large B-Cell Lymphoma Derived From Centrocytes in the Germinal Center.
    Am J Surg Pathol. 2017; 41(10):1309-1321 [PubMed] Related Publications
    Primary bone (PB) diffuse large B-cell lymphoma (DLBCL) is rare and has a favorable prognosis, but the underlying biological mechanisms remain unknown. In this study we analyzed the clinicopathologic features of 160 patients with PB-DLBCL in comparison with 499 nonosseous DLBCL. Compared with patients with nonosseous DLBCL and secondary involvement of bone by DLBCL, PB-DLBCL patients less frequently had elderly age, B-symptoms, elevated serum lactate dehydrogenase levels, and high International Prognostic Index at diagnosis, more frequently had germinal center (GC) subtype (approximately 90%) and complete remission, and had significantly better survival. The 5-year progression-free and overall survival rates of PB-DLBCL patients were 80% and 93%, respectively, superior to both GC B-cell-like (GCB) and activated B cell-like subtypes of DLBCL. Further stratifying nonosseous DLBCL cell-of-origin subtypes by clinical factors showed that PB-DLBCL had similar survival rates as the centrocyte-origin (CC) subtype of DLBCL-GCB classified by the B-cell-associated gene signature algorithm. To better understand the favorable outcome of PB-DLBCL patients, gene expression profiling and microRNA profiling were performed in a small subset of PB-DLBCL. The gene expression profiles of PB-DLBCL resembled those of nonosseous DLBCL-GCB-CC, but were distinct from other DLBCL cell-of-origin especially the centroblast-origin (CB) subtype. Compared with DLBCL-GCB-CB, PB-DLBCL and DLBCL-GCB-CC also had much higher levels of miR-125a-3p, miR-34-3p, and miR-155-5p, and significantly lower levels of miR-17-5p and miR-17-3p. These results demonstrated that PB-DLBCL is clinically distinct, and the cell-of-origin of PB-DLBCL stems from centrocytes in the GC, that are biologically attributed for the favorable prognosis of PB-DLBCL.

    Huan Y, Qi Y, Zhang W, Chu J
    Primary bone lymphoma of radius and tibia: A case report and review of literature.
    Medicine (Baltimore). 2017; 96(15):e6603 [PubMed] Free Access to Full Article Related Publications
    RATIONALE: Primary bone lymphoma (PBL) is a rare malignant entity. There is a better survival of PBL than any other malignant bone tumors and extranodal lymphomas.
    PATIENT CONCERNS: We report a rare case of PBL involving radius and tibia. The patient was a 14-year-old girl with left forearm pain and swelling after trauma. Six months later after the last chemotherapy and radiotherapy, pain and swelling of left knee was presented.
    DIAGNOSES: Radiological imaging revealed a lytic destruction, periosteal reaction, and pathological fracture of radius and tibia with soft tissue mass. Surgical biopsy was performed, and the result of histopathological diagnosis was diffused large B-cell lymphoma (stage IV, group A).
    INTERVENTION: Chemotherapy combined with radiotherapy was applied before curation.
    LESSONS: Due to its uncommon presentation, PBL should be taken into consideration if differential diagnosis from other bone tumors is necessary in clinic.

    Ahmad I, Chufal KS, Goyal N, Bhatt CP
    Case of polyostotic primary bone lymphoma successfully treated with immunochemotherapy and consolidation radiotherapy.
    BMJ Case Rep. 2017; 2017 [PubMed] Free Access to Full Article Related Publications
    A 47-year-old woman presented with symptoms of sharp pain over the left anterior thigh with radiation from the groin to the knee. She subsequently developed numbness in that region and reduced motor strength in extensors of the left knee. Plain radiography of the spine and knee was normal. An MRI of the spine revealed an irregular extramedullary mass with intradural and extradural components, extending from the L3 to L5 vertebrae. She underwent a laminectomy and posterior spinal decompression based on a working diagnosis of nerve sheath tumour. Histopathology revealed a primary bone lymphoma. A positron emission tomography CT (PET-CT) performed as part of the staging workup revealed fluorodeoxyglucose avid lesions in the spine and left femur. She received immunochemotherapy (Rituximab-Cyclophosphamide, Hydroxydaunorubicin, Oncovin and Prednisone) for eight cycles with an interim PET-CT revealing complete response. Subsequently, she received consolidation radiotherapy, 36 Gy in 20 fractions over 4 weeks to both lesions. She is now disease-free on follow-up for the past 1 year.

    Liu Y
    The role of 18F-FDG PET/CT in staging and restaging primary bone lymphoma.
    Nucl Med Commun. 2017; 38(4):319-324 [PubMed] Related Publications
    PURPOSE: Primary bone lymphoma (PBL) is one of the rarest primary bone malignancies. Very limited data are available on the role of fluorine-18-fluorodeoxyglucose (F-FDG) PET/CT in PBL.
    PATIENTS AND METHODS: Overall, 16 relevant patients had F-FDG PET/CT for staging or staging/restaging PBL. The cases with extraskeletal lesions such as lymphadenopathy or with osseous involvements in disseminated lymphoma were excluded on the basis of the definition of PBL. F-FDG PET/CT findings were correlated to pathologic and/or anatomic image information.
    RESULTS: F-FDG PET/CT was positive with markedly increased uptake in all known PBL lesions, with a sensitivity of 100%. In 15 of 16 (93.8%) cases, the bone lesions infiltrated the adjacent soft tissue with visible soft tissue components. Except for known primary bone lesion, F-FDG PET/CT showed additional 24 bone lesions in seven patients, which accounted for 44% of all cases. With maximum standardized uptake value of 2.5 as a cutoff for differentiating residual lymphoma from metabolically inactive disease on restaging scan, the sensitivity, specificity, positive predictive value, and negative predictive value of F-FDG PET/CT for PBL were 100% (4/4), 75% (9/12), 57% (4/7), and 100% (9/9), respectively. The diagnostic accuracy of restaging F-FDG PET/CT was 81% (13/16). Two of three false-positive scans were because of post-therapeutic osteonecrosis.
    CONCLUSION: F-FDG PET/CT is sensitive for showing PBL that is typically highly F-FDG avid and has the ability to depict extraskeletal soft tissue involvement and additional bone lesions on staging. More importantly, F-FDG PET/CT may accurately and reliably evaluate therapeutic response with excellent sensitivity and negative predictive value. However, false-positive F-FDG uptake poses a concern on restaging PET/CT. Post-therapeutic osteonecrosis is a potential source of false-positive findings on F-FDG PET/CT.

    Wang J, Fan S, Liu J, Song B
    A rare case report of primary bone lymphoma and a brief review of the literature.
    Onco Targets Ther. 2016; 9:4923-8 [PubMed] Free Access to Full Article Related Publications
    Primary bone lymphoma is a rare and peculiar extranodal presentation of non-Hodgkin's lymphoma, which threatens human health. It can be defined as a lymphoma that occurs in the bone, consisting of a single bone lesion with or without regional lymphadenopathies, and its underlying causes are largely unknown. In this case report, we describe a male who presented with left-sided distal forearm pain, swelling of 2 months duration, and progressive limited wrist motion for about 1 month. The patient had no significant medical history except diabetes. Magnetic Resonance Imaging demonstrated a sheet-like bone destruction area in the left-sided radius, localized discontinuous bone cortex, and adjacent soft tissue masses. Finally, a bone biopsy examined by histopathological and immunochemical methods confirmed a diagnosis of primary bone diffuse large B-cell lymphoma. Due to the rarity of this disease, the level of evidence supporting some diagnostic and therapeutic decisions remains low, and therefore, the details of the rare case may facilitate treatment of similar diseases and provide insight about this obscure lymphoproliferative malignancy. Also, related recent literature reports of primary bone lymphoma are reviewed.

    Chisholm KM, Ohgami RS, Tan B, et al.
    Primary lymphoma of bone in the pediatric and young adult population.
    Hum Pathol. 2017; 60:1-10 [PubMed] Related Publications
    Primary lymphoma of bone (PLB) accounts for 3% to 7% of primary neoplasms of bone and must be distinguished from more common bone tumors in the pediatric population such as osteosarcoma, Ewing sarcoma, and other small round blue cell tumors. In this study, pathology databases from 4 institutions were queried for PLB in individuals 1 to 21 years old. A total of 54 cases of PLB were identified, including 41 diffuse large B-cell lymphomas (DLBCL, 76%), 8 B-lymphoblastic lymphomas (BLL, 15%), 3 anaplastic large cell lymphomas (ALCL, 6%), and 2 low-grade follicular lymphomas (4%). The male/female ratio was 1.8:1 and median age was 16 years (range, 2-21). Patients with DLBCL were significantly older (P<.001), and patients with ALCL and BLL were significantly younger (P=.050 and P=.008, respectively) when compared with the other patients. Due to necrosis, crush artifact, and/or insufficient material, 30% of cases required multiple biopsies for diagnosis. The femur, tibia, pelvic bones, humerus, and vertebrae were most commonly involved. DLBCL patients had significantly more solitary bone involvement (P=.001), whereas BLL had significantly more polyostotic involvement (P<.001). Of the 37 patients with outcome data, all had no evidence of disease on last follow-up. This largest pediatric series of PLB identifies DLBCL as the most frequent subtype and documents rarer occurrences of BLL, ALCL, and follicular lymphomas. The differential diagnosis of bone neoplasms in pediatric patients, including those with necrosis, should include PLB.

    Ishiguro K, Hayashi T, Aoki Y, et al.
    Other Iatrogenic Immunodeficiency-associated Lymphoproliferative Disorder Presenting as Primary Bone Lymphoma in a Patient with Rheumatoid Arthritis.
    Intern Med. 2016; 55(16):2259-64 [PubMed] Related Publications
    Primary bone lymphoma (PBL) is a rare disorder. We herein present a case of other iatrogenic immunodeficiency-associated lymphoproliferative disorder (OIIA-LPD) presenting as PBL. A 63-year-old woman was diagnosed with rheumatoid arthritis and had been treated with methotrexate for seven years. Two months before admission, she suffered from pain in the limbs. Magnetic resonance imaging revealed multiple irregular lesions in the bones of the limbs, which showed an uptake of (18)F-FDG on positron emission tomography. A biopsy of the right radius revealed diffuse large B-cell lymphoma, leading to the diagnosis of OIIA-LPD. She received rituximab-containing regimens resulting in a complete response.

    Zhang X, Zhu J, Song Y, et al.
    Clinical characterization and outcome of primary bone lymphoma: a retrospective study of 61 Chinese patients.
    Sci Rep. 2016; 6:28834 [PubMed] Free Access to Full Article Related Publications
    Primary bone lymphoma(PBL) is a rare disease. To assess the clinical characteristics, outcome, and prognostic factors of this entity in Chinese population, we retrospectively analyzed 61 PBL patients initially treated in our institution between 1997 and 2014. The median age was 45 years. The most common histological subtype was diffuse large B-cell lymphoma (DLBCL) (55.7%), followed by T-cell lymphoma (18.0%). All patients underwent systemic chemotherapy as initial treatment while 24 patients (39.3%) were additionally treated with radiotherapy. The 5-year overall survival (OS) and the 5-year progression-free survival (PFS) rates of 57 cases with completed follow-up were 52.3% and 40.1%, respectively. In further analysis of the primary bone DLBCL (PB-DLBCL) subgroup, the 5-year OS and PFS rates were 53.0% and 47.0%, and a multivariable analysis revealed that baseline Eastern Cooperative Oncology Group (ECOG) score and response to initial treatment (complete remission versus no complete remission) were independent prognostic factors for both OS and PFS. The proportion of T-cell lymphoma is higher in China than in western populations. High baseline ECOG scores (≥2) and unachieved CR in initial therapy were factors for poor PB-DLBCL prognosis. The role of radiotherapy and rituximab in PLB therapy remains to be confirmed in further investigation.

    Jacobs AJ, Michels R, Stein J, Levin AS
    Socioeconomic and demographic factors contributing to outcomes in patients with primary lymphoma of bone.
    J Bone Oncol. 2015; 4(1):32-6 [PubMed] Free Access to Full Article Related Publications
    BACKGROUND: Primary lymphoma of bone (PLB) is a rare disease, comprising a malignant lymphoid infiltrate of bone. The goal of this study was to identify socioeconomic, demographic, and anatomic factors as prognostic indicators of survival for this disease using the Surveillance, Epidemiology, and End Results (SEER) database.
    METHODS: The SEER database was used to identify a study population of 692 patients diagnosed with PLB in the United States from 1989 to 2003. Survival was analyzed using the Kaplan-Meier method, with effects of potential prognostic factors on survival analyzed using the log-rank test. Multivariable analysis was performed by Cox proportional hazards regression.
    RESULTS: The overall 5-year survival rate was 49.6%, with a 10-year survival rate of 30.2%. Median overall survival was 4.9 years (95% CI: 3.9, 6.1). In multivariable analysis, age (p<0.0001), marital status (p=0.006), and appendicular vs. axial tumor location (p=0.004) were found to be independent predictors of survival.
    CONCLUSIONS: This population-based study of PLB identified age, marital status, and tumor location as independent indicators of prognosis. This finding supports the clinical suspicion that an appendicular tumor location confers a better prognosis than an axial tumor location.

    Wang LJ, Wu HB, Wang M, et al.
    Utility of F-18 FDG PET/CT on the evaluation of primary bone lymphoma.
    Eur J Radiol. 2015; 84(11):2275-9 [PubMed] Related Publications
    PURPOSE: Primary bone lymphoma (PBL) is a rare type of malignant lymphoma. Few data have been reported regarding the utility of F-18 FDG PET/CT in this disease. The aim of this study was to assess the role of F-18 FDG PET/CT in the diagnosis and therapeutic effect evaluation of PBL.
    MATERIALS AND METHODS: A total of 19 consecutive patients with PBL were enrolled. Whole-body PET/CT scan was performed for all patients. The diagnosis of PBL was established by histopathology and immunohistochemistry.
    RESULTS: F-18 FDG PET/CT was positive in 94.7% (18/19) of patients. Uptake of FDG in lesions was intense with SUVmax of 15.14 ± 11.82. Multiple involved lesions were found in 47.4% (9/19) patients, while 52.6% presented with a single involved lesion. Based on the lesions, PET detected 98.9% (87/88) lesions. Among them, 71.6% (63/88) lesions were found to be located in axial skeleton and 28.4% (25/88) in the extremity skeleton. FDG PET/CT also found the lesions infiltrate to the surrounding soft tissue in 84.2% (16/19) patients. On the syn-modality CT, the bone destruction was noted in 43.2% (38/88) of the lesions, of which 50.0% lesions presented as slight change in bone density and 50.0% as severe change. The diagnostic sensitivity of PET was much higher than that of CT (98.9% vs. 43.2%, P=0.000). PET/CT was performed for evaluation of treatment response in 13 patients. In 12 patients with complete response(CR), PET/CT found the 25 lesions were F-18 FDG fully resoluted after treatment, however, bone destruction was still presented in 72.0% (18/25) lesions.
    CONCLUSIONS: The present study suggests that F-18 FDG PET/CT was a sensitive imaging modality for diagnosis and treatment response evaluation of PBL.

    Inklab M, Steingart RH, Freeman JK
    Primary lymphoma of bone presenting as spindle cell neoplasm of the vertebral body: a case report and review of the literature.
    Case Rep Hematol. 2015; 2015:518307 [PubMed] Free Access to Full Article Related Publications
    Spindle cell variant of lymphoma is a very rare but known disease entity that can mimic a sarcoma. Diagnosis can be even more challenging if the only site of the disease is in the bone. We report a case of primary lymphoma of bone with spindle cell morphology which was successfully treated with a combination of surgery, chemotherapy, and radiotherapy.

    Hayase E, Kurosawa M, Suzuki H, et al.
    Primary Bone Lymphoma: A Clinical Analysis of 17 Patients in a Single Institution.
    Acta Haematol. 2015; 134(2):80-5 [PubMed] Related Publications
    Primary bone lymphoma (PBL) comprises less than 1% of all malignant lymphomas. Because few studies of PBL have been conducted in Japan, the characteristics of Japanese patients with PBL have not been fully elucidated. We retrospectively analyzed 17 patients diagnosed with PBL at our institution between 2001 and 2011. Median patient age was 60 years. Eleven patients had diffuse large B-cell lymphoma and 2 patients had T-cell lymphoma histology. The spine was the most frequently involved site at the time of presentation. There were 11 patients with stage IV disease and 11 patients with high or high-intermediate risk according to the International Prognostic Index (IPI). Thirteen patients achieved complete response (CR) after initial treatment. At a median follow-up of 31 months, the 3-year overall survival (OS) and progression free survival were 63.5 and 49.9%, respectively. Localized disease, low or low-intermediate IPI, and CR after initial treatment were associated with a good outcome in patients with PBL and significantly associated with a better OS. Spine involvement and T/NK-cell phenotype are more frequent in Japanese than in Caucasian patients with PBL.

    Ibrahim I, Haughom BD, Fillingham YA, et al.
    Primary Lymphoma of Bone Complicating Total Knee Arthroplasty: An Unexpected Mode of Prosthesis Failure: A Case Report.
    JBJS Case Connect. 2015; 5(2):e34-e5 [PubMed] Related Publications
    CASE: Primary lymphoma of bone is a rare musculoskeletal malignancy that typically arises in long bones. Few cases of bone lymphoma occurring alongside orthopaedic implants have been reported in the literature. We describe the complex case of a seventy-eight-year-old woman who developed diffuse large B-cell lymphoma of the proximal part of the tibia five years after a successful total knee arthroplasty.
    CONCLUSION: Several studies have suggested a link between orthopaedic implants and genomic instability, although true carcinogenesis remains questionable. Lymphoma complicating joint arthroplasty poses a unique problem for orthopaedic physicians due to diagnostic challenges and the unique implications of disease and treatment-related complications.

    Jamshidi K, Jabalameli M, Hoseini MG, Bagherifard A
    Stage IE Primary Bone Lymphoma:Limb Salvage for Local Recurrence.
    Arch Bone Jt Surg. 2015; 3(1):39-44 [PubMed] Free Access to Full Article Related Publications
    BACKGROUND: Primary bone lymphoma or non-Hodgkin lymphoma of bone is a rare disease. There are only a few case series of stage IE of this condition in medical literature. The aim of this study is to determine the rate of survival for stage IE after combined modality treatment, the rate of local recurrence, and the results of limb salvage in cases of local recurrence.
    METHODS: We collected data from 61 patients with histologically confirmed PBL treated at the Musculoskeletal Oncology Department of our hospital from 2000 to 2010. Retrospective evaluation included demographics, symptoms, tumor locations, outcomes of surgical treatment for local recurrence and survival rates.
    RESULTS: All patients received Combined Modality Therapy. Overall,five year survival was 89% and five year disease free survival rate was 78%. Local recurrence occurred in 6 patients during follow up period, which was treated surgically by wide excision and reconstruction. The mean follow-up for the local recurrence group was 36(24-54) months and mortality rate in this group was 17%.
    CONCLUSIONS: Combined Modality Therapy for stage IE primary bone lymphomaresults in good survival rate. In case of local recurrence, wide excision and reconstruction improves the outcomes.

    Fox MG, Marti JK, Bachmann KR, et al.
    Epiphyseal presentation of non-Hodgkin's lymphoma of bone in two pediatric patients--one with primary lymphoma of bone.
    Skeletal Radiol. 2015; 44(4):587-95 [PubMed] Related Publications
    We report two children with lymphoma of bone centered in the distal femoral epiphysis who presented with knee pain. Radiographs, magnetic resonance imaging (MRI) and computed tomography (CT) were performed on both patients prior to biopsy. Following biopsy, both patients had fluorodeoxyglucose ((18) F-FDG) positron emission tomography/CT (PET/CT) and whole-body technetium-99m (Tc-99m) scintigraphy performed for staging. One patient met the criteria for primary lymphoma of bone. One patient did not meet the criteria for primary lymphoma of bone because of PET uptake in a popliteal, external iliac and possibly lower abdominal node. Both patients responded well to chemotherapy and are disease free more than 7 years after diagnosis. While an epiphyseal presentation of lymphoma of bone is rare, the efficacy of treatment and the compromised outcome associated with diffuse spread of the disease make early recognition by clinicians important. We present these two cases to increase awareness of the disease and to have clinicians consider it in the differential diagnosis of adolescent epiphyseal lesions.

    Zhou HY, Gao F, Bu B, et al.
    Primary bone lymphoma: A case report and review of the literature.
    Oncol Lett. 2014; 8(4):1551-1556 [PubMed] Free Access to Full Article Related Publications
    Primary lymphoma of the bone (PLB) primarily arising from the medullary cavity is an extremely rare entity, with only retrospective studies and sporadic cases reported in the literature. The current study presents one case of PLB treated with chemotherapy and radiotherapy, and a review of the literature to elucidate the optimal treatment of PLB. A 73-year-old female presented with pain in the left hip that had persisted for two months. Plain X-ray and magnetic resonance imaging of the left hip showed lytic areas involving the left innominatum. Technetium-99m radionuclide imaging showed increased tracer uptake in the ilium, acetabulum and ischium. An

    Tachibana A, Robinson RJ, Patel CN
    18F-FDG PET/CT demonstrating primary bone lymphoma of the extremities mimicking an inflammatory peripheral arthropathy.
    Clin Nucl Med. 2015; 40(2):169-71 [PubMed] Related Publications
    A 66-year-old woman presents with progressive bilateral swelling of her fingers, elbows, and toes. Initially thought to represent an inflammatory peripheral arthropathy, the patient underwent rheumatology review, but subsequent tissue biopsy confirmed diffuse large B-cell lymphoma. Results of bone marrow biopsy and staging CT were negative. F-FDG PET/CT demonstrates FDG-avid bony and adjacent soft tissue disease limited to the extremities with an excellent metabolic response to primary chemotherapy. This is a rare case of primary bone lymphoma limited to the extremities mimicking an inflammatory peripheral arthropathy.

    Guzik G
    Primary bone lymphoma - experience of oncological orthopaedics department in Brzozów.
    Ortop Traumatol Rehabil. 2014 May-Jun; 16(3):327-38 [PubMed] Related Publications
    BACKGROUND: Primary bone lymphomas are very rare. The diagnosis is based on a histopathological examination and imaging studies where other disease foci should be excluded. The lymphomas are most often found in the axial skeleton, pelvis, femur and humerus. The tumour causes extensive lytic bone destruction with soft tissue infiltration. Surgical treatment is currently not used routinely. The aim of this study was to present five clinical cases and their most characteristic imaging features.
    MATERIAL AND METHODS: Over the last three years, 1327 patients with bone tumours have been hospitalized in the Oncological Orthopaedics Department in Brzozów, and most of these tumours (1184) were metastatic cancers. A total of 57 primary malignant bone tumours were diagnosed, of which five fulfilled the diagnostic criteria of primary bone lymphomas. Patient age and tumour sites varied. All the tumours were large, infiltrated soft tissues and caused osteolysis. No periosteal reactions or sequesters were observed. Two patients were treated surgically by radical resection and implantation of a prosthesis. The indication for surgery was tumour location enabling R0 resection. All patients received chemotherapy and radiotherapy. They have been followed up at the haematological and orthopaedic clinics. There is currently no evidence of generalized disease.
    RESULTS: The most characteristic feature of bone lymphomas is extensive osteolysis with soft tissue infiltration. The absence of false capsules helps distinguish lymphomas from sarcomas. No bone sequesters or osteosclerotic lesions described in the literature were observed in imaging examinations at our centre. Discussion. Diagnosis of a primary bone lymphoma is difficult as the tumour often resembles metastatic lesions. Secondary bone involvement is particularly difficult to differentiate from multifocal lymphoma. The Coley criteria are useful in diagnosis. If radical resection is possible, surgical treatment significantly improves prognosis. Radiotherapy has been associated with frequent recurrences of generalized disease and it is postulated that it should only be employed in palliative patients.
    CONCLUSIONS: 1. Primary bone lymphomas are rare tumours. 2. Imaging studies characteristically show extensive lytic bone destruction with soft tissue infiltration. 3. No sclerotic lesions or periosteal reactions were observed. 4. Surgical treatment should be performed when radical tumour resection is possible.

    Cıraklı A, Elli M, Dabak N, et al.
    Evaluation of primary bone lymphoma and the importance of positron emission tomography.
    Acta Orthop Traumatol Turc. 2014; 48(3):371-8 [PubMed] Related Publications
    Primary lymphoma of the bone is an extremely rare tumor in the form of non-Hodgkin lymphoma or Hodgkin lymphoma. The majority of primary bone lymphomas are non-Hodgkin lymphoma, of which the most common subtype is diffuse large cell lymphoma. Patients can present with pain, swelling or pathologic fracture. Definitive diagnosis is made after biopsy examination. Treatment consists of chemotherapy, radiotherapy and surgery. We report 3 male patients who presented with pain and swelling. Involvement was in the distal femur, proximal fibula and iliac crest in all patients. Patients were diagnosed with non-Hodgkin lymphoma in biopsy examination and underwent chemotherapy. The patient with distal femoral involvement underwent distal femoral resection prosthesis. Another patient with involvement of the fibular head experienced foot drop and delayed wound healing. Fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography revealed complete response to the treatment. Patients are in remission and continue schooling.

    DeLauro NM, Sharma S, Shah N, Ahmed I
    Incidental pedal manifestation of primary bone lymphoma: a case report.
    J Am Podiatr Med Assoc. 2014; 104(3):291-4 [PubMed] Related Publications
    Primary lymphoma of bone (PLB) is an uncommon entity and is extremely rare in the foot and ankle. In this case, PLB was identified from the bone specimen after a bunionectomy of the first and fifth metatarsals. The diagnosis was confirmed with pathologic analysis, genetic karyotyping, positron emission and computed tomography scans, and fluorescence in situ hybridization (FISH). We felt that reporting this case was essential due to the rarity of its pedal occurrence and the lack of preoperative signs or symptoms.

    Schmidt-Tanguy A, Houot R, Lissandre S, et al.
    Results of a Prospective Study of High-Dose or Conventional Anthracycline-Cyclophosphamide Regimen Plus Radiotherapy for Localized Adult Non-Hodgkin's Primary Bone Lymphoma.
    Adv Hematol. 2014; 2014:512508 [PubMed] Free Access to Full Article Related Publications
    Background. Primary bone lymphoma (PBL) is a rare entity that has only been reviewed in one prospective and small retrospective studies, from which it is difficult to establish treatment guidelines. We prospectively evaluated high-dose or conventional anthracycline-cyclophosphamide dose and radiotherapy for PBL. Patients and Methods. The GOELAMS prospective multicenter study (1986-1998) enrolled adults with localized high-grade PBL according to age and performance status (PS). Patients <60 years received a high-dose CHOP regimen (VCAP) and those ≥60 years a conventional anthracycline-cyclophosphamide regimen (VCEP-bleomycin); all received intrathecal chemotherapy and local radiotherapy. Results. Among the 26 patients included (VCAP: 19; VCEP-bleomycin: 7), 39% had poor PS ≥2. With a median follow-up of 8 years, overall survival, event-free survival, and relapse-free survival were 64%, 62%, and 65%, respectively, with no significant difference between treatment groups. Poor PS was significantly associated with shorter OS and EFS. Conclusions. Our results confirm the efficacy of our age-based therapeutic strategy. High-doses anthracycline-cyclophosphamide did not improve the outcome. VCEP-bleomycin is effective and well tolerated for old patients. The intensification must be considered for patients with PS ≥2, a poor prognostic factor.

    Liu M, Liu B, Han F, Song Y
    Primary bone lymphoma of the left radius: a case report and related literature review.
    Eur J Med Res. 2014; 19:19 [PubMed] Free Access to Full Article Related Publications
    Primary bone lymphoma (PBL) is a rare but distinct clinicopathological disease. Because it is not common, the optimal treatment strategy has not been established. Here, we present a patient with PBL of the left radius and review the related literature. We focus on the standard treatment for PBL. Many aspects such as rehabilitation, local control and overall survival need to be considered. Studies on this disease should be carried out to clarify the optimal treatment in the future.

    Wu H, Zhang L, Shao H, et al.
    Prognostic significance of soft tissue extension, international prognostic index, and multifocality in primary bone lymphoma: a single institutional experience.
    Br J Haematol. 2014; 166(1):60-8 [PubMed] Related Publications
    Primary bone lymphoma (PBL) is a rare disease. The literature is inconsistent in regard to definition, stage and prognostic factors. We examined the PBL cases seen at the Moffitt Cancer Center between 1998 and 2013 using the 2013 World Health Organization criteria for bone/soft tissue tumours. Seventy PBL patients were included, of whom 53 (75.7%) patients were histologically classified as primary bone diffuse large B-cell lymphoma (PB-DLBCL). Femur was the most commonly involved site in PBLs with unifocal bone lesions, whereas PBLs with multifocal bone lesions most frequently presented with spine disease. Further analysis of the PB-DLBCL subgroup showed that these patients had 3- and 5-year progression-free survival (PFS) of 61.2% and 46.9%, respectively and 5- and 10-year overall survival (OS) of 81.1% and 74.7%, respectively. Multivariate analysis identified soft tissue extension and International Prognostic Index (IPI) score as the most important unfavourable prognostic factors for both PFS and OS. Multifocality was also highly significantly associated with a worse PFS (P = 0.002) and OS (P < 0.001), although it was not identified in multivariate analysis due to its incorporation into the IPI. The results warrant further investigation regarding whether PBL with multifocal bone lesions could be considered as a systemic and more aggressive disease rather than a conventional PBL.

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