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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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Recent Research Publications
Non-Hodgkin's Lymphoma
Bone Cancers

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Information for Health Professionals / Researchers (5 links)

Recent Research Publications

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] 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; [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.

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 (18)F-fluorodeoxyglucose-positron emission tomography-computed tomography (FDG-PET-CT) scan showed high FDG uptake. A fine-needle aspiration biopsy of the lesion was performed, and histopathological and immunohistochemical examination confirmed a diagnosis of B-cell lymphoma. The patient received radiation therapy followed by six cycles of CHOP regimen (1,000 mg cyclophosphamide, 80 mg epirubicine and 2 mg vincristine on day one, and 100 mg prednisone on days one to five, every three weeks) and achieved a complete response, as confirmed by FDG-PET-CT. At present, the patient is in a good condition. This case is noteworthy, as it is a well-documented case in which the patient received successful treatment. This case demonstrates that PLB has an improved prognosis compared with primary lymphoma of other sites; however, combined therapy may further improve the patient outcome.

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.

Kreutz J, Khamis J, Bauduin E, et al.
Primary bone lymphoma in a 10-year-old boy.
JBR-BTR. 2013 Nov-Dec; 96(6):381-2 [PubMed] Related Publications
Primary bone lymphoma has been defined as a solitary lesion in bone, without concomitant involvement of the extra osseous hematopoietic system, with no evidence of extra osseous disease within 6 months of the onset of symptoms. The vast majority of cases are of the large B-cell non-Hodgkin type. They are rare bone tumor. Distinguishing primary bone lymphoma from other bone tumors is important because the former has a better response to therapy and a better prognosis.

Messina C, Ferreri AJ, Govi S, et al.
Clinical features, management and prognosis of multifocal primary bone lymphoma: a retrospective study of the international extranodal lymphoma study group (the IELSG 14 study).
Br J Haematol. 2014; 164(6):834-40 [PubMed] Related Publications
'Multifocal bone lymphoma' or 'polyostotic lymphoma' is a neoplasm with exclusive multifocal involvement of the skeleton, without affecting lymph nodes or other soft tissues. Knowledge on this uncommon condition is limited because the related literature is sparse and fragmentary. We reviewed cases of multifocal bone diffuse large B-cell lymphoma (MB-DLBCL) registered in a clinico-pathological database of the International Extranodal Lymphoma Study Group that includes 499 cases of bone lymphoma. Clinical features, management and prognosis of 37 MB-DLBCL patients and 63 'controls' (stage-IV DLBCL and skeletal involvement) were analysed. Presentation and treatment of MB-DLBCL and controls were identical. At a median follow-up of 52 months (10-189), MB-DLBCL patients exhibited a significantly better response rate (92% vs. 65%; P = 0·002), progression-free survival (5-year: 56 ± 9% vs. 34 ± 6%; P = 0·003) and overall survival (5-year: 74 ± 8% vs. 36 ± 7%; P = 0·002). Among MB-DLBCL patients, the use of post-chemo radiotherapy was associated with better overall survival (5-year: 83 ± 12% vs. 55 ± 16%; P = 0·003). Two MB-DLBCL patients (5·4%) with spine and skull involvement experienced central nervous system (CNS) relapse. Thus, MB-DLBCL patients exhibit a significantly better prognosis compared to patients with advanced-stage DLBCL, and should be treated with conventional anthracycline-based chemotherapy, keeping intensified treatment for relapsing cases, considering involved-field radiotherapy, and CNS prophylaxis in high-risk patients.

Dar SH, Wazir HS, Dar IH, Singh JB
Primary bone lymphoma with multiple vertebral involvement.
J Cancer Res Ther. 2013 Jul-Sep; 9(3):487-9 [PubMed] Related Publications
A 20-year-old student presented with 2 months history of fever and night sweats, 15 days history of low backache, progressive weakness of both limbs of 7 days duration, and urinary retention for last 24 h. Examination revealed a sensory level at D 10 dermatome and grade two power in both the lower limbs with absent reflexes. Examination of spine revealed a knuckle at T8 level, which was tender on palpation. MRI spine showed erosion of D11-12 and L1 in vertebral bodies with destruction of left pedicles, transverse processes and lamina, and a prominent psoas abscess. Post gadolinium study revealed ring-enhancing lesions in the D11-12 and L1 vertebrae as well as the dural sac. Fine needle aspiration cytology (FNAC) and bone biopsy demonstrated a non-Hodgkin's lymphoma (NHL, large cell high-grade) of the spine (primary), which as per age is the youngest case of NHL ever reported in literature with multiple vertebral involvement.

Matikas A, Briasoulis A, Tzannou I, et al.
Primary bone lymphoma: a retrospective analysis of 22 patients treated in a single tertiary center.
Acta Haematol. 2013; 130(4):291-6 [PubMed] Related Publications
BACKGROUND: Primary bone lymphoma is a rare disease, representing less than 5% of all extra-nodal non-Hodgkin lymphomas.
MATERIALS AND METHODS: We retrospectively searched the database of the lymphoma unit, Hematology/Lymphoma Department, Athens General Hospital 'Evangelismos' for primary bone lymphoma patients. Demographic and clinicopathologic data were collected and overall survival was analyzed. A log-rank test was used in a univariate analysis to identify factors affecting overall survival.
RESULTS: We identified 24 and analyzed data from 22 patients. 12 were male (54.5%) and 10 female (45.4%) and their median age was 55 years (range: 19-83). Most patients had localized disease at the time of diagnosis (n = 19, 86.3%), the most common site was the spine (n = 11, 50%) and the most common histology was diffuse large B-cell lymphoma. 21 patients received chemotherapy as initial therapy and 16 received combined chemoradiation. 81.8% of the patients (n = 18) achieved complete remission. 5-year survival rate was 86.3% and overall survival was found to be affected by the patients' initial response to treatment.
CONCLUSIONS: Primary bone lymphoma is usually associated with a good prognosis. Prospective studies are needed in order to clarify the effect of immunochemotherapy in overall survival.

Scoccianti G, Rigacci L, Puccini B, et al.
Primary lymphoma of bone: outcome and role of surgery.
Int Orthop. 2013; 37(12):2437-42 [PubMed] Free Access to Full Article Related Publications
PURPOSE: Primary bone lymphoma is a rare disease. Little is reported about surgical procedures in these patients. We evaluated a single-centre consecutive series of 21 patients for results, prognostic factors and surgical treatment.
METHODS: Patient ages ranged from 19 to 82 years. The most frequently affected site was the spine (six cases), followed by the ileum, femur and mandible (three cases each). Four patients were treated with chemotherapy and 17 with chemotherapy and radiation therapy. Six patients were affected by a pathological fracture. Surgery was performed in four patients (19%), in two cases before chemotherapy, in one case during chemotherapy and in one case after chemotherapy and radiotherapy. Five patients died within the range of three to 36 months after diagnosis. Average follow-up of the remaining patients was 62.8 (19-145) months.
RESULTS: Overall survival was 74.2% at five years. The only positive prognostic factor was complete remission after chemotherapy. A trend for better survival was present for International Prognostic Index (IPI) score (P = 0.051), under 40 years of age (P = 0.10) and abnormal lactate dehydrogenase (LDH) values (P = 0.10), but it did not reach statistical significance.
CONCLUSIONS: Surgical treatment should be aimed at restoring function and eliminating pain while minimising delays in the chemotherapy schedule. When feasible, postponing surgery until after chemotherapy is advisable.

Mohamed M, Brain T, Sharma S
Multifocal primary bone lymphoma: durable complete remission after R-CHOP chemotherapy.
BMJ Case Rep. 2013; 2013 [PubMed] Free Access to Full Article Related Publications
Primary bone lymphoma (PBL) is a type of non-Hodgkin's lymphoma predominantly affecting the skeletal system. PBL is an extremely rare cancer in adults affecting mainly the axial skeleton. The extent of bone involvement in these patients is variable. Most of the cases reported had single or a few skeletal lesions. We report a patient who had extensive multifocal lymphoma involving the axial skeleton and a very good and durable response to R-CHOP chemotherapy.

Koens L, Heyning FH, Szepesi A, et al.
Nuclear factor-κB activation in primary lymphoma of bone.
Virchows Arch. 2013; 462(3):349-54 [PubMed] Related Publications
Primary lymphoma of bone is a rare type of extranodal diffuse large B-cell lymphoma with a relatively favourable outcome. Recent scientific interest has focused on elucidating the role of nuclear factor-κB pathway in lymphomagenesis and its potential significance as a therapeutic target. In nodal B-cell non-Hodgkin lymphomas, constitutive activation of nuclear factor-κB appears to be involved in tumour cell survival, notably in the non-germinal centre type of diffuse large B-cell lymphoma. We investigated nuclear factor-κB activation via the classical and alternative pathway in primary lymphoma of bone, through immunohistochemical staining for nuclear factor-κB family members on tumour tissues of 50 patients. Nine cases (18 %) showed nuclear staining for p50, and one case showed nuclear co-expression of p52. None of the cases showed nuclear staining for c-Rel. The nuclear staining of p50 suggests that in a minority of primary lymphomas of bone nuclear factor-κB is constitutively activated via the classical pathway. In contrast to other extranodal types of diffuse large B-cell lymphoma, there was a lack of nuclear co-expression of p65, which might suggest activation of a different pathway. Activation of nuclear factor-κB through the alternative pathway does not appear to be significantly involved, as only one case showed significant nuclear expression for p52. Finally, nuclear expression of p50 was neither preferentially detected in non-germinal centre type or germinal centre type primary lymphoma of bone, nor related to poor prognosis. Therefore, in contrast to nodal diffuse large B-cell lymphoma, the nuclear factor-κB pathway does not appear to be an attractive therapeutic target in primary lymphoma of bone.

Kim SY, Shin DY, Lee SS, et al.
Clinical characteristics and outcomes of primary bone lymphoma in Korea.
Korean J Hematol. 2012; 47(3):213-8 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: This study evaluates the effectiveness of immunochemotherapy and radiation therapy in the treatment of patients with primary bone lymphoma (PBL).
METHODS: We retrospectively reviewed the medical records of 33 patients with PBL who were treated at 6 medical centers in Korea from 1992 to 2010. Clinicopathological features and treatment outcomes were analyzed.
RESULTS: The median age of the patients participating in our study was 40 years. The most common sites of involvement were the pelvis (12.36%) and femur (11.33%). CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisolone) or CHOP-like regimens were administered to 20 patients (61%), and R-CHOP (rituximab plus CHOP) was administered to the remaining 13 patients (39%). The overall response rate was 89% (complete response, 76%; partial response, 12%). The overall survival (OS) of patients with solitary bone lesions was longer than that of patients with multiple bone lesions (median OS: not reached vs. 166 months, respectively; P=0.089). Addition of rituximab to CHOP did not significantly affect either OS or progression-free survival (P=0.53 and P=0.23, respectively). Combining radiation therapy with chemotherapy also did not improve the OS or progression-free survival of patients with solitary bone lesions.
CONCLUSION: Conventional cytotoxic chemotherapy remains an effective treatment option for patients with PBL. Additional benefits of supplementing chemotherapy with either rituximab or radiation therapy were not observed in this study. Further investigation is needed to characterize the role of immunochemotherapy in treating patients with PBL.

Mikhaeel NG
Primary bone lymphoma.
Clin Oncol (R Coll Radiol). 2012; 24(5):366-70 [PubMed] Related Publications
Primary bone lymphoma is a distinct clinical entity that accounts for 5% of extra-nodal lymphoma. Most patients have diffuse large B-cell lymphoma and present with bone pain, a mass or both. The involvement could be in a single focus or disseminated. There are no prospective clinical studies in this disease. Patients have been treated with radiotherapy, chemotherapy or a combination. There is a trend towards improved outcome with combined modality treatment and further improvement with the addition of rituximab. Assessment of response may be difficult with current imaging techniques. The prognosis of primary bone lymphoma is generally good. Here, the current evidence for the optimal treatment of primary bone lymphoma is reviewed and questions for future investigation are addressed.

Nasiri MR, Varshoee F, Mohtashami S, et al.
Primary bone lymphoma: a clinicopathological retrospective study of 28 patients in a single institution.
J Res Med Sci. 2011; 16(6):814-20 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: Primary bone lymphoma (PBL) is a rare disease and distinct clinicopathological entity. The optimal treatment strategy is still unclear. Because of rarity of PBL, we report our institute experience in PBL clinicopathological feature and treatment results.
METHODS: 28 patients diagnosed with PBL were referred to Omid Hospital, cancer research center (CRC), between March 2001 and February 2009. Immunophenotype studies on 16 out of 28 pathological blocks were performed. We analyzed disease free survival (DFS) and overall survival (OS) rates.
RESULTS: 14 patients with PBL were analyzed retrospectively. 17 patients (60.7%) were male and 11 (39.3%) were female with a median age of 41 years (range: 11-79). Long bones were the most primarily site of involvement (71%). 26 (93%) patients had diffuse large B cell lymphoma and 2 (7%) had small lymphoblastic lymphoma. One (3%) patient received radiation alone, 18 (66%) cases received combined modality (chemotherapy + radiotherapy) and 8 (30%) received only chemotherapy during their treatment period. The median follow up was 18 months (range: 1-82). Mean DFS was 51 months (range: 37-66). Overall survival (OS) was 54 months (range: 40-68). OS was significantly better in the chemoradiotherapy group compared with other two groups (64 versus 27 months, respectively, p=0.014). DFS was also significantly better in combined modality arm compared with other two groups (64 versus 21 months, respectively, p=0.003).
CONCLUSIONS: In spite of small number of patients reported in this study, combined modality treatment (chemotherapy and radiotherapy) was shown to be useful as an effective treatment strategy in PBL.

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