Primary Bone Lymphoma Research Articles

Clinical features of 11 cases of primary bone lymphoma

目的探讨原发性骨淋巴瘤患者的临床特征、治疗及预后。方法回顾性分析11例原发性骨淋巴瘤患者的临床资料，对其临床特征，诊治过程及预后进行分析总结。结果11例患者中女7例，男4例，中位发病年龄45岁，仅1例患者以无痛性肿块起病，其余均以骨痛为首发症状，主要病理类型为弥漫大B细胞淋巴瘤和间变大细胞淋巴瘤。Ann Arbor临床分期ⅠE期3例，ⅡE期2例，ⅣE期6例。6例患者采取化疗联合放疗，2例行骨关节置换术联合化疗，3例选择单纯化疗。11例患者经初始治疗后5例达完全缓解，4例达部分缓解，2例疾病稳定。中位随访时间为21（6~58）个月，中位无进展生存期为17（5~58）个月。结论原发性骨淋巴瘤诊断时多为晚期，临床表现及影像学检查缺乏特异性，目前治疗主要采取以化疗为主的综合治疗，预后相对较好。

Primary Lymphoma of Bone

Lymphoma of bone is not uncommon, accounting for 7% of primary bone malignancies and 5% of extranodal lymphomatous involvement.1 Osseous lymphoma is divided into four groups based on extent of disease: (1) a solitary osseous lesion, (2) multifocal osseous involvement without soft tissue or nodal involvement, (3) simultaneous osseous and nodal/soft tissue involvement, and (4) osseous involvement presenting more than 6 months after an established diagnosis of lymphoma.1 Primary bone lymphoma is defined by the World Health Organization as a malignant lymphoid neoplasm of bone, either at a single bone site with or without regional lymph node involvement, or at multiple bone sites without visceral or lymph node involvement. The majority of cases are diffuse large B-cell lymphoma, but other subtypes of non-Hodgkin lymphoma also are seen. Primary Hodgkin lymphoma of bone has been reported; however, extranodal manifestations of Hodgkin lymphoma of any type are extremely rare. Primary bone lymphoma is rare in children younger than 10 years of age, as are most primary bone tumors. Primary bone lymphoma usually is seen in the second to eighth decades; most cases, however, are found in young adults (late teens to early 30s), with a male-to-female ratio of about 2:1.

Primary Lymphoma of Bone

Primary Lymphoma of Bone

Primary bone lymphoma (PBL) real world experience of a single cancer hospital. results, prognostic factors, treatment and long term follow up of 12 patients.

e19033 Background: PBL is a very rare condition which constitutes less than 1% of all lymphomas. Due to the uncommon entity of this disease there is a lack of concordance in the characteristics, optimal management, treatment strategies and the associated outcome. Methods: Herein, we retrospectively review 12 consecutive patients initially diagnosed with PBL and also treated at our institution from 2001 to present. Results: The median age of the patients was 49 years old. Eight patients were female and 4 male. The histological type of all 12 patients presented was DLBCL. Ten patients (83%) were presented with Ann Arbor Stage I or II disease. While 2 patients (17%) had stage IV disease, none of them showed bone marrow involvement at their initial diagnosis. The median follow-up after achieving CR was 80 months. Our analysis demonstrated that age less than 47 years, lack of B symptoms, normal LDH level, Stage I or II and female gender were found to be favorable prognostic factors for achieving CR and OS. All 12 patients underwent chemotherapy with most of them (10; 83%) receiving 6 to 8 cycles of the RCHOP regimen. The majority (11; 92%) of them received chemotherapy followed by radiotherapy. After front line chemotherapy followed by radiotherapy most patients (9; 75%) achieved CR. Among the remaining three patients, the one patient died before completing first line chemotherapy due to an infection during chemotherapy-induced neutropenia, the other patient is undergoing autologous stem-cell transplantation due to refractory PBL and the third one achieved CR after he underwent high-dose chemotherapy and autologous stem-cell transplantation due to refractory PBL. Both patients that had refractory disease were male and initially diagnosed with stage IV PBL. The median OS was 100 months. Conclusions: The data from our study supports that most PBL patients have had early stage disease (IE-IIE) on diagnosis which also was the most important favorable prognostic factor. Overall, patients with primary lymphoma involving the bone with DLBCL have an excellent prognosis and only a limited number of patients underwent autologous stem-cell transplantation in order to achieve CR.

The application value of apparent diffusion coefficient in monitoring the response to chemotherapy of primary bone lymphoma

Objective To investigate the application value of apparent diffusion coefficient (ADC) in monitoring the response to chemotherapy of primary bone lymphoma (PBL) . Methods Sixteen consecutive patients with PBL confirmed by pathology were enrolled in this retrospective study. All patients underwent a controlled MRI including a DWI sequence before chemotherapy and after the first chemotherapy session. The ADC values of PBL lesions were measured before and after the first chemotherapy session. All patients were divided into responders and non-responders groups according to the combined response assessment criteria. The difference in ADC values between the responders and non-responders groups before and after chemotherapy and the rate of change of ADC values were compared using Wilcoxon test. The paired t test was used to compare ADC values before and after chemotherapy in each group. Results Twenty-three cases of PBL lesions were diagnosed in 16 patients before the chemotherapy. After the first chemotherapy session, 10 patients (12 lesions) and 6 patients (11 lesions) were categorized into the responder and non-responder group, respectively. The mean pre-treatment ADC value of the responder group and non-responder group were 737.1 (301.7, 928.4) ×10-6mm2/s and 749.2 (624.4, 1 186.5) ×10-6mm2/s, which had no statistically significant difference (Z=1.51, P=0.132). The mean post-treatment ADC value of the responder group and non-responder group were 1 843.0 (721.5, 2 745.8) ×10-6mm2/s and 1 026.3 (382.8, 2 100.8) ×10-6mm2/s, which had no statistically significant difference (Z=-1.75, P=0.079). The post-treatment ADC value of the responder group significantly increased [156.7 (60.9, 227.5) %] while the post-treatment ADC value of the non-responder group slightly increased [31.7 (-44.7, 147.4) %]. The difference in the percentage changes of ADC values in the responder and non-responder groups before and after the treatment was statistically significant (Z=-3.66, P<0.001) . Conclusion The measurement of the percentage changes of ADC values enable a good short-term response evaluation for PBL. Key words: Bone neoplasms; Lymphoma; Antineoplastic combined chemotherapy protocols; Treatment outcome; Magnetic resonance imaging

The role of 18F-FDG PET/CT in staging and restaging primary bone lymphoma

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. 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. 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. 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.

Primary bone lymphoma of radius and tibia

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.

Primary B-cell lymphoma of the pelvic bone in a young patient: Imaging features of a rare case

Primary B-cell lymphoma of the pelvic bone in a young patient: Imaging features of a rare case

Unexpected primary bone lymphoma

Unexpected primary bone lymphoma

Primary Lymphoma of Bone Literature Review

Primary Lymphoma of Bone (PLB) is a very rare neoplasm presenting most commonly with bone pain and swelling or as an incidental finding on imaging. PLB diagnosis requires tissue biopsy and Non-Hodgkin’s lymphoma (NHL) is the commonest sub-type. Disease staging includes thoracic-abdominal-pelvic computed tomography scan (CT), bone marrow biopsy and cerebrospinal fluid cytology. Chemo-immunotherapy with radiotherapy (RT) consolidation is a widely applied treatment strategy for PLB with good outcomes achieved.

Primary Bone Lymphoma Mimicking as Osteomyelitis: An Unusual Presentation

Primary Bone Lymphoma is a rare disorder, even though secondary involvement of bone marrow in systemic Lymphoma is common. Most of Primary Bone Lymphomas are Primary Bone Diffuse Large B-cell Lymphomas. We describe a case of Primary Bone Lymphoma, initially treated as Osteomyelitis of unknown etiology. This case shows the difficulties that can occur while diagnosing this entity. We provide an overview of Clinical, Radiological, Pathological characteristics of these rare tumours along with review of Literature.

Primary Lymphoma of Bone: A case report and brief review of literature

Primary Lymphoma of Bone: A case report and brief review of literature

Primary Bone Burkitt’s like Lymphoma of the Skull Mimicking Meningioma - Case Report and Review of Literature

Primary Bone Lymphoma of the skull is extremely rare and has a variable clinical course and prognosis. We are describing a case of a 26 year old woman who presented with a 2 year history of intermittent scalp swelling and skull pain. Brain MRI showed thickening of the bilateral frontal bones and band thickening of the underlying meninges suggestive of band meningiomatosis. Patient was presumably diagnosed with a meningioma based on radiological findings and underwent resection; however, pathology was consistent with a B-cell lymphoma with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma. A PET CT scan showed sacral bone hypermetabolic lesion which was biopsied and consistent with same lymphoma features. She received chemotherapy with Rituximab and Hyper-CVAD protocol (Table 1) and had a sustained ongoing complete remission 19 months after her initial diagnosis. Several cases of primary lymphoma of the skull or cranial vault have been described in the literature, but only one other case with the same pathology of Burkitt- like lymphoma was found.

Unexpected primary bone lymphoma.

Unexpected primary bone lymphoma.

Long-Term Follow-up of Primary Bone Diffuse Large B-Cell Lymphoma Treated with m NHL-BFM-90

Background: Primary bone lymphoma (PBL) accounts for about 5% of extranodal lymphomas, the most common type are Diffuse Large B-cell Lymphoma (PBL-DLBCL). R-CHOP chemotherapy combined with or no radiation therapy can induce 5-year overall and progression free survival about 63% and 60% for localized-stage and only 41% and 35% for advanced stage and with adverse factors (AF). Due to the rarity of the disease the optimal treatment strategy still remains unknown. Further research is thus needed to increase treatment efficacy for patients with advanced stage PBL-DLBCL and with AF.Aims: To evaluate the efficacy of mNHL-BFM-90 program in adult patients with advanced stage PBL-DLBCL and with AF.Methods: Twenty three previously untreated pts with PBL-DLBCL underwent mNHL-BFM-90 treatment between May 2007 and July 2014; mean age 41 years (range 17-65); age ≥60 years 3 pts (13,6%); M\\F=14\\9. Tumor stage according to the Ann-Arbor classification: stage I (involvement of 1 bone) - 7 pts (30,4%), II (involvement of regional lymph nodes) - 3 pts (13%), IV (multiple bone involvement) - 13 pts (56,6%); stage >I 16 pts (69,5%). All pts with stage I had large-size tumors (>6sm) with soft tissue contact invasion. LDH elevation was observed in 10 pts (43,5%), B-symptoms in 15 pts (65%), large-size tumors - 20 pts (87%). The most common tumor sites were the skull bones, vertebrae, and femur. All patients received treatment according to the mNHL-BFM-90 intensive program This program was modified in the following way: adriamycin was administered on the 3rd day of course AA at a dose 50 mg/m2; methotrexate was administered on the 1st day of course C at a dose 1.0 g/m2 for 12 hours. All patients underwent from 4 to 6 courses. Twelve (52%) pts received rituximab on day 0 prior to each course of therapy. Five pts received radiotherapy, all pts received intrathecal prophylaxis of central nervous system (CNS) involvement.Results. Complete remissions were achieved in 23 pts (100%). Relapses were observed in three patients in 6, 9 and 40 months after completion of treatment. From 2 pts with early relapse, one was diagnosed as «double-expression lymphoma» (MYC expression >70%, BCL2 >60%), both pts proliferation index Ki-67 accounted 100%. All 3 pts received salvage therapy and died from progression of disease.With a median follow-up of 69 months (range 17-110) both progression-free and overall survival of 23 pts constituted 87%. Grades 3, 4 hematological toxicity was observed in all pts. No deaths related to mNHL-BFM-90 treatment occurred. No second malignancies were observed.Conclusion The mNHL-BFM-90 demonstrated high efficacy and acceptable toxicity in patients with advanced-stage PBL-DLBCL and with AF. [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Clinical Retrospective Analysis of 42 Patients with Primary Bone Lymphoma Treated at Maisonneuve-Rosemont Hospital

IntroductionPrimary bone lymphoma (PBL) is an uncommon form of lymphoma. Diffuse large B-cell lymphoma (DLBCL) represents the main histology associated with PBL. Clinicopathological understanding and management of PBL rely only on retrospective series.MethodsCases of DLBCL diagnosed by bone biopsy treated in one referring center between 1993 and 2014 were retrospectively reviewed (Montreal, Canada). Bone biopsies done as part of the bone marrow analysis were excluded. Patients files were analyzed to determine if patient had primary bone lymphoma or systemic lymphoma with bony involvement. We excluded patient with distant lymph node or other extranodal involvement. Data on clinical presentation, staging procedures and treatment management including use of radiotherapy was collected. Staging was performed according to WHO classification of soft tissue tumors (2002). Survival time and time to recurrence were calculated from the date of the first documented treatment until recurrence or death with the Kaplan-Meier method. Prognostic factors of recurrence or death were explored with log-rank tests and Cox proportional hazards models. Competing risks analysis was attempted to isolate deaths from lymphoma and death from other causes. Our study was approved by local research and ethic committees.ResultsWe retrieved 42 cases of PBL with a median age of 63 years (23-83) treated between October 1995 and April 2014. We identified 3/14 (21%) GCB and 11/14 (79%) non-GCB subtypes based on the modified Hans algorithm (Meyer et al., JCO 2011). The most common presenting symptom was pain (88%). 12/42 (33%) patients had an IPI score ≥ 3. We identified 18 (43%) stage I, 11 (26%) stage II and 13 (31%) stage IV. 20/42 (48%) had bulky disease (≥ 10cm). Among the 37 patients treated with curative intent, 36 (97%) received CHOP-based regimen and 23 (62%) received rituximab. 30 of these 37 (81%) patients received additional radiotherapy of which 67% received a dose of radiotherapy between 36-50 Gy. Overall response rate for patients treated with curative intent was 86%. With a median follow up of 64.8 months for the whole cohort, the 5- and 10-year overall survival was 73% and 54%, respectively. The 5- and 10-year progression-free survival was 70% and 49% respectively. Age, LDH, stage (I-II vs IV), ECOG (0-1 vs 2-4), IPI (0-2 vs 3-5), use of radiotherapy or addition of rituximab were associated with differences in survival and progression rates that did not reach statistical significance given the limited number of patients in our cohort and the fact that half of the deaths were attributed to other causes. A larger cohort would be required to demonstrate a benefit with rituximab. Response was based on positron emission tomography-computed tomography (TEP-CT) imaging in 12 patients. There was an insufficient number of patients to evaluate the role of adding radiotherapy in patient complete remission defined by PET-CT.ConclusionOur survival rates reproduce published data from series of PBL (Ramadan et al., Ann Oncol 2007; Bruno Ventre et al., Oncologist 2014), although the benefit of adding rituximab did not reach statistical significance in our cohort. The role of radiotherapy in the era of response defined by PET-CT remains to be defined. DisclosuresFleury:Gilead: Consultancy, Speakers Bureau; Amgen: Consultancy, Speakers Bureau; Roche: Consultancy, Speakers Bureau; Novartis: Consultancy, Speakers Bureau; Lundbeck: Consultancy, Speakers Bureau; Seattle Genetics: Consultancy, Speakers Bureau.

911P - Outcome of primary bone lymphoma: Single center experience

911P - Outcome of primary bone lymphoma: Single center experience

Primary lymphoma of bone in the pediatric and young adult population

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.

Prognosis and staging of primary bone lymphoma: an analysis of 40 patients

Objective To retrospectively analyze the treatment outcomes and prognostic factors for primary bone lymphomas (PBL). Methods Forty patients with PBL who were admitted to our center from 1964 to 2014 were enrolled as subjects. In those patients, 10 were treated with chemotherapy alone, 10 with radiochemotherapy, 10 with postoperative chemotherapy, 9 with postoperative radiochemotherapy, and 1 with surgery alone. The median radiation dose was 36 Gy. The Kaplan-Meier method was used to calculate survival rates. The log-rank test was used for survival difference analysis and univariate prognostic analysis. Results The follow-up rate was 100%. The 3-year sample size was 36. In all patients, the 1-and 3-year overall survival rates were 60% and 42%, respectively, while the 1-and 3-year disease-free survival rates were 45% and 34%, respectively. The univariate analysis showed that no pathological fracture at diagnosis, normal lactate dehydrogenase level, an International Prognostic Index score of ≤1, early clinical stage (stage ⅠE), complete response after initial treatment, no less than 6 cycles of chemotherapy, a radiation dose of ≥40 Gy, no progression outside radiation field after radiotherapy, and grade<3 bone marrow suppression during the treatment were prognostic factors for survival (P=0.027, 0.037, 0.000, 0.016, 0.000, 0.000, 0.022, 0.014, and 0.030). Conclusions The incidence of PBL is low. Comprehensive treatment can achieve satisfactory outcomes. As a PBL staging system, Ann Arbor has limitations. The staging of PBL should be based on local bone destruction and metastasis. Key words: Lymphoma, bone/radiotherapy; Lymphoma, bone/chemotherapy; Lymphoma, bone/radiochemtherapy; Prognosis

Clinical characterization and outcome of primary bone lymphoma: a retrospective study of 61 Chinese patients.

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.