Primary Bone Lymphoma Research Articles

Clinical Characteristics and Disease Outcomes in Primary Bone Lymphoma: Retrospective Study

Clinical Characteristics and Disease Outcomes in Primary Bone Lymphoma: Retrospective Study

Radiological Features of Bone Lymphoma on CT and MRI. A Retrospective Monocentric Series of 56 Patients

Bone lymphoma is a rare bone neoplasm consisting of malignant lymphoid cells. It is classified as primary bone lymphoma (PBL) or secondary bone lymphoma (SBL). PBL is a neoplasm producing one or more tumor masses within the bone, with no extra-regional lymph-node involvement or other extranodal lesions (1). It accounts for approximately 7% of all malignant bone tumors (1,2). SBL is a bone lymphoma with systemic disease (1). The diagnosis of bone lymphoma requires histopathologic analysis of a tumoral tissue sample.

PET/MR of pediatric bone tumors: what the radiologist needs to know.

Integrated 2-deoxy-2-[fluorine-18]fluoro-D-glucose (18F-FDG) positron emission tomography (PET)/magnetic resonance (MR) imaging can provide "one stop" local tumor and whole-body staging in one session, thereby streamlining imaging evaluations and avoiding duplicate anesthesia in young children. 18F-FDG PET/MR scans have the benefit of lower radiation, superior soft tissue contrast, and increased patient convenience compared to 18F-FDG PET/computerized tomography scans. This article reviews the 18F-FDG PET/MR imaging technique, reporting requirements, and imaging characteristics of the most common pediatric bone tumors, including osteosarcoma, Ewing sarcoma, primary bone lymphoma, bone and bone marrow metastases, and Langerhans cell histiocytosis.

PRIMARY BONE LYMPHOMA OF FEMUR: A CASE REPORT

Primary Bone Lymphoma (PBL) is a subtype of lymphoma that exclusively affects the skeletal tissue.Primary Bone Diffuse Large B Cell Lymphoma (DLBCL) is the most common pathological type, comprising about 80% of PBL. In this case report, we discuss a patient, a 50-year-old lady who presented with solitary bone lesion, confirmed by biopsy and IHC as DLBCL. She was diagnosed as stage IAE as per WHO staging system. Chemotherapy with R-CHOP for a total of 6 cycles were given followed by IFRT. The patient is under follow up with complete response at present. Being rare case, no randomised study can be taken up for standard treatment protocol. Case to case individualised treatment is done based on stage, histopathological subtype and predictive biomarkers to response. PBL is more common in paediatric population more than the adults.The prevalence of PBL is estimated to be 3-7% amongst all lymphomas in adults.1 WHO classification of soft tissues and bone tumors defined PBL as either single osseous lesions without regional Lymph node involvement , or tumor involvement of multiple osseous sites without associated visceral or Lymph Node disease.2

Nomogram-Based Prediction of Overall and Cancer-Specific Survival in Patients with Primary Bone Diffuse Large B-Cell Lymphoma: A Population-Based Study.

Background Primary bone diffuse large B-cell lymphoma (PD-DLBCL) accounts for more than 80% of primary bone lymphoma. We created two nomograms to predict overall survival (OS) and cancer-specific survival (CSS) in patients with PD-DLBCL for this rare disease. Methods In total, 891 patients diagnosed with PB-DLBCL between 2007 and 2016 were extracted from the Surveillance, Epidemiology, and End Results (SEER) database. Univariate and multivariate Cox analyses were performed to explore independent prognostic factors and create nomograms for OS and CSS. The area under the curve (AUC), the calibration curve, decision curve analysis (DCA), and Kaplan–Meier (K-M) curve analysis were used to evaluate the nomograms. Results Four variables were identified as independent prognostic factors for OS, and three variables were identified as independent prognostic factors for CSS. The receiver operating characteristic (ROC) curves demonstrated the strong discriminatory power of the nomograms. The calibration and DCA curves showed that the nomograms had a satisfactory ability to predict OS and CSS. The K-M curves showed that age, gender, primary site, chemotherapy, and tumor stage affected patient survival. Conclusions In patients with PD-DLBCL, age, race, primary site, and chemotherapy affected OS, while age, race, and chemotherapy affected CSS. The two nomograms created based on the aforementioned variables provided more accurate individual survival predictions for PD-DLBCL patients and can help physicians make appropriate clinical decisions.

Linfoma No-Hodgkin primario de sacro. Presentación de un caso clínico y revisión de la literatura.

Introduction Bone lymphoma is a very rare entity. It constitutes less than 2% of lymphomas and the sacrum is an unusual primary location. Only 11 cases have been described to date. Objective To describe a case of primary sacral lymphoma in a 46-year-old patient after reporting lumbar pain with radiculopathy of one month's evolution. Material and methods A 46-year-old female patient with lumbar pain of one month's evolution of mechanical characteristics with radiculopathy S1 of the right leg. No acute bone injury was observed in the X-rays performed. A CT scan was performed in a private center that reported a lytic lesion of 28 x 97 mm diameter in the right sacral wing with malignant characteristics. MRI is requested, which confirms the lesion with extension to soft tissues. Tumor markers and plasma proteins were not elevated. Biopsy with histopathological analysis reported a diffuse large B-cell lymphoma with positivity for cMYC, BCL2, BLC6, CD20, CD79a, PAX5, MUM1, with a Ki67 index of 75%. Results After diagnosis of primary diffuse large B-cell lymphoma without distant metastasis, chemotherapy treatment was started with 6 cycles of R-CHOP followed by radiotherapy. Conclusion Primary bone lymphoma is a very rare entity, and even more rare at the bone level, but knowledge of this entity is necessary to include it as a differential diagnosis of low back pain with radiculopathy.

Clinical Characteristics and Long-term Prognosis Analysis of Patients with Primary Bone Lymphoma

To analyze the clinical characteristics and long-term prognosis of patients with primary bone lymphoma (PBL). The clinical data of 21 patients with PBL treated in our center from 2005 to 2018 were analyzed retrospectively, the clinical characteristics and the factors affecting prognosis of the patients were analyzed. The median age of all the 21 newly diagnosed PBL patients was 40(12-71) years old. Ostealgia was the initial symptom in most of the patients (19/21,90.5%). 42.9%(9/21) of the patients showed single bone lesion only. 571% (12/21) of the patients showed diffuse large B cell lymphoma. 28.6% (6/21) of the patients showed anaplastic large cell lymphoma and 9.5% (2/21) of the patients showed T cell lymphoblastic lymphoma. All the patients received chemotherapy (CHOP or CHOP like regimen, 33.3% plus rituximab) with or without radiotherapy and/or autologous hematopoietic stem cell transplantation (ASCT). 18 patients achieved clinical remission (including 15 for CR and 3 for PR). The median follow-up time was 48 months. The 5-year overall survival rate and progression-free survival rate of the patients were was 67.5% and 63.7%, respectively. The single factors analysis showed that ASCT was the important prognostic factor of PFS, while the single or multiple bone lesion was the factors affecting OS of the patients. There were no statistical differences with the effects of age, sex, stage, ECOG score, LDH level, B symptoms and radiotherapy for the prognosis of patients. Diffuse large B cell lymphoma is the most common pathological type of PBL. Chemotherapy is the main treatment, which can be combined with radiotherapy and/or ASCT. The ASCT and the number of bone lesion are the factors for long time survival of the patients.

THE SMALLEST PRIMARY BONE LYMPHOMA

Primary lymphoma of bone (PLB) is a rare malignant condition with lymphocytic infiltration of the bone; it accounts for 2–3% of all primary bone tumours in adults and children .Here we report a little girl with isolated PLB of B cell lineage focussing on diagnosis, evaluation and treatment strategy. Our case can help to get acquaintance with PBL,it should be taken into consideration as a different diagnosis for osteolytic lesions of bone.

Spinal Manifestation of Malignant Primary (PLB) and Secondary Bone Lymphoma (SLB).

Manifestation of malignant lymphoma in the spine is rare; there have only been a few cases reported in the literature. Due to its rarity, there is no gold standard for the management of patients suffering from spinal lymphoma manifestations. Methods: We retrospectively reviewed the data for 37 patients (14 female, 23 male) with malignant lymphoma in the spine receiving intervention in our center from March 2006 until June 2020. Neurological impairment, pain, diagnostics, and/or surgical instability were the criteria for surgery in this patient cohort. Otherwise, only CT-guided biopsies were conducted. Analysis of the patient cohort was based on the Karnofsky performance status scale (KPSS), location of the lesion, spinal levels involved, spinal instability neoplastic score (SINS), surgical treatment, histopathological workup, adjuvant therapy, and overall survival. The following surgical procedures were performed: posterior stabilization and decompression in nine patients; decompression and/or tumor debulking in 18 patients; a two-staged procedure with dorsal stabilization and vertebral body replacement in four patients; decompression and biopsy in one patient; a two-stage procedure with kyphoplasty and posterior stabilization for one patient; posterior stabilization without decompression for one patient; a vertebroplasty and cement-augmented posterior stabilization for one patient; and a CT-guided biopsy alone for two patients. Twenty-one patients (56.78%) had ≥1 lesion in the thoracic spine, 10 patients (27.03%) had lesions in the lumbar spine, two patients had lesions in the cervicothoracic junction, two patients had lesions in the thoracolumbar junction, one patient had a lesion in the lumbosacral junction, and one patient had a lesion in the sacrum. The diagnoses of the histopathological workup were diffuse large B-cell lymphoma in 23 (62.16%) cases, indolent lymphoma in 11 (29.74%) cases, anaplastic T-cell lymphoma in one case (2.70%), T-cell lymphoma in one case (2.70%), and Burkitt lymphoma in one (2.70%) case. The median overall survival was 7.2 months (range 0.1–266.7 months). Pre- and postoperative KPSS scores were 70% (IQR 60–80%). Manifestation of malignant lymphomas in the spine is rare. Similar to the approach taken for spine metastases, a surgical intervention in cases of neurological impairment or manifest or potential instability is indicated, followed by chemoimmunotherapy and radiotherapy.

ABCL-006: High Grade Primary Bone Lymphoma with TdT Expression

Context Distinguishing mature B-cell lymphomas from immature or precursor B-cell neoplasms is important since different treatments are usually given. It is known that some mature B-cell neoplasms can have relatively immature morphologic features and present similarly to a leukemia. Expression of TdT in lymphomas that are typically negative for TdT can occur rarely and present a diagnostic challenge. This entity can present de novo or in the setting of a relapsed/refractory lymphoma, usually follicular lymphomas. Primary bone lymphoma, characterized by exclusive bone or marrow involvement, represents less than 2% of all lymphomas in adults. Its frequency is lower in children and young adults. Objective To describe a case of a high-grade primary bone lymphoma with TdT expression. Setting A 35 yo man was admitted for severe bone pain, fever, and night sweats. Laboratory showed no abnormalities; HIV, HCV, and HBV serologies and blood cultures including atypical germs were negative. Magnetic resonance showed disseminated bone lesions predominant in column and pelvis. Bone marrow biopsy showed an infiltration by a CD20, CD 10 BCL2 positive high grade B cell lymphoma. MUM1 and TdT were negative. He initiated treatment with R-DA-EPOCH presenting refractory disease after 3 cycles: PET/CT uptake in both clavicles, humerus, scapula, dorsolumbar raquis, iliac bones and ribs. Max SUV was 20 in the 7th right rib. A new biopsy was done which result was consistent with an infiltration by a B cell neoplasm with 2 different populations: both positive for CD10, PAX5, BCL2 and one positive for TdT and CD20 while the other was CD20 and TdT negative. High-grade lymphoma and areas of a B cell lymphoblastic like neoplasm expressing TdT was the final diagnosis. His treatment was changed to R Hyper-CVAD plus triple intrathecal chemotherapy, and he is currently undergoing that treatment. Consolidation with allogeneic marrow transplant is planned. Summary/Conclusion TdT positive neoplasms diagnostic is challenging. Whether this case represents a composite neoplasm (high grade lymphoma with lymphoblastic leukemia/lymphoma) or if it is a transformation from a previous asymptomatic lymphoma is a matter of debate. Distinguishing mature B-cell lymphomas from immature or precursor B-cell neoplasms is important since different treatments are usually given. It is known that some mature B-cell neoplasms can have relatively immature morphologic features and present similarly to a leukemia. Expression of TdT in lymphomas that are typically negative for TdT can occur rarely and present a diagnostic challenge. This entity can present de novo or in the setting of a relapsed/refractory lymphoma, usually follicular lymphomas. Primary bone lymphoma, characterized by exclusive bone or marrow involvement, represents less than 2% of all lymphomas in adults. Its frequency is lower in children and young adults. To describe a case of a high-grade primary bone lymphoma with TdT expression. A 35 yo man was admitted for severe bone pain, fever, and night sweats. Laboratory showed no abnormalities; HIV, HCV, and HBV serologies and blood cultures including atypical germs were negative. Magnetic resonance showed disseminated bone lesions predominant in column and pelvis. Bone marrow biopsy showed an infiltration by a CD20, CD 10 BCL2 positive high grade B cell lymphoma. MUM1 and TdT were negative. He initiated treatment with R-DA-EPOCH presenting refractory disease after 3 cycles: PET/CT uptake in both clavicles, humerus, scapula, dorsolumbar raquis, iliac bones and ribs. Max SUV was 20 in the 7th right rib. A new biopsy was done which result was consistent with an infiltration by a B cell neoplasm with 2 different populations: both positive for CD10, PAX5, BCL2 and one positive for TdT and CD20 while the other was CD20 and TdT negative. High-grade lymphoma and areas of a B cell lymphoblastic like neoplasm expressing TdT was the final diagnosis. His treatment was changed to R Hyper-CVAD plus triple intrathecal chemotherapy, and he is currently undergoing that treatment. Consolidation with allogeneic marrow transplant is planned. TdT positive neoplasms diagnostic is challenging. Whether this case represents a composite neoplasm (high grade lymphoma with lymphoblastic leukemia/lymphoma) or if it is a transformation from a previous asymptomatic lymphoma is a matter of debate.

Primary non-Hodgkin's lymphoma of the temporal bone

We present a 6-year-old child, hospitalized in MSPI Institute of Mother and Child, Emilian Coţaga Clinic, Pediatric Otorhinolaryngology Department, Chisinau, Republic of Moldova, with primary non-Hodgkin’s lymphoma (NHL) of the temporal bone, with paresis of facial nerve.

Primary Lymphoma of the Distal Femur Presenting as Spontaneous Deep Vein Thrombosis and Knee Monoarthritis: A Case Report.

A 68-year-old man with a history of unprovoked deep vein thrombosis (DVT) presented with recurrent knee effusions after the initiation of anticoagulation. Radiographs showed soft-tissue swelling without a distinct skeletal lesion. Magnetic resonance imaging, however, showed a marrow-replacing process in the distal femur. Subsequent biopsy demonstrated B-cell lymphoma. This is a rare case of a primary lymphoma of bone mimicking synovial disease. It also highlights the fact that an unprovoked DVT may be a harbinger of an undiagnosed underlying malignancy.

Clinical and imaging features of primary bone lymphoma: a pictorial essay

Primary bone lymphoma (PBL) is rare and histologically in most of the cases is non-Hodgkin. Imaging plays a crucial role in early detection, Staging and assessing response to treatment. This pictorial essay presents epidemiological, clinical and imaging features of PBL. Conventional radiographs can appear normal, even in the presence of a large tumour. CT is used primarily for image-guided biopsy. MR imaging is the method of choice for early detection. Positron emission tomography is useful for Staging and reStaging after treatment.

Unexpected Primary Extranodal Marginal Zone Lymphoma of Bone in Amputation and Arthroplasty Specimens.

Amputation due to gangrene and arthroplasty for degenerative joint disease are common orthopedic procedures and are expected to increase as populations age. Histopathologic examination of these specimens can identify unsuspected diseases. We reviewed gangrenous amputations and large joint arthroplasty specimens for diagnosis of unexpected lymphoma, January 2014 to January 2020. Pathology and medical records were reviewed to determine diagnosis, treatment, and outcome. Five cases (0.08%) of unexpected primary extranodal marginal zone lymphoma (MZL) centered in bone were identified in 1,624 amputations for gangrene and 4,163 arthroplasty specimens. The female-to-male distribution was 3:2. Median age was 71 years (range, 62-87). The 3 cases arising in the setting of gangrene involved the first toe phalanges and metatarsals, and the femoral head was involved in all cases of joint disease (2 cases). The bone showed variable (10%-80%) infiltration by dense populations of small lymphoid cells with MZL immunophenotype. One patient died from sepsis 18.5 months after diagnosis; all others are alive with a median follow-up of 27.45 months. Histopathologic examination of nonneoplastic orthopedic specimens identifies unexpected primary bone extranodal MZL in a small percentage of cases. This neoplasm may be the result of chronic antigenic stimulation in some circumstances.

A RARE CASE OF CONCURRENT PRIMARY GASTRIC AND BONE DIFFUSE LARGE B CELL LYMPHOMA.

Primary bone lymphoma (PBL) is an uncommon clinical entity and a rare non-Hodgkin's lymphoma presentation. PBL accounts for less than 5% of malignant bone tumours. Gastric lymphoma (PGL) is also rare cancer, but it is the most common site for extranodal non-Hodgkin lymphoma. We report a rare case of concurrent occurrence of bone lymphoma and gastric lymphoma. The patient presented with a slow-growing swelling around the knee joint for two years. Positron emission tomography incidentally picked up an FDG avid lesion in the fundus of the stomach. Tissue specimens of both the lesions were obtained and conformed to have diffuse large B cell lymphoma on histology and immunochemistry. He is on follow up with the oncology department and being treated with an R-CHOP regimen (rituximab, cyclophosphamide, hydroxydaunorubicin, oncovin (vincristine), and prednisone.

Primary diffuse large B-cell lymphoma of the parietal bone

Primary diffuse large B-cell lymphoma of the parietal bone

Ankle Localization of a Primary Bone Large B-Cell Lymphoma

Plain X-Ray of a primary bone large B-cell lymphoma in an 84-year-old patient (Figure 1 and 2). She consulted for a swelling of the right ankle with functional impotence, in a context of altered general condition for many months. The radiographic appearance was typical, showing a shaft and metaphyseal “moth-eaten” osteolytic lesion, with periosteal reaction and thickening of the opposite soft tissues. The diagnosis of B-cell lymphoma was confirmed with histological examination of tissue samples from percutaneous computed tomography-guided biopsy of the lesion. Primary bone lymphoma is rare, accounting for 5% of malignant bone tumors, and mostly large B-cell lymphomas. Moreover, localization usually predominating at the roots of the axial skeleton, explained by the persistence of red bone marrow. Even if it presents in an unusual site, this case highlights the importance of considering typical imaging characteristics of a lesion.

Les lymphomes non-Hodgkiniens osseux de l’adulte

Two forms of bone lymphomas can be distinguished: the primary bone lymphoma (PBL) and the secondary bone lymphoma (SBL). PBL is a rare disease with a good prognosis. Clinical manifestations and imaging findings are usually non-specific. Patient can present with pain, swelling of affected bone or pathologic fracture. Positron emission tomography-CT scan is a sensitive imaging modality and very useful for staging, restaging, surveillance of recurrence, and monitoring of treatment response of lymphoma. The diagnosis of PBL is often difficult and made after biopsy examination. Most patients have diffuse large B-cell lymphoma. Patients have been treated with radiotherapy, chemotherapy or combination of both. Localized disease, low IPI (International Prognostic Index) and complete remission after initial treatment were associated with a better outcome. Management of late sequelae deserves particular attention. SBL is more common than PBL; this is a disseminated lymphoma with concomitant involvement of the skeleton. We review the clinical, imaging and pathologic features of bone lymphomas; and discuss therapeutic modalities as well as prognosis of these lymphomas in the era of immunochemotherapy.

PO-0918: Early-stage primary bone lymphoma: a single-center experience.

PO-0918: Early-stage primary bone lymphoma: a single-center experience.

DLBCL of Clavicle: Role of FNA in diagnosis of Rare Lesion

Primary bone lymphoma is rare, among which commonly involved site at time of presentation are femur, pelvic bones, tibia and most common type is Large B cell lymphoma which usually occurs in adults. Cases of large B cell lymphoma in clavicle as primary bone lymphoma is extremely rare finding. According to our knowledge this case may be first reported case of world. We are here reporting a case of 62-year female presented as sternoclavicular joint swelling, without fever or pain in local area. Patient was diagnosed on X ray by radiologist, as well as by clinician as, osteosarcoma of right clavicle. Patient came to Department of Cytopathology for FNA for confirmation of X ray finding. On FNA diagnosis of Non-Hodgkin lymphoma was given, and advise for biopsy and immunohistochemistry (IHC) was given for confirmation of diagnosis and to subtype NHL. On IHC Ki67 index was 85%, with diffuse positivity noted for MUM1, CD79a, and CD138 along with cytoplasmic positivity for ALK 1 and patchy positivity for PAX 5. Tumor was negative for BCL6, BCL2, CD20, CD5, CD23, CD10, CD19, CD4, CD8, CD7, CD3. Diagnosis of ALK positive DLBCL was given on biopsy after IHC. Patients with PBL treated with combined modality were found to have a superior outcome, with a significantly better survival (5-year cause-specific survival 95%). So correct and timely pathological diagnosis of DLBCL as PBL is significantly important for prognosis.