Conflict of interest: Nothing to report
Diagnostic Imaging in Hematology
Primary bone lymphoma involving bilateral tibia†
Article first published online: 28 MAY 2012
Copyright © 2012 Wiley Periodicals, Inc.
American Journal of Hematology
Volume 87, Issue 9, pages 924–925, September 2012
How to Cite
Giardino, A. A., Shinagare, A. B., Shinagare, S. A., Dewar, R., Weckstein, D., Mauch, P., Ramaiya, N. H. and Freedman, A. S. (2012), Primary bone lymphoma involving bilateral tibia . Am. J. Hematol., 87: 924–925. doi: 10.1002/ajh.23245
- Issue published online: 23 AUG 2012
- Article first published online: 28 MAY 2012
- Accepted manuscript online: 26 APR 2012 07:43AM EST
- Manuscript Accepted: 18 APR 2012
- Manuscript Revised: 14 APR 2012
- Manuscript Received: 29 MAR 2012
A 77-year-old man with history of Helicobacter pylori-negative gastric mucosa-associated lymphoid tissue (MALT) lymphoma without bone marrow involvement which was treated with involved field radiation was in complete remission for 7 years. He presented in April 2010 with a fall and right knee pain. Magnetic resonance imaging (MRI) in June 2010 showed a meniscal tear and abnormal marrow signal in the tibial plateau. Persistent knee pain prompted biopsy of the proximal right tibia.
Hematoxylin and eosin stain showed a dense infiltrate of intermediate to large-sized lymphocytes with dispersed chromatin and variably distinct nucleoli (Image 1A,B), which, on immunohistochemistry, were positive for CD20 (+) (Image 1C), BCL-2, and BCL-6, but negative for CD5, CD10, CD43, and Cyclin D1. MIB-1 proliferative index was around 40–50% within the larger neoplastic cells (Image 1D). All the findings were consistent with large B cell type non-Hodgkin lymphoma and were not suggestive of transformation of prior indolent gastric marginal zone lymphoma. [18F] fluorodeoxyglucose positron emission tomography (FDG-PET/CT) showed very intense focal uptake in proximal tibia bilaterally (SUVmax 48.3 on right side and 46.4 on left), more extensive on the right side (Image 2A,B). An FDG-avid right popliteal lymph node (SUVmax 4.2) was also noted.
The patient was treated with six cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) chemotherapy completed in April 2011. FDG-PET/CT after three cycles (Image 2C,D) showed near-total resolution of tibial uptake as well as the popliteal lymph node. FDG-PET/CT after completion of treatment showed no evidence of disease (not shown). Patient remains well after 15 months, with no evidence of disease noted on FDG-PET/CT obtained in January 2012.
Primary lymphoma of bone (PBL) is a rare form of non-Hodgkin's lymphoma, first described in 1939 . It represents 3% of all primary bone malignancies, <2% of all adult lymphomas, and 5% of extranodal lymphomas [2–5]. Diffuse large B-cell lymphoma accounts for approximately 80% of cases [3, 4]. PBL occurs in older patients with a slight male preponderance, commonly involves femur, pelvis, humerus, skull, and tibia, and presents with local pain or B symptoms [3, 6–8]. PBL is defined as lymphomatous involvement of a single osseous site with or without regional lymph node involvement, or involvement of multiple bones without visceral or nodal involvement [3, 6].
Plain radiographs and CT usually show a lytic metaphyseal lesion with periosteal reaction; however, with limited sensitivity and specificity [9, 10]. CT may also appear normal, or rarely, can show a sclerotic area . MRI is the preferred modality on which PBL appears isointense or hypointense on T1-weighted images, isointense to slightly hyperintense on T2-weighted images, and shows moderate homogeneous enhancement . The differential considerations include metastatic carcinoma, Ewing's sarcoma, osteosarcoma, and chronic ostoemyelitis. The imaging appearance is nonspecific, and pathologic diagnosis is required in all cases.
CHOP-based chemotherapy has become the standard of care, with or without radiation treatment . The reported 5- year and 10-year survival rates of PBL are 58–62% and 41–45%, respectively [3, 8]. Progression free survival (PFS) has improved with the addition of rituximab (88% 3-year PFS for CHOP plus rituximab versus 52% for CHOP only) .
In summary, we present a case of PBL involving tibia, which was treated with R-CHOP, and patient is in remission at 15 months. Although rare, this case demonstrates the typical features of PBL including age, gender predilection, a common anatomic location, typical imaging features, as well as treatment and outcome. The remote history of gastric MALT lymphoma did not dissuade the necessity for biopsy of the bone abnormality.
- 1Primary reticulum cell sarcoma of bone. Surg Gynecol Obstet. 1939; 68: 45–53., .