Introduction
Background
A 57-year-old woman with knee pain. Lateral radiograph of the right lower leg reveals a large, permeative, lytic lesion with a moth-eaten appearance in the metadiaphysis of the tibia. Note the cortical destruction, aggressive periosteal reaction, and soft-tissue mass.
A 65-year-old man with right hip pain. Axial computed tomography (CT) scan demonstrates a destructive lesion of the right acetabular roof with multiple small sequestra and soft-tissue extension.
Typical scintigraphic appearance. A 65-year-old woman with right leg pain. Whole-body image from technetium-99m (99mTc) bone scan reveals a solitary area of intensely increased tracer uptake suggestive of Paget disease. An old left rib fracture and degenerative changes in both hands are evident.
Primary lymphoma of bone (PLB) is a rare, malignant, neoplastic disorder of the skeleton. In 1939, it was described as a distinct clinical condition by Parker and Jackson.1 Later that year, Ewing included it among the bone tumors listed in the Bone Sarcoma Registry, under the heading of reticulum cell lymphosarcoma.2 In 1963, the term PLB was introduced by Ivins and Dahlin.3
Pathophysiology
Most (94%) PLB cases result from non–Hodgkin lymphoma. In the past, most authorities considered all cases of Hodgkin disease in bone to be metastatic. Currently, Hodgkin disease is reported as occurring as a primary bone tumor. In one referral series, 6% of PLB cases resulted from Hodgkin disease. A review of Hodgkin-type PLB was provided by Gerbert and colleagues in 2004.4 PLB tumors produce osteoclast-stimulating factors that cause lytic bone destruction.
See also the following related eMedicine topics:
Lymphoma, Non-Hodgkin
Non-Hodgkin Lymphoma
Frequency
United States
In most series, PLB is responsible for approximately 5% of primary malignant bone tumors and 5% of all cases of extranodal non-Hodgkin lymphoma; however, the incidence of all types of lymphoma is increasing in the United States. The Leukemia and Lymphoma Society reported a 73% increase in the number of patients with all types of non-Hodgkin lymphoma from 1973-1991, based on statistics from the National Cancer Institute.5 This increase may in part be the result of an aging population and a rise in the number of immunocompromised patients.
Mortality/Morbidity
A report by Fidias and colleagues indicated that 5- and 10-year survival rates of 91% and 87% (respectively) are achievable with combined modality therapy6 ; in a 2004 study by Barbieri and colleagues, patients who underwent such treatment had an overall survival rate of 88% at 15 years.7 A report from the Cleveland Clinic demonstrated a 100% 5-year survival rate.8Combined modality therapy includes a method of local control, usually radiation therapy, and a systemic treatment, usually combination chemotherapy.9,10 Recurrence at the initial primary site is uncommon. Late, distant soft-tissue metastases to lung, liver, and brain account for most of the mortality and much of the morbidity.
Race
Patients of all races are affected.
Sex
Male-to-female ratio ranges from 1.5-2:1.
Age
PLB has been reported in patients as young as 2 years and as old as 88 years. The incidence of disease is distributed fairly evenly in the second through eighth decades. This disease is rare in children younger than 10 years, as are most primary bone malignancies.11
Presentation
Prolonged pain is the usual clinical symptom. Patients may detect an area of swelling at the site of pain. The diagnostic criteria enumerated by Coley and co-authors in 1950 include the following12 :
- A primary focus in a single bone
- Histologic confirmation
- No evidence at diagnosis of distant soft-tissue or distant lymph node involvement. Regional lymph node involvement at diagnosis is not considered exclusionary using these criteria.
It is now recognized that PLB may involve multiple bones, as long as the other 2 criteria are met. The World Health Organization recognizes the following 4 groups of lymphoma involving bone:
- A single primary bone site with or without regional nodes
- Multiple bone sites but no visceral involvement
- A bone lesion and involvement of multiple visceral or lymph node sites
- Soft-tissue lymphoma, with bone involvement detected by bone biopsy or marrow aspirate
This author would consider only groups 1 and 2 to represent primary lymphoma of bone. Groups 3 and 4 would most likely represent metastatic involvement of bone.
Preferred Examination
After biopsy of the bone lesion confirms the diagnosis of lymphoma, computed tomography (CT) scanning of the chest, abdomen, and pelvis is needed to exclude a primary, soft-tissue origin or distant disease. The CT scan may be combined with fluorodeoxyglucose (FDG) positron emission tomography (PET). Technetium-99m (99m Tc) bone scintigraphy also can be used to look for additional sites of involvement that may be clinically silent.13
Once soft-tissue origin and distant disease are excluded, magnetic resonance imaging (MRI) is the preferred examination to stage the extent of disease within the affected bone.
Differential Diagnoses
Bone Metastases
Ewing Sarcoma
Multiple Myeloma
Osteomyelitis
Other Problems to Be Considered
Other round cell tumors (can mimic appearance of PLB)
Primitive neuroectodermal tumor and primary rhabdomyosarcoma of bone (depending on patient's age)
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References
Parker F, Jackson H. Primary reticulum cell sarcoma of bone. Surg Gynecol Obstet. 1939;68:45-53.
Ewing J. A review of the classification of bone tumors. Surg Gynecol Obstet. 1939;68:971-6.
Ivins JC, Dahlin DC. Malignant lymphoma (reticulum cell sarcoma) of bone. Mayo Clin Proc. 1963;38:375-85.
Gebert C, Hardes J, Ahrens H, et al. Primary multifocal osseous Hodgkin disease: a case report and review of the literature. J Cancer Res Clin Oncol. Dec 15 2004;[Medline].
Howell D. State of the Blood Cancers. Washington, DC: Leukemia Society of America; 1999.
Fidias P, Spiro I, Sobczak ML, et al. Long-term results of combined modality therapy in primary bone lymphomas. Int J Radiat Oncol Biol Phys. Dec 1 1999;45(5):1213-8. [Medline].
Barbieri E, Cammelli S, Mauro F, et al. Primary non-Hodgkin's lymphoma of the bone: treatment and analysis of prognostic factors for Stage I and Stage II. Int J Radiat Oncol Biol Phys. Jul 1 2004;59(3):760-4. [Medline].
Kirsch J, Ilaslan H, Bauer TW, et al. The incidence of imaging findings, and the distribution of skeletal lymphoma in a consecutive patient population seen over 5 years. Skeletal Radiol. Aug 2006;35(8):590-4. [Medline].
Maruyama D, Watanabe T, Beppu Y, et al. Primary bone lymphoma: a new and detailed characterization of 28 patients in a single-institution study. Jpn J Clin Oncol. Mar 2007;37(3):216-23. [Medline]. [Full Text].
Ford DR, Wilson D, Sothi S, et al. Primary bone lymphoma--treatment and outcome. Clin Oncol (R Coll Radiol). Feb 2007;19(1):50-5. [Medline].
Mulligan ME, McRae GA, Murphey MD. Imaging features of primary lymphoma of bone. AJR Am J Roentgenol. Dec 1999;173(6):1691-7. [Medline]. [Full Text].
Coley BL, Higinbotham NL, Groesbeck HP. Primary reticulum-cell sarcoma of bone; summary of 37 cases. Radiology. Nov 1950;55(5):641-58. [Medline].
O'Connor AR, Birchall JD, O'Connor SR, et al. The value of 99mTc-MDP bone scintigraphy in staging primary lymphoma of bone. Nucl Med Commun. Jul 2007;28(7):529-31. [Medline].
Mulligan ME, Kransdorf MJ. Sequestra in primary lymphoma of bone: prevalence and radiologic features. AJR Am J Roentgenol. Jun 1993;160(6):1245-8. [Medline]. [Full Text].
Heyning FH, Kroon HM, Hogendoorn PC, et al. MR imaging characteristics in primary lymphoma of bone with emphasis on non-aggressive appearance. Skeletal Radiol. Oct 2007;36(10):937-44. [Medline].
Melamed JW, Martinez S, Hoffman CJ. Imaging of primary multifocal osseous lymphoma. Skeletal Radiol. Jan 1997;26(1):35-41. [Medline].
Bjersand AJ. Bone involvement pattern in hypervascular lesions. Skeletal Radiol. 1982;9(2):103-8. [Medline].
Ramadan KM, Shenkier T, Sehn LH, et al. A clinicopathological retrospective study of 131 patients with primary bone lymphoma: a population-based study of successively treated cohorts from the British Columbia Cancer Agency. Ann Oncol. Jan 2007;18(1):129-35. [Medline]. [Full Text].
Further Reading
Keywords
primary lymphoma of bone, PLB, primary bone lymphoma, PBL, primary lymphocytic lymphoma, reticulum cell sarcoma, non-Hodgkin lymphoma, non-Hodgkin's lymphoma
