Presentation
History
The most common presentation of primary lymphoma of bone (PLB; also referred to as reticulum cell sarcoma, malignant lymphoma of bone, or osteolymphoma) consists of several months of bone pain and the appearance of a soft-tissue mass. Constitutional symptoms (B symptoms), such as weight loss, fever, and night sweats, are present in fewer than 10% of patients with PLB. Hypercalcemia is seen in some pediatric patients and has been associated with a poorer prognosis. PLB may be rarely seen in patients with AIDS, immunosuppression, and Paget disease of bone.
The severity of bone pain should be ascertained, particularly with weightbearing. Activity-related pain that subsides with rest or worsening pain despite adjuvant chemoradiation may be indicative of structural insufficiency and the need for prophylactic fixation.
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Physical Examination
Patients with PLB may present with a prominent mass, even with minimal radiographic changes. All lymph nodes should be palpated. Nodal involvement is rare with primary soft-tissue or bone sarcomas, but is an important factor in managing lymphoma. (See the image below.)
Clinical photo of a left shoulder shows a prominence in the midportion of the left clavicle. This 45-year-old man was suffering from local pain and tenderness but had no history of prior trauma.
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Total skeleton technetium-99m (99mTc) nuclear medicine scan reveals an isolated increased uptake in the left proximal femur at the site of this patient's bone lymphoma.
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Total skeleton technetium-99m (99mTc) nuclear medicine scan shows increased uptake in the left acromion, the site of bony involvement by lymphoma in this patient. The initial differential diagnosis suggested metastatic disease to bone in addition to multiple myeloma and lymphoma, in that order.
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Coronal, T1-weighted magnetic resonance imaging (MRI) scan of the left shoulder reveals the replacement of the left acromion by a low-signal process extending into the surrounding soft tissue.
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Coronal, T2-weighted magnetic resonance imaging (MRI) scan of the left shoulder reveals a high-signal process involving the left acromion and extending to the surrounding soft tissue. The MRI scan's features are suggestive only of a very high cellularity fluid-containing process, but they are nonspecific. Biopsy is required for a specific diagnosis.
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Sections of the biopsy show a diffuse infiltrate of atypical large lymphoid cells with vesicular nuclei, small nucleoli, and moderate cytoplasm. Small reactive lymphocytes are in the background.
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An immunohistochemical stain using an antibody directed against CD20 (B-cell marker) shows strong positivity in the large lymphoid cells. This is an example of a diffuse large B-cell lymphoma.
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A woman who is in the early part of her fifth decade presents with progressive left thigh pain and a limp. An anteroposterior radiograph of her left proximal femur reveals a lytic destructive process involving the subtrochanteric region, with medical cortical erosion, soft-tissue extension, and an associated lesser trochanteric avulsion fracture. The proximal femur is the most common site for primary bone lymphoma.
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An elderly woman presents with complaints of left shoulder pain of several months duration. A plain radiograph of the left shoulder (glenoid view) reveals a destructive lytic process eroding the cortical margins of the acromial process.
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Diffuse infiltrate of large lymphoid cells is present, with cleared cytoplasm and hyperchromatic nuclei. Admixed small, reactive lymphocytes also are noted.
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Immunohistochemical stain using an antibody against CD20 is positive in the large cells; this is a diffuse large B-cell lymphoma.
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Clinical photo of a left shoulder shows a prominence in the midportion of the left clavicle. This 45-year-old man was suffering from local pain and tenderness but had no history of prior trauma.
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Technetium-99m (99mTc) total skeleton nuclear medicine scan shows increased uptake in the midportion of the left clavicle, an area corresponding to the clinical site of bone enlargement.
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Anteroposterior radiograph of the left clavicle reveals a mixed lytic and sclerotic destructive process within the midportion of the bone, with indistinct, permeative borders.
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Another example of a diffuse large cell lymphoma. In addition to the large lymphoid cells with moderate cytoplasm, a few cells with lobate nuclei also are seen. Such cells are often observed in large cell lymphoma of the bone.
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Plain radiographs in this 12-year-old patient with severely progressive right shoulder pain were interpreted as being normal. At most, they showed the existence of localized osteopenia in the right proximal humerus, but they did not demonstrate the presence of a discrete lesion within the bone. Based on the initial evaluation and plain radiographs, the patient was thought to have referred pain from the cervical region or brachial plexus.
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Coronal, T1-weighted magnetic resonance imaging (MRI) scan of the upper thorax and bilateral shoulders reveals a marrow replacement low-signal process involving the entire right proximal humerus. The corresponding T2-weighted MRI scan showed a high-signal process in this area. This MRI scan was produced after plain radiographs were interpreted as normal and an MRI scan of the cervical spine and brachial plexus revealed the unsuspected findings in the humerus.
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Histologic sections reveal a highly cellular neoplasm composed of cells with a high nucleus-to-cytoplasm ratio, scant cytoplasm, and fine nuclear chromatin. The cells showed immunohistochemical evidence of B-cell lineage and expressed terminal deoxynucleotidyl transferase (TdT), consistent with a precursor B-lymphoblastic lymphoma.
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