Primary (Malignant) Lymphoma of Bone Treatment & Management

Updated: Oct 29, 2018
  • Author: Vincent Y Ng, MD; Chief Editor: Harris Gellman, MD  more...
  • Print
Treatment

Nonoperative Therapy

Radiation therapy for primary lymphoma of bone (PLB; also referred to as reticulum cell sarcoma, malignant lymphoma of bone, or osteolymphoma) provides adequate local control rates; however, when used alone, it results in high rates of systemic failure. The addition of chemotherapy provides superior overall survival and a decreased incidence of relapse. [17, 18]  Multimodal chemoradiation can often even allow bone healing. Only approximately one third of patients with PLB ultimately require surgery for impending pathologic fractures. [8]

Patients should be monitored radiographically and clinically during chemoradiation for bone healing. Those with persistent activity-related pain should be evaluated for surgery before progressing to less restrictive weightbearing precautions.

Chemotherapy typically consists of cyclophosphamide, doxorubicin (Adriamycin), vincristine, and prednisone (CHOP), either with (RCHOP) or without the anti-CD-20 antibody rituximab. Intrathecal methotrexate may be given for central nervous system (CNS) prophylaxis. Radiation therapy of 40-60 Gy, fractionated over several weeks, usually follows chemotherapy. [19, 20, 21]  Refractory cases have been treated with allogeneic bone marrow transplantation. [22]

In children, aggressive chemotherapy alone appears to be as effective as combined-modality therapy. Because radiation therapy in children is associated with an increased incidence of adverse, growth-related consequences, it should be avoided in this population.

Next:

Surgical Management

Surgery is not a primary modality of treatment for PLB, aside from biopsy and treatment of skeletal complications. Before the advent of effective radiation and chemotherapy, some patients were treated with amputation. Indications for the operative involvement of orthopedic surgeons in PLB most commonly fall into one of the following three categories:

  • Diagnosis
  • Treatment of pathologic or impending pathologic fractures
  • Decompression of spinal canal compromise

No clear role for surgical debulking or wide resection currently exists for PLB.

Indications for prophylactic stabilization of impending pathologic fractures

Because lymphomas involving bone may result in pathologic fractures, appropriate steps should be taken to prevent such fractures before they occur.

In most patients, particularly those who are younger and compliant, protected weightbearing and use of a gait-aid devices suffice during chemoradiation and until there is improvement of the radiographic appearance. Prophylactic stabilization may be indicated in selected patients who are either unable or unwilling to take such measures to avoid fracture, or if there is an increase in activity-related pain indicating structural insufficiency and microfractures.

The evaluation for impending pathologic fracture in PLB is similar to that for metastatic bone lesions. The highest-risk lesions are those of a lytic nature that involve more than one half to two thirds of the bone diameter. This is particularly true for lesions that are located in the peritrochanteric region and cause pain with weightbearing. Lesions that have cortical destruction are at higher risk than those that are primarily marrow-replacing.

Prophylactic stabilization typically involves curettage, cementation, and augmentation of the lesion with internal fixation. An adjuvant such as cryotherapy may be used.

Indications for fixation of pathologic fractures

If a pathologic fracture occurs, the surgical decision-making changes. On average, approximately one third of all pathologic fractures from metastases to bone heal after surgical fixation. This rate varies depending on the type of primary malignancy. There are no published rates for PLB, but with the addition of radiation therapy that further hinders bone healing, the surgeon should assume that the fracture will not heal.

Because the survival for PLB is fairly high, the construct chosen should be very durable. Several trauma studies have demonstrated the ability of a statically locked intramedullary nail at least 12 mm in diameter to support early weightbearing of an average adult male, even with comminution or a segmental bone defect. Nevertheless, the bone stock in patients with malignancy may be compromised and it is likely that long-term weightbearing on a nail with no bony support will result in hardware failure.

If the pathologic fracture occurs in an area amenable to bone resection and replacement with a megaprosthesis, this should be strongly considered. Cemented endoprostheses provide immediate stability and early resumption of activity.

Surgery for spinal involvement

Lymphoma can occur in the spine and cause nerve or cord compression. In cases of foraminal or central stenosis from soft-tissue extension of PLB, decompression may be necessary to prevent progressive neurologic deficit. If there is spinal instability either from the decompression or from a lytic lesion, spinal fusion also may be necessary.

Contraindications

Contraindications for surgery are similar to other conditions and are not specific to PLB. Needle biopsies are often adequate and can obviate the need for surgical biopsy. Because radiation and chemotherapy are effective modalities of treatment, a cure can be achieved without surgical intervention.

Previous
Next:

Complications

Complications from PLB and its treatment can include the following:

  • Fracture [23]
  • Fracture nonunion
  • Wound infection
  • Failure of prophylactic fixation
  • Secondary arthritis following collapse of osteonecrotic bone
  • Toxicity of chemotherapy
  • Thrombophlebitis
  • Avascular necrosis (AVN) of bone
  • Secondary malignancy from chemotherapy or radiation
Previous