|Year : 2013 | Volume
| Issue : 3 | Page : 487-489
Primary bone lymphoma with multiple vertebral involvement
Showkat Hussain Dar1, Hardeep Singh Wazir1, Ishrat Hussain Dar1, Jang Bahadur Singh2
1 Department of Medicine, Government Medical College, Srinagar, Jammu and Kashmir, India
2 Department of Pathology, Government Medical College, Srinagar, Jammu and Kashmir, India
|Date of Web Publication||8-Oct-2013|
Ishrat Hussain Dar
Department of Medicine Government Medical College, Srinagar-190 010, Jammu and Kashmir
Source of Support: None, Conflict of Interest: None
A 20-year-old student presented with 2 months history of fever and night sweats, 15 days history of low backache, progressive weakness of both limbs of 7 days duration, and urinary retention for last 24 h. Examination revealed a sensory level at D 10 dermatome and grade two power in both the lower limbs with absent reflexes. Examination of spine revealed a knuckle at T8 level, which was tender on palpation. MRI spine showed erosion of D11-12 and L1 in vertebral bodies with destruction of left pedicles, transverse processes and lamina, and a prominent psoas abscess. Post gadolinium study revealed ring-enhancing lesions in the D11-12 and L1 vertebrae as well as the dural sac. Fine needle aspiration cytology (FNAC) and bone biopsy demonstrated a non-Hodgkin's lymphoma (NHL, large cell high-grade) of the spine (primary), which as per age is the youngest case of NHL ever reported in literature with multiple vertebral involvement.
Keywords: Cord compression, involvement, large cell, multiple vertebral, paraparesis, primary non-Hodgkin′s lymphoma, radiotherapy
|How to cite this article:|
Dar SH, Wazir HS, Dar IH, Singh JB. Primary bone lymphoma with multiple vertebral involvement. J Can Res Ther 2013;9:487-9
| > Introduction|| |
Lymphomas are a heterogeneous group of malignancies of B or T cells that usually originate in the lymph nodes, but may originate from any other organ of the body.  Primary lymphoma of bone (PLB) is an extremely rare condition that is usually confused with other primary injuries of the bone. It is characterized by the involvement of one or more bone locations, with or without involvement of regional lymph nodes and viscera. It is found at all ages, being most frequently seen in adult life. Any part of the skeleton can be involved, but a trend exists in favor of bones with persistent bone marrow. PLB constitute about 5% of all extra nodal non-Hodgkin's lymphomas (NHL) and 7% of primary bone tumors.  First described by Oberling in 1928,  it was not until 1939 that Parker and Jackson  described 17 cases of primary bone lymphomas. Spinal cord compression is a rare presentation of NHL occurring in 0.1-3.3% of patients.  A case of primary NHL in a 25-year-old woman involving ten different vertebrae has been reported first time in 2002 by Ebus et al.  The case under study is the youngest and only the second case in literature reported with multiple vertebral involvement.
| > Case Report|| |
A 20-year-old male student presented with low-grade fever and night sweats for last 2 months, low backache for last 15 days, progressive weakness of both limbs of 7 days duration, and urinary retention for last 24 h. He was non-diabetic, nonalcoholic and nonsmoker. His general examination was normal. Chest, cardiovascular (CVS), and abdominal examination was unremarkable. Central nervous system (CNS) examination revealed normal higher mental functions and cranial nerves. Motor system, sensations, and deep tendon reflexes were normal in upper limbs. Motor system examination of lower limbs revealed grade II power in all groups of muscles, sensory system showed impairment of all modalities of sensation below T 10 dermatome. Deep tendon reflexes were absent in the lower limbs. Examination of spine revealed a knuckle at T8 level which was tender on palpation. His complete hemogram, renal and liver functions, and urinalysis were normal. Radiographs of chest and dorsolumbar spine were normal. Magnetic resonance imaging (MRI) of the spine showed erosion of D11-12 and L1 in vertebral bodies with destruction of left pedicles, transverse processes and lamina, and a prominent psoas abscess. Postgadolinium study revealed ring-enhancing lesions in the D11-12 and L1 vertebrae as well as the dural sac [Figure 1] and [Figure 2]. Fine needle aspiration cytology (FNAC) and bone biopsy revealed NHL(large cell high-grade) [Figure 3]. Immunohistochemistry was positive for CD20 and negative for CD3, CD4, and CD56. Bone marrow examination was normal. Computed tomography (CT) of abdomen and chest was normal. A diagnosis of primary lymphoma of multiple vertebral bodies presenting as extradural cord compression was made. Patient has subsequently shifted to the Department of Neurosurgery where surgical decompression and spine stabilization was done. Bone biopsy again revealed features of NHL. Treatment with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) plus etoposide was successful in establishing complete remission. Consolidation with radiotherapy was done and the patient responded to treatment by way of improvement in power and regression in the symptoms and till date was doing well on follow-up.
|Figure 1: MRI of spine showing erosion of D11-12 and L1 in vertebral bodies with destruction of left pedicles, transverse processes and lamina, and a prominent psoas abscess. Post gadolinium study revealed ring-enhancing lesions in the D11-12 and L1 vertebrae as well as the dural sac|
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|Figure 2: MRI of spine showing erosion of D11-12 and L1 in vertebral bodies with destruction of left pedicles, transverse processes and lamina, and a prominent psoas abscess. Post gadolinium study revealed ring-enhancing lesions in the D11-12 and L1 vertebrae as well as the dural sac|
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|Figure 3: Fine needle aspiration cytology (FNAC) and bone biopsy revealed non-Hodgkin's lymphoma (large cell high-grade)|
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| > Discussion|| |
PLB are defined as malignant lymphoid infiltrates within bone with or without arterial invasion or extension into contiguous soft tissue but without concurrent involvement of regional lymph nodes or of distant viscera.  The peak incidence of presentation is in the 5 th decade with a male:female ratio of 1.8:1. Most patients of primary bone lymphomas present with localized disease.  Beal et al.,studied the clinical characteristics of 82 PLB patients and reported the most common sites to be involved as the femur (27%), pelvis (15%), tibia (13%), humerus (12%), spine (9%), mandible (2%), skull (1%), scapula (1%), radius (1%), and ulna (1%).  Ramadan et al., studied 131 cases of PLB and reported that approximately one-third [42/131] presented with disease in the spine and one-third [42/131] with disease in the long bones.  CNS presentation of NHL of bone includes spinal cord compression, leptomeningeal spread or intracerebral mass lesion. Spinal cord compression is commonly caused by extradural disease, either because of an isolated deposit within the spinal cord or by an extension from an adjacent nodal mass or bone involvement. Less commonly NHL may arise subdurally or within the spinal cord.  Spinal cord compression typically presents with back pain, leg numbness and tingling, radicular pain followed by extremity weakness and paresis. ,,, The case under study presented with backache, fever, lower limb paraparesis with impaired sensations below T 10 level.
Patients of NHL of the spine can be investigated by CT/MRI. Especially in patients with a history of lymphoma and neurological signs in absence of abnormalities on the conventional radiographs, the performance of MRI of the spine is strictly indicated.  MRI is a good technique to view the spinal cord and is useful to evaluate mechanical disorders of the lumbosacral spine. It can delineate the extent of the extradural tumoral invasion of the spinal cord and compression or displacement of the spinal cord. FNAC or biopsy of the tissue gives the conclusive evidence of the presence of NHL of the bone.
Majority of the PLBs appear to have Bcell tumors of follicular center cell origin with a diffuse mixed or diffuse large cell histology.  The present patient also had a large cell, high-grade histological type of NHL.
The treatment modalities of the primary bone lymphomas are surgical, chemotherapy, and radiotherapy. Unlike other tumors of the bone, the role of surgery in primary bone lymphomas is limited to the diagnostic biopsy, laminectomy, and to the repair of fractures that either present at initial diagnosis or that arise during the period posttreatment.  Radiotherapy is usually given to the primary lesion of the bone and chemotherapy is given in the form of nitrogen mustard, oncovin, procarbazine, prednisolone, or an anthracycline containing regimen. Primary bone lymphomas are a potentially curable form of malignancies; therefore, a policy of combined modality treatment has been adopted. However, because of its rarity, no final guidelines exist for the treatment of primary bone lymphomas. 
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[Figure 1], [Figure 2], [Figure 3]