malignant lymphoma ( ml ) can involve the central nervous system either primarily or by secondary spread , which tends to occur late in the disease as part of widespread dissemination8,18 ) .
the rate of epidural mass formation in all cases of mls is 0.8 - 2.86,14,19,21 ) . owing to this rate of occurrence , a small number of research studies and case reports
we report a rare case of primary spinal epidural lymphoma that presented with progressive myelopathy .
a 47-year - old man presented with progressive lower extremity weakness and numbness associated with fecal and urinary incontinence over 2 weeks .
a physical examination revealed hypoesthesia on the medial aspect of both thighs with bilateral motor weakness ( gr 4 - 4 + ) .
there was no abnormality of laboratory findings except the erythrocyte sedimentation rate was elevated ( esr-32mm / h ; reference value - less than 20mm / h ) .
preoperative magnetic resonance imaging ( mri ) of the thora columbar region showed an isointense spinal medulla on t1-weighted images and a slightly hyperintense t2-weighted images of the spinal cord extending from t9-l2 .
intraoperatively , a grayish epidural mass was identified and resected to decompress the cord and cauda equina .
proliferation of small blastic cells , with a lymphoblastic aspect and starry sky pattern , was seen .
further immunohistochemical characterization showed that results from tumor testing were positive for cd20 , cd3 , cd5 , bcl-2 , cyclin d1 and negative for cd23 , cd10 and t - cell markers ( fig .
further work - up , including computed tomography ( ct ) scans of the chest and the abdomen , iliac crest bone marrow biopsy , and ultrasound of the lymph nodes showed no extramedullary lymphoma manifestation .
the patient had no history of immune disorder , and human immunodeficiency virus ( hiv ) testing was negative .
the treatment regimen consisted of radiotherapy , 40gy administered in 20 fractions , from t9-l2 , followed by combination chemotherapy with cyclophosphamide , adriamycin , oncovin , and prednisone ( chop ) .
a follow - up mri one year after the surgery showed that the lesions of lymphoma had disappeared ( fig .
postoperatively , the patient was faring well with no evidence of local recurrence or new lesions at any other site .
primary central nervous system lymphoma is a rare form of extranodal lymphoma , particularly isolated primary spinal lymphoma7,8 ) .
compression of the spinal cord as the first manifestation of the primary spinal lymphoma is particularly rare , with an incidence of < 5%15 ) . in our report , the patient initially presented with compression of the spinal cord manifesting as hypoesthesia and motor weakness of the lower extremities . although the mechanism of epidural mass formation without systemic lymphoma has not been clarified , the origin of the mass is thought to be bone marrow11 ) .
a small amount of lymphocytic cells can migrate to the spinal epidural space directly through the harversian canals of the vertebral bone or hematogenously via the epidural venous plexus , followed by a mass formation even before systemic lymphoma .
findings from the ct and mri provided only indirect diagnostic evidence . on t1-weighted mri ,
primary spinal lymphomas are most frequently isointense to hypointense , while t2-weighted images are commonly isointense to hyperintense , with contrast enhancement4 ) .
primary spinal lymphomas are often confused with other spinal tumors , especially metastasis12 ) . in the present case the spinal lesion
the mri demonstrated an epidural mass lesion extending from t9 to l2 , causing spinal cord compression .
the lesion appeared slightly hyperintense on the t2-weighted image and exhibited continuous homogenous enhancement after gadolinium injection .
this is similar to the description by negendank et al of lymphomas elsewhere in the body and li et al of spinal epidural lymphomas12,17 ) .
most primary spinal lymphomas are comprised of diffuse large b - cell lymphomas with a minority being follicular lymphoma , precursor b - lymphoblastic lymphoma , ki - lymphoma , diffuse lymphoblastic lymphoma , small lymphocytic lymphoma or t - cell lymphoma5 ) .
small lymphocytic lymphoma is composed mostly of small mature appearing lymphocytes with round nuclei and scant basophilic cytoplasm9 ) . in our case
, immunohistochemical studies demonstrated that the tumor cells expressed the b cell markers cd19 and cd20 , with coexpression of the t cell marker cd5 ( fig .
cyclin d1 staining yielded positive results for both the primary lesion and the recurrence1 ) .
haddad et alobserved that patients with the histopathologic subtype of mixed histiocytic and small lymphocytic lymphoma seemed to have a better survival than the other subtypes ( 61% at 10 years ) , but a small number ( n=17 ) of patients in this group did not allow statistical confirmation of this apparent observation10 ) . in the study by monnard
et al , out of 52 patients , 48 ( 92% ) underwent a laminectomy , with a partial resection performed in 22 cases ( 42% ) and a complete resection for 7 cases ( 13% ) . in surgery , each lesion was brownish or reddish purple and soft to firm in consistency .
, we performed a hemilaminectomy of t9-l2 and a partial resection of a grayish epidural mass .
however , a surgical resection does not have the survival benefits and the reported median overall survival durations with surgery alone are similar to those of untreated patients2,3 ) .
some reports suggest that well - established treatments , such as radiotherapy and chemotherapy or a combination of both should remain as the mainstays and that surgery should be used in combination with radiotherapy and/or chemotherapy13 )
. therefore , concurrent chemoradiotherapy treatment was provide ( chop ; cyclophosphamide , adriamycin , oncovin , and prednisone+radiotherapy ; 40gy administered in 20 fractions ) after pathologic confirmation .
monnard et al observed local control of 88% and a 5-year overall survival of 69% with combined modality treatment . in a multivariate analysis
, they found that the combined modality treatment was statistically superior to rt alone16 ) .
also , they reported a post - treatment complete neurologic response in 25% of patients who had initial motor deficits .
the remaining 75% had partial recovery . in their multivariate analysis , they have found that complete neurologic response was the most significant favorable prognostic finding with regard to overall survival ( p=0.001)16 ) . in the present case , because of the manifestations of compression , timely surgical decompression was required to allow for a recovery of nerve function even before the diagnosis had been made . after surgery , the neurologic function in our patient was completely recovered .
a primary small lymphocytic lymphoma of the spine should be considered in the differential diagnosis of primary spine tumors .
surgical decompression and tumor removal are mandatory to recover neurologic function , if the patient had a neurologic deficit .
the combined modality treatment after surgical decompression resulted in improvement of the neurologic symptoms and remission of the lymphoma .
 we report the case of a 47-year - old man who presented with progressive paraparesis and sphincter changes over 2 weeks . 
 magnetic resonance imaging revealed a spinal epidural mass from t9 to l2 . 
 we performed a decompressive laminectomy and mass removal . 
 the histopathology was consistent with a small lymphocytic lymphoma . 
 no metastatic lesion was noted in the chest and abdomen - pelvic computerized tomography ( ct ) and positron emission tomography computerized tomography ( pet - ct ) scan . 
 the final diagnosis was primary spinal lymphoma , so we performed chemotherapy combined with radiotherapy . at one year follow - up , he had no neurological deficit and no recurrence on neurologic and radiologic exams . 
 primary spinal cord lymphomas should be considered in the differential diagnosis of spinal cord tumors . 
 early surgical management is mandatory to achieve a recovery of neurologic function , especially if the patient has a neurological deficit . 
INTRODUCTION
CASE REPORT
DISCUSSION
CONCLUSION