CASEREPIMMT
uA,lltr4,W{f{ED rflJ RNALil 5(2)t7 2-7 5 .ilr"rRE2fi13
Primaryspinalcord epiduralnon-Hodgkin's Lymphomaas causeof paraplegia: report of 2 cases N Mkandawire Department of 5urgery, College of Medicine, Blantyre, Malawi
Introductiomr Two casesof paraplegia due to primary spinal cord epidural non-Hodgkin's lymphoma (NHL) are presented. This is a rare cause of paraplegia. The clinical presentation, investigations and management of this rare condition are outlined. A review ofthe literature is presented. Primary spinal epidural NHL should be considered in patients who give a history of back pain; followed by rapid development of features of spinal cord compressionl have normal plain x-rays but whose CAT / myelogram reveal an extradural mass. Urgent surgical decompression followed by chemotherapy and radiotherapy improves the outcome of such patients. Primary epidural Non Hodgkin's Lymphoma (NHL) with no evidence of parenchymal central neryous system (CNS) or systemic involvement is rare. Although it may present as a localised diseaseit is in fact a systemic disease.This disease entity must be differentiated from the more common clinical situation of primary CNS parenchymal lymphoma with
meningeal involvement or systemic lymphoma, such as adlulrl T cell lymphoma, complicated by lymphomatous meningitrni" A 20-year experience with NHL at the Memorial Sloaur Kettering Cancer Centre showed that primary epidural NHL accounted for 2 out of 256 (1.9Eo)of NHL casesl.A l{lyear experience (1979 - 1989) at the Mayo Clinic report prmmary spinal epidural NHL accounting for 6.67a of all casr of intraspinal NHL'z. Levitt et al report that among 592 case*. of NHL seenbetween 1967 and1977,52 patients (11%) hed CNS lymphomatous involvement. However they did n,u,m. specify how many were primary spinal epidural NHL'. With the current AIDS/HIV pan epidemic it is expectrd that the incidence of primary spinal NHL will increas*. Distinguishing primary spinal NHL from an infectiotr+ spinal mass may be difficult as both would present with sinmilar features of meningitis, feyer, night sweats and weighn loss.
Cases
prednisolone)regime; six cycles over six months was recontmended. However the patient has not been able to purchaserhr drugs and chemotherapyhas not been commenced.
LT 39-year-old self-employed businessmanadmitted to Queen Elizabeth Central Hospital on 9'hJanuary2002 with loss of function in the lower limbs and inability to walk since mid December 200 I . He stafied having mid thoracic back pain in October 2001. By end November 2001 he developed weakness in the lower limbs but was able to walk and was admitted at a rural mission hospital. Within two weeks, by mid December 2001, he developed almost complete loss of function in the lower limbs and was unable to walk. He had control of his bowels and the bladder and sensationwas preserved. He denied any trauma and systemically he was well with no significant constitutional features.He had no significant past medical history. He looked well nourished and was afebrile. He had tendernessin the dorsal spine but there was no deformity. Neurological assessment of the lower limbs revealedpower of 215;increasedtone; hyperreflexia with sustainedclonus; up going plantar reflexes; normal sensation. He was classified as Fraenkel grade C of paraplegia. Plain x-rays of the spine were normal. Myelography revealed a right-sided posterolateral extradural compressivemass at T9 T10 (Fig la, 1b). A CT scan,done some days after the myelogram, confirmed the compressivemass (Fig lc).The rest of his investigationsare as outlined in table 1. He was taken to theatre on 28th January 2002. He underwent hemilaminectomy at T9 and T10 on the right side. There was an extradural fibrinous soft tissue mass causing spinal cord compression.The tumour peeled off easily from the dura. Histology confirmed this to be non-Hodgkin's lymphoma. Chemotherapy using a CHOP (cyclophosphamide, adriamycin, vincristine,
Figure 1a:
Malawi Medical Joumal
l|tn
causeof paraplegia 73 recover\ post operativelyand was He made -soodneurolo-sical unit on I l"'march1002mobilising to a rehabilitation discharged \\'ith a \\ alkins fiame.He n a: sradedar FraenkelD on discharge t o t h e r e h a b i l i t a t i ounn i t a n dh e . o n t l n u e st o l n l p r o v e . 'AIDS anti reiroviral He i. also .onsiderins.ontntencing HI\'
i r g u r el b :
rnedication..
MC l--1 ear-old:es-onJrn .chtrrl >ludenitrrnsielredtiom a rural mis:ion hs.pital 1s Queen Eliz.rt-erhCentral Horprtal on l5th Februaryl00l r'.ith a trro-d:', frtr'ttrn.ri >uddenilnd conlplete and taecal loss of tunctionin the lo$er llnlhr: unin"in'r€riifl{ir.rn
Figure 1a, 1b: Myelogram for LI showing a right posterolateralcompressivemass at T9 T10 level. No evidenceofbone destructionnoted.
Figure 1c:
Figure lc
CT scanat levelof mass.A right sidedlesionis seendisplacingthe spinal fbramen cordto the left. Thereis an extensionthroughtheinterveftebral
incontinence. He had complainedof upperthoracicb.:c}'p.rirl.ri a non-traumatic origin since November100L Thir $as "i)iocieted\\'ith generalmalaise.On 4'nFebruar) 2001 he lr a: rdnlitted on the medical ward at QECH with increasedback pain and terer. He had an elevatedESR; was mildly anaemic:a negati\e blood culture; and a negative HIV test. After a course of intravenous mobilisin-s antibioticshis fever subsidedand he was dischar-eed 7 dayslater.On22"oFebruary2002he was admittedat a misslon hospitalwith suddenand completeloss of function of the lo*'er to QECH on 25* February2002. limbs and was transfer:red Systemicallyhe was unwell with weight loss; persistentt-ever: neck pain and stiffnesssuggestiveof meningitis'He had flaccid paralysisof the lower limbs with loss of sensationfrom T4 distally. He had no bowel or bladder control. He was assessedas Fraenkel A. He had developed small pressure sores on the sacrum and the greater trochanter areas. Blood cultures grew bacillus species,which was thoughtto be a contaminant.He was anaemicwith haemoglobinof 4.9. A lumbar puncturerevealed clear CSF with increasedprotein; no organismseen;and a negative culture. Plain x-rays of the spine were normal' Other investigationsare as shown in table 1. He was commencedon intravenousantibioticsincluding chloramphenicol,penicillin and flucloxacillin. A doseof praziquantel was given. His fever persistedand on 7th March, two weeks after admission, TB treatment was commenced empirically. A privately sponsoredCT Myelogram was done on 7th March 2002 and revealed a posterior extradural compressive mass extendingfrom T2 to T7 (Fig 2a,2b,2c,2d). The diagnosiswas now consideredto be spinal abscessand intravenousantibiotics as well as the TB treatment, were continued.
ILLUSTRATIONS Figure 2a:
Table: Investigations and results INYESTIGATIONS CASE:LT Full Blood Count / Picture
WBC -5.0:Hb 9.7: Plat 345 Nomochronic. normoc,vtic anaemia:neutrophils6E-t : lymph ?8-r: mono J'i Not done Not done
CASE:MC
WBC 3.21Hb 4.9; Plat 92
60 mnVHr Bacillussp.'l Contaminant C l e a rC S F : Not Done Lumbar puncture protein++++:no organisn seen: culture no gro$th \on Reactile Non Reactive VDRL \ormal Nomal Urinalysis / Stool \e gatire Positive HIV \omirl Normal Plain Spine x-ray Ertradural compressire Extradural compressive mass CAI / Myelogram at T9, T1 0 on the posterolateral massTl to T7 posteriorll' aspect on the dght. Normal Nomal Chest x-ray Normal Abdominal Ultrasound Normal Non Hodgkin's Non Hodgkin's Lymphoma / Histology ESR Blood culture
Iymphocytic lymphoma
Malawi Medical Joumal
lymphoma
Figure 2a: Lateral view myelogram of MC showing a posterior compressivemass extending over several vertebral segments.
74 Causeof paraplegia Figure 2b:
surgerybecauseif lymphoma were confirmed, they would con sider buying the chemotherapy. He receivedfour units of blood prior to surgeryand he was taken to theabe on l9'' March 2002. Laminectomieswere done bilaterally from T3 to T5. A soft, fibrinous, epidural mass was partially removed ant the spinal cord decompressed.Total excision was not possibledue to the extent of the lesion. Histology of the tissue confirmed a diagnosis of non-Hodgkin's lymphonia.' Worseningpressuresoresand breakdownof the surgical wound complicatedthe postoperativeperiod. The neurologicaldeficit in the lower limbs did not improve, however power in the upper limbs retumed to normal. Post operatively he required transfusion of four units of blood. Chemotherapy using a CHOP regime was recommendedbut the parentscould not afford the medications.His general condition detedoratedand he died on 26'r'April 2002 from a combination of anaemiaand septicaemia from infecledprersuresore\.
Figure 2c:
Discussion ClinicalPresentation / Features Primary spinal epidural NHL can presentat any age although it is more common in elderly patientswith a median age 70 years. A prodrome period of back pain with a median duration of 3 months is common. This is followed by an acute neurological deteriorationwith a median duration of 6 daysr. Neurological findings may include paraparesisor paraplegia;a discretesensorylevel; hyperreflexia;clonus and loss of sphincter contlol. In most patients,diagnosisof primary spinal epidural NHL is made when neurologic deficit is already established and histological specimenshave been obtainedat surgery.
Investigations Figure 2d:
Figure 2b, 2c, 2di CT scaDsat various levels. Fig 2b shows a posterior mass displacing the cord anleriorly- Fig 2c and 2d are scansat levels above and below the massrespectivelyshowing that the spinal cord is centrally located in the spinal canal
An orthopaedicconsultationwas hrst made on 7th March 2002. On reviewing the CT Myelogram the opinion was that the diagnosis was epidufal lymphoma. He was now noted to also have weaknessof the upper limbs and was developing weaknessof chest wall muscles.After discussingwith the parentsit was felt that surgery would help to arrive at a tissue diagnosisbut was unlikely to reverse the neurological deficit. The general poor prognosiswas explained.They felt we should go aheadwith
Blood tests: Routine blood investigationsinclude FBC, ESR and peripheral blood film. These may reveal a normocytic, normochronic anaemia;pancytopaeniaand atypical cells; elevatedESR. Bone manow assessmentis impofiant to exclude systemiclymphoma. Blood culture is necessaryto exclude septicaemiain patient presenting with featuresof meningitis and fever. VDRL and HIV testsshould be done after appropriatecounselling. Lumbar puncture Analysis of CSF is important. Young et al o report that CSF abnormalitiesare found in 9770 of patients of NHL with CNS involvement. Routine microscopy,culture and sensitivitytesting of CSF must be done. This may reveal raised protein and cell count with a predominanceof lymphocytes. Cytology of the CSF may be done but has beenshown to be positive in only 677o of casesof lymphoma with CNS involvement4.India ink staining should be done to exclude cryptococcalmeningitis. Urine and stool Urine and stool analysis are important to investigateschistosomiasis as a causeof paraparesis/ paraplegia. Radioktgical Plain x - rays of the spine are invariably normal with no evidence of bone destruction. Myelography and CAT scan will reveal an exhadural mass compressingthe spinal cord, usually extending over more than one vertebral segment. MRI scan should be done where availableto further delineatethe extent of Malawi Medical Joumal
C a u s eo f p a r a p l e g i a 1 5
i
.r lesion.Abdominal ultrasound,chestx-ray, CAI scanof the There is a belief that sith the HI\r / AIDS pan epidemicthe -r.domen and chest are necessaryto exclude systemic disease number of casesof priman spinal epidural NHL rvill increase. The literature suggeststhat the responseto chemotherapyand '.nd tbr stagingthe disease. radiation in AIDS patientsis sirnilar tcrnon--\IDS patients. Both patientsrer.ealthe difiicultl in rliagnosingthis condition. Histology Histological specimensfrom primary epidural NHL are obtained During the prodromeperiod of brck pain L'othpatientsdid seek rt the time of surgical decompressionof the spinal cord. CAT medical attention.Hos.everbr the rime the] \1erereterredto the guided biopsy of lesion may be attemptedprior to surgery if the central hospital, neurolo-eicdet'icit ha,trt'een presentfor some time. A high index of clinical suspicionis neededto consider l e s i o ni s a c c e s s i b l e . In patients with NHL and CNS involvement, between 807o and t h i s r a r e c o n d i t i o n a s a d i f f e r e n t i a l d i r g n o . i : i n p a t i e n t s 987o have a diffuse histological subtype CNS involvement in presentingwith paraplegia.The other ma-iorconstreintin arrivnodular lymphoma is uncommon and is reported in only about ing at a diagnosisis the lack of radiologicalinrestrgationsespecially Myelography. In both these patients CT \helography 3Voof patientswith NHL4. For proper sub typing immunohistochemistry and immunocyto- was done after somedelay becausethe patientshad to pa1'for it themselvesat a private institution becauseQECH did not have chemistry are imporlant tests. Myelography contrastmedia and the CT scanis out of commission. Treatment Most patients with primary epidural NHL present with rapidly MC had a more rapid progressionand on presentationhad no progressiveor advancedneurologicaldeficit. Diagnosisis usual- motor function and already had developedsome pressuresores. ly confirmed by histological specimenstaken at time of emer- Although surgery was done, it was known that this u.as mainly gency surgical decompression of the spinal cord. Surgery is to aid in tissuediagnosisand would not reversethe neurolo-sical therefore impofiant for decompression and tissue diagnosis. deficit. His neurological deficit has remained. LI had a slower Surgery involves laminectomy and resection of the tumour. progressionand had residual motor function at the time of presSpinal inadiation and systemic chemotherapy are important entation. Surgic4l decompressionhas resulted in improvement adjuvant treatment, which have been shown to increase disease of the neurological deficit. free survival. Intrathecal chemotherapy can be considered in Chemotherapyin both caseshasto be purchasedby the patients, relapsecases.The ten-year experienceat the Mayo clinic report as these drugs are not on the hospitals pharmacy list. They are shows that 8 patients had primary spinal epidural NHL and all expensiveand both patients were not able to purchasethe medunderwent decompressive laminectomies, subtotal tumour ications. This undoubtedly will affect outcome in patient LT' It resection and local spinal radiotherapy. Four patients died and is debatable whether chemotherapy would have prevented the four remain alive and well and free from disease.The twenty- death of patient MC. Radiotherapyhas been shown to be a useyear experience at the Memorial Sloan - Kettering Cancer ful adjuvant therapy but unfortunately is not availablein Malawi. Primary spinal epidural NHL should be considered in patients Centre showed that 5 patients had primary spinal epidural NHL. Two patients died and three remained alive and diseasesfree who give a history of back pain; followed by rapid development of featuresof spinal cord compression;have normal plain x-rays after a median follow up period of ten years. Responsein AIDS 5. but whose CAI / myelogram reveal an extradural mass. Urgent / HIV patients is similar to that seenin non-AIDS patients surgical decompressionfollowed by chemotherapy and radiotherapy improves the outcome of such patients. Prognosis Some poor prognosticfactors especiallywith respectto resolution of paraplegiaand diseaserelapseinclude: a Rapidly progressiveparaplegia with lack of motor function at presentation (Fraenkel A, B) and long duration of complete paraplegiabefore presentationhave a poor chance of neurological recovery. o Poorly differentiated, diffuse histological grade is associatedwith increasedrelapserate. Thesetwo casesraise severaldiscussionpoints. Primary spinal epidural NHL can present at any age, although the median age in most seriesis 70 years.MC is a young man who is HIV nega t i v e w h e r e a sL T i s m i d d l e a g e d m a n a n d i s H I V p o s i t i v e .
Malawi Medical Joumal
References 1. Mora J, Wollner N. Primary epidural non-Hodgkin's lymphoma: spinal cord compression syndrome as the initial fom of presentation in childhood non-Hodgkin's lymphoma. Med Pediatr Oncol 1999 Feb; 32(2): 107 - 5 2. Lyons MK, O'Neill BP, Marsh WR, Kurtin PJ Primary spinal epidural non - Hodgkin's lymphoma: report of eight patients and review of the literature. Neurosurgery I992May:30(5): 675 - 80. 3. 4.
5.
Levitt LJ. Dawson DM, Rosenthal DS, Moloney WC. CNS involvement in the non - Hodgkin's lymphomas.Cmcer 1980 Feb; 45(3): 5'15- 52. Young RC, Howser DM, Anderson T, et. al. Central nenous slstem complications of non-Hodgkin's lymphoma. The potential role for prophl lactic therap). Am J Med 1979 Mr: 66(3): 435 - 43 Chamberlain MC, Kormanik PA. AIDS - related central nenous s)stem lymphomas. J Neurooncol 1999 July: 43(3):269
76.
uA,lltr4,W{f{ED rflJ RNALil 5(2)t7 2-7 5 .ilr"rRE2fi13
Primaryspinalcord epiduralnon-Hodgkin's Lymphomaas causeof paraplegia: report of 2 cases N Mkandawire Department of 5urgery, College of Medicine, Blantyre, Malawi
Introductiomr Two casesof paraplegia due to primary spinal cord epidural non-Hodgkin's lymphoma (NHL) are presented. This is a rare cause of paraplegia. The clinical presentation, investigations and management of this rare condition are outlined. A review ofthe literature is presented. Primary spinal epidural NHL should be considered in patients who give a history of back pain; followed by rapid development of features of spinal cord compressionl have normal plain x-rays but whose CAT / myelogram reveal an extradural mass. Urgent surgical decompression followed by chemotherapy and radiotherapy improves the outcome of such patients. Primary epidural Non Hodgkin's Lymphoma (NHL) with no evidence of parenchymal central neryous system (CNS) or systemic involvement is rare. Although it may present as a localised diseaseit is in fact a systemic disease.This disease entity must be differentiated from the more common clinical situation of primary CNS parenchymal lymphoma with
meningeal involvement or systemic lymphoma, such as adlulrl T cell lymphoma, complicated by lymphomatous meningitrni" A 20-year experience with NHL at the Memorial Sloaur Kettering Cancer Centre showed that primary epidural NHL accounted for 2 out of 256 (1.9Eo)of NHL casesl.A l{lyear experience (1979 - 1989) at the Mayo Clinic report prmmary spinal epidural NHL accounting for 6.67a of all casr of intraspinal NHL'z. Levitt et al report that among 592 case*. of NHL seenbetween 1967 and1977,52 patients (11%) hed CNS lymphomatous involvement. However they did n,u,m. specify how many were primary spinal epidural NHL'. With the current AIDS/HIV pan epidemic it is expectrd that the incidence of primary spinal NHL will increas*. Distinguishing primary spinal NHL from an infectiotr+ spinal mass may be difficult as both would present with sinmilar features of meningitis, feyer, night sweats and weighn loss.
Cases
prednisolone)regime; six cycles over six months was recontmended. However the patient has not been able to purchaserhr drugs and chemotherapyhas not been commenced.
LT 39-year-old self-employed businessmanadmitted to Queen Elizabeth Central Hospital on 9'hJanuary2002 with loss of function in the lower limbs and inability to walk since mid December 200 I . He stafied having mid thoracic back pain in October 2001. By end November 2001 he developed weakness in the lower limbs but was able to walk and was admitted at a rural mission hospital. Within two weeks, by mid December 2001, he developed almost complete loss of function in the lower limbs and was unable to walk. He had control of his bowels and the bladder and sensationwas preserved. He denied any trauma and systemically he was well with no significant constitutional features.He had no significant past medical history. He looked well nourished and was afebrile. He had tendernessin the dorsal spine but there was no deformity. Neurological assessment of the lower limbs revealedpower of 215;increasedtone; hyperreflexia with sustainedclonus; up going plantar reflexes; normal sensation. He was classified as Fraenkel grade C of paraplegia. Plain x-rays of the spine were normal. Myelography revealed a right-sided posterolateral extradural compressivemass at T9 T10 (Fig la, 1b). A CT scan,done some days after the myelogram, confirmed the compressivemass (Fig lc).The rest of his investigationsare as outlined in table 1. He was taken to theatre on 28th January 2002. He underwent hemilaminectomy at T9 and T10 on the right side. There was an extradural fibrinous soft tissue mass causing spinal cord compression.The tumour peeled off easily from the dura. Histology confirmed this to be non-Hodgkin's lymphoma. Chemotherapy using a CHOP (cyclophosphamide, adriamycin, vincristine,
Figure 1a:
Malawi Medical Joumal
l|tn
causeof paraplegia 73 recover\ post operativelyand was He made -soodneurolo-sical unit on I l"'march1002mobilising to a rehabilitation discharged \\'ith a \\ alkins fiame.He n a: sradedar FraenkelD on discharge t o t h e r e h a b i l i t a t i ounn i t a n dh e . o n t l n u e st o l n l p r o v e . 'AIDS anti reiroviral He i. also .onsiderins.ontntencing HI\'
i r g u r el b :
rnedication..
MC l--1 ear-old:es-onJrn .chtrrl >ludenitrrnsielredtiom a rural mis:ion hs.pital 1s Queen Eliz.rt-erhCentral Horprtal on l5th Februaryl00l r'.ith a trro-d:', frtr'ttrn.ri >uddenilnd conlplete and taecal loss of tunctionin the lo$er llnlhr: unin"in'r€riifl{ir.rn
Figure 1a, 1b: Myelogram for LI showing a right posterolateralcompressivemass at T9 T10 level. No evidenceofbone destructionnoted.
Figure 1c:
Figure lc
CT scanat levelof mass.A right sidedlesionis seendisplacingthe spinal fbramen cordto the left. Thereis an extensionthroughtheinterveftebral
incontinence. He had complainedof upperthoracicb.:c}'p.rirl.ri a non-traumatic origin since November100L Thir $as "i)iocieted\\'ith generalmalaise.On 4'nFebruar) 2001 he lr a: rdnlitted on the medical ward at QECH with increasedback pain and terer. He had an elevatedESR; was mildly anaemic:a negati\e blood culture; and a negative HIV test. After a course of intravenous mobilisin-s antibioticshis fever subsidedand he was dischar-eed 7 dayslater.On22"oFebruary2002he was admittedat a misslon hospitalwith suddenand completeloss of function of the lo*'er to QECH on 25* February2002. limbs and was transfer:red Systemicallyhe was unwell with weight loss; persistentt-ever: neck pain and stiffnesssuggestiveof meningitis'He had flaccid paralysisof the lower limbs with loss of sensationfrom T4 distally. He had no bowel or bladder control. He was assessedas Fraenkel A. He had developed small pressure sores on the sacrum and the greater trochanter areas. Blood cultures grew bacillus species,which was thoughtto be a contaminant.He was anaemicwith haemoglobinof 4.9. A lumbar puncturerevealed clear CSF with increasedprotein; no organismseen;and a negative culture. Plain x-rays of the spine were normal' Other investigationsare as shown in table 1. He was commencedon intravenousantibioticsincluding chloramphenicol,penicillin and flucloxacillin. A doseof praziquantel was given. His fever persistedand on 7th March, two weeks after admission, TB treatment was commenced empirically. A privately sponsoredCT Myelogram was done on 7th March 2002 and revealed a posterior extradural compressive mass extendingfrom T2 to T7 (Fig 2a,2b,2c,2d). The diagnosiswas now consideredto be spinal abscessand intravenousantibiotics as well as the TB treatment, were continued.
ILLUSTRATIONS Figure 2a:
Table: Investigations and results INYESTIGATIONS CASE:LT Full Blood Count / Picture
WBC -5.0:Hb 9.7: Plat 345 Nomochronic. normoc,vtic anaemia:neutrophils6E-t : lymph ?8-r: mono J'i Not done Not done
CASE:MC
WBC 3.21Hb 4.9; Plat 92
60 mnVHr Bacillussp.'l Contaminant C l e a rC S F : Not Done Lumbar puncture protein++++:no organisn seen: culture no gro$th \on Reactile Non Reactive VDRL \ormal Nomal Urinalysis / Stool \e gatire Positive HIV \omirl Normal Plain Spine x-ray Ertradural compressire Extradural compressive mass CAI / Myelogram at T9, T1 0 on the posterolateral massTl to T7 posteriorll' aspect on the dght. Normal Nomal Chest x-ray Normal Abdominal Ultrasound Normal Non Hodgkin's Non Hodgkin's Lymphoma / Histology ESR Blood culture
Iymphocytic lymphoma
Malawi Medical Joumal
lymphoma
Figure 2a: Lateral view myelogram of MC showing a posterior compressivemass extending over several vertebral segments.
74 Causeof paraplegia Figure 2b:
surgerybecauseif lymphoma were confirmed, they would con sider buying the chemotherapy. He receivedfour units of blood prior to surgeryand he was taken to theabe on l9'' March 2002. Laminectomieswere done bilaterally from T3 to T5. A soft, fibrinous, epidural mass was partially removed ant the spinal cord decompressed.Total excision was not possibledue to the extent of the lesion. Histology of the tissue confirmed a diagnosis of non-Hodgkin's lymphonia.' Worseningpressuresoresand breakdownof the surgical wound complicatedthe postoperativeperiod. The neurologicaldeficit in the lower limbs did not improve, however power in the upper limbs retumed to normal. Post operatively he required transfusion of four units of blood. Chemotherapy using a CHOP regime was recommendedbut the parentscould not afford the medications.His general condition detedoratedand he died on 26'r'April 2002 from a combination of anaemiaand septicaemia from infecledprersuresore\.
Figure 2c:
Discussion ClinicalPresentation / Features Primary spinal epidural NHL can presentat any age although it is more common in elderly patientswith a median age 70 years. A prodrome period of back pain with a median duration of 3 months is common. This is followed by an acute neurological deteriorationwith a median duration of 6 daysr. Neurological findings may include paraparesisor paraplegia;a discretesensorylevel; hyperreflexia;clonus and loss of sphincter contlol. In most patients,diagnosisof primary spinal epidural NHL is made when neurologic deficit is already established and histological specimenshave been obtainedat surgery.
Investigations Figure 2d:
Figure 2b, 2c, 2di CT scaDsat various levels. Fig 2b shows a posterior mass displacing the cord anleriorly- Fig 2c and 2d are scansat levels above and below the massrespectivelyshowing that the spinal cord is centrally located in the spinal canal
An orthopaedicconsultationwas hrst made on 7th March 2002. On reviewing the CT Myelogram the opinion was that the diagnosis was epidufal lymphoma. He was now noted to also have weaknessof the upper limbs and was developing weaknessof chest wall muscles.After discussingwith the parentsit was felt that surgery would help to arrive at a tissue diagnosisbut was unlikely to reverse the neurological deficit. The general poor prognosiswas explained.They felt we should go aheadwith
Blood tests: Routine blood investigationsinclude FBC, ESR and peripheral blood film. These may reveal a normocytic, normochronic anaemia;pancytopaeniaand atypical cells; elevatedESR. Bone manow assessmentis impofiant to exclude systemiclymphoma. Blood culture is necessaryto exclude septicaemiain patient presenting with featuresof meningitis and fever. VDRL and HIV testsshould be done after appropriatecounselling. Lumbar puncture Analysis of CSF is important. Young et al o report that CSF abnormalitiesare found in 9770 of patients of NHL with CNS involvement. Routine microscopy,culture and sensitivitytesting of CSF must be done. This may reveal raised protein and cell count with a predominanceof lymphocytes. Cytology of the CSF may be done but has beenshown to be positive in only 677o of casesof lymphoma with CNS involvement4.India ink staining should be done to exclude cryptococcalmeningitis. Urine and stool Urine and stool analysis are important to investigateschistosomiasis as a causeof paraparesis/ paraplegia. Radioktgical Plain x - rays of the spine are invariably normal with no evidence of bone destruction. Myelography and CAT scan will reveal an exhadural mass compressingthe spinal cord, usually extending over more than one vertebral segment. MRI scan should be done where availableto further delineatethe extent of Malawi Medical Joumal
C a u s eo f p a r a p l e g i a 1 5
i
.r lesion.Abdominal ultrasound,chestx-ray, CAI scanof the There is a belief that sith the HI\r / AIDS pan epidemicthe -r.domen and chest are necessaryto exclude systemic disease number of casesof priman spinal epidural NHL rvill increase. The literature suggeststhat the responseto chemotherapyand '.nd tbr stagingthe disease. radiation in AIDS patientsis sirnilar tcrnon--\IDS patients. Both patientsrer.ealthe difiicultl in rliagnosingthis condition. Histology Histological specimensfrom primary epidural NHL are obtained During the prodromeperiod of brck pain L'othpatientsdid seek rt the time of surgical decompressionof the spinal cord. CAT medical attention.Hos.everbr the rime the] \1erereterredto the guided biopsy of lesion may be attemptedprior to surgery if the central hospital, neurolo-eicdet'icit ha,trt'een presentfor some time. A high index of clinical suspicionis neededto consider l e s i o ni s a c c e s s i b l e . In patients with NHL and CNS involvement, between 807o and t h i s r a r e c o n d i t i o n a s a d i f f e r e n t i a l d i r g n o . i : i n p a t i e n t s 987o have a diffuse histological subtype CNS involvement in presentingwith paraplegia.The other ma-iorconstreintin arrivnodular lymphoma is uncommon and is reported in only about ing at a diagnosisis the lack of radiologicalinrestrgationsespecially Myelography. In both these patients CT \helography 3Voof patientswith NHL4. For proper sub typing immunohistochemistry and immunocyto- was done after somedelay becausethe patientshad to pa1'for it themselvesat a private institution becauseQECH did not have chemistry are imporlant tests. Myelography contrastmedia and the CT scanis out of commission. Treatment Most patients with primary epidural NHL present with rapidly MC had a more rapid progressionand on presentationhad no progressiveor advancedneurologicaldeficit. Diagnosisis usual- motor function and already had developedsome pressuresores. ly confirmed by histological specimenstaken at time of emer- Although surgery was done, it was known that this u.as mainly gency surgical decompression of the spinal cord. Surgery is to aid in tissuediagnosisand would not reversethe neurolo-sical therefore impofiant for decompression and tissue diagnosis. deficit. His neurological deficit has remained. LI had a slower Surgery involves laminectomy and resection of the tumour. progressionand had residual motor function at the time of presSpinal inadiation and systemic chemotherapy are important entation. Surgic4l decompressionhas resulted in improvement adjuvant treatment, which have been shown to increase disease of the neurological deficit. free survival. Intrathecal chemotherapy can be considered in Chemotherapyin both caseshasto be purchasedby the patients, relapsecases.The ten-year experienceat the Mayo clinic report as these drugs are not on the hospitals pharmacy list. They are shows that 8 patients had primary spinal epidural NHL and all expensiveand both patients were not able to purchasethe medunderwent decompressive laminectomies, subtotal tumour ications. This undoubtedly will affect outcome in patient LT' It resection and local spinal radiotherapy. Four patients died and is debatable whether chemotherapy would have prevented the four remain alive and well and free from disease.The twenty- death of patient MC. Radiotherapyhas been shown to be a useyear experience at the Memorial Sloan - Kettering Cancer ful adjuvant therapy but unfortunately is not availablein Malawi. Primary spinal epidural NHL should be considered in patients Centre showed that 5 patients had primary spinal epidural NHL. Two patients died and three remained alive and diseasesfree who give a history of back pain; followed by rapid development of featuresof spinal cord compression;have normal plain x-rays after a median follow up period of ten years. Responsein AIDS 5. but whose CAI / myelogram reveal an extradural mass. Urgent / HIV patients is similar to that seenin non-AIDS patients surgical decompressionfollowed by chemotherapy and radiotherapy improves the outcome of such patients. Prognosis Some poor prognosticfactors especiallywith respectto resolution of paraplegiaand diseaserelapseinclude: a Rapidly progressiveparaplegia with lack of motor function at presentation (Fraenkel A, B) and long duration of complete paraplegiabefore presentationhave a poor chance of neurological recovery. o Poorly differentiated, diffuse histological grade is associatedwith increasedrelapserate. Thesetwo casesraise severaldiscussionpoints. Primary spinal epidural NHL can present at any age, although the median age in most seriesis 70 years.MC is a young man who is HIV nega t i v e w h e r e a sL T i s m i d d l e a g e d m a n a n d i s H I V p o s i t i v e .
Malawi Medical Joumal
References 1. Mora J, Wollner N. Primary epidural non-Hodgkin's lymphoma: spinal cord compression syndrome as the initial fom of presentation in childhood non-Hodgkin's lymphoma. Med Pediatr Oncol 1999 Feb; 32(2): 107 - 5 2. Lyons MK, O'Neill BP, Marsh WR, Kurtin PJ Primary spinal epidural non - Hodgkin's lymphoma: report of eight patients and review of the literature. Neurosurgery I992May:30(5): 675 - 80. 3. 4.
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Levitt LJ. Dawson DM, Rosenthal DS, Moloney WC. CNS involvement in the non - Hodgkin's lymphomas.Cmcer 1980 Feb; 45(3): 5'15- 52. Young RC, Howser DM, Anderson T, et. al. Central nenous slstem complications of non-Hodgkin's lymphoma. The potential role for prophl lactic therap). Am J Med 1979 Mr: 66(3): 435 - 43 Chamberlain MC, Kormanik PA. AIDS - related central nenous s)stem lymphomas. J Neurooncol 1999 July: 43(3):269
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