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21 June 2022: Articles

Primary Central Nervous System Burkitt Lymphoma, Presenting with Long-Term Fluctuating Level of Consciousness: A Case Report and Literature Review on Challenges in Diagnosis and Management

Challenging differential diagnosis, Rare disease

Salem M. Bahashwan A* , Osman O. Radhwi D , Hatem M. Alahwal B , Abdullah T. Almohammadi C , Ahmed S. Barefah A , Yara M. Daous E , Fahad A. Alghamdi B

DOI: 10.12659/AJCR.936401

Am J Case Rep 2022; 23:e936401

Table 1. Reported cases of primary central nervous system lymphoma in the literature, the multiple approaches used for treatment, and the outcomes.

AuthorYearAgeGenderPresenting symptomsModality of diagnosisLocation
Valsamis et al []6 19766 monthMFever, vomiting, seizureTissue biopsyLeft parietal,bilateral temporal with abdominal and peeriaortic lymph node involvement
Gawish et al []7 19768 yearMLeft frontal fungating mass without neurological symptomsTissue biopsyLeft frontal lobe
Gigormini et al []18 198111 yearMHeadache and confusionTissue biopsyLeft temporo-occepital
Kobayashi et al []27 198455 yearFHeadacheTissue biopsyRight temporo-parietal
Hegedus []28 198450 yearFSeizures, vomitingPost mortum autopsyRight lower parietal
Pui et al []29 19856 yearMParaplasia, loss of sensation below T8Tissue biopsyEpidural mass T2–T5
Pui et al []29 198514 yearFParesis and parasthesia of right lower extremityTissue biopsyEpidural mass L5–S1, left axillary lymph nodes, pleura, and abdomen.
Pui et al []29 19857 yearMParaplasiaTissue biopsyEpidural mass C7–T4
Pui et al []29 198512 yearMParaplasia, loss of sensation below T11Tissue biopsyEpidural mass T7–T10
Mizugami et al []30 19876 yearMBack pain, paraplasiaTissue biopsyEpidural mass T10
Mizugami et al []30 19875 yearMParasthesia in lower limb, difficulty in walkingTissue biopsyEpidural mass L2–L3
Mizugami et al []30 19877 yearFSensory and motor disturbancesTissue biopsyEpidural mass T11
Shigemori et al []31 199149 yearMHeadache, memory disturbance and weakness in right lower extremitiesCT, MRI and carotid angiogram and tissue biopsyLeft frontal lobe
Tekkok et al []32 19915 yearMHeadache and loss of vision in left eyeCT and tissue biopsyParasellar mass extended to bilateral sphenoid bone and sella turcica
Toren et al []33 19946 yearFAtaxia, external ophthalmoplegia, facial diplegia, chewing and swallowing difficulties and irritabilityCSF analysisBrain CSF?
Spath-Schwalbe et al []8 199940 yearMPositional vertigo, nausea and vomitingMRI and tissue biopsyCerebellum and pons
Mora et al []9 19999 yearMLow back pain then unable to walkMyelogram and histopathologyEpidural T9-11 mass
Mora et al []9 199918 yearMLow back pain, then unable to walk, sever constipation and bladder incontinenceMyelogram and histopathologyT11
Wilkerning et al []10 200143 yearFLumboischialgia, hypaesthesia of the right leg and radicular painSpine MRI and histopathologyL2-3 epidural tumor involving the dura and cauda equina
Monabati et al []11 200249 yearFEmotional disorder, left-sided weaknessMRI and histopathologyRight parietal mass
Daley et al []12 200313 yearFLow back pain, a burning sensation in the vaginal area, and difficulty voiding then unable to walkSpine MRI and histopathologyL1-2 epidural mass
Shehu et al []13 20038 yearMHeadache, lower right limb weakness and protrusion of the right eye. Later on blurred vision in the right eye and diplopiaCT brain and tissue biopsyLeft temporal and right orbit masses
Huisman et al[]14 200312 yearMRight ophthalmoplegia, exophthalmos, headache, nausea and vomitingBrain CT, MRI, tissue biopsy and BM aspirationRight cavernous sinus mass extending into the orbital apex
Gobbato et al []15 200638 yearMHeadacheCT, craniotomy, excisional biopsySubdural, fronto-temporo-parietal
Takasu et al []16 201071 yearMGeneralized fatigue, disorientationCSF, CT, MRI, biopsyHypothalamus, 3 ventricle
Gu et al []17 201075 yearFHeadache, speech disturbanceMRI, excisional biopsy3r and left ventricles
Lim et al []19 201143 yearFHeadacheCSF, MRI, biopsyMedulla-leptomeningeal seading
Jiang et al []20 201114 yearMHeadacheMRI, excisional biopsyRight lateral ventricle
Akhaddar et al []21 201213 yearFRight facial painMRI, biopsyTemporal dura of the skull base, maxillary sinus
Yoon et al []22 201210 yearMHeadacheCSF, MRI, biopsySuprasellar, cerebellum, 3 ventricle
Yoon et al []22 201232 monthMLethargic featuresCSF, MRI, biopsySellar area extend to orbit
Jiang et al []23 201269 yearMBilateral lower limb weakness and painCSF, MRI, biopsyRight temporal and occepital lobe
Alabdulsalam et al []24 201418 yearMAtaxia, double vision, dysphagia, facial asymetryCT, MRI, biopsyPosterior fossa, 3 ventricle
Bower et al []25 201855 yearMBilateral lower limb weaknessMRI, biopsySuprasellar, hypothalamus, right ventricle
Patel et al []26 20197 yearMHeadache, decreased vision in left eyeMRI, excisional biopsyMiddle cranial fossa
AuthorTreatmentinitial responseEFSOS
Valsamis et al []6 Steroid, whole brain and spinal radiation with relapse, IT MTXPartial response3 months20 months
Gawish et al []7 Total resection of tumor, chemotherapy (cyclophosphamide)Complete response>3 years>3 years
Gigormini et al []18 Total resection of tumorComplete response>1 year>1 year
Kobayashi et al []27 Total resection of tumorComplete response1 month2 months
Hegedus []28 None3 months
Pui et al []29 Laminectmy T2 to T5, chemotherapy CHOP without prednisoneComlete response>2 years
Pui et al []29 Raditherapy L3–S2, chemotherapy CHOPComlete response22 months
Pui et al []29 Laminectomy C7 to T4, radiotherapy C7–T12, dexamethasone, cyclophosphamideComlete response5 months
Pui et al []29 Laminectomy T7 to T10, chemotherapy CHOPComlete response4 months
Mizugami et al []30 Laminectomy T7 to T11, surgical resection, spinal irradiation, systemic chemotherapy, then leukemic transformation and CSF recurrence, IT MTX and cranial irradiationComplete response20 months
Mizugami et al []30 Laminectomy T12 to T4, spinal irradiation, chemotherapy with systemic recurrenceComplete response7 months
Mizugami et al []30 Laminectomy T9 to L2, surgical resection, spinal irradiation, systemic chemotherapyRefractory3 months
Shigemori et al []31 Resection, radiation, Chemotherapy, CHOP, MTX.Good response>6 months
Tekkok et al []32 Resection, chemotherapy, CHOP and MTX, craniospinal radiation.Good response>18 months
Toren et al []33 Steroids, IVIG, doxorubicin, vincristine, HD MTX, with IT MTX, cytarabine, and hydrocortisone. Changed to CHOP with MTX and IT MTX, cytarabine, hydrocortisoneComplete remission> 2 years
Spath-Schwalbe et al []8 Chemotherapy combined with MTX then subsequent radiotherapyPartial response initially then full remission after radiotherapy>1 year
Mora et al []9 Laminectomy, chemotherapy via LSA3 protocol. Then received palliative radiation in second recurrenceRelapse>1 year
Mora et al []9 Laminectomy, CHOP substitute daunorubicin for doxorubicin and radiation.Relapse and refuse treatment>8 months
Wilkerning et al []10 Surgical resection, radiation and IT MTX, cyclophosphamide, vincristin, methotrexate, ifosfamid, adriamycin, and dexamethasoneGood response>2 years
Monabati et al []11 Complete resection, CHOP, and craniospinal radiation. Refused further treatment.Good response>6 months
Daley et al []12 Laminotomy, CHOP with MTX, and IT MTX and cytarabine and steroids.Good response>5 years
Shehu et al []13 Cy, vincristine, and MTX with IT cytosine arabinosideDeteriorated progressively due to viral hepatitis then died11 months
Huisman et al[]14 IV and intrathecal chemotherapy according to the NHL-BFM-1995 protocol. Then anti-CD20 antibodies (rituximab) after relapseRelapse, and died due to liver failure3 months
Gobbato et al []15 Total resection of tumorRefractory11 days
Takasu et al []16 Partial resection, WBRTGood partial response
Gu et al []17 Total resection of tumor, WBRTComplete response>9 months>9 months
Lim et al []19 Chemotherapy (HD-MTX based protocole)Refractory6.7 months
Jiang et al []20 Total resection, gamma-knife therapy, chemotherapy (MPV)Complete response>18 months>18 months
Akhaddar et al []21 ChemotherapyPartial response3 months
Yoon et al []22 Systemic chemotherapy (HD-MTX and HD cytarabine), ITComplete response>86 months>86 months
Yoon et al []22 Systemic chemotherapy (HD-MTX and HD cytarabine), ITComplete response6 months9 months
Jiang et al []23 WBRT, chemotherapy (HD-MTX, cytarabine,rituximab)Partial response
Alabdulsalam et al []24 Total resection, chemotherapy(CHOP-R and HD-MTX), ITComplete response>18 months>18 months
Bower et al []25 WBRT, chemotherapy (HD-MTX, cytarabine)Complete response>30 months>30 months
Patel et al []26 Total resection, WBRT, chemotherapy(HD-MTX, cytarabine), ITComplete response>12 months>12 months

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American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923