Precursor B-lymphoblastic lymphoma mimicking: An acute subdural hematoma

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Case report/ Kazuistyka

Precursor B-lymphoblastic lymphoma mimicking:

An acute subdural hematoma

Ant Uzay

^1,

*, Siret Ratip

¹

, Ilhan Elmaci

²

, Metin Ozdemirli

³

1DepartmentofHaematology,AcıbademHospital,Istanbul,Turkey

2DepartmentofNeurosurgery,MemorialHospitalsGroup,Istanbul,Turkey

3DepartmentofPathology,GeorgetownUniversityHospital,WashingtonD.C.,USA

Introduction

Presentation of patients with solitary involvement of the durabylymphomaisrareandistypicallythatofasubacute to chronic process. We present the ﬁrst case of a patient with precursor B-lymphoblastic lymphoma mimicking an acutesubduralhematoma.

Case report

A 19-year-old male patient was referred to Marmara Uni- versityHospitalfromaperipheralhospitalfollowingunsuc- cessful burr-hole drainage of a subdural hematoma.

Presenting complaints included severe headache, nausea, andvomiting.Aphysicalexaminationrevealedpapilledema article info

Articlehistory:

Received:03.01.2016 Accepted:04.10.2016 Availableonline:14.10.2016

Keywords:

Lymphoblastic

Lymphoma

Subdural

Hematoma

abstract

Objective and importance:We present the ﬁrstcase of a precursor acute subdural B- lymphoblasticlymphomamimickinganacutesubduralhematoma.Clinicalpresentation:

A19yearoldmalepresentedwithanacuteonsetofheadache,nauseaandvomiting.CT scanshowedcrescenticright-sided,frontoparietalsubduralmassisointensewithcortex and showing homogeneous enhancement after gadolinium. Intervention: The patient underwentacraniotomyandagraysubduraltumorwithinvasionofbothduraandbrain wasobserved.Theinvadeddurawasresectedandduraplastyperformed.Histopathologi- cally,thetumorwascomposedofsmallroundcellsinﬁltratingsofttissue.Insomeareas of the tumor, cells were arranged in a linear, “Indian ﬁle” fashion between collagen bundles.Theirnucleiweregenerallyuniform,roundtoovoidinshape,smalltomedium insize,andfeatured delicatechromatin.Accompanying cytoplasmwasscant.Necrosis wasabsent.Onimmunohistochemicalanalysis,thetumorcellswerepositiveforCD79a, TdT, CD10 andCD34. Conclusion:Subdural lymphomacan presentas a neurosurgical emergency,andlymphomashouldbeconsideredasararebutpossiblediagnosisbefore operation.

*Corresponding author at: AcıbademUniversity Hematology, Betül sokak,Çesan sitesi,2 Blok,D8, Acıbadem mahallesi, Kadıköy, Istanbul,Turkey.Tel.:+905365123022;mobile:+905365123022.

E-mailaddresses:teteantuzay@yahoo.com,ant.uzay@acibadem.com.tr(A.Uzay).

ContentslistsavailableatScienceDirect

Acta Haematologica Polonica

journal homepage:www.elsevier.com/locate/achaem

http://dx.doi.org/10.1016/j.achaem.2016.10.003

zo.o.Allrightsreserved.

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butnootherneurologicalsigns.ACTscanshowedcrescen- tic right-sided,frontoparietalsubdural masswhich hadthe appearance of a subdural hematoma (Fig. 1A). The lesion wasisointensewithcortexonT1-weightedandT2-weighted MRexaminations and showed homogeneous enhancement after gadolinium (Fig. 1B, C). The patient underwent acraniotomy and a gray subdural tumor featuring hemor- rhage withinvasion of both dura and brain was observed.

The invaded dura was resected and duraplasty was

performed. The postoperative course was uneventful. The formalin-fixedtissuewasroutinely processed for histologic examination.Immunohistochemicalstainingwasperformed usingtheperoxidaseantiperoxidasetechniqueandobtained from Dako Corp, Carpinteria, CA, and antibodies directed against CD3, CD20, CD79a, CD10, CD34, TdT, myeloperoxi- dase, lysozyme, synaptophysin, GFAP. Histopathologically, the tumor was composed of small round cells infiltrating soft tissuewithouta specificpattern. Insomeareas of the Fig.1–A:AxialCTscan.Ahyperdense,subduralmassintherightfrontoparietalregioncausingmidlineshiftand

compressionofthelateralventricle.B,C:AxialT1-andT2-weightedMRimagesshowthemasstobeisointensewithcortex

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Fig.2–Hematoxyleneandeosinestainofthebrainbiopsy,revealingdiffuseinfiltrationoftheuniformlargeimmature lymphoidcells(A).TheneoplasticcellsareimmunopositiveforTdT(B-1.Positive79astainofthespecimen;B-2.Strong positiveTdTstainingofthespecimen)andCD10(C).(A,BandC,T250)

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tumor,cellswerearrangedinalinear,“Indianﬁle” fashion between collagen bundles (Fig. 2A). Their nuclei were generally uniform, round to ovoid in shape, small to mediuminsize,andfeatureddelicatechromatin.Accompa- nyingcytoplasmwasscant.Necrosiswasabsent.Onimmu- nohistochemicalanalysis, the tumorcells werepositivefor CD79a,TdT (Fig. 2B), CD10 (Fig. 2C) andCD34; CD3, myelo- peroxidase,lysozyme, GFAP, synaptophysin,and CD20 pre- parations were negative. A diagnosis of precursor B- lymphoblastic lymphoma was made based on combined cytologicandimmunohistochemicalﬁndings.

Further assessment of the patient’s hematologic status was undertaken. The patient had no B symptoms such as weightlossof greaterthan10%body weight,fever ornight sweats.The physicalexamination revealedneitherlympha- denopathy nor hepatosplenomegaly. Pertinent laboratory valuesincludedahemoglobinof14.4g/dL,aleukocytecount of 7800/mm³, and a platelet count of 287000/mm³. The erythrocyte sedimentation rate was 5mm/h. Liver function tests and LDH levels were normal as was the peripheral blood smear. The patient was referred to the hematology department. Bilateral posterior iliac crest bone marrow aspiratesandtrephineswereperformed.Noinvolvementby lymphoma was detected. A CT scan of the thorax and abdomen showed nosign of lymphadenopathy or hepatosplenomegaly. A diagnosis of primary precursor B-lymphoblastic lymphoma of the subdural space and brain was made. The patient was transferred to the care of the hematology service but, unfortunately, failed to keep out- patientclinic appointmentsand couldnot becontacted.He presented twomonths thereafter with complaintsof weak- nessandlethargy.AfullbloodcountrevealedaHbof8.1g/

dL, aleukocytecount of 4800/mm³, and aplatelet count of 54000/mm³. A repeat bone marrow aspirate and trephine showed heavy inﬁltration by lymphoblasts. He underwent cranial irradiation, and intrathecal as well as systemic chemotherapyas partof theUKALL X regime, including L- asparaginase,vincristine,daunorubicine,prednisolone,cytar- abine, methotrexate, 6-mercaptopurine, thioguanine, cyclo- phosphamide and etoposide. Despite an initial favorable response,herelapsedat 11monthsafteronsetof treatment anddiedofthedisease.

Discussion

Central nervous system (CNS) involvement by non-Hodg- kin’s lymphoma(NHL) isusually encounteredat advanced stagesofthedisease[1,2].PrimaryinvolvementoftheCNS by lymphoma remains relatively uncommon [3], and pre- sentationwithduralinvolvementaloneisrare[4–11].

With the single exception of an extradural malignant lymphoma of mixed type presenting acutely as a surgical emergency [11], the presentation of patients with solitary involvement of the dura bylymphoma is typically that of asubacutetochronicprocess[4–10].Thereasonforthelack ofacutepresentationsisexplainedbythelow-gradenature of NHLs involving the dura. These include MALT lympho- mas[4,5],smalllymphocyticlymphomas[6–8],andaT-cell richB-celllymphoma[9].

Lymphoblastic lymphoma as reported herein is a very aggressive form of NHL that usuallypresents with rapidly progressive lymphadenopathy andshows a high frequency of CNS involvement [3]. Its subtype, B-LBL is uncommon and accounts for less than 10% of cases of lymphoblastic lymphoma [12]. B-LBL has the feature of involving extra- nodal sites,mostoftentheskinand thebones.Inareport of B-LBL,therewasnoevidenceof bonemarrow diseaseat the time of diagnosis in 23 out of 25 patients, and the primarysitesofdiseasewereskin(9cases),bones(5cases), softtissue(4cases),lymphnodes(3cases),breast(2cases), stomachand colon(1case), andmediastinum (1 case)[12].

ThepatientinthiscaseistheﬁrstreportofB-LBLpresent- ing with dural involvement. Presentation of lymphoblastic lymphoma, oranyother form of NHL,withacute subdural hematoma related to a solitary lesion has also not been previouslydescribed.Rapidtumorgrowth,areﬂectionofthe very aggressive nature of lymphoblastic lymphoma seems toexplainthe“acutesubdural”presentationofthispatient.

Even modern neuroimaging methods, such as CT and MRI scans, usually fail to distinguish hematoma from bleeding into tumor at the subdural space [10, 11]. Proton density MRI scans may be suggestive of lymphoma as a hyperintense image and allow differentiation of hematoma and lymphoid tissue [13]. However, when dural lymphoma is associated with hematoma, even proton density MRI sequences are unlikely to differentiate lym- phoidtissuefrom the hematoma.Precursor B-Lymphoblas- tic lymphoma is a rare subtype of lymphoma, and thus ararecauseofsubduralhemorrhage,buttumorsassociated with haematomas are not rare in neurosurgical practice.

Therefore, it is always important to have a differential diagnosis inmind, even for whatappearsto beastraight- forward case,and toalwaysbeaspreparedas possiblefor unexpectedintraoperativeﬁndings.

In the present case, an apparently primary subdural lymphoma presented with acute neurological symptoms unaccompaniedbysystemicmanifestations.Despiteinvesti- gations,includingCTscansaswellasbilateralbonemarrow aspirates and trephine biopsies, no other site of involvement was identiﬁed at the time of presentation. Blood countsandbiochemicalparameterswerealsonormalatthis stage. Nonetheless, two months thereafter, the patient presented withbonemarrow involvement.Itispossible,of course,thatasystemicfocus ofsystemiclymphomamight initially have escapeddetectionand ledto hematogeneous involvementofthedura.Althoughthepathogenesisofdural involvementbylymphomasisuncertain,mostpatientswith CNS NHL do have bone marrow involvement [14]. Thus, intracranial disease at presentation isusually theresult of hematogeneous dissemination.However, as in the present case, manifest marrow inﬁltration isnot a prerequisite for CNSdissemination[1].

Conclusion

Lymphoblastic lymphoma mimicking an acute subdural hematomaandrequiringemergencysurgicaldecompression hasnotbeenpreviouslyreported.Thepresentcaseindicates

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that subdural lymphoma can present as a neurosurgical emergency.Thus,lymphomashouldbeconsideredasarare butpossiblediagnosisbeforeoperation.

Authors’ contributions/ Wkład autorów

AU–studydesign,datacollection,manuscript preparation, literature search.SR– datacollection, manuscript preparation, literature search. İE – data collection and interpreta- tion.MÖ–datainterpretation.

Conﬂict of interest/ Konﬂikt interesu

Nonedeclared.

Financial support/ Finansowanie

Nonedeclared.

Ethics/ Etyka

Thework described inthis article has been carriedout in accordance with The Code of Ethics of the World Medical Association(Declaration of Helsinki)for experimentsinvol- ving humans; EU Directive 2010/63/EU for animal experi- ments;UniformRequirementsformanuscriptssubmittedto Biomedicaljournals.

Acknowledgements/ Podziękowania

We thank ProfessorBernd W. Scheithauer for his editorial assistance.

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