Indian J Otolaryngol Head Neck Surg (January–March 2010) 62(1):92–95; DOI: 10.1007/s12070-010-0003-y 92
Indian J Otolaryngol Head Neck Surg (January–March 2010) 62(1):92–95
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$QDWWHPSWWRGHÀQHWKHW\SHRIELRSV\LQDVLQRQDVDOOHVLRQVKRZLQJERQ\ HURVLRQ Indranil Pal · Amlan Gupta · Subhabrata Sengupta
Abstract Objectives To present a case of sino-nasal destructive mass LQLWLDOO\ GLDJQRVHG DV DQ LQÀDPPDWRU\ OHVLRQ IROORZLQJ SXQFK ELRSV\ IURP WKH OHVLRQ KRZHYHU WKH SRVW VXUJLFDO KLVWRSDWKRORJ\ ZDV GLDJQRVWLF RI *UDGH DQJLRFHQWULF immunoproliferative lesion (AIL). The reasons for the initial misdiagnosis are analyzed. Materials and methods A 76-year-old male patient presenWLQJZLWKSURJUHVVLYHELODWHUDOQDVDOREVWUXFWLRQIRU\HDU 5HSHDWHGSXQFKELRSVLHVIURPWKHPDVVZHUHVXJJHVWLYHRI DQLQÀDPPDWRU\OHVLRQ Result 7KH SDWLHQW XQGHUZHQW VXUJLFDO H[HQWHUDWLRQ RI WKHPDVVDQGWKH¿QDOKLVWRSDWKRORJ\UHSRUWVXJJHVWHG$,/ *UDGH 7KH SDWLHQW ZDV WKHUHDIWHU WUHDWHG ZLWK FKHPR therapy and radiotherapy. Conclusion ,QLWLDO VXSHU¿FLDO SXQFK ELRSVLHV OHDG WR incorrect diagnosis leading to an unnecessary surgical H[HQWHUDWLRQ7KHH[SODQDWLRQVIRUWKHLQLWLDOPLVGLDJQRVLV DUHJLYHQEHORZDQGDSSURSULDWHGLDJQRVWLFSURWRFROVPRGH and depth of biopsy are suggested based on the case study. Keywords Angiocentric immunoproliferative lesion · Incisional biopsy · Angiocentricity
I. Pal1 · A. Gupta2 · S. Sengupta1 Department of Ear Nose Throat and Head and Neck Surgery, Sikkim Manipal Institute of Medical Sciences, Gangtok, Sikkim, India 2 Department of Pathology, Sikkim Manipal Institute of Medical Sciences, Gangtok, Sikkim, India
1
I. Pal ( ) E-mail:
[email protected]
,QWURGXFWLRQ The nasal mucosa is affected by a variety of tumor and tumor like lesions. The most enigmatic are the hematoO\PSKRLGWXPRUV7KLVLVGXHWRDYDULHW\RILQÀDPPDWRU\ FHOOLQ¿OWUDWHVWKDWDSSHDULQERWKQHRSODVWLFDVZHOODVQRQ neoplastic conditions. Radiologic investigations and initial VXSHU¿FLDOELRSVLHVDUHNQRZQWRKDYHWKHLUOLPLWDWLRQV Angiocentric immunoproliferative lesions (AIL) DUH D JURXS RI H[WUDQRGDO O\PSKRSUROLIHUDWLYH GLVRUGHUV that share histological, immunophenotypic and clinical features. They are graded into three grades according to their cellular morphology and atypia. AIL, Grade 3 or angiocentric lymphoma is the end point in this sequence of grading [1]. We describe one such case of AIL Grade 2 and GLVFXVVWKHQHHGWRHYDOXDWHSRO\PRUSKRXVFHOOLQ¿OWUDWHLQ VXSHU¿FLDOQDVDOPXFRVDOELRSVLHV7KHGH¿QLWLYHGLDJQRVLV ZDV PDGH E\ KLVWRSDWKRORJLFDO H[DPLQDWLRQ IROORZLQJ VXUJLFDO H[HQWHUDWLRQ 7KH UHSRUW LV SUHVHQWHG DORQJ ZLWK recommendations for appropriate biopsy modality and depth so that unnecessary surgeries can be avoided.
&DVHUHSRUW $ \HDUROG PDQ SUHVHQWHG ZLWK SURJUHVVLYH ELODWHUDO nasal obstruction for 1 year, starting from the right side and then progressing to involve the left side also. He also had recurrent episodes of severe frontal headache and persistent ZDWHULQJIURPKLVULJKWH\HGXULQJWKLVSHULRG7KHUHZDVQR history of bleeding from nose or pain. 2QH[DPLQDWLRQWKHUHZDVD¿UPPDVVLQWKHULJKWQDVDO cavity covered by foul smelling slough and discharge. The PDVV ZDV QRW VHQVLWLYH WR WRXFK DQG WKH SUREH FRXOG EH LQVLQXDWHGDOODURXQGWKHPDVV7KHUHZDVEURDGHQLQJRIWKH ULJKWQDVRIDFLDOJURRYHDQGSLWWLQJHGHPDRIWKHULJKWORZHU H\HOLGDORQJZLWKSURSWRVLVRIWKHULJKWH\HEDOO7KHUHZHUH palpable bilateral multiple discrete, non-tender, mobile level ,O\PSKQRGHVHDFKFPLQPD[LPXPGLDPHWHU
Indian J Otolaryngol Head Neck Surg (January–March 2010) 62(1):92–95
$PPSXQFKELRSV\ZDVWDNHQIURPWKHQDVDOPDVV using endoscopic optical biopsy forceps, after a short FRXUVHRIRUDODQWLPLFURELDODJHQWV7KHELRSV\UHSRUWZDV VXJJHVWLYHRIDQLQÀDPPDWRU\OHVLRQ7KH&7VFDQLPDJHV )LJVKRZHGDVRIWWLVVXHPDVVLQYROYLQJWKHULJKWQDVDO FDYLW\ H[WHQGLQJ LQWR WKH QDVRSKDU\Q[ HWKPRLGDO DQG VSKHQRLGDO VLQXVHV LSVLODWHUDOO\ 7KH PHGLDO ZDOO RI WKH RUELW DSSHDUHG WR EH HURGHG DQG WKH PDVV H[WHQGHG LQWR WKH RUELW FDXVLQJ SURSWRVLV7KH QDVDO VHSWXP ZDV SXVKHG to the contralateral side. The mass also had pushed the ODWHUDO ZDOO RI WKH QRVH ODWHUDOO\ 6LQFH WKH PDVV DSSHDUHG QHRSODVWLF DQG SRVVLEO\ QRW LQÀDPPDWRU\ RQ &7 VFDQ WKH SXQFK ELRSV\ ZDV UHSHDWHG 7KH PLFURVFRS\ UHYHDOHG D OHVLRQ OLQHG E\ VWUDWL¿HG VTXDPRXV HSLWKHOLXP DQG VPDOO IRFL RI SVHXGRVWUDWL¿HG FROXPQDU HSLWKHOLXP 7KH VWURPD ZDVH[WHQVLYHO\LQ¿OWUDWHGE\QHXWURSKLOVDQGO\PSKRF\WHV DORQJZLWKDIHZPDFURSKDJHV)RFLRIO\PSKRLGDJJUHJDWHV poorly formed clusters of epitheloid cells and necrosis ZHUHSUHVHQW1RVSHFL¿FHYLGHQFHRIPDOLJQDQF\FRXOGEH determined in the given specimen. Thus only a diagnosis of DFXWHRQFKURQLFLQÀDPPDWRU\OHVLRQFRXOGEHUHQGHUHG
93
E\WKLFN¿EURWLFWLVVXH7KHWXPRUH[WHQVLRQVZHUHFDUHIXOO\ FXUHWWHGRXWDQGVHQWIRUKLVWRSDWKRORJLFDOH[DPLQDWLRQ Grossly the specimen consisted of irregular fragments of WLVVXH7KHODUJHVWPHDVXULQJîîFPZKLOHWKHVPDOO HVWZDVFPDFURVV6RPHZHUHSRO\SRLGDOLQDSSHDUDQFH &XW VXUIDFH VKRZHG D KRPRJHQRXV SDOH DSSHDUDQFH ZLWK focal gelatinous areas. Microscopy revealed a lesion lined on RQHVXUIDFHE\SVHXGRVWUDWL¿HGHSLWKHOLXPDQGDIHZIRFLRI VTXDPRXVFHOOV7KHLPPHGLDWHXQGHUO\LQJVWURPDVKRZHG H[WHQVLYH JHRJUDSKLFDO QHFURVLV ZLWK D SRO\PRUSKRXV LQ¿OWUDWHRIERWKDFXWHDQGFKURQLFLQÀDPPDWRU\FHOOVDORQJ ZLWKJUDQXODWLRQWLVVXH)LJLHWKHVXUIDFHKDGWKHVDPH DSSHDUDQFH DV WKH SUHYLRXV VPDOOHU ELRSV\ +RZHYHU WKH GHHSHU DUHDV RI WKH VWURPD VKRZHG D O\PSKRSODVPDF\WLF LQ¿OWUDWH DORQJ ZLWK PDQ\ ODUJH KLVWLRF\WH OLNH FHOOV ZLWK moderate cytoplasm and oval vesicular nuclei. Some of these cells had a clearing around them and some had K\SHUFKURPDWLF QXFOHL 1R VLJQL¿FDQW PLWRVHV ZHUH VHHQ The stains for microbes like PAS, Ziehl-Neilsen and Gram ZHUH QHJDWLYH 5HWLFXOLQ VWDLQ ZKLFK VWDLQV WKH HODVWLQ RI WKH YHVVHO ZDOOV VKRZHG GLVUXSWLRQ RI YHVVHO ZDOOV ,Q *UDGHPRVWO\PDWXUHO\PSKRF\WHVDUHVHHQZLWKVSDUVH plasma cells or lymphoblasts. In Grade 2 polymorphous LQ¿OWUDWHZLWKDIHZRUUDUHDW\SLFDOO\PSKRF\WHVDUHVHHQ ,Q *UDGH WKHUH LV DQ LQ¿OWUDWH RI DW\SLFDO FHOOV ZLWK D FDYLWDU\ OHVLRQ E\ WKH LQL¿OWUDWLQJ FHOOV 7KXV WKH ¿QGLQJV GHVFULEHGDERYHPDWFKHGZLWK*UDGHDQGWKHGLDJQRVLVRI
Fig. 1a &76FDQRIWKHSDWLHQWVKRZLQJWXPRUPDVVRFFXS\LQJ WKH ULJKW QDVDO FDYLW\ HURGLQJ WKH PHGLDO RUELWDO ZDOO WKH ODWHUDO QDVDOZDOODQGWKHQDVDOVHSWXP(b and c). $[LDO&7VFDQRIWKH SDWLHQWVKRZLQJWKHWRPRUPDVVRFFXS\LQJWKHZKROHRIWKHULJKW QDVDOFDYLW\H[WHQGLQJLQWRWKHQDVRSKDU\Q[
7KH SDWLHQW ZDV SUHSDUHG IRU VXUJHU\ DQG D ODWHUDO UKLQRWRP\ ZDV GRQH XQGHU JHQHUDO DQHVWKHVLD 7KH PDVV ZDVWKHUHDIWHUUHPRYHGE\EOXQWGLVVHFWLRQ7KHPDVVZDV IULDEOHDQGSDUWLDOO\QHFURWLFHVSHFLDOO\WKHH[WHQVLRQVLQWR the ethmoidal sinuses and at its central area. The lamina SDS\UDHFHDZDVHURGHGEXWWKHRUELWDOSHULRVWHXPDSSHDUHG uninvolved. The nasal bones and the lateral margins of the S\ULIRUPDSHUWXUHZHUHHURGHGGHPLQHUDOLVHGDQGUHSODFHG
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Indian J Otolaryngol Head Neck Surg (January–March 2010) 62(1):92–95
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'LVFXVVLRQ This patient had a localized sinonasal destructive lesion KLVWRORJLFDOO\FODVVL¿HGDV$,/*UDGH$,/DUHPRGHUDWHO\ aggressive (intermediate grade) lymphomatous lesions. They are a heterogeneous spectrum of hematolymphoid malignancies that share a particular histological characteristic, QDPHO\DQDQJLRFHQWULFRUSHULYDVFXODUJURZWKSDWWHUQ7KH\ include a variety of T-cell lymphomas, B-cell lymphomas, and natural killer-cell derived lymphomas (NK/T-cell lymphomas). ,Q WKLV OHVLRQ WKH LQÀDPPDWRU\ RXW SRXULQJ RI ERWK WKHDFXWHDQGFKURQLFLQÀDPPDWRU\FHOOVZDVWKHFDXVHRI LQLWLDOPLVGLDJQRVLVDVDQLQÀDPPDWRU\OHVLRQ7KHGDPDJH WRWKHYHVVHOZDOOFRXOGQRWEHDVFHUWDLQHGGXHWRWKHVPDOO DQG VXSHU¿FLDO ELRSV\ VDPSOH ZKLFK RQO\ VKRZHG WKH LQÀDPPDWRU\ UHVSRQVH DQG QRW WKH QHRSODVWLF SURFHVV 7R GHWHUPLQH WKH GHSWK DW ZKLFK WKLV QHRSODVWLF OHVLRQ ZDV HYLGHQWDPHDVXUHPHQWZDVGRQHXVLQJ9HUQLHUVFDOHLQWKH KLVWRORJLFDOVOLGHWDNHQDIWHUH[HQWHUDWLRQ7KLVZDVVHHQWR UDQJH EHWZHHQ WR PP IURP WKH HSLWKHOLDO RU XOFHUDWHG surface. The issues discussed are (i) The failure to locate the SXWDWLYHFHOOVLQVXSHU¿FLDOELRSVLHVLL WKHSUREOHPVUHOD ted to grading angioimmunoblastic lymphoproliferative GLVHDVHV DQG LLL WKH FULWHULD E\ ZKLFK WKH GLDJQRVLV FDQ EHDUULYHGDWEDVHGRQWKHKHPDWR[\OLQDQGHRVLQVHFWLRQV The angioimmunoblastic lymphoproliferative disease has DQH[XEHUDQWLQÀDPPDWRU\UHDFWLRQFRQVLVWLQJRIDQLQWHQVH LQ¿OWUDWH RI D PL[HG SRSXODWLRQ RI ERWK DFXWH DQG FKURQLF LQÀDPPDWRU\FHOOVSUREDEO\UHODWHGWRWKH(SVWHLQ%DUUYLUXV LQIHFWLRQZLWKZKLFKLWLVLQYDULDEO\DVVRFLDWHG>@7KHIDFW that early lesions are misdiagnosed as benign process due WR WKLV PL[HG SRSXODWLRQ RI LQÀDPPDWRU\ FHOOV KDV EHHQ documented [4]. 7KH WZR PRVW LPSRUWDQW GLDJQRVWLF FULWHULD XVHG LQ histology are angiocentricity and atypical lymphocytes. The FULWHULDIRUDQJLRFHQWULFLW\QHHGHGWREHGH¿QHGSUHFLVHO\7KH DQJLRFHQWULFLW\LVGH¿QHGDVSUHVHQFHRIWXPRUFHOOVDURXQG DQGZLWKLQYDVFXODUVSDFHVZLWKLQ¿OWUDWLRQDQGGHVWUXFWLRQ RIYHVVHOZDOO3HULYDVFXODUORFDOL]DWLRQLVQRWVXI¿FLHQWIRU angiocentricity [5]. Presence of nuclear hyperchromasia DQGSUHVHQFHRIQXFOHROLZHUHXVHGDVFULWHULDWRGHWHUPLQH DW\SLD%HFDXVHRIWKHQHFURVLVDQGRWKHULQÀDPPDWRU\FHOOV WKHVH DW\SLFDO FHOOV DUH YHU\ GLI¿FXOW WR EH LGHQWL¿HG DQG most series report multiple biopsies [5]. We hypothesized WKDWWKHGLI¿FXOW\PD\EHUHODWHGWRWKHGHSWKDWZKLFKWKHVH vessels are situated. We searched ‘Medline’ and ‘Pubmed’ for any article related to depth and vessels in relation to the DQJLRFHQWULFO\PSKRPDVRU$,/ZLWKQRUHVXOW:HWKHUHIRUH ZRUNHGRQWKH9HUQLHUVFDOHRIWKHPLFURVFRSHWRGHWHUPLQH WKH GHSWK DW ZKLFK VXFK GHVWUXFWLYH YHVVHOV DUH GHWHFWHG 7R LGHQWLI\ WKH YHVVHOV ZLWK ZDOO GDPDJH UHWLFXOLQ VWDLQ ZDVXVHG2XUUHVXOWVVKRZHGPRVWRIWKHYHVVHOVZHUHDWD
GHSWKRIEHWZHHQDQGPPIURPWKHVXUIDFHHSLWKHOLXP RU XOFHUDWLRQ 7KH WXPRU PDUNHU VWXGLHV ZHUH GRQH WR GHWHUPLQH ZKHWKHU WKH\ DUH 7 RU % RU 1. FHOOV &' (also called leukocyte common antigen) is present in all OHXNRF\WHVVRLWFRQ¿UPHGWKDWWKHQHRSODVWLFHOHPHQWVZHUH QRWHSLWKHOLDOFHOOV&'DQG&'DUHVSHFL¿FIRU7FHOOV DQG %FHOOV UHVSHFWLYHO\ 6R WKH SUHVHQFH RI &' PDUNHU FRQ¿UPHG D 7FHOO RULJLQ QHRSODVLD 7KH PHVVDJH LV WKDW LQFDVHVRIGHVWUXFWLYHVLQRQDVDOOHVLRQVLIWKHVXSHU¿FLDO ELRSV\VKRZVDPL[HGSRSXODWLRQRILQÀDPPDWRU\FHOOVLW PD\ EH SUXGHQW WR WDNH D GHHSHU ELRSV\7KH TXHVWLRQ ZH KDYH WULHG WR DQVZHU LV ZKDW VKRXOG EH WKH DSSUR[LPDWH required depth and modality of the biopsy to be taken. $VLQJOHFDVHUHSRUWFDQQRWREYLRXVO\JLYHD¿UPDQVZHUEXW LWLVLQGLFDWLYH6LQFHWKHVXUURXQGLQJFXIIRILQÀDPPDWRU\ WLVVXH LQ RXU FDVH ZDV ± PP WKLFN LW LV UHFRPPHQGHG WKDWLQVWHDGRIDSXQFKELRSV\ZLWKDQHQGRVFRSLFRSWLFDO ELRSV\ IRUFHSV ZKLFK JLYHV D YHU\ VXSHU¿FLDO WLVVXH sample (around 3 mm depth), a deeper incisional biopsy be performed of at least 10 mm depth so as to get enough tissue to visualize blood vessels in all cases of nasal masses ZKLFKVKRZHYLGHQFHRIERQHGHVWUXFWLRQFOLQLFDOO\DQGRU UDGLRORJLFDOO\7KHREYLRXVULVNRIHSLVWD[LVLVPDQDJHDEOH DQGLVDQ\ZD\PXFKOHVVVHULRXVWKDQWKRVHDVVRFLDWHGZLWK full scale surgical intervention. The look for atypia of the lymphocytes revealed only DIHZLQIUHTXHQWDW\SLFDOFHOOV3UHVHQFHRIQXFOHDUK\SHU FKURPDVLD DQG SUHVHQFH RI QXFOHROL ZHUH XVHG DV FULWHULD WRGHWHUPLQHDW\SLD+RZHYHUWKHSUHVHQFHRIQXFOHROLZDV QRW D FRQVSLFXRXV ¿QGLQJ 7KXV D *UDGH $,/ FRXOG EH ascertained. &RQFOXVLRQ Angiocentric lymphoma like all angioimmunoproliferative OHVLRQV LV DQ HQLJPDWLF FRQGLWLRQ ZKLFK LV OLNHO\ WR EH misdiagnosed on routine surgical biopsy sampling due to its VXUURXQGLQJFXIIRILQÀDPPDWRU\WLVVXH,QFDVHRIVXVSLFLRQ RIDQHRSODVWLFOHVLRQDGHHSHULQFLVLRQDOELRSV\H[WHQGLQJ VXI¿FLHQWO\EH\RQGPPIURPWKHVXUIDFHLVUHFRPPHQGHG in order to ensure a representative histopathological sample. This is especially pertinent for peripheral centers ZKHUH IDFLOLWLHV IRU GHWHFWLQJ &' PDUNHUV PD\ EH DEVHQW EXW SUHVHQFH RI VXI¿FLHQWO\ DZDUH RWRUKLQRODU\QJRORJLVW head and neck surgeons and pathologists may prevent an XQQHFHVVDU\VXUJHU\DORQJZLWKWKHDVVRFLDWHGPRUELGLW\DQG initiate treatment on the correct lines. We learnt our lessons DQGZRXOGOLNHWRVKDUHLWZLWKRWKHUV 5HIHUHQFHV 1.
Wenig BM, Pilch BZ (2000) Tumors of the upper respiratory WUDFW ,Q )OHWFKHU &'0 (G 'LDJQRVWLF KLVWRSDWKRORJ\ RI WXPRUV QG HGLWLRQ /RQGRQ &KXUFKLOO /LYLQJVWRQH
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Jaffe ES (2002) Lymphoid lesions of the head and neck: A model of lymphocyte homing and lymphomagenesis. Mod Pathol 15(3):255–263 Jaffe ES (1995) Post thymic T cell lymphoma. In, Jaffe ES (Ed.). Surgical pathology of the lymph node, 2nd edition. 3KLODGHOSKLD:%6DXQGHUV