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Case R eport
Clinical manage me nt of six case s of low-risk
primary tonsillar non-Hodgkin´s lymphoma
Discipline of Hematology and Hemotherapy,
Universidade Federal de São Paulo/Escola Paulista de Medicina, São Paulo, Brazil
Gisele Wally Braga Colleoni, José Salvador Rodrigues Oliveira, Antonio Correa Alves, Davimar Miranda Maciel Borducchi, Roberto Araújo Segreto, Onivaldo Cervantes, José KerbauyINTRODUCTION
W a ld e ye r´ s ring is the se c o nd mo st c o mmo n site o f extra -no da l lympho ma s in the g a stro intestina l tra c t.1 Amo ng the no n-Ho dg kin lympho mas (N HL) fo und in W aldeyer´s ring , the to nsils a re the prima ry lo c a tio n fo r the disea se in 8 0 % o f the cases.2 Mo st o f them have recently b een rec o g niz ed a s MALT (muc o sa -a sso c ia ted lympho id tissue),3 whic h sho w a pro g ressio n fro m lo w -g ra d e to hig h-g ra d e lymp ho ma indisting uisha b le fro m o ther hig h-g ra de B-c ell lympho ma s. The o rig in o f the tumo r ma y b e defined in 3 0 -4 0 % o f c a ses, b ut o nly if residua l a rea s o f lo w-g ra de lympho ma c a n b e identified in the b io psy spec imens.1 , 4
In the last few years, there have been many repo rts tha t fa vo r a g g ressive systemic trea tment with c hemo thera py a nd ra dio thera py, even fo r suc h well-lo c a liz ed lympho ma s, a vo iding the need fo r to nsillec to my o f the no rma l to nsil.2 ,5 ,6
CASE REPORT
W e repo rt six c a ses o f prima ry to nsilla r no n-Ho dg kin´s lympho ma , dia g no sed b etween Ma rc h 1 9 8 6 a nd July 1 9 9 6 . There were five ma le p a tie nts a nd o ne fe ma le , w ith a g e s
ABSTRACT
Contex t: There ha ve b een ma ny repo rts tha t fa vo r a g g ressive systemic trea tment with c hemo thera py a nd ra dio thera py, even fo r well-lo c a liz ed lympho ma s, a vo iding the need fo r to nsillec to my o f the no rma l to nsil.
Ca se Report: W e repo rt six cases o f primary to nsillar lympho ma with a median patient ag e o f 4 2 years. There were two lympho ma cases with diffuse larg e cells, two cases with mixed small and larg e cells, o ne with small cells and o ne indeterminate. They were treated with six cycles o f chemo therapy and cervical radio therapy. All patients achieved durable co mplete remissio n. O ur data ag ree with previo us repo rts that sug g ested that primary to nsillar hig h-g rade B-cell N HL has a h-g o o d pro h-g no sis if ah-g h-g ressively treated.
Key w ords: N o n-Ho dg kin´s lympho ma. To nsil. Treatment. Pro g no sis.
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ra ng ing fro m 2 0 to 6 4 yea rs o ld (a media n o f 4 2 yea rs o ld) (Ta b le 1 ). In a c c o rda nc e with the W o rking Fo rmula tio n c la ssific a tio n, there were two diffuse la rg e c ell lympho ma s, two diffuse mixe d sma ll a nd la rg e c e ll lympho ma s, o ne sma ll lympho c ytic lympho ma a nd o ne c o uld no t b e c la ssified due to intense to nsil nec ro sis. At the time o f this stud y, e mb e d d e d p a ra ffin spec imens were o nly a va ila b le fo r three c a ses (c a se s 1 , 3 a nd 4 ). The y w e re re vie w e d a c c o rding to the REAL c la ssific a tio n3 a nd the d ia g no se s w e re ma inta ine d . C a se 6 w o uld pro b a b ly b e rec la ssified a s MALT lympho ma (Ta b le 1 ).
Fo ur pa tients were sta g ed a s IIA (pa la tine to nsil and cervical adeno meg aly) and two were sta g ed a s IA o r B.7 All o f them were neg a tive fo r HIV.
The pa tients were trea ted with six c yc les o f c hemo thera py (two c a ses with BACO P, o ne with CHO P-Bleo and the three mo re recent cases w ith Pro M A C E-C yta BO M ) a nd a ll p a tie nts exc ept c a se 6 rec eived c ervic a l ra dio thera py (4 0 0 0 c G y), preferentia lly b etween the third a nd fo urth c yc les o f c hemo thera py.
A ll p a tie nts a c hie ve d re missio n w ith c o mb ine d the ra p y. Five p a tie nts w e re in c o mplete remissio n, ha ving b een fo llo wed up fo r 1 5 , 1 7 , 2 0 , 6 1 and 1 3 5 mo nths by O cto ber 1 9 9 7 . O ne o f them wa s lo st fro m the fo llo w-up a t 2 9 mo nths a fter dia g no sis (c a se 2 ).
O ur first c a se s w e re tre a te d w ith c o nve ntio na l sc he d ule s (C HO P-Ble o a nd BAC O P) witho ut C N S pro phyla xis. The la te r three cases (o ne case o f fast g ro wing tumo r and
to nsil ne c ro sis a nd tw o c a se s o f la rg e c e ll ly mp ho ma ) w e re tre a te d w ith Pro M A C E-Cyta BO M, a nd were sub mitted to fo ur mo nthly intra the c a l infusio ns o f me tho tre xa te a nd dexa meta so ne, b ec a use the disea se wa s c lo se to the CN S and their histo lo g y sug g ested a mo re a g g ressive disea se, with a hig her pro b a b ility o f rela pse.
DISCUSSION
N o ne o f o ur pa tients were sub mitted to to nsille c to my o f the no rma l p a la tine to nsil b eca use we b elieved tha t cervica l ra dio thera py wa s eno ug h to prevent lo c a l rela pse.2 ,5 ,6
Ba se d o n the fa c t tha t a pro po rtio n o f pa tients with to nsilla r N HL ma y rela pse in the g astro intestinal tract,1 we have been perfo rming endo sc o pic exa mina tio ns a nd b io psies o f the sto ma c h every 6 mo nths up until five yea rs a fter dia g no sis.
The re sults fro m o ur p a tie nts a re in a c c o rda nc e with Endo et a l,2 who a na lyz ed 3 8 c a ses o f prima ry to nsilla r N HL a nd c o nc luded tha t in p a tie nts w ith sta g e I o r II to nsilla r ly mp ho ma s w ith b ulky tumo r ma ss, c hemo thera py fo llo wed b y ra dio thera py mig ht b e the c ho ic e o f trea tment.
Mo reo ver, Barista et al8 believed that stag e II to nsilla r N HL with a g g ressive histo lo g y c o uld b e trea ted with a c o mb ined thera py. In fa c t, the g ra d e s o f ma lig na nc y, sta g e a nd tumo r burden are the mo st impo rtant pro g no stic facto rs in to nsilla r N HL.2 ,8
Ho wever, co nsidering o ur small number o f
Ta ble 1 – Pa tients, histologic subtype, trea tment a nd follow -up
Patient Ag e Sex Stag e Histo lo g y Treatment Fo llo w-up Risk facto r 1 4 4 M IB Lympho ma+ N ecro sis Pro mace-Cytabo m + RT CR 1 5 + Lo w 2 4 0 M IIA DSLCL BACO P + RT CR 2 9 * Lo w 3 3 6 M IIA DLCL Pro mace-Cytabo m + RT CR 1 7 + Lo w 4 5 6 M IIA DLCL Pro mace- Cytabo m + RT CR 2 0 + Lo w 5 2 0 M IA DSLCL CHO P-Bleo + RT CR 6 1 + Lo w 6 6 4 F IIA SLL BACO P CR 1 3 5 + — * lo st fro m fo llo w-up; CR = co mplete remissio n; DSLCL = diffuse small and larg e cell lympho ma; DLCL = diffuse larg e cell lympho ma; SLL = small lympho cytic lympho ma
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patients, we decided to apply the internatio nal ind e x o f the Inte rna tio na l N o n-Ho d g kin´ s Lymphoma Factors Project 9 ,1 0 fo r the five aggressive N HL included in this repo rt (Table 2 ). All o f them were sco red as lo w-risk (Table 1 ), with pro bable disease-free survival o f 7 0 % after five years.
O ur data ag reed with previo us repo rts that sug g ested tha t prima ry to nsilla r hig h-g ra de B-c ell N HL ha s a g o o d pro g no sis if a g g ressively tre a te d w ith c o mb ine d c he mo the ra p y a nd ra dio thera py.
O ne po ssible explanatio n fo r this behavio r is the o rig in o f the tumo r, c o ming fro m a lo c a liz e d a nd no na g g re ssive muc o sa -a sso c i-a ted lympho id tissue (MALT) lympho m-a .
REFERENCES
1. Wright DH. Lym p ho m as o f Wald e ye r´s ring. His to p atho lo gy 1994;24:97-9.
2. Endo S, Kida A, Sawada U, et al. Clinical analysis o f malignant lympho mas o f to nsil. Acta Oto laryngo l (Sto ckh) 1996;523:263-6. 3. Harris NL, Jaffe ES, Stein H, et al. Revised Euro pean-American
c las s ific atio n o f lym p ho id ne o p las m s : a p ro p o s al fro m the internatio nal lympho ma study gro up. Blo o d 1994;84:1361.
4. Menarguez J, Mo llejo M, Carrio n R, et al. Waldeyer ring lympho mas: a clinico patho lo gical study o f 79 cases. Histo patho lo gy 1994;24:13-22.
Ta ble 2 - N umber ofrisk fa ctors present in a
ggres-sive N HL, compa red w ith proba bility of complete
remission (CR) a nd five-yea r surviva l (SV)1 0
Pro g no stic Risk CR rate 5 year SV lo w (0 o r 1 facto r) 8 7 % 7 3 % lo w-intermediate (2 facto rs) 6 7 % 5 1 % lo w-intermediate (2 facto rs) 5 5 % 4 3 % hig h (4 o r 5 facto rs) 4 4 % 2 6 % Risk facto rs co nsidered: age, perfo rmance status, disease stage, LDH (lactic dehydro g enase) level, number o f extra-no dal sites.
5. Barto n JH, Osb o rne BM, Butler JJ, et al. No n-Ho dgkin´s lympho ma o f the to nsil: a c linic o p atho lo gic al stud y o f 65 c ase s. Canc e r 1984;53:86-95.
6. Fu j ita n i T, Ta ka h a ra T, Ha tto ri H, Im a j o Y, O g a s a wa ra H. Rad io c he m o the rap y fo r no n-Ho d gkin´s lym p ho m a in p alatine to nsil. Cancer 1984;54:1288-92.
7. Carb o ne P, Kaplan HS, Musho lf K. Re po rt o f the co mmitte e o f Ho d g kin ´s d is e a s e s ta g in g c la s s ific a tio n . Ca n c e r Re s 1971;31(11):1860-1.
8. Barista I, Tekuzman G, Güllü I, et al. No n-Ho dgkin´s lympho mas o f the to nsil: a retro spective analysis o f twenty-eight patients with primary to nsillar lympho ma. Tumo ri 1995;81:234-7.
9. Shipp MA, et al. A predictive mo del fo r aggressive no n-Ho dgkin´s lym p h o m a : th e In te rn a tio n a l N o n - Ho d g kin ´s Lym p h o m a Pro gno stic Facto rs Pro ject. N Engl J Med 1993;329(14):987-94.
10. Ship p MA. Pro g no s tic fac to rs in ag g re s s ive no n- Ho d g kin´s lympho ma: who has high-risk disease? Blo o d 1994;83(5):1165-73.
Gisele W a lly Bra ga Colleoni –Asso ciate pro fesso r, Discipline o f Hemato lo g y e Hemo therapy, Universidade Federal de São Paulo / Esco la Paulista de Medicina.
José Sa lva dor Rodrigues O liveira – Asso ciate pro fesso r, Discipline o f Hemato lo g y e Hemo therapy, Universidade Federal de São Paulo / Esco la Paulista de Medicina.
Antonio Correa Alves – Asso ciate pro fesso r, Departament o f Pato lo g y, Universidade Federal de São Paulo / Esco la Paulista de Medicina.
Da vim a r M ira nda M a ciel Borducchi – Po stg raduate, Discipline o f Hemato lo g y e Hemo therapy, Universidade Federal de São Paulo / Esco la Paulista de Medicina.
Roberto Ara újo Segreto –MD, PhD. Pro fesso r, Discipline o f Radio therapy, Universidade Federal de São Paulo / Esco la Paulista de Medicina.
O niva ldo Cerva ntes – Asso ciate pro fesso r, Head and N eck Surg ery Departament, Universidade Federal de São Paulo / Esco la Paulista de Medicina.
José Kerba uy – MD, PhD. Full pro fesso r, Discipline o f Hemato lo g y e Hemo therapy, Universidade Federal de São Paulo / Esco la Paulista de Medicina.
Sources of funding: N o t dec la red
Conflict of interest: N o t dec la red
La st received: 2 4 Ma rc h 1 9 9 8
Accepted: 1 2 Feb rua ry 1 9 9 9
Address for correspondence:
G isele W a lly Bra g a Co lleo ni
Rua Bo tuc a tu, 7 4 0 – 3 º a nda r – Hema to lo g ia Sã o Pa ulo / SP – Bra sil - CEP 0 4 0 2 3 -9 0 0
RESUMO
Contex to: Muito s trabalho s têm pro po sto tratamento sistêmico ag ressivo co m quimio terapia e radio terapia para o s linfo mas de to nsila palatina, mesmo tratando -se de tumo res bem lo caliz ado s, sem a necessidade de amidalecto mia co ntralateral.
Rela to de Ca so: N ó s relatamo s seis caso s de linfo ma primário de to nsila palatina, co m idade mediana de 4 2 ano s. Havia do is caso s de linfo ma difuso de g randes células, do is caso s de linfo ma de pequenas e g randes células, um caso de linfo ma linfo cítico de pequenas células e um caso indeterminado . Fo ram tratado s co m seis ciclo s de quimio terapia e radio terapia cervical. To do s o s pacientes ating iram remisão co mpleta mantida. N o sso s dado s estão de aco rdo co m relato s prévio s que sug erem que o s linfo mas de células B têm bo m pro g nó stico se ag ressivamente tratado s.
Pa la vra s-cha ve: Linfo ma não -Ho dg kin. To nsila palatina. Tratamento pro g nó stico .
