L y m p h o m e d e B u r k i t t g a s t r i q u e :
p r o p o s d ' u n c a s e t r e v u e d e la litt( rature
Fedoua ROUIBAA, H. SEDDIK, A. AOURARH, I. SASSENOU, A. BENKIRANE, M. HACHIM, Farida TOLOUNE
Serv ice de m~dec ine A - HOpital d ' ins truct ion mil i taire M V - R a b a t (Maroc)
Burkitt's lymphoma of stomach : a case report and review of literature
RI~SUMIE
Le lymphome de Burkitt est une entit6 histologique distincte des lymphomes non Hodgkiniens. La forme sporadique est rare chez l'adulte immunocomp6tent.
Nous rapportons un cas de lymphome de Burkitt gastrique chez un patient s6ronOgatif pour le virus de l'immunod6ficience humaine, rOv616 par une hOmorragie digestive. L'aspect endoscopique Otait celui de <~ coffee cup-like ulcers >> caract6ristique de ce type de lymphome. Le diagnostic a 6t~ confirm0 par les biopsies gastriques et ganglionnaires. Le patient est d6c6d6 avant Finstauration de toute chimioth6rapie.
La localisation gastrique du lymphome de Burkitt est inhabituelle, et ses aspects endoscopiques sont encore mal d6crits. Notre travail est une revue de la littOrature du lymphome de Burkitt dans sa localisation gastro-duod6nale, ainsi que ses caractOristiques endoscopiques.
S U M M A R Y
Burkitt' s lymphoma is a distinct histologic type of non-Hodgkin ' s lymphoma. Non-endemic Burkitt' s lymphoma is rare in non-HIV adult population.
We hereby report Burkitt's lymphoma of stomach in an immuno-competent adult revealed by digestive haemorrhage. Gastric and lymph node biopsy confirmed the diagnosis. The endoscopic findings are small ulcers called "coffee cup-like" ulcers characteristically present in Burkitt's lymphoma. Patient died before starting chemotherapy.
The presentation o f Burkitt's lymphoma with gastroduodenal disease is unusual, and the endoscopic features have not been previously reported. Gastrointestinal involvement of Burkitt's lymphoma is reviewed and endoscopic characteristics are presented.
I N T R O D U C T I O N
Le lymphome de Burkitt est une prolif6ration maligne de lymphocytes de ph6notype B. C'est un lymphome agressif qui a le plus souvent un point de d6part extra ganglionnaire (mgchoire, tractus ORL, tube digestif...) [1]. Lorsqu'il touche le conduit digestif, il si~ge 61ectivement au niveau de la r6gion il6o-c~ecale, l'atteinte gastrique est extramement rare [2].
L'aspect endoscopique de ce type de lymphome, de par sa raret6, reste mal connu. Nous profitons de cette observation pour 6tayer les caract6ristiques cliniques et surtout endoscopiques du lymphome de Burkitt gastrique.
O B S E R V A T I O N
Madame O. A, ~g6e de 33 ans, est admise au service en septembre 2002, pour des 6pigastralgies avec un 6pisode de m616na, 6voluant dans un contexte d 'al t6ration de l '6tat g6n6ral, avec un amaigrissement de 10 kg en 2 mois, et une fi~vre 38 ~ L'examen clinique a r6v616 la pr6sence d'une ad6nopathie cervicale gauche mesurant 3 cm de diam6tre et une sensibilit6 6pigastrique.
Le bilan biologique a objectiv6 un syndrome inflammatoire avec une vitesse de s6dimentation
80 mm h la 1TM heure et une thrombop6nie 70 000 616ments/mm 3, la s6rologie HIV 6tait n6gative.
La fibroscopie (esogastroduod6nale a montr6 une gastrite 6ryth6mateuse avec des 16sions nodulaires 6rod6es h leur sommet, diffuses antrales et fundiques, de couleur blanc nacr6 (Fig. 1, 2). L '6tude histologique des biopsies gastriques et de l'ad6nopathie cervicale ont r6v616 la pr6sence d'une prolif6ration lymphomateuse faite de cellules de taille moyenne h noyaux ovoides hyperbasophiles nucl6ol6s avec mitoses nombreuses et pr6sence de macrophages /a corps tangibles au sein de la prolif6ration, r6alisant un aspect en << ciel 6toi16 ,> (Fig. 3, 4).
L '6tude immuno-histochimique a confirm6 le diagnostic de LB en montrant la pr6sence de cellules tumorales exprimant le CD20 et le LCA.
Le bilan d 'extension comprenant un scanner thoraco-abdominal et une biopsie ost6o-m6dullaire 6tait en faveur d 'un stade IV selon la classification d 'Ann Arbor modifi6e par Musshoff.
L'6volution a 6t6 caract6ris6e par le d6c~s de la patiente quelques jours aprbs son admission, dans un
Tir6s h part : D r Fedoua ROUIBAA - APP 20 cit6 IBN SINA, Immeuble 14, Agdal 10000 Rabat Agdal Ryad (Maroc).
Mots-clOs : adulte, estomac, immunocomp6tent, lymphome de Burkitt.
Key-words: adult, Burkitt's lymphoma, non-HIV, stomach.
Acta Endoscopica Volume 35 - N ~ 5 - 2005 775
Figure 1 Aspect endoscopique de ,, coffee cup-like ,, ulcers caracteristiques
du lymphome de Burkitt gastrique. The endoscopic features of "coffee-cup-like" ulcers characteristic
of smach Burkitt's lymphoma.
Figure 2 Infiltration diffuse de l'antre par les lesions nodulaires.
Diffuse infiltration of the antrum by the nodulaire lesions,
Figure 3 Examen histologique de la biopsie gastrique (x 20) : montrant I'aspect
,, ciel ~toilee ,, de la proliferation tumorale typique du lymphome de Burkitt. Histological examination of the gastric biopsy (• "starry sky"
lymphoid cell infiltration of Burkitt's lymphoma.
Figure 4 Examen histologique de la biopsie gastrique (x 40) : proliferation lympho-
mateuse realisant t'aspect en ,, ciel etoilee ,,. Histological examination of the gastric biopsy (• "starry sky"
lymphoid cell infiltration of Burkitt's lymphoma.
tableau de d6faillance multivisc6rale (insuffisance h6patocellulaire et r6nale) avant l'instauration d'une chimioth6rapie.
DISCUSSION
Les localisations digestives repr6sentent 12,5 % de l 'ensemble des lymphomes non hodgkiniens (LNH) et sont les plus fr6quentes des formes extra ganglionnaires (36 %) [1].
Dans les pays occidentaux, les localisations gastriques sont les plus souvent rencontr6es, suivies de celles du gr61e et du c61on-rectum [1-3].
Selon la classification d'Isaacson, les lymphomes B du type MALT sont les plus cornmuns ; ils peuvent ~tre de bas grade ou de haut grade. Par ailleurs, Le lymphome de Burkitt est une entit6 histologique bien distincte qui reste plus rare [2]. C'est un lymphome cellule B de haut grade de malignit6. I1 est plus r6pandu en Afrique o0 il survient sous forme end6mique, surtout chez le garqon autour de 5 ans. Les formes sporadiques int6ressent l 'Europe et l 'Am6rique [4-5].
Le lymphome de Burkit t est une affection rare chez l 'adulte immunocornp6tent. I1 repr6sente uniquement 1 ~ 2 % de tout les LNH [6]. En cas d ' immunod6pression li6e h l ' infection par le VIH, cette pr6valence atteint les 35-40 % [6].
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TABLEAU I
LYMPHOMES DE BURKITr ~ LOCALISATION GASTRIQUE RAPPORTI~S
DANS DIFFI~RENTE SI~RIES DE LNH
Brooks 1975 [6] Lewin 1978 [7] Dragosis 1985 [8] Sen Gupta 1990 [9]
Nombre Si~ge Burkitt LNH gastrique gastrique
58 117 133 42
58 48
10
0 1
11 0
D a n s sa f o r m e s p o r a d i q u e , la l oca l i s a t i on d iges t ive la p l u s c o u r a n t e e s t c e l l e d u c a r r e f o u r i l6o-c~ecal. L a l o c a l i s a t i o n g a s t r i q u e e s t e x t r a m e m e n t r a r e ( T a b l e a u I).
L ' a s p e c t e n d o s c o p i q u e t y p i q u e d u L B r e s t e d i f f 6 r e n t d e s a u t r e s l y m p h o m e s m a l i n s . Si l ' a s p e c t h a b i t u e l des l y m p h o m e s g a s t r i q u e s est s o u v e n t ce lui d ' u n ulc~re avec d e s b e r g e s tr~s sur61ev6es, associ6 ~i u n e m u q u e u s e g a s t r i q u e i r r6gu l i~ re et m a m e l o n n 6 e ; e n cas d e L B g a s t r i q u e , o n n o t e la p r 6 s e n c e de m u l t i p l e s pe t i t s u lc~res m e s u r a n t 3-5 m m de d i a m 6 t r e avec u n e b o r d u r e n o d u l a i r e , a p p e l 6 s ~ u lc~re en tasse
c a f 6 ~ [10-12]. M a i s s e l o n P r i b e , ce t a s p e c t e n d o s c o p i q u e c a r a c t 6 r i s t i q u e p e u t v a r i e r s e l o n le deg r6 de l ' i n f i l t r a t i o n e t l ' e x t e n s i o n t u m o r a l e [13] ; et p e u t 6 g a l e m e n t c h a n g e r ap r~s c h i m i o t h 6 r a p i e et p a r c o n s 6 q u e n t , l a r 6 c i d i v e d e l a m a l a d i e p r e n d r a un a s p e c t e n d o s c o p i q u e d i f f6 ren t [12].
C h e z n o t r e p a t i e n t , l ' a s p e c t e n d o s c o p i q u e r e s t e p a r t i c u l i e r ; en ef fe t , l ' i n f i l t r a t i o n t u m o r a l e 6ta i t dif- f u se 5 t o u t l ' e s t o m a c , s o u s f o r m e d e 16sions n o d u - l a i r e s , u l c 6 r 6 e s fi l e u r s o m m e t , r a p p e l a n t l ' a s p e c t d '~ u lc~res en t a s se h caf6 ~ m a i s se d i s t i n g u a n t de la
m u q u e u s e g a s t r i q u e p a r c e t t e c o u l e u r b l a n c nac r6 qu i n ' a j a m a i s 6t6 r a p p o r t 6 e d a n s la l i t t 6 ra tu re .
L a c o n f i r m a t i o n d i a g n o s t i q u e e s t a p p o r t 6 e p a r l ' 6 t u d e h i s t o l o g i q u e e t i m m u n o h i s t o c h i m i q u e q u i m o n t r e u n e i n f i l t r a t i on m o n o t o n e p a r des ce l lu les d e t a i l l e m o y e n n e , d o n t les n o y a u x c o n t i e n n e n t p l u - s ieurs nuc l6o l e s associds ~ d e n o m b r e u x m a c r o p h a g e s
co rps t ang ib l e s , r e s p o n s a b l e s d e l ' a s p e c t ca rac t6 r i s - t i q u e en ~ c ie l 6toi16 ~. L e s ce l lu l e s t u m o r a l e s exp r i - m e n t une Ig M, C D 2 0 et le p lus s o u v e n t le C D 1 0 [12].
L e t r a i t e m e n t d o i t ~ t r e i n s t i t u 6 d ' u r g e n c e v u la r a p i d i t 6 de la c ro i s s a nc e t u m o r a l e . I1 cons i s t e en u n e c h i m i o t h 6 r a p i e s y s t 6 m i q u e i n t e n s i v e ( c y t a r a b i n e , d o x o r u b i c i n e , c y c l o p h o s p h a m i d e , a n t h r a c y c l i n e ) a s s o c i a n t u n e c h i m i o t h 6 r a p i e i n t r a t h 6 c a l e ~ v i s 6 e p r 6 v e n t i v e ou cura t ive . L a c h i r u r g i e n ' a p a s d e p l a c e e n d e h o r s d ' u n e c o m p l i c a t i o n ( h 6 m o r r a g i e , o c c l u - s ion) [14].
L e p r o n o s t i c d e la m a l a d i e r e s t e sdv6re ; e n e f fe t , p o u r n o t r e p a t i e n t e , le d6c6s es t s u r v e n u a v a n t d e p o u v o i r d 6 m a r r e r u n e c h i m i o t h 6 r a p i e . M a i s l ' a v ~ - n e m e n t d e n o u v e l l e s m o l 6 c u l e s , n o t a m m e n t l e s a n t i c o r p s m o n o c l o n a u x , l a i s s e e s p 6 r e r u n a v e n i r m e i l l e u r [14].
C O N C L U S I O N
L e l y m p h o m e de B u r k i t t es t un l y m p h o m e agress i f . L a l o c a l i s a t i o n g a s t r i q u e es t e x c e p t i o n n e l l e ma i s e l le r e s t e c a r a c t 6 r i s 6 e e n d o s c o p i q u e m e n t p a r l ' a s p e c t t y p i q u e d e s u l c ~ r e s d i t s ~ e n t a s s e g ca f6 ~. S o n d i a g n o s t i c es t h i s t o l o g i q u e e t i m m u n o h i s t o c h i m i q u e . S o n p r o n o s t i c es t s o m b r e , e t la su rv ie r e s t e m e n a c 6 e t o u t m o m e n t p a r les c o m p l i c a t i o n s p r o p r e s ~ la m a - l a d i e e t ce l les de la c h i m i o t h 6 r a p i e .
Rt~FI~RENCES
1. Ruskone-Fourmestraux A, Lavergne-Slove A, Delmer A. Les lymphomes gastro-intestinaux. Gastroenterol Clin Biol 2002; 26 : 233-41.
2. Isaacson PG. Les lymphomes gastro-intestinaux : classification et pr6sentation anatomoclinique. Acta Endoscopica 1998 ; 28 : 479-87.
3. De Mascarel A, Belleannee G, Parrens M. Les lymphomes de l'intestin : Aspects anatomo-pathologiques. Acta Endoscopica 1998 ; 28 : 547-55.
4. Collins J. Katon R. Harty-Golder B. Burkitt's lymphoma pre- senting with gastroduodenal involvement : Endoscopic des- cription and review of the literature. Gastroenterology 1983 ; 85 : 425-9.
5. Sharma A, Raina V, Gujral S, Kumar R, Tandon R, Jain P. Burkitt's lymphoma of stomach : a case report and review of literature. American Journal of Hematology 2001 ; 67 : 48-50.
6. Olinie CD. Vasiu R. Gastric Burkitt's lymphoma : case report and review of the literature. Morphol Embryol (Bucur) 1990 ; 36 : 39-41.
7. Lewin K, Ranchod J, Dorfman M. Lymphoma of the GIT : a study of 117 cases presenting with gastrointestinal disease. Cancer 1978 ; 42 : 693-707.
8. Dragosis B, Bauer P, Radaszkiewies T. Primary gastrointesti- nal non-Hodgkin's lymphoma : A retrospective clinicopatholo- gic study of 150 cases. Cancer 1985 ; 55 : 1060-73.
9. Sen Gupta SK, Sinha SN. Clinicopathologic features of pri- mary gastrointestinal lymphoma : a study of 42 eases. Aust NZ J Surg 1991, 6 : 133-6.
10. Delmotte JS. Les lymphomes, aspects endoseopiques. Acta Endoscopica 1998 ; 28 : 489-93.
11. Michael Pruebe W. The endoscopic appearance of Burkitt's lymphoma involving the stomach and colon. Gastrointestinal Endoscopy 1986 ; 32 : 352-3.
12. Miyaguchi S, Hibi T, Kanai T, Tashiro H, Suematsu M, Gue- vara FM, Suchiya MT. Burkitt's Lymphoma of the Stomach : A case presenting with a cubmucosal tumor and coffee-cup-like ulcers. Endoscopy 1993 ; 25 : 494-5.
13. Pribe WM. The endoscopic appearance of Burkitt's lymphoma involving the stomach and colon. Gastrointest Endoscopy 1986; 32 : 352-62.
14. Magrath IT, Adde M, Shad A, Venzon D, Seibel N, Gooten- berg J, Neely J, Arndt C, Wittes RA, Horak ID. Adult and children with small non-cleaved cell lymphoma have a similar excellent outcome when treated with the same chemotherapy regime. J Clin Oncol 1996 ; 14 : 425.
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I N T R O D U C T I O N
Burkitt' s lymphoma & a B-cell high-grade lymphoma. It is an aggressive lymphoma and the starting point is usually extra-nodaL When the gastrointestinal tract is involved, the most common site o f involvement is the ileocecal area. The stomach is rarely involved.
Endoscopic descriptions o f Burkitt's lymphoma are not well known, because o f its rarity. We report this case, in order to review clinical and endoscopic characteristics o f gastric Burkitt's lymphoma.
CASE R E P O R T
A 33-year old female was admitted in our hospital in September 2002 with a 2-month history o f epigastric pain, melena, weight loss o f lO kg and fever (38~
Clinical examination revealed a 3 cm left supra- clavicular lymph node and abdominal palpation revealed epigastric pain.
The biological test showed an inflammatory syndrome with a sedimentation rate o f 80 m m at the first hour, thrombopenia with 70 000 platelets/mm 3. H I V serology was negative.
Upper gastrointestinal endoscopy demonstrated an erythematous gastritis with diffuse infiltration o f the fundus and antrum by white nodular lesions excavated on their top.
Histological examination o f the endoscopic gastric biopsy and the lymph node revealed the so called "starry sky" lymphoid cell infiltration consisting o f intermediate cells with many macrophages in the cytoplasm. These intermediate cells had ovoid basophilic nucleus with multiple mitosis. Immu- nohistochemistry confirmed the diagnosis o f Burkitt's lymphoma (CD20 and leukocyte common antigen positivity). Abdomina l computed tomography scan and bone marrow biopsy specimens showed a stage IV lymphoma according to the Ann-Arbor classification modified by Musshof f
The patient developed multivisceral failure and died within few days before any chemotherapy could be set up.
DISCUSSION
Primary gastrointestinal lymphoma are among the most common extranodal lymphomas accounting for 36% and about 12,5% o f all non Hodgkin 's lymphomas (NHL) [1]. In western countries, the most common site involved is the stomach followed by the small intestine and colon-rectum [1-3].
According to the Issacson classification, B-cell lymphomas o f Malt type are the most common and may be o f low or high grade. Burkitt's lymphoma is a rare and distinct histological entity [2]. Burkitt 's lymphoma is a B-cell high-grade lymphoma that is endemic in Africa, especially in children. Sporadic cases are reported in America and Europe [4-5].
Burkitt 's l ymphoma is rare in immunocompetent adult. It represents only 1-2% of aU N H L [6]. However, in the setting o f H I V infection, Burkitt's lymphoma may account for about 35-40% o fa l l N H L patients [6].
TABLE I
NUMBER OF GASTRIC BURKITT'S LYMPHOMAS R E P O R T E D IN DIFFERENT STUDIES
Author Year (ref) Number Stomach Gastric Burkitt 's
Brooks 1975 [6]
Lewin 1978 [7] Dragosis 1985 [8]
Sen Gupta 1990 [9]
58
117 133
42
58
48
10
0
1
11
0
For non-endemic Burkitt's lymphoma, the ileocecal area represents the most common site o f involvement o f gastrointestinal tract. Burkitt 's lymphoma o f stomach is extremely rare in adults (Table I).
Typical endoscopic aspects in BL are different from other malignant lymphomas. The usual endoscopic findings in malignant lymphoma are ulcers with high margins and mucosal irregularity. In Burkitt 's lymphoma, small ulcers 3-5 m m in diameter with nodular borders so called "coffee cup-like" ulcers are characteristically present [10-12]. The endoscopic features o f Burkitt's lymphoma can vary, depending on the area involved and the degree o f tumor infiltration or extension, as has been suggested by Pribe [13]. Furthermore, the endoscopic features can also be modif ied by chemotherapy and subject to further change on recurrence o f the disease [12].
For our patient, the endoscopic aspect was particular. In fact, there was a diffuse infiltration o f all the stomach by nodular lesions excavated on their top reminding the aspect o f "coffee cup-like ulcers". These lesions were characterised by their white colour which has never been reported in literature.
Histological examination and immunochemistry confirm the diagnosis. It shows monotonous infiltration by intermediate cells, o f which nucleus presents multiple nuclei, with many macrophages realizing the classical "starry sky" appearance o f Burkitt's lymphoma. Tumorous cells express surface immunoglobulin (IgM), CD20 and more often CDIO [12].
Treatment must be started as soon as possible because of the rapid growth o f the tumor. It consists o f intensive chemotherapy (Cyclophosphamide, doxorubicin, cytarabine, anthracycline) including prophylactic or curative intrathecal chemotherapy. Surgery is limited to complicated cases (obstruction, bleeding) [14].
The prognosis o f disease remains dismal Our patient died before any chemotherapy could be started. The use of new drugs such as monoclonal antibodies is promising for a better outcome [14].
C O N C L U S I O N
Burkitt's lymphoma is a malignant lymphoma. Stomach involvement is exceptional and & characte- rised by the typical endoscopic aspect o f "coffee cup- like ulcer". The histological examination and immunochemistry allows for the diagnosis. The pro- gnosis is fatal and the survival is threatened at any moment by the risk o f complications due to the disease itself and to chemotherapy.
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