Indian J Pediatr 1987; 54 : 859-862

Characteristics of Burkitt's lymphoma in India V.P. Choudhry, R.K. Agarwal and R.K. Marwah*

Department o f Pediatrics, Al l India Inst i tute o f Medical Sciences, New Delhi and *Department of" Pediatrics, Postgraduate Insti tute o f Medical

Educatzon and Research, Chandigarh

Clinical presentation o f Burkitt's lymphoma in India does not resemble either endemic or non-endemic areas. Jaw involvement was observed in 27 of 52 (51" 9%) cases with Burkitt's lymphoma on retrospective analysis o f published cases along with 14 of our cases. Higher incidence o f jaw involvement is unusual as compared to the pattern o f Burkitt's lymphoma of non-endemic areas o f the world. Abdominal involvement was seen in 30 o f 52 patients (58.8%) in India. Burkitt's lymphoma in India presents with jaw and abdominal involvement in almost equal number and this may be the third mode of clinical presentation.

Key words : Burkitt's lymphoma; Jaw involvement; Abdominal involvement.

Dennis Burkitt from Africa observed predominance of jaw involvement in lymphoma with a distinct morphological features) Lymphoma with primarily abdo- minal involvement but with similar his- tological features has been seen in other parts of the world (non endemic area). 2-4 The prevalence of Burkitt 's lymphoma among series of non Hodgkin lymphoma from non endemic areas varied between 0-56.6%. 5.9

Pattern of Burkitt 's lymphoma from this country is not well documented since only few cases have been reported as either case reports or as very small series. Fourteen cases of Burkitt 's lymphoma have been seen at All India Institute of Medical Sciences over the last eight years. All these cases along with thirty eight cases published from this country were

Reprint requests : Dr. V.P. Choudhry, Associate Professor, Department of Pediatrics, All India Institute of Medical Sdences, New Delhi 110029.

analysed to study the clinical presenta- tion of Burkitt 's lymphoma in India (three of our cases were documented earlier)3-33

Material and Methods

Fifty two cases of Burkitt's lymphoma including 14 of ours constituted the subjects of the present study.

Results

The ages varied between nine months to 70 years with mean of 10.33 years. Male to female ratio was 6" 66 : 1. The male predominance was seen only in cases below 13 years (M : F : 1 3 : 1 ) while Burkitt 's lymphoma was more common in women of over 13 years age ( M : F : 2 : 3 ) . The pattern of organ involvement is depicted in Table I.

Abdominal involvement was seen in 30 (58.8~o), abdominal pain in 16 (30.8~,)

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860 THE INDIAN JOURNAL OF PEDIATRICS Vol. 54, No. 6

and vomiting in 21 (40.4%) cases. Three patients presented with intestinal obstruc- tion. Diagnosis of Burkitt 's lymphoma was made on laparotomy in 20 subjects. Various organ involvement in these cases along with organ involvement in six patients on autopsy (two patients had earlier laparotomy) is given in Table II.

Table I. Clinical presentation of Burkitt's lymphoma in India

Site of lesion No. ~o n----52

Facial bones 18 34" 6 Abdominal viscera 21 40.4 Facial and abdominal 9 17" 3 Peripheral lymphnodes 7 13" 4 Facial abdominal & other 2 3.8 bones Others* 1 1' 95

*Soft tissue tumor from thigh

Table IL Abdominal involvement on laparotomy and autopsy

Organ involved Laparotomy Autopsy

No. of cases 20 6 Gastrointestinal tract I0 5 --upper 3 2 --lower 7 3 Mesentry and omentum 9 1 Mesentrie and para-aortie 12 2 lymphnodes Ascites 9 1 Kidney 0 5 Ovary 3 0 Adrenal 0 3 Pancreas 1 2 Liver - - 2 Spleen - - 2 Diaphragm - - 1

Facial bones were involved in 27 (51 "9,%o) cases. In 11 cases (21 "0,%o) tumor orignated f rom maxila while it originated from mandible in nine cases. More than one facial bone was involved in seven patients. Proptosis was seen in one while palate was involved in two patients. Facial bones were involved more often in younger patients as jaw involvement was seen in only two patients beyond 13 years as compared to 17 of 41 (41.4~o) children below 13 years of age.

Bone marrow and central nervous system was involved in two and one patients respectively. Systemic symptoms like fever, anorexia and weight loss were seen in 16 patients (30-8yo).

Discussion

Two distinct patterns of Burkitt 's lymphoma have been described. First presentation was described by Dennis Burkitt f rom South Africa where tumor predominant ly involved the facial bones. 1 Second mode of presentation was from non-endemic African and non-African areas with predominant abdominal in- volvement.34-37 Inspite of many environ- mental similarities like humidity, tem- perate climate, prevalence of infections (malarial or otherwise), altitude in some parts of this country, Burkitt 's tumor remains a rari ty in India.

Endemic Burkitt 's lymphoma is characteristically seen over broad ' lympho- matous belt ' extending across the tropical Africa. 3a Endemic Burkitt 's lymphoma occurs over areas with a rainfall exceed- ing 20 inches per year with a mean tem- perature over the year above 60~ and at altitudes of less than 5,000 feet. 39 Jaw involvement accounts for 56-72% of Burkitt 's lymphoma in endemic areasl, 41

CHOUDHRY ET AL : BURKITT'S LYMPHOMA IN INDIA 861

while j aw involvement was seen in 4-35"7% while j a w invo lvemen t was seen in 4-35.7% of cases over endemic areas. 36-37

In o ther areas, the t u m o r occurs spo rad i - ca l ly (non-endemic) . Assoc ia t ion o f EB virus was seen in 96% of cases in Af r i ca and New Guinea,40 while in non endemic areas, the Burk i t t l y m p h o m a was asso- c ia ted with EB virus between 20-30%36 o f cases. S imi la r ly the c h r o m o s o m a l abe r ra t ions t (8-14q), t (8-22) have been observed in subjects f rom endemic and non-endemic areas. 4x

Pa t t e rn of cl inical involvement in our coun t ry shoa ld have resembled non- endemic areas. Surpr is ingly the p resen t s tudy revealed t ha t 51 .9% of cases h a d facial involvement e i ther a lone or a long with abdomina l lesion. In con t r a s t to endemic areas, onset o f t u m o r was in o lder ch i ldren a n d the systemic s y m p t o m s were seen in 30 .8% of cases. However , involvement o f centra l nervous system, bone m a r r o w a n d p leura were infrequent . A b d o m i n a l involvement in 57-6% of cases a long with pauc i ty of CNS involve- ment in present series is ak in to the non- endemic areas.2,3,8,34,35,42

Whi le the p re sen t a t i on with j a w in- volvement in over 50% of cases is s imi la r to endemic areas , the cl inical p resen ta - t ion o f Burk i t t ' s l y m p h o m a in I n d i a has a lmos t equal invo lvement o f facial bones and a b d o m i n a l organs . These observa- t ions are o f grea ter ep idemio log ica l a n d e t io logical significance which needs to be s tudied fur ther .

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