Vol.8, No.2, Summer/Fall 2008

NETWORK……… THE NEWSLETTER OF THE INTERNATIONAL NETWORK FOR CANCER TREATMENT AND RESEARCH Volume 8, Number 2, Special Issue: Burkitt lymphoma (Replaces Summer and Fall Issues 2008) — Inside: REPORT: Progress Report My Child Matters (MCM) Program – Kenya - 8 - report: The UICC My Child Matters Project in Tanzania - 10 - case report: Methotrexate-induced Skin necrosis in a child receiving low-dose methotrexate - 13 - report: NNCTR/INCTR Nepal: The Cancer Program in Nepal - A Brief Progress Report - 15 - FORUM: NCI’s Office of International Affairs Addresses Global Burden of Cancer - 20 - News - 22 - partner profiLE: Comité Héraultais de la Ligue Contre le Cancer - 23 - profiLE IN cancer MEDICINE: Saving Eyes, Saving Lives - 24 THE PRESIDENT’s MESSAGE DENIS BURKITT AND THE AFRICAN LYMPHOMA by Ian Magrath DISCOVERY OF THE TUMOR Denis Parsons Burkitt was born in 1911 in Enniskillen, the picturesque county town of Fermanagh, now in Northern Ireland. “Enniskillen” is derived from a Gaelic word meaning Ceithleann’s island, the town being situated on an island between two loughs (lakes) connected by the river Erne. According to Irish mythology, Ceithleann was the wife of Balor, the one-eyed king of a race of giants – a mythology that has echoes in Burkitt’s life. Sadly, at the age of 11, young Denis suffered Figure 1. Lake Victoria, Uganda. The regions surrounding the lake are high incidence an injury that led to the loss of an regions for Burkitt lymphoma. Picture from Wikipedia Commons taken by D. Luchetti. eye. Although this hampered his eyesight and, to a degree, his subse- him to meticulously map their ter- footsteps of two Irish literary giants, quent career as a surgeon, it had no ritories. James Burkitt’s maps must Oscar Wilde and Samuel Beckett effect on his insight. Burkitt doubt- have impressed his elder son, who who, like Burkitt, also continued less inherited some of his observa- was later to map the distribution of their education at Trinity College, tional skills from his father, James the “African lymphoma” (Figure 1). Dublin. Burkitt, without a clear idea Parsons Burkitt, a civil engineer, Dennis Burkitt, attended the Portara of his future, had taken up engi- but also an amateur ornithologist Royal School at Enniskillen, one of neering at Trinity College. He joined who was one of the first to use the five free schools founded by Royal the University Christian Society, technique of ringing or banding to Charter in 1608 by King James I. which gave new meaning to his life; recognize individual birds, allowing In this respect he followed in the he decided that his calling was to 1 NETWORK become a missionary. This, perhaps spent leave in Uganda and visited coupled to sharing a room with a the old Mengo Hospital, where the medical student, led him to give up first missionary doctor to Africa, Sir his engineering studies and instead, Albert Cook, had worked, as well take up medicine. After completing as the Mulago teaching hospital in his studies he decided to become Kampala, where he himself would a surgeon – somewhat surprising, later work. This experience, cou- perhaps, in view of his lack of bin- pled to his evangelical zeal – and ocular vision – and completed his possibly the example of his Uncle basic training shortly before the Roland, who practiced surgery in Second World War. Nairobi – convinced him that he was Burkitt took a position as ship’s destined to serve in Africa. In 1946, doctor before applying for a post therefore, Burkitt again applied to in the colonial medical services. the British Colonial Office for a post. Unfortunately, he was turned down This time he was accepted and was because he had only one eye. After appointed to the position of District Figure 2. Map of Africa showing the other unsuccessful applications for Medical Officer in Lira, a small town “lymphoma belt” in which Burkitt lym- an overseas posting, he decided to in the Northern Lango district of phoma occurs at high incidence. Lower join the Army Medical Corps and, Uganda. While there, he noted a incidence areas (e.g., highlands) within after working in England for a while, high incidence of hydrocele, caused this zone are not shown. was sent to Africa, where he served by mosquito-born filarial worms in Somalia and Kenya. Once, he that block lymphatic vessels, and mandibles) in a 5 year-old-boy he was able to show that the incidence was asked to see in the children’s was much higher in the eastern part ward at Mulago hospital by the of Lango (30% of men) than the pediatrician, Hugh Trowell. A biopsy western region (1%). This experi- report described the tumor as a ence, too, must have sensitized him “small round cell sarcoma.” Burkitt EditoriaL Staff to geographic epidemiology while was unable to offer any advice Ian Magrath, Editor impressing upon him the important on treatment, although the gross Marcia Landskroener, Managing Editor role of arthropod vectors in trans- facial distortions caused by the jaw Bénédicte Chaidron, Assistant Editor Sophie Lebedoff, Lay-out mitting disease in Africa – a lesson tumors made a big impression on that served him in good stead in the him. Shortly afterwards he saw a ADDRESS correspondence to: context of the African lymphoma. second child, with tumor in all four INCTR aisbl at the Institute Pasteur Burkitt had been in Lira for only 18 jaw quadrants, during a regular visit Rue Engeland 642 1180 Brussels • Belgium months when he received a tele- to a hospital in Jinja, a small town +32 2 373 93 23 • [email protected] gram summoning him to Mulago situated where the river Nile flows www.inctr.org Hospital (Figure 2) where Ian out of Lake Victoria (one of the McAdam, the only other formally illusive sources of the Nile sought Network: INCTR Newsletter c/o Marcia Landskroener trained surgeon in Uganda at the by the European explorers, Burton 3817 McGinnes Road time, had became ill. McAdam, who and Speke). This second child also Millington, Maryland 21651 USA subsequently became the head of had tumors in the abdomen. Like [email protected] the Department of Surgery in the the first, his disease had been diag- University Hospital, was to become nosed as a small round cell sarcoma. Views, comments and statements a strong supporter of Burkitt’s sub- The coincidence of seeing two chil- are not necessarily shared or sequent work. dren with jaw tumors in quick suc- endorsed by INCTR. All patients' It was not until 1957 that Burkitt cession led Burkitt to examine the photographs are published with their consent. saw his first case of multiple jaw records of other patients seen in tumors (i.e., bilateral maxillae and Mulago hospital. He identified 29 2 PRESIDENT’S MESSAGE other children who had presented pathologists also working at Mulago childhood malignancy in the USA, with jaw tumors, although many, Hospital, were in the process of sur- was the least common in East Africa, like the child in Jinja, had additional veying the malignant tumors in chil- accounting for only 1-3% of child- disease at other sites, including the dren in the Mulago Hospital Registry hood cancers in several published orbit and abdomen, salivary glands, that had been initiated seven years series. ALL remains uncommon in nervous system and elsewhere before. In fact, Davis had earlier Equatorial Africa today – but that is (Figure 3). Most of these tumors observed that approximately half of another story. were diagnosed as small round the childhood tumors were derived cell tumors and variably reported, from the “reticulo-endothelial sys- DISCOVERY OF according to the sites of disease, as tem” and O’Connor’s and Davis’ more EPSTEIN-BARR VIRUS sarcoma, retinoblastoma, germino- extensive review, which included A few months after the publication blastoma, Ewing’s sarcoma, Wilms’ children without jaw tumors, con- of Burkitt’s first paper, Dr George tumor or neuroblastoma. firmed this. It soon became clear Oettle, a cancer specialist from Although several European pathol- that the tumor described by Burkitt South Africa, visited Kampala, and ogists working in Africa had observed was a lymphoma and, in 1961, Burkitt Burkitt was intrigued to learn from the high incidence of jaw tumors, and O’Connor published additional him that he had never seen children and of lymphomas in children with papers in the journal Cancer, bring- with a similar clinical syndrome in cancer many years before Burkitt ing it to the attention of cancer South Africa. This observation, influ- saw his first case, Burkitt was the first specialists. Although it seemed ini- enced, perhaps, by his earlier work to describe the clinical syndrome. He tially that the tumor was confined on hydrocele, led Burkitt to think proposed that all the children with to Africa, it was subsequently rec- about the geographical distribution jaw tumors, regardless of other sites ognized that histologically identical of the tumor. Was it widespread of disease, were probably suffer- lymphomas occur throughout the throughout Africa, or confined to ing from the same disease. His first world, although at a much lower certain regions? Burkitt hung a map paper, entitled “A sarcoma involving incidence and with some differences of Africa on the wall of his office and the jaws of African Children” was at clinical (Figure 4) and molecular started to indicate places where published in the British Journal of levels from the African lymphoma. children with jaw tumors had been Surgery in 1958 (vol. 46, 218-213). The relationship of Burkitt lympho- seen. He sent 1,000 brochures to This aroused little interest, since the ma to ALL was frequently discussed government and mission hospitals disease offered limited scope for sur- since the latter disease was rarely throughout Africa and started to gery.

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