BURKITTS LYMPHOMA

Burkitt's lymphoma (BL) is a highly malignant tumor first described during the late 50's in African children (jaw), endemic in nature, and composed of undifferentiated lympho-reticular cells with uniform appearance. The American BL variety is non-endemic, mostly attacks children between 8-12 years of age, predominantly (>75%) with abdominal disease such as unexplained mass, pain, or intussusception. The head and neck region follows. The tumor can appear as a localized, diffuse (multifocal, non-resectable) or metastatic abdominal mass (bone marrow and CNS). It's considered the fastest growing tumor in humans with a doubling time around 12-24 hrs. Chemotherapy is the primary treatment modality due to its effectiveness in rapidly proliferating cells. The role of surgery is to establish the diagnosis (using open biopsy), stage the tumor, remove localized disease, relieve intestinal obstruction and provide vascular access. Complete resection whenever possible offers the patient improved survival. Is more readily accomplished in patients with localized bowel involvement operated on an emergency basis due to acute abdominal symptoms. The only predictor of event free survival is extent of abdominal disease at diagnosis. Debulking (cytoreductive) procedures increases morbidity and delays initiation of chemotherapy worsening prognosis. Extensive tumors should be managed with minimal procedure and immediate chemotherapy (a/o radiotherapy). Bone marrow and CNS involvement are ominous prognostic signs.