A 16-yrs-old girl presents with bowel obstruction. Laparotomy reveals markedly enlarged para-aoic lymph nodes. Biopsy of lymph nodes exhibit a diffuse neoplastic infiltrate of small, round lymphocytes with a "starry sky" appearance on low power. The cytoplasm of some of the lymphocytes is vacuolated and fat stains are positive. You would expect the neoplastic cells to demonstrate a
The patient has Burkitt lymphoma (Lukes-Collins; small non-cleaved), which is the most common malignant lymphoma in children. Approximately one-third of all childhood lymphomas in the United States are of the Burkitt type. There is an American and a Noh American variant. The relationship of Burkitt to the Epstein-Barr virus is more secure with the Mrican than the Noh American variant. The American variant has a predilection for the jaw, while the Noh American variant is more often an abdominal mass, located in the ileocecal region, ovary, or retroperitoneum. The lymph nodes are diffusely involved with monotonous, round, non-cleaved cells with numerous mitoses (very aggressive cancer) punctuated by clear spaces with macrophages containing debris ("tingible" macrophages) giving the impression of a "starry sky appearance," the stars representing the macrophages and the black of night the lymphocytes. The neoplastic B cells have a characteristic t(8;14) translocation where the c-myc oncogene is translocated from chromosome 8 to chromosome 14. Burkitt cells contain vacuoles that contain lipid, which stains positively with oil red O. It is a high-grade lymphoma with a predilection for metastasis to the bone marrow and other organs.
Positive non-specific esterase stain of the cells
Positive specific esterase stain of the cells
Low leukocyte alkaline phosphatase score
t(8;14) translocation
General pathology