Burkitt lymphoma presenting as a conjunctival mass.

PURPOSE: A sporadic Burkitt lymphoma, which presented as a primary conjunctival mass, demonstrates the approach to clinical diagnosis, staging, and current management of this rapidly growing malignant tumor, as well as newer concepts of its pathogenesis. PATIENT AND METHODS: A 16-year-old girl had rapid development of a conjunctival mass 6 weeks after an infectious mononucleosis-like illness, associated with a positive monospot test. A biopsy of the tumor was immunostained for lymphocyte markers. Additional clinical workup included computed tomographic scanning, nasopharyngeal biopsy, and studies of blood, bone marrow, and spinal fluid. In situ hybridization for Epstein-Barr virus (EBV)-encoded nuclear RNA was performed on the tumor and adenoidal tissue. RESULTS: The conjunctival biopsy showed a typical Burkitt lymphoma with markers positive for predominantly B lymphocytes. Computed tomographic scans indicated residual tumor in the anterior orbit. Biopsy of thickened adenoidal tissue showed only benign lymphoid hyperplasia. Evidence of EBV infection was found in the adenoidal tissue but not in the tumor cells. The patient was treated with combined chemotherapy on a Pediatric Oncology Group Study protocol for localized non-Hodgkin lymphoma, and is disease-free 4 1/2 years after diagnosis. CONCLUSIONS: A sporadic Burkitt lymphoma arising in the conjunctiva appears to be the first reported in this site. The lymphoma responded well to chemotherapy, which is now the usual course in stage 1 disease. A direct role for EBV in the oncogenesis of this sporadic tumor, similar to that in the endemic form, could not be supported because EBV-encoded nuclear RNA was absent in the tumor cells.

Inflammatory myofibroblastic tumor in children.

The authors presented the cases of two children with inflammatory myofibroblastic (IMF) tumor and reviewed the literature to facilitate the preoperative recognition, delineate the clinical features, and describe the natural history of this entity. The first child had IMF tumor arising from the mesentery of the small intestine. He presented with an abdominal mass associated with severe inflammatory response manifested by fever, impaired growth, thrombocytosis, and microcytic, hypochromic anemia. After surgical resection, his fever resolved and his growth rate and the laboratory abnormalities normalized. Five months after initial diagnosis, the fever, anemia, and thrombocytosis recurred along with two tumors arising from the omentum and the abdominal soft tissue. After the second surgery, he remains free of recurrent disease for 30 months. The second child presented with a lung mass that was radiologically indistinguishable from pulmonary sequestration. After surgical resection, she remains free of recurrent disease for 18 months. IMF tumor should be considered in any solid tumor that occurs in association with a chronic inflammatory response. IMF tumor should also be considered in the differential diagnosis of pulmonary sequestration.

Polycythemia: a review article and case report of erythrocytosis secondary to Wilms' tumor.

We describe a child with Wilms' tumor whose only finding at presentation was polycythemia. A review of the mechanism of erythropoiesis regulation as well as the differential diagnosis, work-up, and management of polycythemia in children is presented. Our report emphasizes the need for a thorough evaluation of children who present with polycythemia.

Isolated thrombocytopenia in children with acute lymphoblastic leukemia: a rare event in a Pediatric Oncology Group Study.

To determine how many children with acute lymphoblastic leukemia were initially referred to a pediatric hematologist because of isolated significant thrombocytopenia ([platelet count less than 50,000/mm3] and an otherwise normal complete blood cell count and physical findings), a retrospective review of the Pediatric Oncology Group's charts was undertaken. Review of the records of 2239 children enrolled in the past two acute lymphoblastic leukemia protocols showed that none of these children had significant thrombocytopenia with no other hematologic or physical manifestations of acute lymphoblastic leukemia when they were first seen by the hematologist. The results suggest that routine bone marrow aspiration in the child with isolated thrombocytopenia may be unnecessary to rule out acute lymphocytic leukemia.

Controversies in the management of acute idiopathic thrombocytopenic purpura: a survey of specialists.

A total of 322 physicians, Board-certified in pediatric hematology, responded to a survey designed to determine several aspects of their management of children with acute idiopathic thrombocytopenic purpura. The survey demonstrates that, in practice among specialists, a controversy exists as to whether or not bone marrow examination needs to be performed, and how often corticosteroids should be prescribed. Seventy-four percent of practitioners would perform the bone marrow examination, whereas 26% would not do so. Forty-six percent of responders prescribe steroids more than half the time, whereas 54% prescribe steroids less than half the time. The questionnaire ascertained the reasons why physicians performed marrow aspirations and prescribed steroids.

Activated partial thromboplastin time and minor coagulopathies.

Five commercially available activated partial thromboplastin time (APTT) test systems were compared with the kaolin partial thromboplastin time (KPTT) method to determine sensitivity in detecting minor coagulation defects. All reagent systems detected severe factor VIII-, IX-, and XI-deficient hemophilia. Homozygous states of factor XII deficiency, Fletcher factor deficiency, and high-molecular-weight kininogen deficiency (Fitzgerald trait) also showed abnormally long APTTs by all systems. Of 19 samples from patients with deficiencies of factors XII, VIII, IX, XI, and II ranging from 2.5 to 52%, eight had deficiencies that were not detected by reagent A (ellagic acid); two, by reagent B (ellagic acid); two, by reagent C (kaolin); one, by reagent D (silica); one, by the KPTT method. All deficiencies were detected by reagent E (celite). Heparin effect on plasma was less well detected by reagent A (ellagic acid) than with the other test systems. APTT test systems can vary greatly in their abilities to detect minor coagulation abnormalities.