Multiple granulocytic sarcomas in acute myeloblastic leukemia with simultaneous occurrence of t(8:21) and trisomy 8.

This report describes a rare case of multiple intracranial, extradural chloromas. A five year old African American male presented with headache, fever, and vomiting. The peripheral blood smear showed myeloblasts with Auer rods. The CTscan of the brain showed three intracranial, epidural lesions as well as soft tissue masses in the retroorbital region and sphenoid sinuses. CTscan of the chest showed two paraspinal epidural thoracic masses. Pathology of the epidural intracranial mass revealed a granulocytic sarcoma. Cytogenetic analysis showed simultaneous occurrence of t(8;21) and trisomy 8. Following induction therapy, he is now in complete remission.

Malignant teratoma of the thyroid with primitive neuroepithelial and mesenchymal sarcomatous components.

A 15-year-old black girl had a near total resection of a malignant thyroid teratoma with bilateral nodal involvement and mediastinal extension. A predominant neuroepithelial pattern had ependymal rosettes and mitoses, stained for neuron-specific enolase, neuron-specific B tubulin, and synaptophysin. A malignant spindle cell component stained for smooth-muscle actin, muscle actin, and to a lesser extent S-100. Loose myxoid tissue resembled primitive cartilage. Epithelial membrane antigen and cytokeratin identified epithelial foci. Chromogranin A, MIC2, glial fibrillary acidic protein, and thyroid stimulating hormone receptor stains were negative. There was focal anaplasia. DNA ploidy by laser scanning cytometry was 1.2. The tumor from the left and right thyroid lobes exhibited trisomy 8, the right also had hyperdiploid cell lines. She was treated with aggressive combination chemotherapy and radiation. Presently there is no residual disease 16 months after diagnosis. Malignant thyroid teratoma is an aggressive tumor, with 15 of 27 reported patients dying 2 weeks to 3 years after diagnosis. Survivors have been treated with total or subtotal resection, combination chemotherapy with agents effective in the treatment of germ cell tumors as well as sarcomas, and radiation for either recurrent or residual disease. The heterologous elements, lacking MIC2 staining and t(11;22), support the diagnosis of malignant teratoma rather than a neuroepithelial tumor. Trisomy 8 is the first cytogenetic abnormality described in malignant thyroid teratoma. Therapy should be tailored to the management of all transformed histologies.

Focal segmental glomerulosclerosis in children with acute lymphocytic leukemia: case reports and review of literature.

PURPOSE: To report the occurrence of focal segmental glomerulosclerosis (FSGS) in children with acute lymphocytic leukemia (ALL), discuss pathogenesis and problems in management. PATIENTS AND METHODS: Progressive renal dysfunction developed in two adolescent black girls with high-risk ALL who underwent renal biopsies that were consistent with FSGS. In both patients, no known etiologic factors, such as systemic lupus erythematosus, poststreptococcal glomerulonephritis, sickle cell anemia, or acquired immunodeficiency syndrome, were evident. FSGS induced by Adriamycin (Pharmacia & Upjohn, Columbus, OH) has been observed experimentally in rats. The patients had received anthracyclines and methotrexate, a known nephrotoxic chemotherapeutic agent. RESULTS: One patient progressed to chronic renal failure and required prolonged dialysis followed by renal transplantation, though the leukemia remained in remission. The other patient is also in remission and on maintenance treatment for leukemia. She has persistent proteinuria and is currently undergoing a trial of high-dose steroid therapy. CONCLUSION: The combination of FSGS with leukemia poses a management challenge to the clinician in terms of further treatment with potentially nephrotoxic drugs, complications of nephrotic syndrome (including infections), and timing of renal transplantation. Future studies should address whether FSGS represents a glomerular response to anthracycline-induced injury in susceptible black persons.

Modulation of cardiac intermediate filament proteins in the chick heart by fibric acid derivatives.

The effects of different fibrates (bezafibrate, fenofibrate and gemfibrozil) on intermediate filaments were studied in cultured chick embryo heart cells after treatment for 6 or 24 h. Treatment led to alterations in total protein levels, as well as changes in protein levels, in the cytoplasmic and cytoskeletal fractions of cultured cells. Desmin was increased in the cytoskeletal fraction of all cultures after 6 h of treatment regardless of the drug tested, whereas vimentin was decreased in the cytoskeletal fraction only in cells treated with fenofibrate. These findings suggest that the alterations caused by fibrates in desmin and vimentin protein content may be related with the secondary effects that these drugs have on the cardiovascular system in patients treated with fibrates.