Peripheral neuroectodermal tumors of the chest wall in childhood.

A retrospective review of primary chest wall malignant tumors of childhood collected at the Children's Memorial Hospital of Chicago was undertaken. Among twelve instances of poorly differentiated neoplasms whose uniform, monotonous structure made accurate classification difficult or impossible by conventional histologic study, there were three tumors with features suggestive of neuroectodermal differentiation. Electron microscopic and immunohistologic findings further strengthened this interpretation, despite the fact that none of the patients had evidence of a primary neuroblastoma outside the chest wall. These results and a review of the pertinent literature support the conclusion that neuroectodermal neoplasms in childhood may present in peripheral somatic tissues with greater frequency than is commonly assumed. The importance of this distinction is discussed, particularly the need to distinguish these neoplasms from Ewing's sarcoma.

"Pure" cutaneous histiocytosis-X.

The case histories of two young children who experienced skin rashes involving various areas of the body are reported. The diagnosis of pure cutaneous histiocytosis-X was established after extensive studies revealed no other organ involvement. The patients were treated with oral corticosteroids. Currently, both children are in good health, show no evidence of disease, and have been followed over a four-to-five-year period. Therapy with corticosteroids may not be indicated with pure cutaneous histiocytosis-X unless there is evidence of extracutaneous dissemination or rapid progression of the disease.

An unusual type of alpha1-antitrypsin deficiency in a child.

An unusual variant of serum alpha1-antitrypsin is described in a 15 5/6-year-old white male with a history of chronic pulmonary disease. The patient had a very low level of this protease inhibitor as demonstrated by tryptic inhibitory capacity and electroimmunoassay. Even though the patient's serum alpha1-antitrypsin was partially purified and concentrated, no phenotypic pattern was seen using conventional Pityping procedures (acid starch gel with crossed antigen-antibody electrophoresis or isoelectric focusing). Crossed antigen-antibody electrophoresis using agarose in both steps, immunoelectrophoresis, and agarose electrophoresis followed by immunofixation all revealed a slow-moving alpha1-antitrypsin, cathodal to the Pi Z region. Studies on sera from the patient's mother and two half-sibs showed that all three had clear Pi M phenotypic pattersn. Quantitative date on these sera suggested that the unusual variant may be inherited in a codominant fashion.

Positive monospot tests preceding the diagnosis of acute monocytic leukemia in two adolescents.

The early detection and treatment of two cases of acute monocytic leukemia (AMoL) was hampered by the presence of positive slide tests for infectious mononucleosis (IM). This assumes great importance with the recent development of specific therapy, effective for certain subtypes of nonlymphocytic leukemia.