Malignant fibrous histiocytoma of the inferior vena cava: appearances on contrast-enhanced spiral CT and MRI.

Primary malignant tumors of the inferior vena cava are infrequent. We report a very rare case of primary malignant fibrous histiocytoma of the inferior vena cava and describe the contrast-enhanced spiral computed tomographic and magnetic resonance imaging findings.

Two color flow cytometric analysis of concomitant acute myeloid leukemia and chronic lymphocytic leukemia.

During evaluation for macrocytic anemia and thrombocytopenia, a 74-year-old female was found to have a leukocytosis with two apparent populations of malignant cells identified in her peripheral blood smear and bone marrow aspirate. Morphologic characteristics of the two cell types were unusual, and cytochemical assays yielded ambiguous results. Two-color flow cytometric analysis demonstrated two distinct cell populations with immunophenotyping patterns consistent with chronic lymphocytic leukemia (CD5+/CD20+) and acute myelocytic leukemia (CD33+/CD34+), detected concurrently. The concomitant appearance of CLL and AML has been reported only rarely. The use of two-color flow cytometry to differentiate the populations demonstrates the utility of this technology in resolving unusual hematological malignancies.

Verruciform xanthoma in association with discoid lupus erythematosus.

Verruciform xanthoma (VX) is an uncommon lesion occurring primarily in the oral cavity. Cutaneous lesions are much less common and they preferentially arise on anogenital skin. They are not necessarily associated with a pre-existing inflammatory process. We report a VX in association with a long-standing lesion of discoid lupus erythematosus (DLE) on the scalp of a 34-year-old black woman. This association, which to our knowledge has not been previously reported, is consistent with the proposed pathogenetic mechanism of entrapment and subsequent degeneration of epithelial cells in the papillary dermis of VX. Histological distinction of VX from squamous cell carcinoma, with which this lesion may be clinically confused, is straightforward.

Gross and fine motor development in 45,X and 47,XXX girls.

Neuromuscular deficits have been described in 47,XXY and 47,XYY boys, but gross and fine motor development of girls with sex chromosome aneuploidy has not been extensively studied. Twenty-one propositae 8 to 19 years of age, identified through newborn screening to be 45,X, 47,XXX, or 45,X mosaic, and 11 control girls were evaluated by a physical therapist unaware of their genetic constitution. The Bruininks-Oseretsky Test of Motor Proficiency (BOTMP) was administered, and the quality of neuromuscular function was determined. The 45,X and 47,XXX propositae exhibited both gross and fine motor dysfunction, with 12 of 15 BOTMP composite scores below the 10th percentile. The clinical assessment confirmed the BOTMP findings, with 13 propositae exhibiting dysfunctional sensory-motor integration. A delay in the age of independent walking confirmed the consistency of motor developmental dysfunction throughout time. Sex chromosome mosaics were more similar to control girls. The gross and fine motor delays were frequently associated with a moderate to severe language dysfunction which adversely affected classroom performance. Regular developmental assessments of children with sex chromosome aneuploidy, including sensory-motor integration, should assist in the identification of early developmental delays and permit appropriate intervention.

Gross and fine motor development in 47,XXY and 47,XYY males.

Neuromuscular deficits described in early childhood as motor awkwardness or slow movements are still clinically present in school-aged boys with XXY and XYY sex chromosome aneuploidy. A control group of 14 boys (6 to 19 years of age) and 14 XXY and four XYY boys (6 to 15 years of age), identified by newborn screening, were blindly evaluated by a physical therapist. The Bruininks-Oseretsky Test of Motor Proficiency (BOTMP) was administered and a clinical rating of neurologic status and sensory-motor integration was assigned. On the motor proficiency test, the XXY boys had significantly lower mean scores for upper limb coordination, speed and dexterity, and on gross motor and battery composites. The neuromuscular status of the aneuploid boys was deficient, with hypotonia, apraxia, primitive reflex retention, and problems with bilateral coordination and visual-perceptual-motor integration. This mild to moderate dysfunctional sensory-motor integration, as well as previously described auditory-processing deficits and dyslexia, contributed to school performance below that expected from their cognitive potential.