Endosonographic features of primary esophageal melanoma

Malignant melanoma most commonly arises in the skin; however, primary melanomas can also arise from the mucosal epithelial lining of the gastrointestinal tract. Primary esophageal melanoma is rare and constitutes only 0.1% of primary esophageal tumors. There are only a few reports on the use of endosonography in this condition. We report here a case of a 45‐year‐old male with a history of painful deglutition and describe the endosonographic features of primary esophageal melanoma.     

Intracortical osteosarcoma

A case of osteosarcoma arising in the tibia of a 15-year-old boy is reported. Of particular interest was the benign roentgenographic appearance and the confinement of the tumor within the cortex of the bone, an extremely rare phenomenon consistent with origin within the primitive mesenchyme of the Haversian canals.     

The thymus in hemochromatosis.

Although iron in cases of hemochromatosis has been described in virtually every organ, its presence in the thymus has only been rarely observed. While investigating all cases of hemochromatosis autopsied at The Johns Hopkins Hospital over the past decade, 4 adult cases in which the thymus was available for histologic study were examined in light of recent documentation of this organ's role in lymphopoiesis and secretory activity. Iron-containing moieties were found within both lymphocytes and epithelial cells of these involuted thymic glands. The thymic iron suggests either an uptake of iron moieties from the vascular pool or their synthesis within the cell itself. While the former mechanism provides evidence against the existence of an impermeable blood-thymus barrier, the latter supports the existence of a metabolically active gland with complex function.     

HCV and GBV-c/HGV infection in HIV positive patients in southern Italy

Flaviviridae–hepatitis C virus (HCV) and GB virus C/hepatitis G virus (GBV-C/HGV) – and human immunodeficiency virus (HIV) frequently show similar modes of transmission. HCV and GBV-C/HGV infection was assessed in 134 consecutive patients with evidence of HIV infection, living in Campania, Italy. Data obtained from this cohort were compared with those obtained from 252 age- and sex-matched HCV infected patients without evidence of HIV infection (HCV control group). Following enzymatic immunoassays, samples were tested for the presence of HCV-RNA by RT-PCR. The HCV-RNA positive sera were genotyped by LiPA procedure. The prevalence of HCV infection in HIV patients was 19.40% and the largest group of HIV–HCV co-infected patients (84.62%) was represented by intravenous drug users (IVDU). The distribution of HCV genotypes in HIV–HCV patients was different, compared to that observed in HCV control group. HCV genotypes 1a (50%) and 3a (23.08%) were more frequently detected in HIV–HCV patients, compared to HCV control group (5.16 and 5.56% for 1a and 3a, respectively). Conversely, HCV genotypes 1b (55.70%) and 2a/2c (30.26%) were more represented in HCV control group, compared to HIV–HCV patients (15.38 and 0% for 1b and 2a/2c, respectively). GBV-C/HGV seroprevalence was 41.04% in HIV patients and 6.54% in healthy control individuals. Differently from HCV, GBV-C/HGV infection did not correlate to a preferential risk behaviour in the HIV cohort. Comparative analysis of HCV and GBV-C/HGV infection indicates that the use of injecting drugs might play a key role in the epidemiology of HCV and, in particular, of 1a and 3a HCV genotypes, in HIV patients.     

Hyperphosphatasemia in an adult. Clinical, roentgenographic, and histomorphometric findings and comparison to classical Paget's disease

A 54-year-old man with short stature, diffuse skeletal abnormalities, and elevated alkaline phosphatase was evaluated by bone biopsy with undecalcified sections and morphometry analysis. Microscopically the bone changes were identical to those of classic Paget's disease. Histomorphometric analysis of bone demonstrated a high remodeling activity with increased mineralization rate similar to Paget's disease. However, the early age at onset and severity suggest that this man suffers from hyperphosphatasemia, a different and distinct condition.     

Acidosis-induced osteomalacia: metabolic studies and skeletal histomorphometry

The pathogenesis of osteomalacia was investigated in three patients with chronic metabolic acidosis. Serum levels of parathyroid hormone and vitamin D metabolites were measured, and bone biopsy specimens were analyzed after double tetracycline labeling. Parathyroid hormone concentrations were normal in patients 1 and 3 and slightly elevated in patient 2. Vitamin D metabolism was undisturbed. Static indicators of bone remodeling substantiated the diagnosis of osteomalacia in each case. In patient 1 fluorescent microscopy revealed no evidence of tetracycline uptake. In patients 2 and 3 active mineralization was evident at all osteoid seams, but because double labels were rare, the mineral apposition rate appears to have been substantially reduced in most bone-forming units. Our results indicate that acidosis-induced osteomalacia, unlike that due to vitamin D deficiency, may be associated with mineral deposition at every possible site. Nevertheless, like other causes of osteomalacia, metabolic acidosis prevents mineral apposition at a normal rate even if mineral deposition is ubiquitous. We suggest that titration of newly deposited phosphate causes the observed impairment of mineral apposition and ultimately leads to osteomalacia.     

Near-triploid and near-tetraploid acute lymphoblastic leukemia of childhood

Cytogenetic and DNA flow cytometric analyses of leukemic cells from 1,971 children with newly diagnosed acute lymphoblastic leukemia (ALL) identified stem lines with modal chromosome numbers greater than 65 in 26 patients (1.3%). Near-triploidy (66 to 73 chromosomes) was found in six cases and near-tetraploidy (82 to 94 chromosomes) in 20. A striking morphologic finding was the presence of clumped chromatin with grooved nuclei or Rieder cell formation in 20 cases. Other than a slight excess of the pre-B immunophenotype, the near-triploid cases did not appear to differ substantially from the general ALL population in clinical features. In contrast, near-tetraploid cases were more often associated with a T-cell immunophenotype (47% v 14%, P less than .001) and L2 morphology (30% v 22%, P less than .01), and were older at diagnosis (median age, 8.6 v 4.8 years, P = .01) than cases with other ploidies. Moreover, an unusually high proportion of near-tetraploid cases tested (6 of 15) expressed one or more of the myeloid-associated antigens CD13, CD15, and CD33. Despite the use of contemporary intensive chemotherapy and short follow-up for most patients, 6 of the 20 near- tetraploid cases have relapsed or died. This study suggests that the near-tetraploid subtype differs from other cases of hyperdiploid greater than 50 ALL, which have been associated with a favorable prognosis.     

The microscopic morphology of fluoride-induced bone

To characterize further the bone changes in osteoporotic patients treated by a combined calcium, vitamin D, and sodium fluoride therapy regimen, full-thickness transilial undecalcified bone biopsy specimens from ten postmenopausal white women treated for idiopathic osteoporosis for 18-24 months were compared with those from ten age-, sex-, and race-matched untreated control subjects using standard light microscopy and histomorphometry. Statistically significant bone changes in the treated group consisted of cortical and trabecular new bone formation juxtaposed on underlying normal lamellar bone (p less than 0.001). The new bone showed increased osteocytic cellularity (p less than 0.001), irregular arrangement of osteocytes (p less than 0.001), enlarged osteocyte lacunae (p less than 0.001), and periosteocytic hematoxylinophilic staining intensity (p less than 0.001). Increases were also noted in trabecular bone volume (p less than .025), trabecular osteoid surface (p less than 0.001), and trabecular osteoid volume (p less than 0.001). Osteoid calculations were significantly less than those in the clinical and chemical osteomalacia observed in the authors' laboratory (p less than 0.01). Osteoclastic resorptive activity was increased (p less than .001), but no evidence of hyperparathyroidism was noted. These histologic and histomorphometric changes indicate accretion of new bone but with distinctly abnormal matrix characteristics. These are changes considered characteristic of the treatment and are pathologic markers of fluoride-induced abnormal bone formation.