Prognostic value of MIB-1, E-cadherin, and CD44 in pediatric chordomas.

The prognosis of pediatric chordomas is difficult to predict based on histology. The objective of this study was to assess the expression of a proliferation marker and adhesion molecules in pediatric chordomas and relate the expressions to outcome. In 8 pediatric chordomas, we calculated the MIB-1 labeling index (LI) by counting the number of MIB-1-positive tumor cells in 100 tumor cells. The grade of expression of E-cadherin and CD44 was calculated by estimating the percentage of tumor cells expressing these markers. MIB-1 LI correlated with tumor recurrence (P = 0.007) and low survival rate (P = 0.007). The expression of E-cadherin correlated with disease-free survival (P = 0.009), tumor recurrence (P > 0.0007), and low survival rate (P > 0.0007). CD44 expression did not correlate with recurrence (P = 0.056) or survival rate (P = 0.056). Our results suggest that MIB-1 LI and expression of E-cadherin are helpful to predict outcome in pediatric chordomas.

Benign cutaneous epithelioid Schwannoma: case report and review of the literature.

Benign peripheral nerve sheath tumors are relatively common. They are mostly characterized by the presence of delicate S-shaped spindle cells and myxoid stroma. Although variants with epithelioid foci can be present, the pure epithelioid variant of benign cutaneous schwannoma is extremely rare. It was first reported as cutaneous epithelial schwannoma by Kindblom et al in 1998. Since then, only six cases have been reported. Care should be taken not to misdiagnose them as malignant neoplasms. Their diagnosis can be problematic as their histopathologic features may overlap with those of other soft-tissue and melanocytic tumors. We report a case of cutaneous epithelioid schwannoma and review of the literature.

Amniotic sac infection syndrome features fetal lung neuroendocrine cell hyperfunction.

Neuroendocrine cells (NEC) are abundant in fetal and neonatal lungs, but reduced in infants with hyaline membrane disease. Perinatal neuroendocrine cell hyperplasia (NCH) has been reported in the hypoplastic lung in diaphragmatic hernia, bronchopulmonary dysplasia, and Wilson-Mikity syndrome. Since we are unaware of any reports on NCH in fetal inflammatory conditions, this report addresses the NEC in fetuses with congenital pneumonia. Twenty-one fetuses/neonates with congenital pneumonia, autopsied between 1995 and 2001, were compared to 21 fetuses without a congenital infection matched for gestational age. Lung sections were immunostained for chromogranin, bombesin, calcitonin, and synaptophysin. Proportions of immunopositive cells lining 20 consecutive bronchioles calculated from digital images were significantly higher in the study than the control group for chromogranin (1.8 vs. 0.8%, P = 2.4 E-06), calcitonin (1.2 vs. 0.7%, P = 0.005), and bombesin (1.1 vs. 0.7%, P = 0.005). There was no difference in synaptophysin (11.7% vs. 12.6%, P = 0.07). The absence of significant differences in the synaptophysin ratio excludes simple NCH in the study group. The synchronous increase in three neurohormones is indicative of NEC hyperfunction, due to either altered enzymatic inactivation by neutral endopeptidase, known to be reduced in adult lung inflammation, or by an increase in expression of the neurohormone genes. These data indicate that NEC hyperfunction may be responsible for the deranged fetal/neonatal lung function and circulatory adaptation, and contribute to the lethality of the amniotic sac infection syndrome.