Morphological manifestations of the Dandy-Walker syndrom in female members of a family.

The Dandy-Walker syndrome (DWS) is a hereditary disorder, appearing somewhat more frequently in women. The most important characteristics of the DWS are the lack of the cerebellar vermis, varying from a partial lack to a complete agenesis, and enlargement of the cerebrospinal spaces, especially in the fourth ventricle. The above mentioned morphological changes clinically manifest in ataxia, increased intracranial pressure and hydrocephalus. Here is presented a family with DWS, where the disease is contracted only by female members, in two generations, whereas no signs of DWS have been noticed in male family members. DWS is clinically manifested from early childhood to middle age, with the morphological changes varying from hypoplastic cerebellar vermis to widening of the brain ventricles and hydrocephalus and arachnoid cyst in the occipital part.

Prognostic value of MVD, LVD and vascular invasion in lymph node-negative colon cancer.

BACKGROUND/AIMS: To clarify the influence of microvessel density (MVD), lymphangiogenesis (LVD), and vascular invasion on prognosis in lymph node-negative colon cancer. METHODOLOGY: We performed immunohistochemical analysis from 152 Duke's B colon carcinomas, CD34 and LYVE-1 antibodies. Carcinomas were graded as low or high grade. χ2 test was used to examine their relationships and correlations with clinicopathological parameters. Survival time was analyzed and the differences between groups were assessed. RESULTS: A statistically significantly correlation was found between increasing MVD with age >60 years, tumor size >4cm, and poor tumor differentiation (χ2=40.018, p<0.001). The increase in MVD was associated with shorter DFS (p<0.001) and shorter OS in patients with colon cancer (p<0.001). LVD was statistically significantly associated with increasing the number of newly created blood vessels (χ2=96.6, p<0.001), low degree of tumor differentiation (χ2=96.6, p<0.001), and vascular invasion (χ2=51.8, p<0.001) in colon cancer. Log rank analysis showed that positive staining for MVD and LVD, high histological grade, vascular invasion, male gender, and age >60 years were connected with shorter survival of patients with Dukes B colon cancer 45 vs. 100 months (p=0.016 to <0.001). CONCLUSIONS: Positive expression MVD and LVD was significantly correlation with survival time and with high tumor grade and vascular invasion in patients with Dukes B colon cancer. The correlation of MVD and LVD with vascular invasion in Dukes B colon cancer indicates the need for further confirmation as a possible predictive marker.