Detection of GD2-positive cells in bone marrow samples and survival of patients with localised neuroblastoma.

The impact of bone marrow (BM) GD2-positive cells on survival has been evaluated in 145 Italian children with localised neuroblastoma (NB) evaluated at diagnosis by anti-GD2 immunocytochemistry. Nineteen of these (13.1%) were found to be BM GD2-positive, with the number of positive cells ranging between 1 and 155 out of 1 x 10(6) total cells analysed. Seven/19 (38.8%) GD2-positive vs 12/126 (9.5%) GD2-negative patients relapsed. The 5-year event-free survival (EFS) and overall survival of the GD2-positive patients was significantly worse than that of the GD2-negative ones (62.2 vs 89.9%, P<0.001; and 74.9 vs 95.9%, P=0.005, respectively). GD2 positivity was not associated to other known risk factors, and in particular to Myc-N amplification and 1p deletion. Among Myc-N-negative patients, the EFS of those negative for both GD2 and 1p deletion was significantly better than in children positive for either one of these two markers (EFS=96.9 vs 66.0%, P<0.001). In conclusion, GD2 positivity may represent a prognostic marker for patients with non-metastatic NB without Myc-N amplification, and its combination with genetic alterations might help identifying patients that require a more careful follow-up.

[Gastric fibroid polyp in a 4-month-old girl with Costello syndrome]. / Polipo fibroide gastrico in sindrome di Costello.

We describe the case of a 4-month-old girl with a gastric fibroid polyp. This was an occasional radiographic finding, confirmed by sonography and computerized tomography. This very rare benign tumor was surgically removed. The diagnosis of Costello syndrome was based on clinical appearance. This is the first report of a gastric fibroid polyp in Costello syndrome, a genetic disease with a high tumor frequency.

Multimodal imaging in the congenital pulmonary lymphangiectasia-congenital chylothorax-hydrops fetalis continuum.

We report on three infants with congenital chylothorax (CC) and congenital pulmonary lymphangiectasia (CPL). CPL appears to be a characteristic pathological finding in CC. Through the use of lymphoscintigraphy and computed tomography, this study suggests that CC and CPL are strongly correlated entities and that the dysplasia of the lymphatic system results in a pulmonary lymphatic obstruction sequence. The initial microscopic dilatation of the lymph channels may lead to progressive weeping of lymphatics and, consequently, to pleural effusion. Non-Immune Hydrops Fetalis (NIHF) may be the final consequence of impaired systemic venous return and may help to explain pleural-pulmonary involvement in this generalized lymph-vessel malformation syndrome.

Persistence of Müllerian derivatives and intestinal lymphangiectasis in two newborn brothers: confirmation of the Urioste syndrome.

We describe two newborn brothers with a pattern of malformation characterized by the persistence of Müllerian duct derivatives, intestinal lymphangiectasia, hypertrophied alveolar ridges, and early death. Postmortem examination showed the presence of a rudimentary uterus, fallopian tubes, the upper third of a vagina, a prostate of normal shape, a dilated colon, and generalized intestinal and pulmonary lymphangiectasia. The syndrome was first delineated by Urioste and co-workers [1993: Am J Med Genet 47:494-503]. These cases confirm the existence of a definite and distinct entity.

Nucleolar organizer regions (NORs) in normal and pathological liver: a quantitative analysis.

By means of a silver staining method, Nucleolar Organizer Region-associated proteins (NORs) have been evaluated on paraffin sections of a series of 58 ultrasound-guided liver biopsy specimens. These included 12 normal livers, 12 cirrhotic livers, 12 cases of chronic hepatitis and 22 cases of hepatocellular carcinoma. A significant difference (P less than 0.001) was found between the mean AgNOR scores of the normal and pathological biopsies, and between the non-neoplastic and the carcinomatous lesions. The authors suggest that AgNOR counts, in combination with conventional histocytological criteria, may be a useful method in the diagnosis of liver diseases.

Nucleolar organizer regions and Ki-67 immunostaining in ductal breast cancer: a comparative study.

53 cases of invasive ductal (NOS) carcinomas of the breast were studied by means of an immunostaining method with Ki-67 monoclonal antibody and an argyrophilic method for the demonstration of Nucleolar Organizer Regions (AgNORs). The percentage of cancer cells with nuclear Ki-67 immunoreactivity and the mean number of NORs for each tumour were statistically related. The data obtained showed a good correlation between Ki-67 index and NOR score (rS = 0.47 - P less than 0.001). The authors suggest that the AgNOR method--which is applicable to routinely processed material--might effectively substitute Ki-67 immunostaining as a marker of cell proliferation in ductal breast cancer.

Aggressive angiomyxoma of the pelvis and perineum. Report of a case with immunohistochemical and electron microscopic study.

A case of aggressive angiomyxoma (AAM) of the pelvis and perineum is reported, whose clinical and pathological findings are consistent with the literature. This is a clinicopathological entity recently described by Rosai and colleagues (1983) who stressed its locally infiltrative nature and its tendency to recur. Its differential diagnosis includes a series of benign and malignant soft tissue tumours.

[The nucleolus organizer region in benign and malignant melanocyte lesions of the skin: a quantitative study]. / Le regioni organizzatrici del nucleolo nelle lesioni melanocitarie cutanee benigne e maligne: studio quantitativo.

By means of a silver staining method, Nucleolar Organizer Region-associated proteins (NORs) have been evaluated on paraffin sections of two series of benign and malignant melanocytic skin lesions (represented by 30 intradermal naevi and 30 malignant melanomas). A significant difference (P less than 0.001) was found between the numbers of Ag-NORs in the nuclei of benign lesions and those of malignant lesions. It is suggested that this method could be of value in assessing the biological potential of cutaneous melanocytic neoplasms.

[Quantitative evaluation of the nucleolus organizer regions (NORs) in malignant melanoma of the skin: is it a new prognosis index?]. / La valutazione quantitativa delle regioni organizzatrici del nucleolo (NORs) nel melanoma maligno cutaneo: un nuovo indice prognostico?

Nucleolar Organizer Region-associated proteins (NORs) have been evaluated on paraffin sections of a series of cutaneous malignant melanomas, stage IA at the time of the initial diagnosis. A significant difference (P less than 0.001) was found between the numbers of AgNORs in the cases which developed metastases and those without metastases: increasing values of AgNORs representing increased risk of metastases.