Early characteristic findings in bowleg deformities: evaluation using magnetic resonance imaging

We used magnetic resonance imaging (MRI) to evaluate bowleg deformities in infancy. Twenty-five tibiae of 13 infants were examined and divided into two groups based on MRI findings: group A had high intensity area in the medial epiphyseal cartilage on T2-weighted images. Group B had depression of medial physis and abnormal signal in the perichondrial region in addition to the epiphyseal lesion. At the final follow-up, all cases in group A demonstrated normal lower leg alignments, whereas five cases in group B showed characteristic roentogenographic findings of Blount's disease. The improvement rate of metaphyseal-diaphyseal angle was correlated with this classification. These findings suggested that abnormal findings in physis and perichondrial region might be preliminary findings in early stage of Blount's disease. The high intensity areas in the medial epiphyseal cartilage were commonly found among the cases with bowing deformities, which suggested that there might be a common pathomechanism between physiologic bowing and infantile Blount's disease.     

Cutaneous Rosai-Dorfman disease--a pathologic review of 2 cases

Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare but distinct clinicopathologic entity characterised histologically by a benign s histiocytic proliferation. Isolated involvement of extranodal sites without concomitant nodal disease is rare. We describe the pathological features of 2 cases of Rosai-Dorfman disease that were clinically confined to the skin. In both male adult Chinese patients, proliferation of histiocytes was accompanied by S-100 protein expression demonstrated immunohistochemically within the histiocytes. The pathology of Rosai-Dorfman disease and its microscopic differential diagnoses are discussed.     

Actual half-life of alpha-fetoprotein as a prognostic tool in pediatric malignant tumors

In a retrospective study, the prognostic value of monitoring the decay of alpha-fetoprotein (AFP) was assessed. Serum AFP was determined serially in 18 children with malignant germ-cell or hepatic tumors: 7 endodermal sinus tumor, 3 embryonal carcinoma, 5 malignant teratoma, 2 hepatoblastomas, and 1 hepatocellular carcinoma. The actual half-life (AHL) of AFP was computed after surgical resection of the tumor. In group 1, which had complete resection and no recurrence during follow-up (n = 13), the AHL of AFP was 4.0 ± 0.9 days. In group 2, which had incomplete resection or recurrence during follow-up (n = 5), the AHL of AFP was 24.8 ± 20 days, significantly longer than that of group 1 (P = 0.0026). The increased AHL of AFP indicated residual active tumor after surgical resection. The AHL of AFP may be more sensitive than serial monitoring of AFP in detecting preclinical recurrence after surgical resection of AFP-secreting tumors. Treatment strategies can be based on AFP clearance, and prospective clinical trials are warranted.     

CYTOMEGALY OF PANCREATIC D CELLS IN TRIPLOIDY

Triploidy is a common chromosomal aberration seen in 1 of clinically recognized human pregnancies. Development beyond 10 weeks is unusual. Occasionally fetuses survive past 20 weeks; however, they usually present as a stillbirth with only a few managing some hours of independent life. The clinical features of these infants cover a wide spectrum, but a consistent feature is organ hypoplasia and hypotonia. We observed marked enlargement of somatostatin-producing cells (D cells) in the pancreata of triploid fetuses. Somatostatin-producing cells are widely distributed in normal mammals although concentrated in the pancreas and gastrointestinal tract, generally being antiproliferative and having an inhibitory effect on various functions. The control of fetal growth is not well understood. There is, however, some evidence that somatostatin does play a significant part and our consistent observation of cytomegaly of the pancreatic D cells in growth-retarded triploid fetuses provides more support for this contention.     

Immune response to p53 and HPV-16 E6 proteins in patients with cervical cancer

Park JS, Kim CJ, Um SJ, Hwang ES, Kim HS, Park SN, Namkoong SE, Kim SJ. Immune response to p53 and HPV-16 E6 proteins in patients with cervical cancer. Int J Gynecol Cancer 1998; 8 : 328–335.
To investigate whether p53 autoantibodies could be found in the sera of patients with cervical cancers, we have therefore studied by radioimmunoprecipitation assay, using in vitro translated p53 protein, sera from such patients. The sero-positive patients for p53 were also evaluated in relation to immunoreactivity to p53 antigens by immunohistochemistry, for genomic alterations of p53 by PCR-SSCP, and for the presence of HPV-16/18 DNAs in the cervical cancer cells. In immunohistochemistry, expression of p53 protein was seen in 47% (14/30) of HPV-16 or -18 positive cervical cancers and 13 % (2/15) of HPV-16/18 negative cervical cancers ( P < 0.01). Eight out of 12 control ovarian cancers (67%) showed positive p53 staining in most tumor cells. Cases of cervical cancer and ovarian cancer, which were positively expressed p53 protein in the tissue or the sera, were studied for genomic alterations in exons 5–8 of the p53 by PCR-SSCP. Serum antibodies to in vitro translated p53 protein were found in two cases from 63 cervical cancers; one patient was stage IIA, having HPV-16 DNA in a tumor of squamous cell type, and another patient was stage IIIB, having HPV-16 and -18 DNAs in an adenocarcinoma. The cervical cancer tissues from the two sero-positive patients were also positive for p53 immunostaining. None of the cervical cancer samples showed aberrant bands, but three of eight cases of ovarian cancer which were positive for p53 protein by immunostaining were shown to have aberrant bands by PCR-SSCP. In contrast to ovarian cancers, alteration of p53 tumor suppressor gene and positive antibody response to p53 protein seem to be rare events in patients with cervical cancer.