Angiogenesis in Ocular and Extraocular Sebaceous Carcinoma.

To shed more light on the pathogenesis of sebaceous carcinoma, we analysed the expression of proteins related to angiogenesis in 18 ocular and 22 extraocular sebaceous carcinomas using a broad panel of immunohistochemical markers. To quantify the expression of D2-40, vascular endothelial growth factor, vascular endothelial growth factor receptor-2 and -3, we calculated a quantification score by considering the percentage of positive tumour cells (0=0%, 1=up to 1%, 2=2-10%, 3=11-50%, and 4=>50%) in relation to the staining intensity (0=negative, 1=low, 2=medium, and 3=strong). Additionally, lymphatic microvessel density in the D2-40 stained sections was counted. Vascular endothelial growth factor receptor-3 (quantification score 9.42 ± 2.94) was significantly more strongly expressed than vascular endothelial growth factor receptor-2 (quantification score 2.15 ± 2.42, p < 0.001). Furthermore, epidermal vascular endothelial growth factor expression was negatively correlated with the intratumoural lymphatic vessel density, and the ratio of small lymphatics to large lymphatics was much higher in intratumoural tissue than in paratumoural tissue and in intraindividual control tissue, suggesting a lymphangiogenetic potential of sebaceous carcinoma.

Sebaceous lymphadenoma identified by fine needle aspiration biopsy: a case report.

BACKGROUND: Sebaceous lymphadenoma of the parotid gland is a rare benign neoplasm. This is the first reported case of fine needle aspiration biopsy (FNAB) findings for sebaceous lymphadenoma of the parotid gland. CASE: A 60-year-old male presented with painless, bilateral parotid swelling noted for 5 months. The swelling was more pronounced on the right. Examination revealed bilaterally prominent parotid glands with diffuse firmness but no discrete masses. There was no evidence of facial nerve dysfunction. Laboratory evaluation was negative for infectious and autoimmune etiologies. Magnetic resonance imaging revealed bilateral cystic parotid masses. FNAB of the right parotid was obtained to assist with preoperative counseling. It revealed lymphoid and salivary gland parenchymal cells. The patient underwent a right superficial parotidectomy. The surgical specimen of the parotid mass confirmed the diagnosis of sebaceous lymphadenoma on the tissue section. The contralateral parotid mass had not been excised at this writing. CONCLUSION: This report is the first to describe the FNAB findings of the unusual benign parotid neoplasm sebaceous lymphadenoma. Though the definitive diagnosis of any parotid mass requires tissue, generally obtained via parotidectomy, an FNAB diagnosis can be useful in counseling a patient prior to definitive biopsy.

Abnormal division and gene expression in cultured cells from a patient with tuberous sclerosis.

Tuberous sclerosis is a dominant hereditary disease characterized by the appearance of angiofibromas on the face, epileptic attacks, and mental retardation. Attention has been paid to the atypical cells observed histologically in the stroma of the angiofibroma as they may play an important role in forming lesions. We have established a cell line from an angiofibroma to clarify the nature of these cells at the cellular and biochemical levels. The cultured cells have been stably maintained for four years and show an abnormal morphology resembling that of neuronal cells. In culture, these cells divide into daughter cells, which consist of both normal cells and abnormal ones whose nuclei disintegrate and cannot divide further. The cells resemble normal fibroblasts in their pattern of staining with antibodies against tubulin, actin, vimentin, and fibronectin. However, they also stain with an antibody against the glia-specific acidic protein, which is thought to be a specific marker protein for glial cells. The gel profile of cyclic-AMP binding proteins was not fibroblastic, but rather of the neuron cell type. These results indicate the gene expression of these cells is also abnormal. They are a useful tool for understanding this specific genetic disease.

Estrogen binding component in the pilosebaceous tumor developed in Suncus murinus.

We transplanted a pilosebaceous tumor developed on the sidegland of Suncus murinus to male nude athymic (BALB/c-nu/nu) mice. This tumor can be transplanted to female hosts as well, with a lower rate of graft-taking and slower growth rate. In this study we demonstrated the presence of macromolecules which specifically bind to estrogen. Measurement of 17 beta-estradiol (E2) binding by a dextran-coated charcoal assay revealed that the number of binding sites and the dissociation constant were 22.3 +/- 4.6 fmol/mg protein and 1.4 +/- 0.24 X 10(-9) M, respectively. This binding was specific for E2 and diethylstilbestrol (DES). Sucrose gradient centrifugation of the [3H]E2-labeled cytosol yielded a sharp peak of radioactivity at 3.5S-4S under high salt conditions and a 9S peak with a shoulder at 3.5S under low salt conditions. This 3.5S shoulder was due to dissociation of [3H]E2 from the 9S peak during the centrifugation, since only the 9S peak was obtained by postlabeled density gradient analysis. An assay of the in vivo binding of [3H]E2 showed significant radioactivity in the nuclear extract from the tumor. This nuclear uptake was markedly decreased by simultaneous administration of 100-fold excess of E2. In tumor-bearing castrated nude mice, 1-100 micrograms/day of E2 did not affect tumor growth, whereas it counteracted the stimulative effect of testosterone propionate.

The tuberous sclerosis syndrome: clinical and EEG studies in 100 children.

The evolution of the early clinical and EEG features in 100 children who developed the tuberous sclerosis syndrome is discussed with particular emphasis on individual variability of epileptic manifestations, skin changes, and mental defect. There were 61 boys and 39 girls. Seizures of various kinds occurred in 98 and in the first 2 years of life infantile spasms were a prominent feature (69) with a partial overlap of other kind of seizures (75). Mental defect (88) and poorly pigmented areas of the skin (77) were already detectable in the first 1 to 2 years of life, while fibroangioma of the face (adenoma sebaceum) (77) and intracranial calcifications (35) became increasingly apparent after the age of 2 to 4 years. The EEG abnormalities tended to be gross in the first 2 years of life, but their subsequent evolution was towards multifocal alterations and some areas of relatively better preserved rhythmic activity. The evolution of the various skin lesions did not run parallel either with that of the clinically detectable seizures or with the appearance of intracranial calcifications.