Prim?re sinunasale Melanome Klinik, Therapie und Verlauf Klinik, Therapie und Verlauf

Hintergrund und Fragestellung. Prim?re Melanome der sinunasalen Schleimhaut gelten, verglichen mit der Gesamtzahl an malignen Melanomen, als sehr seltene Entit?t mit schlechter Prognose. Wir untersuchten H?ufigkeit und Charakteristika sinunasaler Melanome in einem geografisch definierten Einzugsgebiet in einem Zeitraum von 16 Jahren.     

Kongenitale bull?se Poikilodermie (Kindler-Syndrom)

Es wird über eine 18-j?hrige Patientin berichtet, die wegen Synechien der Labien zur weiteren Abkl?rung überwiesen wurde. Anamnestisch erhebbare kongenitale Blasenbildung und Photosensitivit?t in der Kindheit sowie ausgedehnte Poikilodermien mit retikul?rer Pigmentierung und Xerose, proximale Synechien der Finger und Fehlen der Papillarleisten an H?nden und Fü?en im aktuellen Status erbrachten die Diagnose einer kongenitalen bull?sen Poikilodermie (Kindler-Syndrom), die histologisch und elektronenmikroskopisch best?tigt wurde. Es handelt sich um ein seltenes, autosomal-rezessives Krankheitsbild, bei dem Verwachsungen der Schleimh?ute und übergangsschleimh?ute im Vordergrund stehen k?nnen [2, 4].     

Cowden syndrome

A patient with multiple hamartoma syndrome or Cowden's disease with multiple gastrointestinal polyps and malignant melanoma is presented. The syndrome is characterized by hamartomatous tumors of the skin, fibrocystic disease of the breasts, gastrointestinal polyps and disease of the thyroid gland such as goiter and adenoma. In addition, other abnormalities and malformations occur in the skeletal system, central nervous system and urogenital tract. Recognition of this syndrome is important because of the association with malignant tumors of the breast and thyroid gland and with malignant melanoma. Gastrointestinal hamartomatous polyps may lead to the diagnosis of Cowden's disease which must be separated from other intestinal polyposis syndromes.     

Pitfalls in the diagnosis of pemphigus vulgaris (early pemphigus vulgaris with features of bullous pemphigoid)

This report draws attention to pitfalls that may be encountered in histologic diagnosis of early stages of pemphigus vulgaris. A man is described who had clinical and histologic features of bullous pemphigoid to begin with and later typical features of pemphigus vulgaris. Intercellular antibodies were demonstrable by direct immunofluorescence from the beginning.     

Gastrointestinal polyposis in the Cowden syndrome

A case of Cowden disease (multiple hamartoma syndrome) with marked gastrointestinal polyposis is presented. The differential diagnosis of gastrointestinal polyposis syndromes is discussed.     

Importance of the proliferating trichilemmal cyst

The biological behavior of proliferating trichilemmal cysts is commented on, based on two cases. Two female patients, 71 and 70 years of age, developed solitary monstrous tumours of the scalp in the course of repeatedly recurring trichilemmal cysts. The biological behaviour of both was marked by infiltration of the galea aponeurotica, and in the latter case, invasion of the calvaria and the venous sinuses of the brain with a lethal outcome. Histologically, the differentiation of the cyst walls ranged from pseudoepitheliomatous hyperplasia to areas largely lacking differentiation, indicating a trichilemmal carcinoma. Therefore, a more cautious evaluation of proliferating trichilemmal cysts, commonly underestimated as pseudocarcinomatous, is indicated. The second case in particular shows that on occasion an invasive carcinoma can arise in a trichilemmal cyst. Radical surgery can not only prevent recurrence with loss of differentiation, but also enable a better histological evaluation of the deep-lying tumour tissue.     

Tissue counter analysis of tissue components in skin biopsies: evaluation using CART (Classification and Regression Trees)

In tissue counter analysis, complex histologic sections are overlaid with regularly distributed measuring masks of equal size and shape, and the digital contents of each mask (or tissue element) are evaluated by gray level, color, and texture parameters. In this study, the feasibility of tissue counter analysis and classification and regression trees for the quantitative evaluation of skin biopsies was assessed. From 100 randomly selected skin biopsies, a learning set of tissue elements was created, differentiating between cellular elements, collagenous elements of the reticular dermis, fatty elements and other tissue components. Classification and regression trees based on the learning set were used to automatically classify tissue elements in samples of normal skin, benign common nevi, malignant melanoma, molluscum contagiosum, seborrheic keratosis, epidermoid cysts, basal cell carcinoma, and scleroderma. The procedure yielded reproducible assessments of the relative amounts of tissue components in various diagnostic groups. Furthermore, a reliable diagnostic separation of molluscum contagiosum versus normal skin and epidermal cysts, benign common nevi versus malignant melanoma, and seborrheic keratosis versus basal cell carcinoma was possible. Tissue counter analysis combined with classification and regression trees may be a suitable approach to the fully automated analysis of histologic sections of skin biopsies.     

Inflammatory cell types in normal human epidermis--an immunohistochemical and morphometric study

Inflammatory cells in 15 specimens of normal human epidermis were selectively stained by a monoclonal antibody immunoperoxidase technique. The quantitative assessment using an interactive image analysis system revealed OKT 11 positive cells (T lymphocytes) and Leu 2a positive cells (suppressor/cytotoxic cells). As there was no significant difference in the distribution of these markers, helper/inducer cells obviously are not present in considerable amounts. OKM 5 positive cells outnumbered OKM 1 positive cells, indicating the presence of a OKM 5+, OKM 1-macrophage subset. The epidermal dendritic cells clearly showed a striking heterogeneity regarding the expression of HLA-DR (62% of OKT 6-positive cells) and Leu 3a (47%), suggesting the existence of immunologically distinct subsets of human epidermal dendritic cells.     

Morphometric diagnosis of melanocytic skin tumors

Checking consecutively sampled routine sections of 206 melanocytic lesions with a maximum vertical diameter of at least 1 mm (133 benign dermal nevi, 20 Spitz's nevi, 53 primary malignant melanomas), we measured the morphometric features of at least 60 nuclei each from the superficial and the deep dermal tumor portion using a computer-assisted interactive image analysis system. Furthermore we calculated the so-called maturation parameter (MP) in each case as the ratio of the mean nuclear area in the deep portion and the superficial portion. When we compared the results with those obtained in a training set, we found that the lowest evidence for the discrimination of benign and malignant melanocytic lesions resulted from the application of the mean values of the nuclear area in the superficial layer (efficiency = 62.1%). The efficiency was higher when we used the mean values of the nuclear area in the deep layer (96.1%) and the maturation parameter (85.4%). By applying the mean nuclear area in the deep portion and the maturation parameter simultaneously, we gained the highest efficiency, specificity, and sensitivity for the distinction between benign dermal nevi and malignant melanomas (0.968, 0.955, 1) as well as for the distinction between Spitz's nevi and malignant melanomas (0.986, 0.950, 1). Our study shows that morphometry provides reliable diagnostic results in routinely sampled melanocytic skin tumors.