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  • 1
    Digitale Medien
    Digitale Medien
    Springer
    Der Pathologe 17 (1996), S. 296-300 
    ISSN: 1432-1963
    Schlagwort(e): Schlüsselwörter Rhabdomyosarkom ; Fibrosarkom ; Immunhistochemie ; Key words Rhabdomyosarcoma ; Fibrosarcoma ; Immunohistochemistry
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Beschreibung / Inhaltsverzeichnis: Summary We report a case of an intrathoracic, extrapleural, infantile rhabdomyofibrosarcoma in a 4-year-old boy. Histologically, the primary lesion showed extensive hyalinization and stroma sclerosis and was composed of relatively uniform spindle-shaped, at least focally rather polygonal tumour cells with scattered intracytoplasmatic globoid inclusions. Although chemo- and radiotherapy was given postoperatively, local recurrences and metastases in the lung and thymus have developed; the patient died of tumour disease 3 years later. Recurrences and metastases showed features of tumour progression with higher cellularity and increased mitotic activity. Immunohistochemically, the tumour cells stained strongly positive for vimentin, desmin, and muscle-specific actin, and at least focally for MyoD1; the tumour did not stain for alpha-smooth muscle actin, neural and epithelial markers, or CD34 and CD31. The differential diagnosis of these aggressive tumours in the spectrum of spindle-cell lesions in early childhood is discussed.
    Notizen: Zusammenfassung Wir referieren den Fall eines intrathorakalen, extrapleuralen, infantilen Rhabdomyofibrosarkomes bei einem 4jährigen männlichen Patienten. Der Primärtumor wies histologisch eine deutliche Hyalinisierung und Sklerose des Stroma auf und bestand aus relativ uniformen spindeligen, fokal polygonalen Tumorzellen mit nachweisbaren globoiden intrazytoplasmatischen Einschlüssen. Trotz adjuvanter Chemo- und Radiotherapie entwickelten sich ausgedehnte Tumorrezidive und Metastasen in der Lunge und dem Thymus; der Patient verstarb 3 Jahre später. In den Tumorrezidiven und Metastasen fand sich eine Tumorprogression zu einem zellreicheren, mitotisch aktiveren Neoplasma. Immunhistochemisch zeigten die Tumorzellen eine deutlich ausgeprägte Positivität für Antikörper gegen Vimentin, Desmin und muskelspezifisches Aktin sowie eine fokal nachweisbare nukleäre Positivität für MyoD1; der Tumor zeigte keine Immunpositivität für alpha-glatt muskuläres Antigen, neurale und epitheliale Marker, sowie für CD34 und CD31. Die Differentialdiagnose dieses aggressiven Tumors im Spektrum der spindelzelligen Läsionen im Kleinkindalter wird diskutiert.
    Materialart: Digitale Medien
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  • 2
    Digitale Medien
    Digitale Medien
    Oxford, UK : Blackwell Publishing Ltd
    Journal of the European Academy of Dermatology and Venereology 6 (1996), S. 0 
    ISSN: 1468-3083
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: Three cases of solitary circumscribed neuroma (so-called palisaded, encapsulated neuroma) arising in the dermis have been studied. Two patients were male and one was female, with ages ranging from 27 to 59 years. All lesions arose on the forehead. Histologically, the neoplasms were partly encapsulated and were composed of broad fascicles, nests, and whorl-like structures of bland appearing spindle cells often separated by artefactual clefts. Immunohistochemically. most of the tumour cells stained positive for S-100 protein, and a considerable number also for CD 34, but failed to stain for epithelial membrane antigen and melanoma associated antigens. Solitary circumscribed neuroma has to be distinguished from long-standing dermal melanocytic nevus, mucosal neuromas seen in type lib multiple endocrine neoplasia (MEN) syndrome, classical and cellular schwannoma, neurofibroma. perineurioma, traumatic neuroma, and angioleiomyoma.
    Materialart: Digitale Medien
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  • 3
    Digitale Medien
    Digitale Medien
    Oxford, UK : Blackwell Science Ltd
    Journal of the European Academy of Dermatology and Venereology 19 (2005), S. 0 
    ISSN: 1468-3083
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Materialart: Digitale Medien
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  • 4
    Digitale Medien
    Digitale Medien
    Oxford, UK : Blackwell Publishing Ltd
    Histopathology 25 (1994), S. 0 
    ISSN: 1365-2559
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: Four cases of perineurioma (storiform perineurial fibroma) arising in the dermis, subcutis, or deep soft tissue have been studied. Two patients were female and two were male with ages ranging from 19 to 45 years. One lesion each arose on the chest wall, shoulder, neck, and elbow. Follow-up information in three patients revealed no recurrence. Histologically, the neoplasms were circumscribed but non-encapsulated lesions and were composed of spindle cells with elongated bipolar cytoplasmic processes, inconspicuous fusiform nuclei and well-defined palely eosinophilic cytoplasm. These cells were arranged in whorls or lamellar-like structures and often demonstrated a storiform growth pattern. In areas, the tumour cells appeared larger with more rounded nuclei. Immunohistochemically, most of the tumour cells stained positive for epithelial membrane antigen and vimentin, but failed to stain for S-100 protein, neurofilament, desmoplakin, and CD34. Ultrastructurally, two cases showed fusiform tumour cells with long, thin cell processes separated by abundant collagen bundles. Tumour cells were covered by discontinuous external lamina, showed many pinocytic vesicles and occasionally desmosome-like structures. The morphology and EMA immunopositivity of perineurioma are similar to meningioma, especially to cutaneous meningioma type II. We believe that perineurioma and meningioma are closely related, but morphologically distinguishable, neoplasms.
    Materialart: Digitale Medien
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  • 5
    Digitale Medien
    Digitale Medien
    Oxford, UK : Blackwell Publishing Ltd
    Histopathology 26 (1995), S. 0 
    ISSN: 1365-2559
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: Low grade fibromyxoid sarcoma is a recently recognized, uncommon soft tissue neoplasm with a tendency to develop in deep soft tissue of young adults. Diagnostic criteria have not been well defined and this tumour has not been widely accepted as a distinct entity. Eleven new cases are reported here for which reproducible histological features are described and in which the immunohistochemical profile of the tumour cells is documented for the first time. Ten of the eleven patients were male and the majority were young or middle-aged adults (median age 45 years). All except one of the tumours were situated in deep soft tissue. Lower limb (four cases) and chest wall (three cases) were the commonest primary sites; one case each arose in the groin, buttock, axilla and retroperitoneum. Follow-up (median duration 6 years) was available in nine patients. Six developed local recurrence and in five cases recurrences were multiple. Pulmonary metastasis occurred in one patient. All tumours were characterized by the presence of bland spindle cells, showing a mainly whorled or focally linear arrangement, set in alternating areas with a fibrous or myxoid stroma. Tumour cells were small, spindle to stellate, with poorly defined, palely eosinophilic cytoplasm and hyperchromatic ovoid nuclei. Most tumour cells showed strong staining with antibodies to vimentin, while occasional cells stained positively for actin, desmin and cytokeratin, in keeping with focal myofibroblastic differentiation. Ultrastructural examination in one case revealed features of fibroblasts. Careful consideration of the morphological and immunohistochemical features of these tumours permits a positive diagnosis of low grade fibromyxoid sarcoma and allows its distinction from a number of other benign and malignant soft tissue neoplasms.
    Materialart: Digitale Medien
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  • 6
    Digitale Medien
    Digitale Medien
    Oxford, UK : Blackwell Publishing Ltd
    Histopathology 26 (1995), S. 0 
    ISSN: 1365-2559
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: One hundred and sixty-eight cellular (‘infantile’) capillary haemangiomas were assessed for the presence of perineurial invasion, a feature that can lead to the erroneous diagnosis of malignancy. Fourteen tumours (8%) showed unequivocal, usually prominent, involvement of small and medium sized nerves. Eleven of these lesions presented in infants at birth or shortly thereafter, two in young adults and one in a middle-aged adult. Ten patients were females. Ten of the lesions arose in the head and neck region, one in the arm, one in the chest wall, and in two the site was not stated. Follow-up revealed a local recurrence in only one of six cases. Histologically, all cases were typical pure capillary haemangiomas composed of lobules of small blood vessels, lined by bland endothelial cells, involving the dermis, subcutis or both. One case was an intramuscular capillary haemangioma. Long-standing cases, especially those in adults, were less cellular, with focal fibrosis and a myxoid stroma. Neural invasion was detected either in the centre or at the periphery of tumour lobules and was characterized by the presence of variable numbers of capillaries within the perineurium and in close contact with Schwann cells. This feature was highlighted by immunostaining for S-100 protein and EMA. In one case, extensive invasion of medium-sized deep dermal veins was also present, focally simulating an intravascular pyogenic granuloma. This study demonstrates that perineurial invasion in infantile capillary haemangiomas is a relatively common finding and should not be regarded as evidence of malignancy.
    Materialart: Digitale Medien
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  • 7
    Digitale Medien
    Digitale Medien
    Oxford, UK : Blackwell Publishing Ltd
    Histopathology 23 (1993), S. 0 
    ISSN: 1365-2559
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: The clinicopathological features of 14 cases of lipoblastoma and lipoblastomatosis are presented. The age of the patients at presentation ranged from 5 days to 6 years (mean 2.7 years); nine patients were male. Histologically, six cases were circumscribed (lipoblastoma) while eight were diffuse and ill-defined (lipoblastomatosis). In both groups and in individual cases there was distinct lobulation, as well as a spectrum of adipocytic maturation. Cytologically, the 10 most mature lesions were composed of uniform adipocytes intermixed with only scattered lipoblasts and primitive mesenchymal cells. A notable feature in the other four cases was a prominent myxoid stroma producing a very close resemblance to myxoid liposarcoma. Mitotic figures were rare and always normal in appearance. Atypical nuclei were not evident. Follow-up in eight patients revealed local recurrence in two. Liposarcoma in patients under 10 years is exceedingly rare, and, in myxoid form, may be almost impossible to distinguish histologically from lipoblastoma. Helpful clues are the lack of lobulation, variable growth pattern and increased nuclear atypia in liposarcoma.
    Materialart: Digitale Medien
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  • 8
    Digitale Medien
    Digitale Medien
    Oxford, U.K. and Cambridge, USA : Blackwell Science Ltd
    Histopathology 29 (1996), S. 0 
    ISSN: 1365-2559
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Materialart: Digitale Medien
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  • 9
    ISSN: 1365-2559
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: Aims:  Dermatomyofibroma (plaque-like dermal fibromatosis) represents a distinct clinicopathological entity in the spectrum of cutaneous mesenchymal neoplasms showing a myofibroblastic line of differentiation. These benign neoplasms occur frequently, but not exclusively, in young women, and the shoulder girdle as well as the upper trunk are common locations. Histologically, dermatomyofibroma is characterized by a plaque-like proliferation of cytologically bland spindle-shaped tumour cells containing an ill-defined, pale eosinophilic cytoplasm and elongated, neuroid nuclei. Neoplastic cells are arranged in bundles and fascicles orientated parallel to the skin surface, adnexal structures are spared and elastic fibres are increased and fragmented. Immunohistochemically, tumour cells express vimentin and variably muscle actin and α-smooth muscle actin, but are negative for desmin, CD34, S100, and epithelial markers. The main differential diagnosis includes hypertrophic scar, dermatofibroma (fibrous histiocytoma), pilar leiomyoma, neurofibroma, adult myofibromatosis, extra-abdominal fibromatosis and plaque-stage dermatofibrosarcoma protuberans.Methods and results:  We report three cases of dermatofibroma arising in male patients aged 31, 36, and 47 years on the thigh, chest wall and back, respectively. All lesions were completely excised and no local recurrence has been reported. Histologically, the neoplasms showed classical features of dermatomyofibroma; however, in addition abundant extravasated erythrocytes, scattered inflammatory cells, numerous capillaries, and sieve- and slit-like spaces, features resembling plaque-stage Kaposi's sarcoma, were noted. In none of the cases did spindled tumour cells stain positively for CD34, and HHV8 was not detected by polymerase chain reaction.Conclusions:  The reported cases widen the clinicopathological spectrum of dermatomyofibroma and emphasize plaque-stage Kaposi's sarcoma as an additional differential diagnosis.
    Materialart: Digitale Medien
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  • 10
    Digitale Medien
    Digitale Medien
    Oxford, U.K. and Cambridge, USA : Blackwell Science Ltd
    Histopathology 35 (1999), S. 0 
    ISSN: 1365-2559
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: Angiosarcomatous differentiation represents the least common form of heterologous differentiation in malignant peripheral nerve sheath tumours (MPNST), and is seen most frequently in patients with neurofibromatosis type 1. More rarely, it has been reported in patients without stigmata of neurofibromatosis, or in benign nerve sheath tumours and peripheral nerves. This study was undertaken to confirm this rare association.〈section xml:id="abs1-2"〉〈title type="main"〉Methods and resultsFour cases of angiosarcoma arising in a peripheral nerve, in a long-standing schwannoma and in two MPNST are described. Immunohistochemical studies were performed on paraffin sections with the alkaline phosphatase–antialkaline phosphatase method. An intraneural high-grade epithelioid angiosarcoma arose in the left posterior tibial nerve of a 78-year-old man, a well to moderately differentiated angiosarcoma was seen in an ancient schwannoma of the lateral neck in a 73-year-old women, and an angiosarcoma of varying grades of differentiation developed in a recurrent MPNST in the thigh of 38-year-old man. In addition a high-grade MPNST in the axillary region of a 30-year-old man showed foci of heterologous high-grade angiosarcomatous differentiation. The neural and endothelial lines of differentiation were confirmed in each case by positive immunohistochemical staining for neural and endothelial markers, respectively. In all cases tested, the neural differentiated cells stained immunohistochemically positive for antibodies against vascular endothelial growth factor.〈section xml:id="abs1-3"〉〈title type="main"〉ConclusionsThis study confirms the rare association of angiosarcoma arising in peripheral nerves, as well as in benign and maligant peripheral nerve sheath tumours.
    Materialart: Digitale Medien
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