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  • 1
    ISSN: 1432-1963
    Keywords: Schlüsselwörter Rhabdomyosarkom ; Fibrosarkom ; Immunhistochemie ; Key words Rhabdomyosarcoma ; Fibrosarcoma ; Immunohistochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: 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.
    Notes: 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.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1173
    Keywords: Schlüsselwörter Angiom ; „tufted angioma“ ; Angioblastom ; kapilläres Hämangiom ; Immunhistochemie ; Key words Angioma ; Tufted angioma ; Angioblastoma ; Capillary haemangioma ; Immunhistochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Two female and three male patients with acquired “tufted angioma” are presented. The age of these patients ranged from 10 to 62 years. Two lesions were sited in the head and neck region, two in the upper extremities, and one on the trunk. Clinically, the angiomatous lesions appeared as elevated plaques, flat lesions with papular and macular areas, or erythematous plaques with small nodules. In four cases a biopsy was done, and in one case the tumour was excised. Histologically, the neoplasms were characterized by irregularly distributed vascular tufts in the dermis, and, in one case, in the upper subcutis. The vascular tufts were composed of plump endothelial cells and spindle-shaped pericytes surrounded by crescent-shaped vascular spaces. The positive staining for CD 31 and for CD 34 and alpha-smooth muscle actin, and the negative staining of endothelial cells for factor VIII underline both the existence of two cellular components in tufted angioma and the immaturity of endothelial cells. Evidence of regular mitotic figures in two cases and increased proliferative activity in three out of four cases tested, emphasize the neoplastic nature of slowly growing tufted angioma. Benign tufted angioma is a distinct entity in the spectrum of capillary haemangiomas and must be distinguished from other vascular neoplasms.
    Notes: Zusammenfassung Es werden fünf Patienten, zwei weibliche und drei männliche Patienten mit einem büschelartigen Hämangiom („tufted angioma“) vorgestellt. Das Alter der Patienten lag zum Zeitpunkt der Exzision zwischen 10 und 62 Jahren, die Läsionen waren in jeweils zwei Fällen im Kopf/Halsbereich bzw. im Bereich der oberen Extremitäten und bei einem Patienten am Körperstamm lokalisiert. Die Tumoren imponierten klinisch als erhabene Plaques, als flache Tumoren mit papulösen und makulären Arealen oder als erythematöse Plaques mit kleinknotigen Anteilen. Vier der Läsionen wurden bioptiert, bei einer Patientin wurde eine operative Entfernung der Läsion mit anschließender plastischer Deckung vorgenommen. Die fünf Tumoren waren histologisch durch irregulär in der Dermis bzw. in einem Fall auch in der oberen Subkutis verteilte, vaskuläre Lobuli gekennzeichnet, die aus plumpen Endothelien und spindeligen Perizyten bestanden und immer wieder von gekrümmten vaskulären Spalträumen unvollständig umfaßt wurden. Die Immunpositivität für CD 31 bzw. CD 34 und alpha-glattmuskulärem Aktin bei Negativität der endothelialen Zellen gegenüber Faktor VIII assoziiertem Antigen verdeutlichen das Vorhandensein zweier zellulärer Komponenten im büschelartigen Hämangiom und die Unreife der endothelialen Tumorzellen. Der Nachweis von regulären Mitosefiguren in zwei Fällen und die erhöhte proliferative Aktivität in drei von vier untersuchten Fällen in den vaskulären Lobuli unterstreichen die neoplastische Natur des klinisch häufig langsam progredienten büschelartigen Tumors. Das benigne büschelartige Hämangiom stellt eine distinkte Entität im Spektrum der kapillären Hämangiome dar und muß von weiteren vaskulären Läsionen unterschieden werden.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Science Ltd
    Journal of the European Academy of Dermatology and Venereology 19 (2005), S. 0 
    ISSN: 1468-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Journal of the European Academy of Dermatology and Venereology 6 (1996), S. 0 
    ISSN: 1468-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Histopathology 26 (1995), S. 0 
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Histopathology 25 (1994), S. 0 
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Aims : To analyse seven cases of cutaneous myomelanocytic tumour histologically and immunohistochemically. Perivascular epithelioid cell tumours (so-called PEComas) are rare and recently delineated neoplasms occurring in the lung, kidney, pancreas, uterus, falciform ligament, vulva, heart, prostate and soft tissues. PEComas are characterized by a perivascular location of neoplastic cells showing a broad spectrum of epithelioid and spindled cells with clear, and granular pale eosinophilic cytoplasm, and a variable expression of melanocytic and muscle markers, whereas S100 protein and cytokeratins are usually absent.Methods and results : We report seven cases of cutaneous myomelanocytic tumour arising on the lower (six cases) and upper (one case) extremities of female adults (age range 30–66 years). In all cases an ill-defined dermal lesion with extension into subcutaneous tissue was noted. The neoplasms contained numerous blood vessels with a lace-like pattern and slightly thickened vessel walls, and were composed of perivascular epithelioid cells containing clear or focally granular pale eosinophilic cytoplasm and round vesicular nuclei with small, sometimes slightly enlarged nucleoli. Increased proliferative activity and tumour necrosis were not seen. Immunohistochemically, tumour cells stained positively for HMB-45, microphthalmia transcription factor, and NKIC3 in all cases, whereas perivascular expression of α-smooth muscle actin and focal positivity for desmin were noted in one case each only. Two out of four cases tested stained focally positive for calponin. No expression of S100 protein and pancytokeratin was present. Despite incomplete/marginal excision in three cases none of the neoplasms has recurred locally so far.Conclusions : With the presented series of cutaneous myomelanocytic tumours the clinicopathological spectrum of PEComas is expanded.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Histopathology 24 (1994), S. 0 
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Nine smooth muscle tumours, arising at a variety of sites and showing granular cell change of their cytoplasm, have been studied morphologically and immunohistochemically. The age of the patients ranged from 6 to 78 years (median 42 years); seven patients were female. Two tumours each arose in the dermis or subcutaneous tissue while the other five cases were situated in deeper soft tissue. Three of the lesions arose in the lower limbs, two in the pelvis and one each in the regions of the elbow, shoulder, breast and buttock. Follow-up in eight patients was available and revealed local recurrence in three and pulmonary metastases in two cases. All cases showed at least focally the light microscopic features of a smooth muscle tumour and demonstrated moderate to strong positivity for α-smooth muscle actin. Five were also HHF-35 positive and three were desmin positive. Noteworthy was strong positivity for the ‘melanoma associated’ antigen NKI/C3 in all cases. Six cases stained also weakly positive for NSE, but all were S-100 protein negative. The former is not specific but is the most reliable marker of lesions showing granular cell change. Granular cytoplasmic change represents simply a cytological phenotype, apparently representing a characteristic metabolic alteration, not exclusively associated with Schwann cell tumours. Tumours containing granular cells are best classified according to their line of specific cellular differentiation if possible.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Histopathology 23 (1993), S. 0 
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Histopathology 19 (1991), S. 0 
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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