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Requena, L; Sitthinamsuwan, P; Fried, I; Kaddu, S; Schirren, CG; Schärer, L; Hantschke, M; Cerroni, L; McCalmont, TH; Kutzner, H.
A benign cutaneous plexiform hybrid tumor of perineurioma and cellular neurothekeoma.
Am J Surg Pathol. 2013; 37(6):845-852
Doi: 10.1097/PAS.0b013e31827edfda
Web of Science
PubMed
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FullText_MUG
- Co-authors Med Uni Graz
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Cerroni Lorenzo
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Fried Isabella
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Kaddu Steven
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- Abstract:
- There are several recent reports describing hybrid peripheral nerve sheath tumors showing a biphasic component of neoplastic cells. These combinations include a mixture of neurofibroma and schwannoma, schwannoma and perineurioma, neurofibroma and perineurioma, and perineurioma and granular cell tumor. A case of a triphasic combination of neurofibroma, schwannoma, and perineurioma has also been described. We describe the clinicopathologic and immunohistochemical characteristics of 9 cases of a benign cutaneous plexiform nerve sheath tumor located on the lips and exhibiting hybrid features of perineurioma and cellular neurothekeoma. Clinically, lesions were solitary dome-shaped papules located on the lips. Histopathologically, the neoplasms consisted of well-circumscribed but uncapsulated dermal nodules with a plexiform pattern. They were composed of nests or rounded aggregations of neoplastic cells embedded in a slightly myxoid stroma. Within the aggregates, cells were distributed in a storiform and lamellar pattern. Immunohistochemically, most neoplastic cells expressed strong immunoreactivity for S100A6, MiTF, NKI/C3, PGP9.5, EMA, and NSE, whereas variable, focal, and weaker positivity for CD34, claudin-1, and Glut-1 was seen in some cases. On the basis of these findings, we believe that this neoplasm is a distinctive benign cutaneous plexiform nerve sheath tumor with histopathologic and immunohistochemical hybrid features of perineurioma and cellular neurothekeoma.
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Adult -
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Aged -
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Female -
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Humans -
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Immunohistochemistry -
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Lip Neoplasms - metabolism Lip Neoplasms - pathology
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Male -
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Middle Aged -
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Neoplasms, Complex and Mixed - metabolism Neoplasms, Complex and Mixed - pathology
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Nerve Sheath Neoplasms - metabolism Nerve Sheath Neoplasms - pathology
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Neurothekeoma - metabolism Neurothekeoma - pathology
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Tumor Markers, Biological - analysis
- Find related publications in this database (Keywords)
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cutaneous peripheral nerve sheath tumors
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plexiform neural tumors
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hybrid neural tumors
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perineurioma
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cellular neurothekeoma
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immunohistochemistry