Luis RequenaPanitta SitthinamsuwanIsabella FriedSteven KadduCarl Georg SchirrenLeo SchärerMarkus HantschkeLorenzo CerroniTimothy H. McCalmontHeinz KutznerUniversidad Autonoma de MadridMahidol UniversityMedizinische Universitat GrazDermatopathology LaboratoryDermatophatologisches GemeinschaftslaborUniversity of California, San Francisco2018-10-192018-10-192013-06-01American Journal of Surgical Pathology. Vol.37, No.6 (2013), 845-85215320979014751852-s2.0-84880158383https://repository.li.mahidol.ac.th/handle/20.500.14594/32326There 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 wellcircumscribed 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. Copyright © 2013 by Lippincott Williams & Wilkins.Mahidol UniversityMedicineArticleSCOPUS10.1097/PAS.0b013e31827edfda