Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0268596 (EMA)
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We describe the clinicopathologic characteristics of 55 oral palisaded encapsulated (solitary circumscribed) neuromas (PEN/SCN). Fifty-five cases of PEN/SCN in 54 patients were reviewed. Lesions were categorized according to their histologic pattern, partial or complete encapsulation, presence of Verocay bodies and presence of a parent peripheral nerve. In 13 selected cases immunohistochemical evaluation for neuronal markers (S-100, GFAP, NFP, EMA) was performed. When immunoreaction with EMA was weak, claudin-1 and glut-1 stains were utilized. Thirty-eight patients were men and 16 were women. Mean patient age was 48 years (SD: +/-14). The vast majority involved the masticatory (palate and gingiva) mucosa (76.4%) followed by the labial mucosa, the tongue and buccal mucosa. Recurrence was recorded in only one case. Histologically, 34 lesions had a lobular pattern, 10 were plexiform, 7 fungating and 4 multilobular. Stroma was limited, but focal myxoid changes were seen at the periphery of the lobules. Only one predominantly myxoid lesion was encountered. The number of intralesional axons varied, but the ratio of Schwann cells to axons was generally less than 1:2. Most lesions (89%) were only partially surrounded by perineurium. Tumor cells were S-100 positive and GFAP negative. The parent nerve was identified in 50% of the cases. Overlying epithelium was generally atrophic. Peritumoral connective tissue was generally unremarkable, but chronic inflammation was present in five cases. PEN/SCN is a relatively common peripheral nerve sheath tumor. Generally, its diagnosis is simple. GFAP may be of help to distinguish PEN/SCN from other peripheral nerve sheath tumors (schwannoma, neurofibroma, traumatic neuroma) in cases where histomorphologic features may be confusing. Finally, pathologists should be aware of the occurrence of plexiform and multilobular PEN/SCN variants, to avoid misinterpretation as plexiform neurofibroma or schwannoma.
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PMID:Palisaded encapsulated ("solitary circumscribed") neuroma of the oral cavity: a review of 55 cases. 2023 84

Background. Solitary circumscribed neuroma (SCN), also known as palisaded encapsulated neuroma (PEN), is a benign neural tumor. It may be mistaken as either schwannoma or neurofibroma in pathology practice. In this study, we aimed to define clinicopathologic and immunohistochemical features and discuss its differential diagnosis. Materials and Methods. The histopathological features of 30 cases of SCN/PEN were reviewed. The presence of intralesional axons, Schwann, and perineural cell distributions were investigated by performing neuronal immunomarkers. Results. Twelve cases were females, and 18 cases were males. The mean age was 48 years. Lesions were mostly located on the face (27/30). Histologically, 18 had a lobular pattern, 9 were plexiform, 2 fungating, and 1 multilobular. Although the majority of cases were well circumscribed, capsular integrity was at least focally disrupted (73%). Verocay body was noted only in 6 cases (20%). One case showed excessive hyperkeratosis, forming a keratin horn. Adipocytic change was detected in another case. The lesions consisted of S100-positive Schwann cell proliferation and were partially surrounded by perineural cells highlighted by EMA or Claudin-1. The amount of intralesional axons revealed by neurofilament immunostaining was variable. Conclusion. SCN/PEN is a relatively common lesion, and usually seen as an asymptomatic papule on the face of elderly patients. A circumscribed lesion composed of bundles of bland-looking spindle cells thought to be of neural origin is seen in the dermis. Pathologists should be aware of the existence of plexiform and multilobular PEN/SCN variants, to avoid misdiagnosis of plexiform neurofibroma or schwannoma.
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PMID:Palisaded Encapsulated (Solitary Circumscribed) Neuroma: A Review of 30 Cases. 3083