PMID- 27816720 OWN - NLM STAT- MEDLINE DCOM- 20170828 LR - 20180114 IS - 1532-8392 (Electronic) IS - 0046-8177 (Linking) VI - 60 DP - 2017 Feb TI - Multivacuolated mucin-filled cells: a unique cell characteristic of plexiform neurofibroma. A report of 11 cases. PG - 167-173 LID - S0046-8177(16)30281-7 [pii] LID - 10.1016/j.humpath.2016.10.010 [doi] AB - The authors present 11 cases of plexiform neurofibroma (PN) that featured a very characteristic type of cell appearing as multivacuolated mucin-filled cells (MMFC). The 11 cases were obtained after reviewing 109 cases of PN. Six out of 10 patients showed clinical features of neurofibromatosis type 1. The size of PN ranged from 0.8 cm to 11.5 cm in the largest dimension. The lesions represented classical PN in all cases with myxoid, hypocellular stroma. The MMFC were found within the most myxoid tumorous nodules and were haphazardly located, typically featuring a variably sized, multivacuolated cytoplasm divided by fine septa with a small polygonal nucleus on one side, which was often compressed or slightly indented by the cytoplasmic mucous substances. In many cases, the cells resembled a soccer ball or a jellyfish. In all tested cases (n = 9), the MMFC stained for CD34; six cases were also positive with GLUT-1 antibody, and two cases expressed Claudin-1, whereas S-100 protein was negative. For comparison, we have reviewed a series of randomly selected non-PN, malignant peripheral nerve sheath tumors (MPNST) and of cases featuring non-neoplastic nerve trunks in our files, in which no MMFC were encountered. MMFC seem to be unique to myxoid areas of PN, where they occur in about 10% of cases. Their exact histogenesis is unclear but they might represent an intermediate type of cell between perineurial cells and fibroblasts. The awareness of this cell type in PN is especially important in limited (small) biopsy specimens where their recognition may provide a clue for the correct diagnosis. CI - Copyright (c) 2016 Elsevier Inc. All rights reserved. FAU - Michal, Michael AU - Michal M AD - Department of Pathology, Charles University, Biomedical Center, Faculty of Medicine in Plzen and Charles University Hospital Plzen, Alej Svobody 80, 304 60 Pilsen, Czech Republic. Electronic address: michael.michal@medima.cz. FAU - Kazakov, Dmitry V AU - Kazakov DV AD - Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Alej Svobody 80, 304 60 Pilsen, Czech Republic. FAU - Hadravsky, Ladislav AU - Hadravsky L AD - Department of Pathology, Charles University, Third Medical Faculty and Charles University Hospital Kralovske Vinohrady, Srobarova 50, 100 34 Prague, Czech Republic. FAU - Michalova, Kvetoslava AU - Michalova K AD - Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Alej Svobody 80, 304 60 Pilsen, Czech Republic. FAU - Rychly, Boris AU - Rychly B AD - Cytopathos, Limbova 5, 833 07, Bratislava 37, Slovakia. FAU - Michal, Michal AU - Michal M AD - Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Alej Svobody 80, 304 60 Pilsen, Czech Republic. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20161102 PL - United States TA - Hum Pathol JT - Human pathology JID - 9421547 RN - 0 (Antigens, CD34) RN - 0 (Biomarkers, Tumor) RN - 0 (CLDN1 protein, human) RN - 0 (Claudin-1) RN - 0 (Glucose Transporter Type 1) RN - 0 (Mucins) RN - 0 (SLC2A1 protein, human) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Antigens, CD34/analysis MH - Biomarkers, Tumor/*analysis MH - Biopsy MH - Child MH - Claudin-1/analysis MH - Female MH - Glucose Transporter Type 1/analysis MH - Humans MH - Immunohistochemistry MH - Male MH - Mucins/*analysis MH - Neurofibroma, Plexiform/*chemistry/pathology MH - Neurofibromatosis 1/*metabolism/pathology MH - Predictive Value of Tests MH - Prognosis MH - Vacuoles/*chemistry/pathology MH - Young Adult OTO - NOTNLM OT - Multivacuolated lipoblast- like cells OT - Multivacuolated mucin-filled cells OT - Perineurial cells OT - Plexiform neurofibroma OT - Soft tissue EDAT- 2016/11/07 06:00 MHDA- 2017/08/29 06:00 CRDT- 2016/11/07 06:00 PHST- 2016/08/15 00:00 [received] PHST- 2016/10/09 00:00 [revised] PHST- 2016/10/14 00:00 [accepted] PHST- 2016/11/07 06:00 [pubmed] PHST- 2017/08/29 06:00 [medline] PHST- 2016/11/07 06:00 [entrez] AID - S0046-8177(16)30281-7 [pii] AID - 10.1016/j.humpath.2016.10.010 [doi] PST - ppublish SO - Hum Pathol. 2017 Feb;60:167-173. doi: 10.1016/j.humpath.2016.10.010. Epub 2016 Nov 2.