Whorling cellular perineurioma: A previously undescribed variant closely mimicking monophasic fibrous synovial sarcoma

Michal M, Kazakov DV, Agaimy A, Hosova M, Michalova K, Grossmann P, Steiner P, Skenderi F, Vranic S, Michal M (2017)

Publication Type: Journal article

Publication year: 2017

Journal

Annals of Diagnostic Pathology WB Saunders

Book Volume: 27

Pages Range: 74-78

DOI: 10.1016/j.anndiagpath.2017.02.001

Abstract

The authors present a distinctive perineurioma (PN) variant which morphologically strongly resembles monophasic fibrous synovial sarcoma (MSS). The patients were 3 males and 1 female. The age ranged from 15 to 61years (mean: 44years). Locations included the sole, lower jaw, palm and foot. The tumor size ranged from 1.3cm to 2.5cm in the largest dimension (mean 1.8cm). Morphologically, all tumors had an identical, monotonous appearance. The perineurial cells were closely packed and created a confluent cellular whorls and/or sheets in a scarce stroma, with only focally discernible long, slender cytoplasmic processes typical for perineurial differentiation. The nuclei were rounded or slightly elongated to tapered, without nuclear atypia. Mitoses were rare to completely absent. Atypical mitoses, hemorrhage, necrosis or calcifications were not present. The proliferative index (Ki-67) was 1-3%. All analyzed tumors were positive for EMA, Claudin-1, GLUT-1 and negative with S100 protein, CD34, OSCAR, CK7 and TLE-1. Two cases were tested by fluorescence in situ hybridization and neither showed alterations of the SYT gene. One case studied by electron microscopy showed characteristic features of perineurial differentiation. Follow-up was available for two patients both of which showed no evidence of disease at 8years and 6months, respectively. Based on their bland morphology, perineurial features and presumably benign clinical outcome we propose the term "whorling cellular perineurioma" for these tumors, which may represent an extremely cellular variant of sclerosing PN. Awareness of this PN subtype and its distinction from MSS is of utmost clinical significance.

Authors with CRIS profile

Abbas Agaimy Lehrstuhl für Allgemeine Pathologie und Pathologische Anatomie

Involved external institutions

Univerzita Karlova v Praze / Charles University in Prague CZ Czech Republic (CZ) University of Sarajevo BA Bosnia And Herzegovina (BA)

How to cite

APA:

Michal, M., Kazakov, D.V., Agaimy, A., Hosova, M., Michalova, K., Grossmann, P.,... Michal, M. (2017). Whorling cellular perineurioma: A previously undescribed variant closely mimicking monophasic fibrous synovial sarcoma. Annals of Diagnostic Pathology, 27, 74-78. https://doi.org/10.1016/j.anndiagpath.2017.02.001

MLA:

Michal, Michael, et al. "Whorling cellular perineurioma: A previously undescribed variant closely mimicking monophasic fibrous synovial sarcoma." Annals of Diagnostic Pathology 27 (2017): 74-78.

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