The clinico-pathologic profile of primary and recurrent orbital/periorbital plexiform neurofibromas (OPPN).

To evaluate and compare the clinical and histopathological profile of primary and recurrent orbital-periorbital plexiform neurofibromas (OPPN) in patients with neurofibromatosis type 1. We retrospectively evaluated 43 primary or recurrent neurofibroma (NF) specimens from 26 patients (2002 to 2018) a...

Descripción completa

Guardado en:
Detalles Bibliográficos
Autores principales: Mohammad Alabduljabbar, Diego Strianese, Osama Al-Sheikh, Hind M Alkatan, Hailah Al-Hussain, Azza M Y Maktabi, Rajiv Khandekar, Malak Abedalthagafi, Deepak P Edward
Formato: article
Lenguaje:EN
Publicado: Public Library of Science (PLoS) 2021
Materias:
R
Q
Acceso en línea:https://doaj.org/article/71842b7e3fb94e08b782100ce57e2094
Etiquetas: Agregar Etiqueta
Sin Etiquetas, Sea el primero en etiquetar este registro!
id oai:doaj.org-article:71842b7e3fb94e08b782100ce57e2094
record_format dspace
spelling oai:doaj.org-article:71842b7e3fb94e08b782100ce57e20942021-12-02T20:16:39ZThe clinico-pathologic profile of primary and recurrent orbital/periorbital plexiform neurofibromas (OPPN).1932-620310.1371/journal.pone.0258802https://doaj.org/article/71842b7e3fb94e08b782100ce57e20942021-01-01T00:00:00Zhttps://doi.org/10.1371/journal.pone.0258802https://doaj.org/toc/1932-6203To evaluate and compare the clinical and histopathological profile of primary and recurrent orbital-periorbital plexiform neurofibromas (OPPN) in patients with neurofibromatosis type 1. We retrospectively evaluated 43 primary or recurrent neurofibroma (NF) specimens from 26 patients (2002 to 2018) at the King Khaled Eye Specialist Hospital, Saudi Arabia. Demographics, clinical presentation, and surgical intervention data were collected. Histopathological specimens were studied with hematoxylin-eosin, Alcian blue, and immunohistochemical markers; S-100, CD44, CD117, smooth muscle actin (SMA), neurofilament, and Ki-67. Of the 43 NFs specimens, 20 were primary and 23 recurrent tumors. For primary NF, the ratio of plexiform to the diffuse type was 13:7, however in recurrent tumors was 3:8 after the first recurrence, and 1:5 after multiple recurrences. Of the 17 patients with primary tumors that had paired recurrent tumors, 12/17 (70.6%) primary NFs were plexiform and 5/17 (29.4%) were diffuse. However, when tumors recurred, 13/17 tumors (76.5%) were diffuse and only 4/17 tumors (23.5%) had a plexiform pattern. The odds of a tumor having a diffuse pattern in recurrent NF was significantly higher than the plexiform pattern [OR = 7.8 (95% confidence interval 1.69:36.1) P = 0.008]. Primary plexiform NFs underwent an excision at a significantly younger age than the diffuse type. Recurrent NFs had significantly higher CD44, CD117, and neurofilament labeling (P = 0.02, P = 0.01 and P<0.001 respectively) but had significantly decreased Alcian blue, and S-100 labeling (P = 0.03, and P = 0.02 respectively) compared to primary tumors. SMA and Ki-67 proliferation index were not different between primary and recurrent NFs (P = 0.86, and P = 0.3 respectively). There appears to be a high risk for primary plexiform NFs to develop a diffuse histologic pattern when they recur. Immunohistochemical staining suggests a role of mast cells (CD117) and expression of infiltration makers (CD44) in the transformation of plexiform tumors to the diffuse phenotype.Mohammad AlabduljabbarDiego StrianeseOsama Al-SheikhHind M AlkatanHailah Al-HussainAzza M Y MaktabiRajiv KhandekarMalak AbedalthagafiDeepak P EdwardPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 16, Iss 10, p e0258802 (2021)
institution DOAJ
collection DOAJ
language EN
topic Medicine
R
Science
Q
spellingShingle Medicine
R
Science
Q
Mohammad Alabduljabbar
Diego Strianese
Osama Al-Sheikh
Hind M Alkatan
Hailah Al-Hussain
Azza M Y Maktabi
Rajiv Khandekar
Malak Abedalthagafi
Deepak P Edward
The clinico-pathologic profile of primary and recurrent orbital/periorbital plexiform neurofibromas (OPPN).
description To evaluate and compare the clinical and histopathological profile of primary and recurrent orbital-periorbital plexiform neurofibromas (OPPN) in patients with neurofibromatosis type 1. We retrospectively evaluated 43 primary or recurrent neurofibroma (NF) specimens from 26 patients (2002 to 2018) at the King Khaled Eye Specialist Hospital, Saudi Arabia. Demographics, clinical presentation, and surgical intervention data were collected. Histopathological specimens were studied with hematoxylin-eosin, Alcian blue, and immunohistochemical markers; S-100, CD44, CD117, smooth muscle actin (SMA), neurofilament, and Ki-67. Of the 43 NFs specimens, 20 were primary and 23 recurrent tumors. For primary NF, the ratio of plexiform to the diffuse type was 13:7, however in recurrent tumors was 3:8 after the first recurrence, and 1:5 after multiple recurrences. Of the 17 patients with primary tumors that had paired recurrent tumors, 12/17 (70.6%) primary NFs were plexiform and 5/17 (29.4%) were diffuse. However, when tumors recurred, 13/17 tumors (76.5%) were diffuse and only 4/17 tumors (23.5%) had a plexiform pattern. The odds of a tumor having a diffuse pattern in recurrent NF was significantly higher than the plexiform pattern [OR = 7.8 (95% confidence interval 1.69:36.1) P = 0.008]. Primary plexiform NFs underwent an excision at a significantly younger age than the diffuse type. Recurrent NFs had significantly higher CD44, CD117, and neurofilament labeling (P = 0.02, P = 0.01 and P<0.001 respectively) but had significantly decreased Alcian blue, and S-100 labeling (P = 0.03, and P = 0.02 respectively) compared to primary tumors. SMA and Ki-67 proliferation index were not different between primary and recurrent NFs (P = 0.86, and P = 0.3 respectively). There appears to be a high risk for primary plexiform NFs to develop a diffuse histologic pattern when they recur. Immunohistochemical staining suggests a role of mast cells (CD117) and expression of infiltration makers (CD44) in the transformation of plexiform tumors to the diffuse phenotype.
format article
author Mohammad Alabduljabbar
Diego Strianese
Osama Al-Sheikh
Hind M Alkatan
Hailah Al-Hussain
Azza M Y Maktabi
Rajiv Khandekar
Malak Abedalthagafi
Deepak P Edward
author_facet Mohammad Alabduljabbar
Diego Strianese
Osama Al-Sheikh
Hind M Alkatan
Hailah Al-Hussain
Azza M Y Maktabi
Rajiv Khandekar
Malak Abedalthagafi
Deepak P Edward
author_sort Mohammad Alabduljabbar
title The clinico-pathologic profile of primary and recurrent orbital/periorbital plexiform neurofibromas (OPPN).
title_short The clinico-pathologic profile of primary and recurrent orbital/periorbital plexiform neurofibromas (OPPN).
title_full The clinico-pathologic profile of primary and recurrent orbital/periorbital plexiform neurofibromas (OPPN).
title_fullStr The clinico-pathologic profile of primary and recurrent orbital/periorbital plexiform neurofibromas (OPPN).
title_full_unstemmed The clinico-pathologic profile of primary and recurrent orbital/periorbital plexiform neurofibromas (OPPN).
title_sort clinico-pathologic profile of primary and recurrent orbital/periorbital plexiform neurofibromas (oppn).
publisher Public Library of Science (PLoS)
publishDate 2021
url https://doaj.org/article/71842b7e3fb94e08b782100ce57e2094
work_keys_str_mv AT mohammadalabduljabbar theclinicopathologicprofileofprimaryandrecurrentorbitalperiorbitalplexiformneurofibromasoppn
AT diegostrianese theclinicopathologicprofileofprimaryandrecurrentorbitalperiorbitalplexiformneurofibromasoppn
AT osamaalsheikh theclinicopathologicprofileofprimaryandrecurrentorbitalperiorbitalplexiformneurofibromasoppn
AT hindmalkatan theclinicopathologicprofileofprimaryandrecurrentorbitalperiorbitalplexiformneurofibromasoppn
AT hailahalhussain theclinicopathologicprofileofprimaryandrecurrentorbitalperiorbitalplexiformneurofibromasoppn
AT azzamymaktabi theclinicopathologicprofileofprimaryandrecurrentorbitalperiorbitalplexiformneurofibromasoppn
AT rajivkhandekar theclinicopathologicprofileofprimaryandrecurrentorbitalperiorbitalplexiformneurofibromasoppn
AT malakabedalthagafi theclinicopathologicprofileofprimaryandrecurrentorbitalperiorbitalplexiformneurofibromasoppn
AT deepakpedward theclinicopathologicprofileofprimaryandrecurrentorbitalperiorbitalplexiformneurofibromasoppn
AT mohammadalabduljabbar clinicopathologicprofileofprimaryandrecurrentorbitalperiorbitalplexiformneurofibromasoppn
AT diegostrianese clinicopathologicprofileofprimaryandrecurrentorbitalperiorbitalplexiformneurofibromasoppn
AT osamaalsheikh clinicopathologicprofileofprimaryandrecurrentorbitalperiorbitalplexiformneurofibromasoppn
AT hindmalkatan clinicopathologicprofileofprimaryandrecurrentorbitalperiorbitalplexiformneurofibromasoppn
AT hailahalhussain clinicopathologicprofileofprimaryandrecurrentorbitalperiorbitalplexiformneurofibromasoppn
AT azzamymaktabi clinicopathologicprofileofprimaryandrecurrentorbitalperiorbitalplexiformneurofibromasoppn
AT rajivkhandekar clinicopathologicprofileofprimaryandrecurrentorbitalperiorbitalplexiformneurofibromasoppn
AT malakabedalthagafi clinicopathologicprofileofprimaryandrecurrentorbitalperiorbitalplexiformneurofibromasoppn
AT deepakpedward clinicopathologicprofileofprimaryandrecurrentorbitalperiorbitalplexiformneurofibromasoppn
_version_ 1718374473739010048