PMID- 37922949
OWN - NLM
STAT- MEDLINE
DCOM- 20240422
LR  - 20240422
IS  - 1538-7151 (Electronic)
IS  - 0277-1691 (Linking)
VI  - 43
IP  - 3
DP  - 2024 May 1
TI  - Fibroepithelial Stromal Polyp of the Vulvovaginal Region as Part of the RB1 
      Family of Tumors: Friend or Foe?
PG  - 215-220
LID - 10.1097/PGP.0000000000000998 [doi]
AB  - Fibroepithelial stromal polyps (FSPs) are benign mesenchymal lesions occurring in 
      the vulvovaginal region. Following the identification of loss of Retinoblastoma 1 
      (RB1) on immunohistochemical staining in routine practice, we stained a series of 
      FSPs and performed additional fluorescence in situ hybridization (FISH) and copy 
      number variation (CNV) sequencing to detect losses/deletions in the 
      Retinoblastoma transcriptional corepressor 1 (RB1) gene. Fifteen FSP cases were 
      stained for RB1, and subsequently, 9 cases were examined by FISH to detect a loss 
      of RB1 (13q). Next, CNV sequencing was performed to assess genomic alterations. 
      The mean age of the patients was 50 years. Loss of RB1 expression on 
      immunohistochemistry was seen in 13 cases, and heterogeneous RB1 staining in the 
      remaining 2 cases. FISH showed deletion of RB1 in all of the cases. CNV 
      sequencing failed in almost all cases due to a low tumor content. Based on our 
      findings, we hypothesize that FSPs are part of a spectrum of genetically related 
      lesions, namely the 13q/RB1 family of tumors (which includes pleomorphic fibromas 
      and spindle cell/pleomorphic lipomas). Due to the clinical, morphologic, and 
      molecular overlap, we suggest that FSPs are pleomorphic fibromas occurring in the 
      specialized stroma of the genital region.
CI  - Copyright (c) 2023 by the International Society of Gynecological Pathologists.
FAU - Cordier, Fleur
AU  - Cordier F
FAU - Van Roy, Nadine
AU  - Van Roy N
FAU - Matthys, Bart
AU  - Matthys B
FAU - De Paepe, Pascale
AU  - De Paepe P
FAU - Van de Vijver, Koen
AU  - Van de Vijver K
FAU - Van Dorpe, Jo
AU  - Van Dorpe J
FAU - Creytens, David
AU  - Creytens D
LA  - eng
PT  - Journal Article
DEP - 20231004
PL  - United States
TA  - Int J Gynecol Pathol
JT  - International journal of gynecological pathology : official journal of the 
      International Society of Gynecological Pathologists
JID - 8214845
RN  - 0 (RB1 protein, human)
RN  - EC 2.3.2.27 (Ubiquitin-Protein Ligases)
RN  - 0 (Retinoblastoma Binding Proteins)
SB  - IM
MH  - Humans
MH  - Middle Aged
MH  - In Situ Hybridization, Fluorescence
MH  - *Retinoblastoma/pathology
MH  - DNA Copy Number Variations
MH  - *Skin Neoplasms/pathology
MH  - *Fibroma/pathology
MH  - *Polyps
MH  - *Retinal Neoplasms
MH  - Ubiquitin-Protein Ligases/genetics
MH  - Retinoblastoma Binding Proteins/genetics
COIS- The authors declare no conflict of interest.
EDAT- 2023/11/06 03:54
MHDA- 2024/04/22 06:43
CRDT- 2023/11/03 20:13
PHST- 2024/04/22 06:43 [medline]
PHST- 2023/11/06 03:54 [pubmed]
PHST- 2023/11/03 20:13 [entrez]
AID - 00004347-990000000-00121 [pii]
AID - 10.1097/PGP.0000000000000998 [doi]
PST - ppublish
SO  - Int J Gynecol Pathol. 2024 May 1;43(3):215-220. doi: 
      10.1097/PGP.0000000000000998. Epub 2023 Oct 4.