Desmoid Tumors Characteristics, Clinical Management, Active Surveillance, and Description of Our FAP Case Series

(1) Background: desmoid tumors (DTs) are common in patients with familial adenomatous polyposis (FAP). An active surveillance approach has been recently proposed as a valuable alternative to immediate treatment in some patients. However, no clear indication exists on which patients are suitable for...

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Main Authors: Lupe Sanchez-Mete, Virginia Ferraresi, Mauro Caterino, Aline Martayan, Irene Terrenato, Elena Mannisi, Vittoria Stigliano
Format: Article
Language:English
Published: MDPI AG 2020-12-01
Series:Journal of Clinical Medicine
Subjects:
MRI
Online Access:https://www.mdpi.com/2077-0383/9/12/4012
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spelling doaj-c7b7b7c6545840a9b66314aa6555c95f2020-12-12T00:04:37ZengMDPI AGJournal of Clinical Medicine2077-03832020-12-0194012401210.3390/jcm9124012Desmoid Tumors Characteristics, Clinical Management, Active Surveillance, and Description of Our FAP Case SeriesLupe Sanchez-Mete0Virginia Ferraresi1Mauro Caterino2Aline Martayan3Irene Terrenato4Elena Mannisi5Vittoria Stigliano6Gastroenterology and Digestive Endoscopy, IRCCS Regina Elena National Cancer Institute, 00144 Rome, ItalyDepartment of Medical Oncology, IRCCS Regina Elena National Cancer Institute, 00144 Rome, ItalyRadiology Unit, IRCCS Regina Elena National Cancer Institute, 00144 Rome, ItalyDivision of Clinical Pathology, IRCCS Regina Elena National Cancer Institute, 00144 Rome, ItalyBiostatistics and Bioinformatic Unit-Scientific Direction, IRCCS-Regina Elena National Cancer Institute, 00144 Rome, ItalyGastroenterology and Digestive Endoscopy, IRCCS Regina Elena National Cancer Institute, 00144 Rome, ItalyGastroenterology and Digestive Endoscopy, IRCCS Regina Elena National Cancer Institute, 00144 Rome, Italy(1) Background: desmoid tumors (DTs) are common in patients with familial adenomatous polyposis (FAP). An active surveillance approach has been recently proposed as a valuable alternative to immediate treatment in some patients. However, no clear indication exists on which patients are suitable for active surveillance, how to establish the cut-off for an active treatment, and which imaging technique or predictive factors should be used during the surveillance period. (2) Results: we retrospectively analyzed 13 FAP patients with DTs. A surveillance protocol consisting of scheduled follow-up evaluations depending on tumor location and tissue thickening, abdominal computed tomography (CT) scan/Magnetic resonance imaging (MRI) allowed prompt intervention in 3/11 aggressive intra-abdominal DTs, while sparing further interventions in the remaining cases, despite worrisome features detected in three patients. Moreover, we identified a possible predictive marker of tumor aggressiveness, i.e., the “average monthly growth rate” (AMGR), which could distinguish patients with very aggressive/life-threatening tumor behavior (AMGR > 0.5) who need immediate active treatment, from those with stable DTs (AMGR < 0.1) in whom follow-up assessments could be delayed. (3) Conclusion: surveillance protocols may be a useful approach for DTs. Further studies on larger series are needed to confirm the usefulness of periodic CT scan/MRI and the value of AMGR as a prognostic tool to guide treatment strategies.https://www.mdpi.com/2077-0383/9/12/4012desmoid tumorssurveillance protocolprognostic factorsCT scanMRItumor growth
collection DOAJ
language English
format Article
sources DOAJ
author Lupe Sanchez-Mete
Virginia Ferraresi
Mauro Caterino
Aline Martayan
Irene Terrenato
Elena Mannisi
Vittoria Stigliano
spellingShingle Lupe Sanchez-Mete
Virginia Ferraresi
Mauro Caterino
Aline Martayan
Irene Terrenato
Elena Mannisi
Vittoria Stigliano
Desmoid Tumors Characteristics, Clinical Management, Active Surveillance, and Description of Our FAP Case Series
Journal of Clinical Medicine
desmoid tumors
surveillance protocol
prognostic factors
CT scan
MRI
tumor growth
author_facet Lupe Sanchez-Mete
Virginia Ferraresi
Mauro Caterino
Aline Martayan
Irene Terrenato
Elena Mannisi
Vittoria Stigliano
author_sort Lupe Sanchez-Mete
title Desmoid Tumors Characteristics, Clinical Management, Active Surveillance, and Description of Our FAP Case Series
title_short Desmoid Tumors Characteristics, Clinical Management, Active Surveillance, and Description of Our FAP Case Series
title_full Desmoid Tumors Characteristics, Clinical Management, Active Surveillance, and Description of Our FAP Case Series
title_fullStr Desmoid Tumors Characteristics, Clinical Management, Active Surveillance, and Description of Our FAP Case Series
title_full_unstemmed Desmoid Tumors Characteristics, Clinical Management, Active Surveillance, and Description of Our FAP Case Series
title_sort desmoid tumors characteristics, clinical management, active surveillance, and description of our fap case series
publisher MDPI AG
series Journal of Clinical Medicine
issn 2077-0383
publishDate 2020-12-01
description (1) Background: desmoid tumors (DTs) are common in patients with familial adenomatous polyposis (FAP). An active surveillance approach has been recently proposed as a valuable alternative to immediate treatment in some patients. However, no clear indication exists on which patients are suitable for active surveillance, how to establish the cut-off for an active treatment, and which imaging technique or predictive factors should be used during the surveillance period. (2) Results: we retrospectively analyzed 13 FAP patients with DTs. A surveillance protocol consisting of scheduled follow-up evaluations depending on tumor location and tissue thickening, abdominal computed tomography (CT) scan/Magnetic resonance imaging (MRI) allowed prompt intervention in 3/11 aggressive intra-abdominal DTs, while sparing further interventions in the remaining cases, despite worrisome features detected in three patients. Moreover, we identified a possible predictive marker of tumor aggressiveness, i.e., the “average monthly growth rate” (AMGR), which could distinguish patients with very aggressive/life-threatening tumor behavior (AMGR > 0.5) who need immediate active treatment, from those with stable DTs (AMGR < 0.1) in whom follow-up assessments could be delayed. (3) Conclusion: surveillance protocols may be a useful approach for DTs. Further studies on larger series are needed to confirm the usefulness of periodic CT scan/MRI and the value of AMGR as a prognostic tool to guide treatment strategies.
topic desmoid tumors
surveillance protocol
prognostic factors
CT scan
MRI
tumor growth
url https://www.mdpi.com/2077-0383/9/12/4012
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