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Journal of Ultrasound

, Volume 21, Issue 3, pp 253–257 | Cite as

Diagnostic imaging and CEUS findings in a rare case of Desmoid-type fibromatosis. A case report

  • Massimo Valentino
  • Mauro Liberatore
  • Fabrizio Maghella
  • Valeria De Soccio
  • Carlo De Felice
  • Daniela Messineo
  • Francesco Maria Drudi
Case Report
  • 57 Downloads

Abstract

Desmoid-type fibromatosis (DF), also known as aggressive fibromatosis, is a locally aggressive benign fibroblastic neoplasm that can infiltrate or recur but cannot metastasize. It is rare, with an estimated annual incidence of two to four new cases per million people. Most DFs occur sporadically, but it may also be associated with the hereditary syndrome familial adenomatous polyposis. Treatment is necessary when the disease is symptomatic, especially in case of compression of critical structures. When possible, surgical resection is the treatment of choice; however, recurrence is common. Due to the high rate of recurrence, imaging plays an important role not only in diagnosis, but also in the management of DF. Although there are a number of studies describing CT and MRI findings of DF, there is no description of contrast-enhanced ultrasound findings.

Keywords

Desmoid-type fibromatosis Aggressive fibromatosis CEUS Ultrasound 

Sommario

La fibromatosi di tipo desmoide (DF), anche conosciuta col nome di fibromatosi aggressiva, è una neoformazione benigna localmente aggressiva con la capacità di infiltrare o recidivare ma incapace di metastatizzare. È rara con un’incidenza annuale stimata di 2-4 nuovi casi per milione, costituendo approssimativamente lo 0.03% di tutte le neoplasie e meno del 3% dei tumori dei tessuti molli. La maggior parte delle DF sono sporadiche ma possono anche essere associate con la sindrome della poliposi familiare (FAP). Il trattamento è necessario quando la malattia è sintomatica, specialmente in caso di compressione di strutture critiche. Quando possibile, la resezione chirurgica è il trattamento di scelta, sebbene la recidiva sia comune. Visto l’elevato tasso di ricadute, l’imaging gioca un ruolo importante non solo nella diagnosi ma anche nella gestione della DF. Sebbene vi siano un certo numero di studi che descrivono le caratteristiche della DF in TC e RMN, non c’è alcun lavoro che descriva la patologia nell’ecografia con mezzo di contrasto (CEUS).

Notes

Compliance with ethical standards

Conflict of interest

The authors declare no competing financial interest.

Ethical approval

Ethics committee approval was obtained before beginning any research involving human subjects or clinical information. All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2000.

Informed consent

All patients provided written informed consent for enrollment in the study and for the inclusion in this article of information that could potentially lead to their identification.

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Copyright information

© Società Italiana di Ultrasonologia in Medicina e Biologia (SIUMB) 2018

Authors and Affiliations

  1. 1.RadiologyGeneral HospitalTolmezzoItaly
  2. 2.Department of Radiological Sciences and Nuclear MedicineUniversity of Rome “La Sapienza”RomeItaly

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