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Aggressive Fibromatosis of Desmoid Type - a Pictorial Review
Autor: Nuno Almeida Costa, João Garrido Santos, Diogo Fonseca, Maria Filipa Ribeiro,
Maria Leonor Malheiro.
Portugués: |
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Deep fbromatosis of desmoid type comprises
rare mesenchymal tumors characterized
histologically by proliferation of fbroblasts and
myofbroblasts. These lesions are characterized
by infltrative growth and local recurrence but
an inability to metastasize.
They can be located in the abdominal wall
(generally in sutures), intra or extraabdominal.
The best imaging modality for evaluation
and staging of the deep fbromatoses is MR
imaging. These well or ill-defned lesions
generally present internal hypointense bands,
with lack of enhancement in post contrast
images (collagen bundles) and are usually
centered in an intermuscular location with a
rim of fat (“split fat sign”), although invasion
of surrounding muscle is frequent. Linear
extension along fascial planes (“fascial tail
sign”) is also a frequent manifestation in deep
fbromatoses and is uncommon with other softtissue neoplasms.
MR image signal intensity has an implication
on tumor recurrence, with a higher recurrence
rate in lesions with high T2 signal. In surgically
untreated lesions, with undergoing radiation or
drug therapy, MR surveillance has been used
to assess response to treatment with positive
results demonstrating decrease in T2 signal,
lesion enhancement and lesion size.
Keywords: Aggressive fbromatosis; desmoid tumor.
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The evaluation of sonographically
indeterminate adnexal lesions should be
performed with MRI (Magnetic Resonance
Imaging). It is fundamental to determine
the exact location of the lesion, since the
differential diagnosis and therapeutic approach
are distinct according to the organ of origin.
Some signs that may indicate an ovarian origin
are: the presence of ovarian follicles and normal
ovarian parenchyma surrounding the lesion,
without a cleavage plane (“embedded organ
sign”); a change in the ovarian contour by the
mass (“beak sign”); visualisation of a vascular
pedicle or the gonadic veins leading to the
lesion (“suspensory ligament sign”); deviation
of the iliac vessels laterally and of the pelvic
ureters posteriorly or postero-laterally.
The majority of ovarian lesions show cystic
components with high signal-intensity on T2
weighted-imaging. Hypointense lesions on T2
are less frequent. The differential diagnosis for
T2 hypointense ovarian lesions can be vast:
haemorrhagic lesions (namely endometrioma);
presence of smooth muscle (leiomyoma);
presence of fbrous tissue (fbroma, thecoma
and cystadenofbroma) and tumours with
mixed cellularity (Brenner tumour, “struma
ovarii” and Krukenberg tumour).
According to the ESUR recommendations
published in 2017, diffusion-weighted imaging
(DWI) should be applied for those lesions,
using high b-values. The lesions that show lowsignal
intensity on DWI are classifed as benign
and do not require further investigation. On
the other hand, for lesions that demonstrate
intermediate or high signal on DWI, it is
essential to administrate intravascular contrast,
ideally with dynamic-contrast enhanced
imaging (DCE).
Keywords: T2-hypointense lesions; Ovarian lesions; MR; Haemorrhagic lesions; Fibrous tissue; Mixedcellularity tumours.
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