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IgG4 Associated Cholangitis and Hepatic Pseudoinfammatory
Tumour: IgG4 Related Disease Mistaken by Neoplastic Disease
Autor: Ana Isabel S. Ferreira, Natália Ferreira, Raquel Gaio, Afonso Gonçalves, Isabel Távora, José Fonseca Santos.
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We report the case of a 71-year-old man presenting
inaugural cholestatic jaundice, with a stricture
and circumferential wall thickening of the distal
common bile duct, as well as an ill-defned hepatic
infltrative mass with “amputation” of peripheral
distended bile ducts. The patient underwent a
pancreaticoduodenectomy and hemihepatectomy
for suspected cholangiocarcinoma. Histology
revealed IgG4 associated cholangitis (IgG4-AC)
and hepatic pseudoinfammatory tumour (HPT).
IgG4-related disease (IgG4-RD) is a recently
established systemic disease, characterized
by fbro-infammatory tissue infltrates and
tumefaction of one or more organs. The
hepatobiliary manifestations comprise IgG4-AC
and associated HTP - which can be mistaken
for cholangiocarcinoma. Clinicians must be
familiar with this disorder, usually responsive
to corticosteroid therapy, in order to avoid
unnecessary invasive procedures.
We present a case of IgG4-AC and associated
HTP and review the literature of this rare
presentation of IgG4-RD.
Keywords: IgG4-related disease; Autoimmune cholangitis; Hepatic pseudoinfammatory tumour; Cholangiocarcinoma; Hemihepatectomy; Cephalic pancreaticoduodenectomy.
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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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