Gallbladder Adenomyomatosis: Imaging Findings, Tricks and Pitfalls
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Insights Imaging (2017) 8:243–253 DOI 10.1007/s13244-017-0544-7 PICTORIAL REVIEW Gallbladder adenomyomatosis: imaging findings, tricks and pitfalls Matteo Bonatti1 & Norberto Vezzali1 & Fabio Lombardo1 & Federica Ferro 1 & Giulia Zamboni2 & Martina Tauber3 & Giampietro Bonatti1 Received: 10 November 2016 /Revised: 13 January 2017 /Accepted: 17 January 2017 /Published online: 26 January 2017 # The Author(s) 2017. This article is published with open access at Springerlink.com Abstract emission tomography (PET) may be helpful for exclud- Gallbladder adenomyomatosis (GA) is a benign alter- ing malignancy in selected cases. Computed tomography ation of the gallbladder wall that can be found in up (CT) and cholangiography are not routinely indicated in to 9% of patients. GA is characterized by a gallbladder the suspicion of GA. wall thickening containing small bile-filled cystic spaces – (i.e., the Rokitansky Aschoff sinuses, RAS). The bile Teaching points contained in RAS may undergo a progressive concentra- 1. Gallbladder adenomyomatosis is a common benign lesion tion process leading to crystal precipitation and calcifi- (1–9% of the patients). cation development. A correct characterization of GA is 2. Identification of Rokitansky–Aschoff sinuses is crucial for fundamental in order to avoid unnecessary cholecystec- diagnosing gallbladder adenomyomatosis. tomies. Ultrasound (US) is the imaging modality of 3. Sonography is the imaging modality of choice for diagnos- choice for diagnosing GA; the use of high-frequency ing gallbladder adenomyomatosis. probes and a precise focal depth adjustment enable cor- 4. Intravenous contrast material administration increases ultra- rect identification and characterization of GA in the ma- sound accuracy in diagnosing gallbladder adenomyomatosis. jority of cases. Contrast-enhanced ultrasound (CEUS) 5. Magnetic resonance is a problem-solving technique for un- can be performed if RAS cannot be clearly identified clear cases. at baseline US: RAS appear avascular at CEUS, inde- pendently from their content. Magnetic resonance imag- ing (MRI) should be reserved for cases that are unclear Keywords Gallbladder . Rokitansky–Aschoff sinuses of the on US and CEUS. At MRI, RAS can be identified with gallbladder . Gallbladder diseases . Ultrasonography . extremely high sensitivity, but their signal intensity Magnetic resonance imaging varies widely according to their content. Positron Based on the EPOS BGallbladder adenomyomatosis: are we sure to know it?^ DOI 10.1594/ecr2014/C-0198 Abbreviations GA Gallbladder adenomyomatosis * Matteo Bonatti RAS Rokitansky–Aschoff sinuses [email protected] OC Oral cholecystography US Trans-abdominal ultrasound 1 Department of Radiology, Bolzano Central Hospital, 5 Boehler EUS Endoscopic ultrasound Street, 39100 Bolzano, Italy CEUS Contrast-enhanced ultrasound 2 Department of Radiology, University of Verona, 10 LA Scuro Place, MRI Magnetic resonance imaging 37134 Verona, Italy ADC Apparent diffusion coefficient 3 Department of Pathology, Bolzano Central Hospital, 5 Boehler CT Computed tomography Street, 39100 Bolzano, Italy PET Positron emission tomography 244 Insights Imaging (2017) 8:243–253 Main text contracted, whereas the uninvolved one maintains its normal shape. Gallbladder adenomyomatosis Annular GA is characterized by a ring-form thickening of the gallbladder wall, usually involving the middle portion. Gallbladder adenomyomatosis (GA) is a benign alteration of The gallbladder appears contracted only in the involved por- the gallbladder wall characterized by excessive epithelial pro- tion, changing its global morphology and becoming liferation associated with hyperplasia of the muscularis Bhourglass-shaped^. In some cases, epithelial proliferation propria, resulting in gallbladder wall thickening. The exces- may be particularly conspicuous and subdivide the gallbladder sive epithelial proliferation leads to epithelial infolding within lumen into two separate compartments. As a consequence, the underlying muscular layer with subsequent formation of biliary sludge and stones may accumulate into the isolated epithelium-lined diverticular pouches, the so-called fundal compartment [8]. According to some authors, annular Rokitansky–Aschoff sinuses (RAS; Fig. 1)[1–4]. The content GA should be considered a subtype of segmental GA [9]. of RAS consists of bile that may undergo progressive dehy- Diffuse GA is characterized by the involvement of the whole dration over time, leading to cholesterine crystal precipitation organ that consequently appears contracted, even after fasting. [5]. Moreover, cholesterine crystals may induce a chronic in- The pathogenesis of GA is not fully understood: an asso- flammatory reaction leading to intramural dystrophic calcifi- ciation with gallbladder stones and chronic inflammatory cation development. The serosa is never involved by GA. changes has been highlighted in many studies [1, 5, 10–13], Adenomyomatosis may involve the gallbladder according but a correlation with acquired wall motility as a consequence to four main patterns: localized, segmental, annular and dif- of increased endoluminal pressure has also been postulated fuse (Fig. 2)[6, 7]. [14, 15]. GA is a benign lesion as the hyperplastic epithelium Localized GA is the most common pattern and is character- of GA has no higher neoplastic potential than that of a normal ized by a focal thickening, usually involving the fundal region gallbladder, even though gallbladder carcinoma may also arise (the so-called Bfundal GA^). The uninvolved gallbladder wall in association with GA [16]. Some studies have shown an appears physiologically thin and the overall gallbladder shape increase in gallbladder cancer prevalence among patients with is usually maintained. segmental type adenomyomatosis compared to patients with- Segmental GA is characterized by the involvement of a out GA or with other patterns of GA, in particular, in the larger portion of the gallbladder wall, typically the fundus elderly. However, these results may have been influenced by and the distal third of the body. The involved portion appears the higher prevalence of cholecystolithiasis in patients Fig. 1 Gallbladder adenomyomatosis: pathology findings. proliferation (arrowheads) is also appreciable and epithelium-lined Macroscopically (a) GA is characterized by gallbladder wall cystic spaces, representing RAS (arrows), can be observed within the thickening (lines) containing small cystic spaces (arrows) muscular layer. Biliary stones (star) may be present within RAS. At representing Rokitansky–Aschoff sinuses. Microscopically, at low high (40×) magnification (d), the proliferative mucosal glandular (2×) magnification (b and c), wall thickening is due to hyperplasia component that leads to epithelial infolding (arrowheads)andRAS of the muscular layer (lines); a variable degree of epithelial formation is better recognizable Insights Imaging (2017) 8:243–253 245 (Fig. 3) in order to avoid unnecessary cholecystectomies. It is also important to accurately describe the gallbladder wall in- volvement pattern, as it can modify patient management. There are no universally accepted guidelines for GA management. Given the lack of malignancy potential, GA is usually considered a Bdon’t touch^ lesion and cholecystectomy should be routinely reserved for symp- tomatic patients only or in case of inconclusive imaging findings. In any case, the surgical option might be con- sidered in patients with segmental type GA, given its higher association with gallbladder cancer, and in pa- tients with diffuse GA, given the possible difficulties in identifying neoplastic foci within the wall thickening [18]. In this article, we review multimodality imaging findings of GA, providing tips that may increase diagnostic confidence Fig. 2 Gallbladder adenomyomatosis: patterns of gallbladder wall and highlighting possible pitfalls. involvement. Drawings showing localized gallbladder adenomyomatosis (a), annular gallbladder adenomyomatosis (b), segmental gallbladder adenomyomatosis (c) and diffuse gallbladder adenomyomatosis (d) Imaging of gallbladder adenomyomatosis affected by segmental type GA, which represents a well- known risk factor for gallbladder carcinoma [10–13, 16]. Besides GA, differential diagnosis of gallbladder wall thick- GA may increase in size over time and this change by itself enings includes the post-prandial state, acute and chronic cho- must not be considered an index of malignancy [17]. Patients lecystitis, cholesterine polyps, neoplasms and many other less affected by GA are usually asymptomatic. When present, common conditions. Independently from the radiological mo- symptoms may include right upper quadrant pain, possibly dality, an imaging clue for diagnosing GA is the detection, also as a consequence of the presence of gallbladder stones. within a thickened gallbladder wall, of Rokitansky–Aschoff GA is frequently observed in cholecystectomy specimens, with sinuses. It must be kept in mind that RAS may show extreme- a reported prevalence of 1–9% in pathology series [1, 4, 9, 13]. ly different imaging features according to their variable con- GA represents about 40% of benign gallbladder lesions [1, 4]. tent that may range from clear bile to calcifications. Given its relatively high prevalence and the continuous Moreover, it must be considered that tiny cystic spaces, increase in imaging studies performance, GA can be frequent- resembling RAS, have been identified also in rare cases ly encountered during everyday practice.