This process allows for characterizing liver lesions and assessing treatment. In this review, we highlighted both major and ancillary features that could define HCC. The distinctive dynamic vascular pattern of arterial hyperenhancement followed by washout in the portal-venous phase is the key hallmark of HCC, with a specificity value close to 100%. However, the sensitivity value of these combined criteria is inadequate. Recent evidence has proven that liver-specific contrast could be an important tool not only in increasing sensitivity but also in diagnosis as a major criterion. Although LI-RADS emerges as an essential instrument to support the management of liver tumors, still many improvements are needed to overcome the current limitations. In particular, features that may clearly distinguish HCC from cholangiocarcinoma (CCA) and combined HCC-CCA lesions and the assessment after locoregional radiation-based therapy are still fields of research.Liver cancer is the sixth most detected tumor and the third leading cause of tumor death worldwide. Hepatocellular carcinoma (HCC) is the most common primary liver malignancy with specific risk factors and a targeted population. Imaging plays a major role in the management of HCC from screening to post-therapy follow-up. In order to optimize the diagnostic-therapeutic management and using a universal report, which allows more effective communication among the multidisciplinary team, several classification systems have been proposed over time, and LI-RADS is the most utilized. Currently, LI-RADS comprises four algorithms addressing screening and surveillance, diagnosis on computed tomography (CT)/magnetic resonance imaging (MRI), diagnosis on contrast-enhanced ultrasound (CEUS) and treatment response on CT/MRI. The algorithm allows guiding the radiologist through a stepwise process of assigning a category to a liver  Liver cancer is the sixth most detected tumor and the third leading cause of tumor death worldwide ,4,5,6,7.Imaging plays a major role in the management of HCC from screening to post-therapy follow-up ,37,38,39(a) US-LI-RADS for HCC screening and surveillance; (b) contrast-material-enhanced US for HCC diagnosis (CEUS-LI-RADS); (c) CT/MRI for HCC diagnosis and radiologic T stage (CT/MRI LI-RADS); and (d) CT/MRI for HCC treatment response assessment (TR LI-RADS) [In order to optimize diagnostic accuracy and using a universal report that allows more effective communication among the multidisciplinary team caring for the patient, several classification systems have been proposed over time. Of these, the most valid and comprehensive is the Liver Imaging Reporting and Data System (LI-RADS), which provides guidance on all aspects of HCC imaging, from techniques for imaging acquisition to assessing treatment response and directing management. The latest version of ACR LI-RADS includes four algorithms: LI-RADS) ,63,64,65LI-RADS) ,16. Non-LI-RADS) ,39. TherThe aim of this narrative review was to evaluate the main current applications of LI-RADS in liver cancer, primarily in HCC and to assess the main radiological features that can guide differential diagnosis.LI-RADS is a system supported by the American College of Radiology (ACR), resulting from the work of radiologists experienced in liver pathology and integrated with the latest guidelines of the American Association for the Study of Liver Diseases (AASLD) . The firCurrently, LI-RADS comprises four algorithms addressing screening and surveillance, diagnosis on CT/MRI, diagnosis on contrast-enhanced ultrasound (CEUS) and treatment response on CT/MRI. Each algorithm has a core document that includes relevant indications to be applied in clinical practice . A speciBoth US-LI-RADS and diagnostic LI-RADS do not address patients with vascular causes of liver cirrhosis  because these conditions are associated with hypervascular benign liver lesions, which increase the risk of false positives and reduce the PPV for the diagnosis of HCC ,79,80,81observation” when referring to identified liver formations during the screening or diagnostic phase [observation” embraces the whole spectrum of possible abnormalities, ranging from simple cysts to clear neoplastic lesions [An important point of the LI-RADS philosophy is the choice to adopt the term “ic phase . The gen lesions ,92,93,94 lesions .The main purpose in the management of patients with HCC is to detect the disease at an early stage, when it is potentially treatable by resection or liver transplantation. Indeed, the average 5-year survival of advanced and untreated cases is only 15% . US is pobservations into three categories according to the probability of HCC, including an evaluation of the overall quality of the examination to define its reliability [The ACR working group developed a standardized algorithm for US screening and surveillance in high-risk patients (US-LI-RADS) ,104. US-iability ,104.In order to achieve high diagnostic accuracy, the ACR US-LI-RADS working group illustrates some guidance on the technical execution of the US examination ,107,108.Each observation should be assessed in three dimensions in the longitudinal and transverse plane, reporting their precise location and anatomical relationships. Optional cine clips may facilitate the comparison in follow-up exams .The US-LI-RADS algorithm includes both an evaluation score of the observations and an analysis of the quality of the images. The three possible categories  should be applied to the entire examination, and each category corresponds to a subsequent clinical management ,107,108.The US-1 category includes the detection of clear benign formations , leading the patient to routine follow-up in 6 months .The US-2 category is assigned when not definitely benign <10 mm formations are detected. Close US follow-up at 3–6 months is recommended to intercept an overthreshold growth (>1 cm), which requires further diagnostic characterization. The stability or subthreshold size in two years, redeploy the patient in the US surveillance program every six months .US-3 corresponds to a positive examination for lesions >10 mm that are probably HCC. In these circumstances, a contrast-enhanced CT, MRI or US is mandatory .During the examination, the radiologist should also pay attention to identifying a new thrombus in a vein, which could be a sign of tumor invasion. The second component of US-LI-RADS is the visualization score, which is an assessment of three categories (A-B-C) of the overall quality and/or perceived sensitivity of the exam ,109. AltAccurate diagnosis and staging of HCC can be achieved by CT  or MRI  in the aThe choice to use CT rather than MRI remains at the discretion of individual institutions.The LI-RADS guidelines provide information on minimum technical requirements for CT and MRI examinations to be considered reliable. CT examinations should be performed on multidetector devices (≥8 detectors), while a magnetic field of at least 1.5 T is indispensable for MRI . IntraveIn-phase and out-of-phase T1-weighted basal sequences and T2-weighted sequences (with or without fat suppression) prior to contrast injection are necessarily integrated into the MRI study, while diffusion-weighted imaging (DWI), subtraction sequences and hepatospecific phase images are optionally conducted .Although there is no guidance on the assessment of the severity of artifacts, the radiologist should assign an LR-NC category (LI-RADS not categorizable), when an observation cannot be fully assessed due to missing or degraded images .observation is benign, HCC, a neoplasm other than HCC or a tumor in a vein (TIV) [LI-RADS includes eight diagnostic categories (-LR), reflecting the probability that an in (TIV) .The algorithm is built to guide the radiologist through a stepwise process, recognizing both major and ancillary features.Currently, LI-RADS major imaging criteria include APHE , washoutobservation from LR-4 to LR-5 [While the major criteria should always be satisfied, ancillary features, such as restricted diffusion , could b to LR-5 ,150,151.observations <20 mm could be categorized as LR-3 if they have APHE as the sole major feature. Observations lacking APHE are categorized as LR-3 if they have a size <20 mm and ≤1 additional major feature or size ≥20 mm and no additional major features. In these circumstances, it should be indicated to repeat enhanced diagnostic imaging in 3 to 6 months [observations <10 mm with nonrim APHE and ≥1 additional major feature; 10 to 19 mm observations with nonrim APHE and “capsule” as the only major feature; observations ≥20 mm with nonrim APHE and no additional major features. The LR-4 category also includes observations without rim APHE: observations <20 mm and ≥2 additional major features and observations ≥20 mm and with ≥1 additional major feature. According to LI-RADS guidelines, LR-4 patients are referred for biopsy, presumptive treatment or follow-up, depending on the multidisciplinary team’s decision [The categories LR-1 (definitely benign) and LR-2 (probably benign) include simple cysts or nodules, <20 mm in size, that do not show features of malignancy. LR-3 (intermediate likelihood) refers to some perfusions or true nodules with one or two major malignant features. Specifically, 6 months . The LR-decision .Malignancy categories include LR-5 (definitely HCC), LR-M  and LR-TIV  .observations >20 mm with ≥1 additional major feature and 10 to 19 mm observations with ≥2 additional major features. Observations measuring 10 to 19 mm can also be categorized as LR-5 if they have only one additional major feature: either nonperipheral “washout” or threshold growth. In the absence of diagnostic doubt, pathologically confirmed lesions and benign lesions of non-hepatocellular origin do not require LI-RADS classification.APHE and size ≥10 mm are required for LR-5 categorization. The LR-5 category includes CT and MRI also contribute to the staging of liver disease, particularly to the evaluation of possible macrovascular invasion, which falls into the LR-TIV category  and contIn addition to the direct identification of vascular invasion by the tumor mass, findings suggestive on imaging of locally invasive HCC are the presence, within an occluded vein, of arterial neovases manifesting as thin, punctuated hyperenhancing “threads and stripes”. Another aspecific sign of TIV is the increased diameter (23 mm or more) of a thrombosed main portal branch . This laobservation that is unequivocally of higher intensity/attenuation than background liver [On imaging, HCC reveals a distinctive dynamic vascular pattern of APHE followed by washout in portal-venous phase. Due to its high specificity, this temporal enhancement hallmark is incorporated in all current diagnostic systems. As emerged from a recent meta-analysis, the APHE and washout are the strongest independent imaging features associated with HCC . APHE isnd liver . This apnd liver .Among the LI-RADS major features, APHE shows the highest sensitivity (85%) for progressed HCC, but it is characterized by a low specificity (57%) . Indeed,These findings were confirmed by Granata et al. in 17 hyperplastic nodules that resulted hypervascular during MRI examinations in a retrospective evaluation of 70 patients with HCC .observations with APHE could potentially be incorrectly classified as HCC; therefore, this criterion alone is considered inadequately accurate [A meaningful number of accurate . The falaccurate ,160.observation refers to the progressively hypointense/hypodense appearance compared to the surrounding liver parenchyma during the portal-venous phase and the delayed phase [The washout of an ed phase ,161.This contrast-enhancement behavior corresponds to a reduced portal supply, increased cellularity and reduced extracellular volume of the tumor.observations [APHE and washout appearance are not a feature exclusive to HCC and could be observed in cirrhotic or dysplastic nodules, raising the issue of specificity when applied as a sole criterion on liver rvations ,171,172.Combining detection of APHE and washout could significantly increase specificity, reaching values close to 100% when detected in high-risk patients with nodules ≥ 20 mm .This advantage is still associated with poor sensitivity, especially in smaller-sized lesions, showing inconspicuous washout .The capsule appearance is the other major criterion that may improve the performance of the LI-RADS algorithm. The “capsule” presence is a distinctive sign of advanced HCC, with the highest specificity value among all major features (90–96%) ,175. HowFavoring specificity is positively accepted in Western countries, where the main purpose is to avoid false positives in order to select patients for liver transplantation. On the other hand, in Asian countries, locoregional therapies are often employed in the first instance, and therefore HCC should be intercepted at an early stage. Since LI-RADS is proposed as a universal guide in HCC management, the problem of sub-optimal sensitivity should be solved .Ancillary features (AFs) could play a decisive role. Although these imaging criteria alone preclude definitive diagnosis of HCC, they provide additional data for tumor characterization, improving the sensitivity rate of major features. The importance of AFs applied especially to MRI emerges from the literature. Between all AFs, the hypointensity in the hepatospecific phase (HPH) appears independently associated with the diagnosis of HCC .observation assessment, even the small ones (10–19 mm). This last clinical scenario is particularly challenging, reaching a failure rate of 87% when only major criteria are identified in small liver lesions [HPH has shown to increase sensitivity in liver  lesions .observations with APHE upgraded to the LR-4 category [Vernuccio et al. recorded a sensitivity of 84% applying HPH to LR-3 small category . This secategory .observation in the hepatobiliary phase depends on the expression of the OATP8 transporter, which provides the contrast agent uptake. The OATP8 expression declines during hepatocarcinogenesis, and therefore the assessment of signal intensity in the hepatobiliary phase may guide the detection and characterization of hepatocellular nodules in the cirrhotic liver. The HPH could also be seen in non-hepatocellular benign lesions such as hemangiomas or non-HCC malignancies due to the physiological absence of the OATP8 expression. Therefore, HPH is not sufficiently specific to be used as the sole criterion, but as shown in several studies, it could be associated with more AFs in order to overcome this limitation [The signal of an mitation ,187,188.mitation .Additionally, Lee et al. observed that the application of AFs both of malignancy in general (mild–moderate T2 hyperintensity and HPH) and favoring HCC in particular (nonenhancing “capsule” and mosaic architecture) to the LR-4 category assigned on the basis of the major criteria significantly increases the sensitivity in the diagnosis of HCC (69.4–76.9% compared with the standard LR-5 66.2%), without impairing specificity (95.3–96.5%) .Several studies have focused on the application of gadobenate dimeglumine (Gd-BOPTA) in the diagnosis of HCC and its possible advantages as a major feature in tumor assessment. Although the diagnostic performance and liver parenchymal enhancement observed during the hepatobiliary phase seem similar for both gadoxetic Gd-EOB and Gd-BOPTA ,192, somTherefore, these peculiarities could be useful when applying the LI-RADS algorithm. Cortis et al. demonstrated increases in sensitivity from 53.2% to 75.8% for lesions of ≥20 mm and from 53.5% to 62.1% for lesions of 10–19 mm when HPH on Gd-BOPTA was included as an additional major feature to the typical APHE/washout pattern in cirrhotic patients with hypervascular HCC . AccordiIntrahepatic cholangiocarcinoma (iCCA)  and combobservations with features suggestive of malignancy but missing major criteria specific for HCC (classic APHE and washout/capsule appearance). The LR-M features include: rimlike APHE, peripheral washout, liver surface retraction, adjacent biliary obstruction, mixed pattern and infiltrative margin [In LI-RADS, the LR-M category collects all those e margin .observations [In several studies, the rimlike APHE emerges as the most common sign suggesting malignancy other than HCC, being present in 50–84% of examined lesions. The second most frequently encountered feature resulted a delayed or progressive central enhancement during PVP or DP after extracellular agent injection, observed in 42–96% of liver rvations . Regardirvations ,198,199.Another ancillary feature associated with the LR-M category is represented by a mild–moderate T2 hyperintensity. Cannella et al. found that a peculiar signal in the T2 sequences, the “targetoid” appearance associated with tumor vascular involvement, could be a key sign of non-HCC primary liver carcinomas (PLCs) . ResearcLR-M features defined by LI-RADS are most closely associated with the imaging appearance of ICC as the second most frequent neoplasm in cirrhotic liver . HistopaIn the literature, the targetoid appearance on HPH, the presence of rimlike APHE and peripheral “washout” would seem sufficiently distinctive of ICCs ,209. HowTo date, only a few publications have described the imaging appearance of cHCC-CCA and the features that can be distinguished only in these lesions compared to HCC or ICC ,16. SeveRegarding other liver tumors, Granata et al.  assessedCEUS is a US examination widely accepted in many clinical contexts, performed by injecting intravenously microbubble-based contrast agents (USCAs). USCAs are small (from 1 to 10 μm) biodegradable particles of low-solubility gases  covered by a flexible lipid shell ,216,217.ACR incorporated CEUS into the LI-RADS system in 2016, driven by working groups of hepatologists and radiologists, who have demonstrated the diagnostic accuracy of CEUS in the characterization of focal liver lesions . Albeit Otherwise, CEUS is a focused exam, being inadequate for staging and response to therapy, lacking the panoramic and standardized visualization of CT/MRI. Considering these premises, CEUS-LI-RADS introduces different diagnostic features, requiring its own algorithm with distinctive unique properties .US equipment should comprise contrast-enhanced imaging setting, which generally includes a dual-screen and on-screen chronometer.® /Luminity®  and Lumason® (in USA)/Sono-Vue® (outside USA).Prior to enhanced imaging, baseline scans are performed to assess the target nodule, choosing the optimal position for the patient in order to reduce out-of-plane motion from breathing. Using the B-mode image as a guide, the probe is positioned over the lesion on both screens simultaneously to facilitate enhancement characterization . Then, aArterial phase begins within 20 s after injection of the contrast agent and persists until 30–45 s, depending on the cardiocirculatory situation. PVP begins 30–45 s after injection and ends at 120 s, while the late phase starts 2 min after injection, lasting until the clearance of microbubbles from liver tissue .It is advisable to perform the examination continuously from contrast medium injection to the peak of arterial phase enhancement, in order to intercept APHE. Conversely, PVP and LP examinations are executed intermittently (every 30 sec) to minimize the destruction of microbubbles, allowing one to observe the late washout .Finally, a survey recording is recommended from the contrast vascular input until the peak of APHE.As in the CT/MRI model, eight diagnostic categories with related imaging work-up suggestions cover the spectrum of lesions possibly discernible at CEUS.Nodules are classified based on major criteria: size , the APHE appearance and the presence and type of washout.CEUS LR-1 and 2 categories correspond to definitely and probably benign observations, respectively. LR-1 comprises formations with unequivocally benign contrastographic behavior, whereas LR-2 is assigned to isoenhancing lesions (solid nodule <10 mm/nonmasslike formations of any size) and LR-3 nodules stable in size for ≥2 years ,220.Patients with CEUS LR-1 and LR-2 can resume routine 6-month US surveillance, without the need for further diagnostic investigations .LR-3  includes solid nodules ≥ 10 mm with isoenhancement in all phases, nodules <20 mm showing late washout onset (≥60 s) and a mild degree of washout. CEUS LR-3 also includes nodules <10 mm, which show only APHE as a vascular criterion. Guidelines suggest repeating the examination with contrast administration within 3–6 months, but in selected cases, the multidisciplinary team could require a biopsy .The LR-4 categories (probable HCC) include nodules ≥10 mm with APHE and no appreciable washout and those with APHE, late washout onset (≥60 s) and a mild degree of washout but measuring <10 mm. Finally, also focalities ≥ 20 mm with no APHE but with late washout onset (≥60 s) and a mild degree of washout may also be characterized as CEUS LR-4 .All these conditions should undergo bioptic assessment or short-term (<3 months) imaging follow-up, relying on the multidisciplinary discussion decision .The LR-5 nodules fulfill all major criteria and are treated as definitely HCC without biopsy confirmation .CEUS algorithm also provides an LR-M class that includes not HCC malignant lesions, which usually need biopsy confirmation. The imaging features for LR-M are rim APHE, early (<60 s) washout or marked washout resulting in a “punched-out” appearance within 2 min after contrast injection. If the image quality is poor and does not allow lesion definition, the radiologist is called upon to assign the LR-NC category ,220.As CT/MRI, LR-TIV refers to tumor tissue in the portal or hepatic veins.Ancillary features favoring malignancy, without specificity for HCC, include definite interval size increase, whereas definite interval size reduction or stability ≥2 years favor benignancy. Features favoring HCC, in particular, include a mosaic appearance of the nodule and a nodule-in-nodule appearance in the arterial phase . These fCEUS enables the possibility of real-time assessment of APHE, which might be missed or misinterpreted in other imaging studies in case of inappropriate sequences/acquisition timing. This property is particularly useful in definitive LR-2 categorization of nodules, presenting dubiously HCC (LR-3-LR4), for a suspected arterial phase appearance on the CT/MRI algorithm. Among all benign focal liver formations, the assessment of hemangiomas and arteriportal shunts could result particularly challenging ,222. IndThe peculiarity of CEUS in intercepting APHE is reflected positively not only in the downgrade of LR-3 or LR-4 formations but also in the definitive diagnosis of HCC, assigning the category LR-5 to probable malignant lesions . Indeed,In particular, Maruyama et al. recorded that 7/27 focal liver lesions (<20 mm) characterized as non-hypervascular (APNHE) on prior contrast-enhanced CT then showed APHE at CEUS and resulted as HCCs on histological specimens .observations even <10 mm that are not necessarily well defined [As highlighted, APHE is a necessary major feature to achieve HCC diagnosis. According to the CEUS algorithm, any nodule ≥ 10 mm with APHE is classified as LR-4 or LR-5, depending on the presence or absence of the washout criterion, respectively. This assumption is based on the “B-mode nodules visibility” as a prerequisite for CEUS evaluation. As a corollary, the LR-3 category of CEUS includes more cases of HCCs compared to the CT/MRI algorithm, since the examination is based on actual nodules, whereas LR-3 formations on CT/MRI include  defined .Albeit less frequently, mass-forming ICCs are encountered during US surveillance. On CEUS, ICC often shows rim-APHE, which is an uncommon finding in HCC. Rim-APHE was a key feature in Huang et al.’s study on 228 nodules (99 ICCs and 129 LR-M HCCs) showing sensitivity and specificity for differential diagnosis with HCCs of 70.4% and 68.8%, respectively. In addition, combing the rim-APHE with elevated CA 19-9 and normal alpha-fetoprotein (AFP) values increased sensitivity to 100% . Rim-APHTherefore, the LR-M category on CEUS-LI-RADS could be confidentially assigned in the presence of rim-APHE, early washout (<60 s), marked washout visible within the first 2 min after contrast injection or any combination of these three features ,225.Response Evaluation Criteria in Solid Tumours (mRECIST) [HCC treatment was traditionally based on surgical or locoregional ablation technique ,236,237.mRECIST) , which emRECIST) .Considering that the management of HCC often requires multiple therapeutic interventions, this approach could guide the multidisciplinary team caring for the patient in closely monitoring the evolution of the disease or in choosing the appropriate therapy.viable, LR-TR equivocal or LR-TR nonviable  could be assigned. The role of the intermediate category is still questioned [Similarly to the LI-RADS diagnostic algorithm, the TRA classifies treated HCC into three different categories: LR-TR onviable , accordieristics . Generalestioned .viable, the researchers observed an increase in sensitivity in predicting complete necrosis to 81–87%, compared to 40–70% when treating the same lesions as nonviable. Furthermore, the majority of lesions classified as LR-TR equivocal (five out of six (83%)) exhibited incomplete necrosis in histopathology specimens [equivocal after transarterial embolization [equivocal category would seem to increase the sensitivity in residual disease detection, intercepting some cases that might escape from a treatment program for the residual presence of HCC [Chaudhry et al.  evaluatepecimens . Similarlization . Hence, e of HCC .On the other hand, what also emerges from these studies is the unsatisfactory diagnostic accuracy of the nonviable LR-TR category in predicting a complete response to therapy. In both Chaudry’s and Shropshire’s investigations, only a low percentage of lesions assigned as nonviable were actually completely necrotic at histopathology  ,241,242.Currently, insufficient data are available in evaluating the impact of nonviable LR-TR, in cases of minimal residual disease at pathological relief on patients’ overall survival or progression-free survival. Given the possibility of residual microfoci of disease that are difficult to distinguish on imaging after local-regional therapy, the interval for imaging follow-up should remain close, regardless of LR-TR category .Although the application of TRA seems plausible in clinical practice, there are still important gaps.equivocal categorizations and provide clinical assessment of residual disease after all types of local-regional therapies.For instance, an area that has not yet been sufficiently investigated concerns the evaluation of TRA performance in assessing treatment response after locoregional radiation-based therapy . Further research should be promoted to improve the algorithm, decrease the rate of LR-TR LI-RADS is the most comprehensive system for the diagnosis and management of HCC. Four LI-RADS algorithms cover three individual clinical contexts: screening and surveillance, diagnosis/staging and assessment of treatment response.observations found on US surveillance in patients considered at risk. In particular, CEUS-LI-RADS can afford a contrast-enhanced examination in absence of radiation exposure. This could be an important advantage, especially in young patients.CEUS-LI-RADS and CT/MRI LI-RADS algorithms could achieve confident HCC diagnosis, providing tailored management recommendations for equivocal categorizations and provide clinical assessment of residual disease after all types of local-regional therapies.Currently, LI-RADS major imaging criteria should always be satisfied, while ancillary features could be used at the discretion of the radiologist to increase or decrease the category. However, the sensitivity value of these combined criteria is inadequate. Recent evidence proved that HPH on Gd-EOB-DTPA could be an important tool not only in increasing sensitivity but also in diagnosis as a major criterion. Although LI-RADS emerges as an essential instrument to support the management of liver tumors, still many improvements are needed to overcome the current limitations. In particular, features that may clearly distinguish HCC from ICC and cHCC-CC lesions and the assessment after locoregional radiation-based therapy are still fields of research. In fact, although the application of TRA seems plausible in clinical practice, there are still important gaps. An area that has not yet been sufficiently investigated concerns the evaluation of TRA performance in assessing treatment response after transarterial radioembolization or external beam radiotherapy. Further research should be promoted to improve the algorithm, decrease the rate of LR-TR


TRAPPC9) mutation presented to the family medicine clinic after not having passed stool for six days. There was a history of chronic constipation. Examination revealed high-pitched “tinkling” bowel sounds; therefore, a plain abdominal X-ray was ordered to rule out the possibility of intestinal obstruction, which showed a large fecaloma in the rectum with dilated bowel loops proximal to it, signifying obstruction. This was successfully treated with the administration of a rectal enema and confirmed by a post-enema radiograph. Although rare in children, a fecaloma should be considered a cause of bowel obstruction, especially where there is a history of chronic constipation. A plain abdominal X-ray can be useful in diagnosing a fecaloma in pediatric cases.A nine-year-old wheelchair-bound female with cerebral palsy and intellectual disability secondary to trafficking protein particle complex subunit 9 ( Constipation is a problem that commonly affects children  with a pTRAPPC9) mutation causing severe developmental and language delay was brought to the family medicine clinic by her mother. The presenting complaint was that the child had not passed stool for six days. She was prone to constipation and had been treated for this in the past with oral laxatives. On this occasion, she had been given a single sachet of macrogol (polyethylene glycol) but without effect. There was no history of fever, vomiting, or abdominal pain. She was eating and drinking well and passing urine. The mother could not comment on whether the child was passing flatus. There was no family history of bowel disorders or constipation. On examination in her wheelchair, she was not in any distress, and vital signs were within normal parameters for her age. Her abdomen was soft, non-tender, and non-distended, and no abdominal masses could be felt in the sitting position. Auscultation revealed high-pitched “tinkling” bowel sounds; therefore, a plain abdominal X-ray was ordered to check for intestinal obstruction, and this showed a large fecaloma in the rectum with dilated bowel loops proximal to it as a sign of a mechanical large bowel obstruction (Figure A nine-year-old female with asthma, cerebral palsy, and intellectual disability secondary to trafficking protein particle complex subunit 9 (n Figure . The chin Figure . She wasConstipation is common in children , and theA detailed history and plain X-ray are usually sufficient to aid the diagnosis of a fecaloma . EvidencThe importance of a thorough history and examination cannot be underestimated, especially in the case of a child with a learning disability or those prone to chronic constipation. Fecalomas are rare but should be considered as a cause of constipation, especially where the history or examination points toward bowel obstruction. An abdominal X-ray is a useful investigation for the diagnosis of a fecaloma in pediatric cases. Fecalomas can be successfully treated with a combination of polyethylene glycol and sodium picosulfate, which produce fecal disimpaction in chronically constipated children. The initial management of fecalomas comprises oral laxative and enema use; however, in extreme cases, manual disimpaction, endoscopy, or surgical resection may be necessary.


Inflammatory Myofibroblastoma Tumors (IMTs) are extremely tumour rare in the intraocular.A ciliary body tumor was found under slit lamp biomicroscopy in a 55-year-old male first diagnosed with cataract. Then this patient underwent trans-sclera resection via partial lamellar sclerouvectomy and par plans vitrectomy to remove the mass. Hematoxylin and eosin (HE) staining and immunohistochemistry findings showed that the characteristics of the tumor were consistent with IMT.We reported a rare case of intraocular IMT, which is confirmed by H&E staining, and IHC positive staining for Vimentin, Desmin and ALK, while negative staining for SMA, S-100, ki-67, CK, CD68, and calponin. Inflammatory myofibroblastic tumour (IMT) is a distinctive lesion composed of myofibroblastic spindle cells with a prominent inflammatory infiltrate of lymphocytes, eosinophils, and plasma cells . IMT is A 55-year-old man presented with a chief complaint of progressive, painless vision loss in the left eye over the course of 20 days. The patient had no significant prior medical or ocular history. The patient had no history of smoking,had no previous trauma,and had no infected lesions.His best-corrected visual acuity (BCVA) and intraocular pressure (IOP) in the left eye were 20/100 and 15 mmHg, respectively. Only cortical opacity in the left lens was evident on slit-lamp biomicroscopy before pupil dilation. Therefore, an initial diagnosis of age-related cataract was made by outpatient physicians. The patient was then referred to a surgeon for cataract surgery. A dilated pupil examination under slit-lamp biomicroscopy revealed a well-defined, ovoid soft tissue amelanotic mass filling the inferonasal quadrants. Superficial vessels were evident on its surface and lens opacity was mainly present at the mass location Fig. A, D. TumThe final diagnosis based on histological and immunohistochemical assessments was IMT of the ciliary body.The patient was followed up regularly after discharge, and the condition of body and eyes was stable. The silicone oil removal operation was performed in our hospital, and the operation went well.IMTs are mesenchymal neoplasms of intermediate biological potential with a predilection for the lung, abdomen, and pelvis . MetastaIMTs are most have been observed in the orbit but extremely rare in the intraocular. Intraocular IMTs presumably arise from uveal tissues and are associated with uveitis . Common Schwannoma is a begin proliferation of Schwann cells that encase the peripheral nerves .HistopatLeiomyomas are rare benign tumours, most commonly found in the uterus and alimentary tract . On lighALK expression is not a specific manifestation of IMT. Some aspects of the ALK activation mechanism in IMT are also present in a subset of ALCLs . On lighAfter perfecting the relevant immunohistochemistry, it was found that EBER Fig. Nwas negaIn our patient, other possible tumours were excluded on the basis of haematoxylin–eosin staining and immunohistochemical analysis. Therefore, a diagnosis of primary intraocular IMT tumour with ALK overexpression was considered.Cytogenetic and molecular interpretations are important in this pathology. Molecular genetics of this type of tumor have previously described about 50% of cases with 2p23 (ALK) rearrangement, partner genes including ATIC, TPM3, TPM4, CLTC, etc. ALK-RANBP2 gene rearrangement is found in most epithelioid inflammatory myofibroblastic sarcomas. There are reports. Tumors negative for ALK gene fusions can have other fusions including ROS1, RET, etc.However, there were several differences between our patient and the findings of previous reports on IMT. Generally, IMTs exhibit SMA expression (92%), because the most common intermediate filament feature expressed by myofibroblasts is the VA phenotype (i.e. expression of vimentin and α-SMA). However, there have been reports of IMTs with the VD phenotype . Our patIn conclusion, we have described a rare instance of intraocular IMT, confirmed by haematoxylin–eosin staining, as well as positive immunohistochemical staining results for vimentin, desmin, and ALK. This patient had negative immunohistochemical staining results for SMA, S-100, Ki-67, CK, CD68, and calponin. Medline search did not reveal any report of IMT of the ciliary body.


A 72‐year‐old man presented with a 1‐year history of painless progressive nasal obstruction. Nasal fiberscope evaluation revealed a necrotic tumor of the nasal septum, and histopathological evaluation of a biopsy specimen showed significant polymorphous lymphocytic infiltration of the nasal mucosa along with necrosis. The patient was diagnosed with extranodal NK/T cell lymphoma (ENKTL) based on immunohistochemical evaluation that revealed lymphocytes showing positivity for CD3, Granzyme B, CD56, and EB virus‐encoded small RNA (EBER). Fiberscopic examination, fluorodeoxyglucose‐positron emission tomography computed tomography (FDG‐PET/CT), and bone marrow biopsy did not reveal distant metastasis including laryngeal lesions. He was treated with concurrent chemoradiotherapy using an MPVIC‐P regimen  [The patient presented with sore throat, dysphonia, and dyspnea, 3 years after his initial treatment. Flexible endoscopy revealed right vocal cord swelling with subglottic stenosis Figure . We perfENKTL is an Epstein Barr virus‐induced malignancy, which commonly affects the upper respiratory tract, particularly the nasal cavity, and laryngeal ENKTL is rare  , 3. NotaThe authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research reported.


Hepatic inflammatory myofibroblastic tumor (HIMT) is a junctional neoplastic lesion of mesenchymal tissue origin that can sometimes become locally invasive and even metastasize or recur. Therefore, the diagnosis and treatment of HIMT is particularly important. However, hepatic inflammatory myofibroblastic tumor lacks a specific clinical presentation and typical imaging manifestations, thus posing a difficulty for us to diagnose and treat this disease.We report here a very rare surgical case of hepatic inflammatory myofibroblastic tumor (HIMT) in a 41-year-old female who was admitted to the hospital for more than half a month for a liver-occupying lesion with fever found on physical examination.After discussion with the hepatobiliary and pancreatic surgery team, we decided to perform surgical treatment. The final postoperative pathology confirmed hepatitis myofibroblastoma.Our review of the domestic and international literature revealed no significant progress in the diagnosis and treatment of this disease, so we report here a case of surgical treatment. One of our aims in this case report is to highlight the efficacy of surgical treatment in HIMT. HIMT is extremely rare and difficult to diagnose. Due to their intermediate biological behavior, surgical resection should be performed whenever feasible and patients should be followed-up in order to detect recurrence and metastasis as early as possible. Hepatic inflammatory myofibroblastic tumor (HIMT) is a very rare tumor in clinical practice.Its etiology and pathogenesis are not clear at present, and its clinical manifestations and imaging data are non-specific, so it poses a difficulty in clinical diagnosis and treatment. This rare tumor was first reported in the lung, but also can occur in a variety of tissues and organs in the body . HIMT waBecause the disease is rare and lacks specific clinical manifestations and imaging features, it is easily misdiagnosed as hepatocellular carcinoma when patients have risk factors for hepatocellular carcinoma. Therefore, how to improve the accuracy of the diagnosis and find the best treatment is the question we have to think about. However, given the characteristics of the disease, it is almost impossible to obtain a definitive diagnosis without pathological examination. When hepatitis myofibroblastoma is suspected, we recommend a puncture biopsy or surgical resection to clarify the diagnosis. Currently, there is a great debate on the treatment of hepatitis myofibroblastoma, with some advocating non-surgical treatment and others suggesting surgical treatment . Thus, tWe have reviewed several Chinese and English literature on hepatic inflammatory myofibroblastic tumor in recent years. and since this case is rare and not much literature is involved, here we briefly report a surgical case of this disease. In this case report, we have two objectives: the first to highlight that HIMT should be considered by clinicopathology in the diagnosis of liver masses. The second is to highlight the effectiveness of surgical treatment in hepatic inflammatory myofibroblastic tumor.9/L, Alpha fetal Protein (AFP):0.820 ng/ml, Carcinoembryonic antigen (CEA):0.91 ng/ml, Carbohydrate antigen125 (CA125): 22.19 U/ml, Carbohydrate antigen199CA199:1.69 U/ml, Calcitonin original (PCT): 0.12 ng/ml, C-reactive protein(CRP): 182.12 mg/L. The patient's liver function was basically normal, no bacterial growth was found in the blood bacterial culture and no fungus was found in the fecal and urine microscopy. Our enhancement MRI showed an abnormal signal in the right lobe of the liver, considering spindle cell tumor or tumor of mesenchymal origin :11.02*10l origin . We evall origin . The patThe patient was discharged in good general condition, the laboratory parameters were normal and we recommended regular follow-up in the outpatient clinic.The last outpatient follow-up was in April 2022 , and theHepatic inflammatory myofibroblastic tumor was once first found in lung tissue, but as the level of ancillary testing has improved, it has been reported in salivary glands, stomach, spleen, skin, and other tissues and organs. Inflammatory myofibroblastoma located in liver tissue is less common and is a rare junctional tumor of mesenchymal origin called hepatitis myofibroblastoma. It has been reported in the literature that hepatitis inflammatory myofibroblastic tumors can occur at any age, mostly in children or young adults, but no significant differences in gender have been observed . The patBecause hepatitis inflammatory myofibroblastic tumor is rare and lacks specific clinical manifestations and imaging features, it is easily misdiagnosed as hepatocellular carcinoma when patients have risk factors for hepatocellular carcinoma. From this case, we can see that the patient was admitted with fever for half a month and treated with various antibiotics before surgical treatment, but the effect did not improve significantly. Moreover, the patient's tumor markers were normal, but magnetic resonance showed an occupancy in the right lobe of the liver, so it was difficult to distinguish it from liver malignancy. Finally, after preoperative discussion and the patient's strong desire for surgery, we performed a right hemihepatectomy and the diagnosis was confirmed by postoperative pathological examination. However, none of the above imaging tests are specific, which makes the diagnosis of hepatitis myofibroblastoma difficult.Overall, hepatitis inflammatory myofibroblastic tumor is a junctional neoplastic lesion, and there is controversy over the treatment of this disease, which includes surgical treatment, high-dose steroid hormones, nonsteroidal anti-inflammatory drugs, and conservative treatment. It has been argued that partial hepatectomy should be the best treatment . HoweverWe contacted the patient in October 2020 and asked her for her views and insights on our treatment. The patient said she was very satisfied with the treatment her doctors had chosen for her throughout the treatment process. Before the operation, she had been suffering from abdominal pain and fever. This caused her distress and seriously affected her daily work life. When asked how her life has been since the surgery, she said she has returned to work and is making regular visits to the hospital. She said the surgery saved her life and wanted to stress how grateful she was that her condition was treated successfully. And she said she was pleased her case would be submitted for publication and believed it would help find treatments for patients with the same diagnosis.


This article discusses the management of an adolescent woman with a delayed diagnosis of adnexal torsion (AT) whose ovaries were successfully preserved.The patient was a 14-year-old female teen admitted with the chief complaint of lower abdominal pain for 3 days and worsening pain for 2 days. Magnetic resonance imaging suggested a high possibility of torsion in the anterosuperior uterine mass and was accompanied by severe ovarian edema, bleeding, and enlargement. Intraoperatively, the left fallopian tube was characterized by thickening and torsion and appeared blackish purple. The left fallopian tube paraovarian cyst was about 20 cm in size, and the left adnexa was twisted 1080° along the left infundibulopelvic ligament (suspensory ligament of the left ovary). The left ovary appeared blackish purple, with an enlarged diameter of about 10 cm. At the request and with the informed consent of the patient’s parents, we preserved the left ovary and removed the left fallopian tube. The results of the endocrine, ultrasound, and tumor marker tests were normal 1 month after surgery. Follicles and blood flow signals seen in ultrasound examinations indirectly proved the successful preservation of the left ovary in the follow-up.Our attempt to preserve the ovaries in an adolescent with a delayed diagnosis of AT was successful. Adnexal torsion (AT) is an anatomical translocation of the ovaries and/or fallopian tubes along the axis of the infundibulopelvic ligament and the suspensory ligaments of the ovaries, resulting in partial or total obstruction of the blood supply to the ovary. It is ranked as the fifth most common acute abdomen in gynecological practice , 2. AT cWith improvements in the understanding of AT, several studies have shown that recovery of ovarian function is possible after torsion release, regardless of the visual appearance of the AT , 3. TherThe patient was a 14-year-old female teen admitted with a chief complaint of lower abdominal pain for 3 days, which exacerbated for 2 days. Menarche had occurred at 10 years of age, the patient had regular menstruation, and her last menstrual period was on January 10, 2022. Three days before admission, she experienced spontaneous, dull pain in the lower abdomen after changing posture. Ultrasonography conducted in another hospital showed a large cyst on the right side of the pelvis measuring 19.6 × 9.6 cm. Moderate to low echogenicity with an area of about 11.5 × 6.3 cm was observed in the mid-upper pelvic cavity; she sought medical attention at our hospital. Two days before admission , she experienced increased abdominal pain with hypothermia and vomiting, but dizziness, headache, palpitations, and shortness of breath were absent. She did not undergo diagnosis or treatment for personal reasons and underwent consultation at our department for further pelvic examination. A gynecological examination showed abdominal distension, a palpable mass, positive tenderness, and rebound tenderness. She denied any history of chronic diseases, drug allergies, surgery, or blood transfusions.At the time of admission, her temperature, blood pressure, and pulse rate were 37.7 °C, 140–145/80–90 mmHg, and 80–90 beats/minute, respectively. On physical examination, abdominal distension, fixed lower abdominal tenderness, rebound tenderness, and shifting dullness were negative. Gynecological examination  revealed a mass that could be palpated at the pelvis, positive and rebound tenderness, and smooth rectal mucosa.9/L [4 × 109–10 × 109]; hemoglobin (HGB), 97 [110–150] g/L; and platelets (PLT), 227 × 109/L [100 × 109–300 × 109].Routine blood test:white blood cells (WBC), 16.88 × 10Tumor markers:human epididymis protein 4 (HE4), 40.44 [0–92] pmol/L; alpha fetoprotein (AFP), < 0.61 [0–7] ng/mL; carbohydrate antigen 199 (CA199), 3.84 [0–39] U/mL; and CA125, 66.97 [0–35] U/mL.Endocrine tests: follicle-stimulating hormone (FSH), 4.06 [1.50–9.10] IU/L; estrogen 2 (E2), 21.3 [52.80–214.20] pg/mL; progestogen (P), 0.289 [4.44–28.03] ng/mL; luteinizing hormone (LH), 5.5 [0.50–16.90] IU/L; testosterone (T), 2.52 [0.31–1.66] nmol/L; and anti-Mullerian hormone (AMH), 6.39 [0.24–11.78] ng/mL.Enhanced MRI: The left uterine horn was stretched upward, the uterine fundus was approximately 4.3 × 3.0 × 5.1 cm in size, and a solid mass was seen in the anterosuperior uterus, approximately 10.3 × 6.4 × 11.5 cm in size, showing iso-T1, a short T2 predominantly mixed signal, a flocculent long T2 signal, and a low signal on DWI, with small cystic foci scattered in the periphery, non-enhanced or mildly heterogeneous, strengthening on enhanced MRI, and another larger cystic foci with long T1 and long T2 signals that were connected with the above solid mass, measuring approximately 19.2 × 9.9 × 14.2 cm, with a thick and enhanced cystic wall on enhanced MRI, and pelvic effusion. No abnormally enlarged lymph nodes were seen in the pelvis and bilateral inguinal regions. Diagnostic conclusions included a solid mass in the anterosuperior uterus and an enlarged left ovary; the right upper cystic mass was considered a left ovarian cystic mass, and the possibility of torsion in the left ovary and its cystic mass was high and accompanied by severe ovarian edema, bleeding, enlargement, and pelvic effusion.The ancillary examinations were as follows:The intraoperative condition Fig.  was as fThe patient’s parents informed us that the patient was young and had a strong willingness to preserve her ovaries and that she could be followed-up closely. They were informed with the following details: there was a possibility of re-torsion if the ovaries were preserved; the blood supply to the ovaries was poor; surgery might be needed if necrosis occurred; the left fallopian tube was twisted and hypertrophied, had poor blood supply, and had lost its normal form and function; and re-torsion, hydrosalpinx, and ectopic pregnancy may occur if the left fallopian tube was preserved. Based on the latest consensus, left ovary preservation was considered, but the recommendation was to resect the left fallopian tube. The patient’s family expressed their understanding. At the request and with the informed consent of the patient’s parents, we preserved the left ovary, removed the left fallopian tube, and performed blood clot cleaning of the left ovary Fig. . For furOn day 1 after surgery, she had a transient temperature increase of 39 °C, which later spontaneously decreased to normal, with good gas expulsion and defecation.9/L [4 × 109–10 × 109]; HGB, 95 [110–150] g/L; and PLT, 365 × 109/L [100 × 109–300 × 109].Reexamination results were as follows: (1) coagulation: fibrinogen (FIB), 6.7 [1.8–4] g/L; activated partial thromboplastin time (APTT), 28.4 [23.3–32.5] s; thrombin time (TT), 14.3 [14–21]s; international normalized ratio (INR), 1.030 [0.8–4]; and D-dimer (D-D), 5.13 [0–0.55] mg/L. (2) Routine blood tests showed the following: WBC, 10.35 × 10A postoperative pathology test suggested a left fallopian tube paraovarian cyst with fibrocystic wall-like tissue in the left ovary, extensive congestion, and hemorrhage, and no apparent lining epithelium, consistent with torsional pathological changes.One month after surgery, the results of the endocrine, ultrasound, and tumor marker tests were normal, and menstruation was normal. The results of the endocrine tests were as follows: FSH, 7.35 [1.50–9.10] IU/L; E2, 35.7 [52.80–214.20] pg/mL; P, 0.223 [4.44–28.03] ng/mL; LH, 17.0 [0.50–16.90] IU/L; T, 1.680 [0.31–1.66] nmol/L; and AMH, 4.14 [0.24–11.78] ng/mL. Ultrasound:The left ovary was 5 cm in the maximum longitude size, with uneven echo. Multiple cysts (13 cysts/plane) were seen, and the diameter of the larger cysts was 0.7 cm. Blood flow signals could be seen in Color Doppler Flow Imaging (CDFI) ovary, Resistance Index (RI) = 0.77, Pulse Index (PI) = 1.97 (yellow arrow). The follicles and blood flow signals revealed by ultrasound, indirectly proved the successful preservation of the left ovary.In recent years, early diagnosis and standardized management of AT have received attention from Chinese and overseas experts. Surgical exploration is considered the standard method to confirm the diagnosis of AT. The incidence of AT in women aged 1–20 years was 5/100,000 in a US study . StudiesThe guidelines of the Royal College of Obstetricians and Gynecologists suggest that in premenopausal women, adnexal masses > 7 cm in diameter have an increased risk of rupture and therefore require elective surgical management . MoreoveMost scholars view that the intraoperative finding of a darkened ovary means that it is necrotic, AT reduction increases the incidence of thromboembolic events, and surgical treatment is mostly performed by resection of the affected adnexa, which has led to many unnecessary adnexal resections. The reported incidence of pulmonary embolism after AT was only 0.2%, and AT reduction did not increase the incidence of thromboembolic events . The intAnother concern is that the preserved ovaries still have the potential for future re-torsion. In addition, whether or not to perform ovarian fixation still needs to be determined in a study with a large sample size. In our patient, we unfortunately removed a unilateral fallopian tube that was edematous, severely deformed, and possibly severely impaired in function; however, fortunately, the ovary was preserved. We observed normal ovarian structures and ovulation under ultrasonography during the follow-up monitoring, indirectly proving the feasibility that even purplish-black ovaries should be preserved. Moreover, we realized that based on the pathological findings, it was not difficult to guess that this young patient could have had a comorbid paraovarian cyst that had twisted after activity, resulting in ovarian congestion, edema, and even ischemic necrosis, but the ovary itself did not have any lesion. The function of the twisted ovary could be preserved if we performed reduction alone, but postoperative clot absorption would increase the risk of fever or even infection, and the enlarged appendage would lead to re-torsion. The follow-up results proved that this method could increase the success of ovary preservation while partially retaining the function of the left ovary. In the literature, the overall incidence of recurrence of AT (rAT) in female teens was reported to be low, approximately 2–12% . A studyIn our patient, the adnexal cyst was seen intraoperatively to be a paraovarian cyst and was large enough to cause overall torsion of the left adnexa, and the ovary was edematous, congested, and even necrotic due to progressive ischemia. We did not see a solid mass during intraoperative dissection of the ovary; hence, we removed the left ovarian necrosis and clots, and a drainage tube was inserted into the pelvis, which reduced the probability of postoperative infection and fever, reduced the size of the ovary, and reduced the likelihood of recurrence of adnexal re-torsion. If the female teen had undergone early detection and treatment of the paraovarian cyst, she might not have been in such a passive situation, with the left fallopian tube necrotic and severely twisted, leading to its removal and the left ovarian function being severely affected. Therefore, we recommend performing laparoscopic exploration in female teens who are found to have adnexal cysts, if the adnexal diameter (including the mass) is > 6 cm, and persists for 3 months , or if it is accompanied by discomfort such as dull pain in the lower abdomen with adnexal cysts persistence. Although the incidence is extremely low, it indirectly suggests that paraovarian cysts should not be ignored. Periovarian cysts and surgery should be recommended if there is intraoperative or imaging evidence of persistent and enlarged masses.The following reasons are summarized in the case of our adolescent female teen who successfully preserved her ovaries: (1) The time to torsion was about 72 h, and we found that the AT was quickly treated surgically to give adequate time for the recovery of the torsioned adnexa. The longer the interval between the onset of AT and surgical treatment, the greater the likelihood of ovarian damage. Therefore, emergency surgical intervention is required once AT is diagnosed. The time between the disruption of the adnexal blood supply and irreversible ovarian damage is unclear; however, some studies have suggested that ovarian function may still be restored beyond 72 h of AT, but most believe that ovarian function begins to decline dramatically after 48–72 h . Early aCurrently, there are no guidelines on the prevention of AT for female teens in the expert AT consensus. For adolescent women who have had their menarche before aged < 20 years, parents and schools often neglect gynecological examinations during routine physical examinations because they have not yet started becoming sexually active, and because there are more activities and sports at this age. Cysts also grow gradually with age; hence, they are often detected only when they are huge and affect patients’ lives or when they are found to have reached an acute stage such as torsion or rupture. The symptoms are also atypical and are easily missed and misdiagnosed. Therefore, prevention is more important than the treatment of children and female teens, and it is recommended to increase gynecological ultrasound screenings every year. Moreover, physiological health-related scientific knowledge should be imparted so that girls will not be embarrassed if they develop abdominal pain and discomfort and will instead seek timely medical attention.Regarding the choice of surgical approach in the consensus, laparoscopic exploratory surgery was suggested to be used as the first choice. However, the ovary and cyst will increase dramatically after adnexal mass necrosis and torsion. Often, the adnexal mass is about 20 cm in diameter, and the thickening of the infundibulopelvic is often accompanied by thrombosis, which might cause significant difficulties for laparoscopic surgery. The surrounding structures cannot be seen clearly, and it is easy to cause collateral damage. Whether laparoscopic surgery has absolute advantages over small-incision open surgery needs to be examined, but laparoscopic surgery is a good option if used only as an experimental procedure to provide an opportunity for adnexal reduction. Even if adnexal masses with diameters > 10 cm have a higher chance of intraoperative spillage, this does not implicate an a priori need for proceeding with open surgery, particularly when the risk of malignancy is low. The presence of ascites and the size of the lesion associated with a high level of CA 125 affected the correct assessment of the risk of malignancy, exposing the patient to overtreatment . ConsideOur patient underwent emergency surgery, and the preoperative investigation of the adnexal mass revealed that the size of the mass reached the level of the umbilicus, the mass was huge, and the patient was considered to have a true cyst present with an unknown multi-compartment-like nature; hence, a longitudinal small-incision open surgery was performed. However, in retrospect, it may have been feasible if laparoscopic exploration had been performed at that time, the left fallopian tube was resected after reduction, and the left ovary was preserved. Ovarian function can be observed after waiting 6–8 weeks to decide on the follow-up treatment.We experienced several cases of ovarian cyst torsion in adolescent women in the last year. After the reduction in adolescents and children, the symptoms are not typical, and many patients are found to have purplish-black ovaries. Some of these affected girls lose unilateral adnexa, which is deplorable. Our attempt to preserve the ovaries in adolescents with delayed diagnosis of AT was successful in this case and further confirmed the validity of the current consensus on AT in the United States and China. We hope that in future guidelines and consensus improvement, scientific education on physiological anatomy and physiological hygiene of adolescents can be increased and the risk management-related issues such as preventive measures and response to the onset of infection and thrombosis should be given attention during postoperative pain management.


Solitary fibrous tumor (SFT) are rare pleura neoplasms often localized to middle or inferior hemithorax. A middle-aged woman presents to the emergency department following a motor vehicle accident, the computed tomography scan revealed a giant tumor occupying the entire left pleural cavity with a complete collapse of the left lung and substantial right deviation of heart and mediastinum. Using preoperative arterial coiling followed by a double-level thoracotomy we successfully resected the giant tumor. The SFT weighed ~10 lbs. At 2-month follow-up visit patient reports mild discomfort during strenuous movement/heavy lifting but denies any shortness of breath. Solitary fibrous tumors (SFT) are defined as mesenchymal neoplasms of submesothelial origin with fibroblastic differentiation, typically benign in nature . SFTs ofWe present the case of a giant pleuro-parenchymal SFT treated with preoperative arterial coiling followed by resection by a double-level thoracotomy.A 33-year-old woman presented to the emergency department following a motor vehicle accident. Imaging showed a large heterogenous intermediate density mass filling and expanding the left hemithorax, causing a complete collapse of the left lung and substantial right deviation of heart and mediastinum . The patMagnetic resonance imaging showed no invasion of chest wall or visceral structures, consistent with resectability . To prepThe following day a double thoracotomy was performed at the fifth and eighth intercostal space via single oblique skin incision, due to the expansion of the tumor from the left apex to the diaphragm. This allowed satisfactory exposure of the upper and lower aspects of the tumor, with the mass being delivered from the chest through the eighth interspace opening. The upper and lower lobes of the left lung were able to expand successfully. The tumor measured 22 × 26 × 11 cm, weighing ~10 lbs. The patient’s left lung expanded almost fully, and she was discharged one week post-op. At 2-month follow-up visit patient reports mild discomfort during strenuous movement/heavy lifting but denies any shortness of breath .The institutional review board waived the need for written consent 24 October 2022.SFTs were first described by Klemperer and Rabin in 1931. Although originally identified as rare primary pleural neoplasms of mesenchymal origin, immunohistochemical analysis has indicated these fibromas originate from the mesenchyme rather than mesothelial cells . SFTs arBecause of the large size of this tumor, we pursued preoperative arterial occlusion, as operative approach to the vascular supply was expected to be difficult due to the exposure constraints of the ribcage and diaphragm. Preoperative angiography is beneficial for identifying and embolizing major feeding vessels . With thThese tumors are rare, and their sheer sizes within the constraints of the thorax make them difficult to resect. Preoperative arterial embolization has been reported as an adjunct, and double thoracotomy has also been reported for these tumors. We found the combination of these maneuvers to be very helpful in our case: the diaphragm surface required some blunt dissection, which was done without massive bleeding, and the double thoracotomy allowed good exposure to the apex, as well as an adequate opening for tumor delivery through the lower thoracotomy. We think that both embolization and double thoracotomy together allowed for safe removal of this challenging giant tumor.


We report a case of orbital metastasis of infiltrative breast carcinoma in a 65-year-old man. The patient was diagnosed with stage four breast cancer one year before, for which a mastectomy was performed. He refused postoperative radiotherapy and chemotherapy at that time. He had a history of lung, liver, and mediastinal metastases. At admission, he presented with blurred vision, diplopia, ocular pain, and mild swelling of the upper lid of the left eye (LE). Computed tomography (CT) of the brain and orbit revealed a front-ethmoidal tissue mass with left orbital and frontal intracranial extension. Ophthalmologic examination revealed exophthalmos on the LE with a downward and outward deviation of the eyeball, proptosis, and intraocular pressure (IOP) of 40 mmHg. The patient’s treatment started with topical maximal anti-glaucomatous drops and radiotherapy sessions. After three weeks of follow-up, there was a gradual improvement of local symptoms and signs and a normal IOP.


Brucella melitensis. He was managed conservatively for splenic hematoma and received one unit blood transfusion and treatment with combination of antibiotics (rifampicin and doxycycline) for brucella for six weeks. On follow-up, the patient reported no further complications. Spontaneous splenic rupture is a clinical rarity and should be considered in patients presenting with acute abdomen and suspected infective, neoplastic, and inflammatory pathology. Spontaneous splenic rupture in acute brucellosis requires prompt clinical recognition and immediate anti-Brucella therapy to prevent the catastrophic progression.Brucellosis is a common zoonotic infection worldwide caused by the bacterial species Brucella. It has a wide range of presentations from asymptomatic infection to multisystem involvement. Splenomegaly is seen in around 30-60% of cases, however, atraumatic spontaneous splenic rupture is extremely rare. We present a case of a 45-year-old man who presented with acute left upper quadrant pain and fever of five days duration without a history of antecedent trauma. He was hemodynamically stable with examination revealing left upper quadrant tender palpable mass. Ultrasonography followed by computed tomography revealed subcapsular hematoma with perisplenic and perihepatic free fluid. Viral markers  were negative. The autoimmune profile was negative. Brucella serum agglutination test was positive (1: 640) and blood cultures grew  Brucellosis, a zoonotic infection seen worldwide, is caused by a bacterial species Brucella, a Gram-negative bacillus. It has a wide range of presentations, ranging from asymptomatic infection, and non-specific symptoms  to having a multisystem involvement. Almost all systems are involved including the spleen ,2.Gastrointestinal manifestations of brucellosis are seen in up to 40% of cases, these include anorexia, abdominal pain, constipation, and weight loss being the commonest. Complications of brucellosis include Brucella hepatitis with abscess formation, splenic abscess, spontaneous rupture of the spleen, cholecystitis, intestinal obstruction or perforation, and pancreatitis which can manifest as acute abdomen .Splenic enlargement in brucellosis is reported in around 30-60% of cases . SpontanTo date, only five cases of SSR as a complication of brucellosis have been reported worldwide -11. HereA 45-year-old male, a farmer by occupation, with no underlying co-morbid illness presented to the emergency department with acute abdominal pain in the left hypochondrium for a few hours duration. He had a history of high-grade fever (maximum of 103°F) for the last five days. Being a farmer, the patient had exposure to cattle. However, there was no history of antecedent trauma in the last two weeks, and no history of any other family member being ill. On clinical examination, there was pallor and tachycardia (114 beats/min) and blood pressure was 110/70 mmHg. He was anxious in appearance and was wincing with abdominal pain. Abdominal examination revealed mild distension with a palpable mass of 4-5 cm below the left costal margin, in addition, there was tenderness  and rebound in the left hypochondrium.Baseline investigations as seen in Table Ultrasonography showed splenomegaly with free fluid in the abdomen. Contrast-enhanced computed tomography (CECT) of the abdomen and pelvis was sought, splenomegaly with a large subcapsular splenic hematoma with moderate free fluid in perisplenic, perihepatic, and pelvic regions suggestive of splenic rupture with hemoperitoneum was found Figure . DiagnosBrucella melitensis with serum agglutination test revealing titers of greater than 1:640 for Brucella melitensis and higher dilution revealing titers of 1:2560. The echocardiographic examination was normal. Conservative management for splenic rupture was considered after consultation with the surgical team. He received intravenous fluids, one unit blood transfusion, and daily clinical and biochemical monitoring was done. Hemoglobin had risen to 10.1 g/dL post blood transfusion, and no further drop in hemoglobin during the course of hospitalization was seen. He was started on triple therapy for brucellosis with rifampicin, doxycycline for six weeks, and streptomycin initially for a total duration of three weeks. Repeat ultrasonography at six weeks revealed resolution of hemoperitoneum and subcapsular splenic hematoma. On follow-up as an outpatient, no complications or sequelae developed.Viral serology for hepatitis B and C, Ebstein-Barr virus (EBV), cytomegalovirus (CMV), and HIV were negative. Malaria by card test and dengue serology were negative. The autoimmune profile  was negative. Blood culture grew Rupture of the spleen is a commonly seen complication following blunt abdominal trauma. An atraumatic rupture in a diseased spleen is seen less frequently but has been widely reported in the literature . In contThe first case of SSR was described in 1874 by Atkinson, on post-mortem analysis . A systeSSR has been widely reported in numerous infective, neoplastic, inflammatory, rheumatological, and hematological diseases. Malaria has been implicated in most cases of all the infective causes, followed by infectious mononucleosis (EBV), CMV, HIV, Salmonella, brucellosis, babesiosis, and tuberculosis ,6.Brucellosis as a cause of SSR is very rare and ours is the sixth case reported in the literature. Table The underlying mechanism contributing to spontaneous rupture of the spleen has been attributed to splenomegaly, especially pathological infiltration of the capsule, splenic infarct with subcapsular hemorrhage (subsequent rupture), and sometimes associated coagulopathy. However, rupture is known to occur from a combination of these mechanisms ,14. As pThe typical presentation involves left upper quadrant (LUQ) pain, vomiting, abdominal distention, tenderness, and dizziness that may occur if hypotension develops. Kehr sign (referred pain to left shoulder) and Ballance sign  may be present, as seen in our case where a tender palpable mass in LUQ was present .Radiological evaluation via ultrasonography forms the mainstay in diagnosis, revealing an enlarged spleen, areas of decreased echogenicity, subcapsular and pericapsular hematomas, and the presence of intraperitoneal free fluid. Computed tomography (CT) having a higher sensitivity and specificity helps in the diagnosis and grading of splenic injury ,17.The treatment decision depends on a case-to-case basis, upon the grade of splenic injury, and the hemodynamic stability of the patient, as there is no clear consensus on treatment. In the presence of certain red flags like hemodynamic instability at presentation, high-grade splenic injury, precipitous fall in hemoglobin to less than 10 g/dL, or acute worsening of clinical condition , a splenectomy along with exploratory laparotomy for draining of hemoperitoneum can be considered the procedure of choice . HoweverSpontaneous splenic rupture is a rare phenomenon and should be considered in patients presenting with acute abdomen and underlying suspected infective, neoplastic, and inflammatory diseases. Our case highlights the occurrence of spontaneous splenic rupture in acute brucellosis which requires prompt clinical recognition and immediate anti-Brucella therapy to prevent the catastrophic progression.


Orbital masses include a broad spectrum of benign and malignant entities. Often these masses are asymptomatic or show a slow growth rate, so that emergence of clinical symptoms is prolonged. In this context, cross-sectional imaging plays an elementary role in the characterization of these lesions. Aside from the characterization of the underlying entity, an evaluation of the involved compartments is possible by sufficient imaging, which also facilitates optimal treatment and surgery planning. The purpose of this review is to explore different benign and malignant orbital tumors and their typical appearance in imaging together with histopathologic findings. Vision is the most important of the five human senses. Human beings receive more than 80% of their sensory perception through the eyes. Traumatic accidents and tumorous lesions are the most common reasons to perform the dedicated imaging of the orbit . ClinicaThis pictorial review describes the most common tumorous orbital disorders. In addition, the imaging techniques and radiological findings for each pathology are described.Detailed knowledge of the anatomy of the orbit is important. The exact description of the anatomical location of the tumors and the involved structures are crucial for diagnosis on the one hand and for further therapy, e.g., surgical treatment, on the other hand . The orbThis pictorial review is based on cases of orbital tumors collected from the Department of Diagnostic and Interventional Radiology at the Ulm University Medical Center . MRI was performed with a 1.5T or 3.0T system , whereas CT images were acquired with a 128 slice scanner .The MRI data were acquired with the use of a phased-array surface coil system. The institutional protocol includes T1- and T2-weighted oblique axial images along the plane of the optic nerves, oblique T2-weighted images, and diffusion-weighted imaging. If an intravenous contrast agent was applied, all sequences after application were acquired with fat suppression. The sequence protocol for orbit imaging is shown in Retinoblastoma is an aggressive eye tumor of children and infants, representing the most common intraocular neoplasm in childhood . Of all Despite aggressive growth patterns, the overall survival rate is very high (>95%). Nevertheless, the prognosis of metastasized retinoblastoma remains poor . For retThe MRI of the orbit is the standard method for assessing extraocular growth and the possible infiltration of the optic nerve. On T2-weighted images, retinoblastoma is hypointense compared to the vitreous body or cerebrospinal fluid. The calcified parts of the tumor can appear as hypointense foci on both T1- and T2-weighted images. On T1-weighted images, retinoblastoma is slightly hyperintense, with moderate to marked enhancement after the administration of a contrast agent. In addition, retinoblastoma shows restricted diffusion on DWI and low signal in the apparent diffusion coefficient (ADC) maps ,14. BothDifferent clinical classification systems exist; the most commonly used ones are the International Intraocular Retinoblastoma Classification (IIRC) and Intraocular Classification of Retinoblastoma (ICRB) ,20. An oThe involvement of the optic nerve is associated with a poor prognosis, with a mortality rate ranging from 13% to 89%, depending on the degree of optic nerve involvement and whether the surgical margin is clear . In thisUveal melanoma is the most common primary intraocular malignancy in adults . It reprUltrasound only measures two-dimensional cross sections of the tumor, whereas MRI makes it possible to image the complete tumor in three dimensions and the possible extrascleral extensions. The typical appearance of the uveal melanoma is a well-defined, hyperintense tumor on non-enhanced T1-weighted images compared to the vitreous body. The hyperintense signal depends on the tumors´ melanin content. A moderate contrast enhancement can be detected; nevertheless, it is hardly visible due to the intrinsic T1 hyperintensity. In addition, uveal melanoma often causes retinal detachment and subretinal hemorrhage .Uveal melanoma can show different morphologic features, such as lentiform, dome, or mushroom shape. Tumor shape itself mirrors the type of tumor development and therefore has a prognostic relevance. Flat lesions may remain small over a long period; however, they can infiltrate the sclera ,27. GrowOn contrast-enhanced T1-weighted images, the tumor shows moderate diffuse enhancement. On T2-weighted images uveal melanomas show marked hypointense signal compared to the vitreous body. In addition, retinal detachment with subretinal fluid is present. Evaluating the tumor in different planes is needed to assess tumor geometry, for accurate measurements, to identify sclera/extrascleral extension, and to plan an adequate surgical resection . TypicalOrbital venous varices can relate to an intra- and/or extraconal mass but are rather rarely observed. They account for less than 2% of all orbital tumors . They tyImaging findings strongly depend on the grade of dilatation of the orbital varices at the time point of image acquisition. Of note, the collapse of varices particularly during image acquisition can be common, given that jugular vein pressure is low during supine position that is, however, standardly used for CT or MRI examinations. Thus, the absence of an orbital mass under these premises does not entirely rule out orbital varices. Consequently, different approaches prior to or during image acquisition have been propagated, including the Valsalva manoeuvre or elastic neck tourniquets to enhance jugular compression ,34,35. FOrbital cavernous venous malformation (cavernous hemangioma) is the most common benign ophthalmic tumor in adults. They make up about 5 to 8% of all orbital tumors, mostly present in the middle aged individuals (between 30 and 50 years) . Women aHemangioma is typically surrounded by a fibrous capsule. Overall, cavernous hemangiomas can be located anywhere within the orbit; however, a majority (>80%) are located in the intraconal compartment, especially in the lateral compartment . On CT sBone remodeling or small calcifications can occur. Due to low vascular flow, mild contrast enhancement is typical. On MRI, cavernous hemangioma shows a heterogeneous signal on T1- and T2-weighted images, with mild contrast enhancement on T1-weighted images after contrast administration. Typically, an isointense signal compared to muscles can be found on T1-weighted images. Hyperintense signal can be found in case of thrombosis within the hemangioma. Flow voids within the mass are common ,40. Typiclassification of the ISSVA  and the Hamburg classification system of vascular malformations [Most of the cases are managed with a conservative therapy without surgery. Typical clinical classification systems are the rmations ,42,43. ACapillary hemangioma is the most common benign tumor in infants . It occuThe diagnosis is commonly made clinically, and the majority of patients do not require special treatment. Capillary hemangiomas are usually located anterior to the globe and are present at birth. When capillary hemangioma appears as a mass of the orbit and periorbital areas, imaging is often necessary to assess the extent of the hemangioma and its possible effect on adjacent anatomical structures . Aside fSuperficial lesions below the dermis tend to have a deep blue hue. These lesions can present as skin discoloration and can be seen from outside. In contrast to this, deep orbital lesions can be invisible from outside and could be detected by clinical symptoms of proptosis, strabismus, or decreased visual acuity. Mixed lesions show a combination of both manifestations .On MRI with T-weighted images, capillary hemangioma appears as an extraconal, well-defined, lobular hyperintense mass . Septa oLymphoid orbital tumors, including benign reactive lymphoid processes and malignant lymphoma, represent 10 to 15% of all orbital tumors and 55% of ophthalmic malignancies . OrbitalMalignant orbital lymphoma typically arises from the mucosa-associated lymphoid tissue of ocular adnexa as a B-cell non-Hodgkin lymphoma. In addition, in 1.5 to 5% of patients with systemic lymphoma, the orbit can be involved . In moreMost patients are elderly and between 50 and 70 years of age. No gender predilection is known in the current literature; however, this varies with the histological subtype . LymphomThe corresponding histopathology is illustrated in However, especially in mantle cell lymphoma, a bilateral tumor can be registered  Figure .On CT and MRI, lymphoma appears as a well-circumscribed, homogenous, typically lobulated mass. Tumors mold around normal anatomic orbital structures. Typically, they do not erode surrounding bony structures. On CT, lymphoma appears isodense to skeletal muscles with homogenous contrast enhancement. A homogenous intermediate signal on T1- and T2-weighted imaging, with homogenous contrast enhancement on T1-weighted imaging after contrast administration, is typical. The signal on T1-weighted images is usually iso- to hypointense compared to muscle signal, reflecting a high cell density. Signal on T2-weighted images is usually hyperintense compared to muscle signal but can be hypointense to the signal of the bulbus or cerebrospinal fluid. Due to its hypercellular nature, high signal on DWI and signal loss on ADC maps can help to distinguish lymphomas from inflammation. An affection of the lacrimal gland is possible.Lacrimal gland tumors are rare and account for about 10% of orbital masses. These tumors include a wide spectrum ranging from benign epithelial and lymphoid lesions to carcinomas, also including lymphomas and sarcomas with respective differences in the choice of treatment and related prognosis . An overHowever, for definite diagnosis, excision or biopsy is required, and radiological imaging can help to distinguish between benign conditions  or malignant masses . Furthermore, CT or MRI findings can be used to plan surgical excision/biopsy and further treatment, e.g., chemotherapy. Pleomorphic adenoma is the most common benign epithelial tumor. On CT images a typically well defined, unilateral tumor with moderate contrast enhancement that involves the orbital lobe of the lacrimal gland can be seen. On MRI pleomorphic adenoma present with low to intermediate T1 signal and intermediate T2 signal compared with orbit muscles. After contrast admission they show moderate enhancement .The most common epithelial tumors are benign pleomorphic adenoma. Among lacrimal gland masses, malignant epithelial tumors account for approximately 5% of all orbital lesions. The most common malignant lacrimal gland tumors are adenoid cystic carcinomas and carcinomas .Patients present with symptoms such as pseudoptosis, exophthalmos, dystopia, pain, and reduced visual acuity . On CT, As a slow growing tumor, optic nerve glioma occurs typically in children. In 12 to 37% of cases, it appears in patients with neurofibromatosis type 1 (NF1), often with bilateral manifestation. In patients with NF1, it is associated with a better prognosis compared to other cases . Optic nOn the CT, a widening of the optic canal can be seen in the bone window. Furthermore, MRI typically shows a well-defined, fusiform enlargement and tortuosity of the optic nerve . A compaOn T1-weighted sequences the tumor shows a hypo- or slight hyperintense appearance compared to the contralateral side. T2-weighted images show a hyperintense mass as compared to the cerebral cortex. Sometimes cystic degeneration may occur. Additionally, a slight contrast enhancement or sometimes no enhancement can be seen on T1-weighted imaging after contrast administration. Optic nerve gliomas with the involvement of the intraorbital nerve and the chiasm can show the shape of a dumbbell.T1-weighted images without fat saturation are suitable for measuring the size and for delineating the course of the optic nerve. The normal maximum diameter in children is 5 mm or less. Hemorrhage or calcification are rare . The invThe optic nerve sheath meningioma (ONSM) accounts for 1 to 2% of all meningiomas. In addition, it is the second most frequent primary optic nerve tumor . ONSM coOn MRI, ONSM typically shows intermediate and homogenous signal on T1- and T2-weighted images. On T1-weighted imaging after contrast administration, ONSM appears as a homogenously enhancing tumor surrounding the optic nerve. CT may show calcification within the lesion. The optic nerve can be located in the center or eccentrically in relation to the mass. Post-contrast T1-weighted, fat-suppressed images are ideal for the diagnosis. Sometimes a characteristic “tram track sign” on axial images or a “doughnut sign” on coronal images can be detected .Meningiomas tend to cause the segmental or diffuse circumferential thickening of the optic nerve sheath so that on sagittal images this produces a tram track sign . The extAn example of a patient with ONSM is shown in Schwannomas are the most common benign peripheral nerve tumors in adults. Any nerve with Schwann cells in the perineurium can be affected, including all cranial nerves (except for optic and olfactory nerve), spinal nerves, and the autonomic nervous system . GeneralIt is often difficult to differentiate schwannomas clinically from other orbital tumors. Typical imaging findings can help to predict the diagnosis of a schwannoma. On MRI, orbital schwannoma presents as a lobular, heterogeneously enhancing mass on T1-weighted imaging after contrast administration, with isointense or hypointense signal on non-contrast T1-weighted and marked hyperintense signal on T2-weighted sequences .Contrast enhancement can either be distributed homogenously or heterogeneously ,76,80. SPeripheral neurofibromas are benign peripheral nerve sheath tumors composed of Schwann cells, fibroblasts, perineurial cells, and mast cells . They caOn cross-sectional imaging plexiform neurofibromas may involve large portions of the face in the form of a serpentine soft tissue mass. On T1-weighted images, they present with a heterogeneous signal, and on T2-weighted images with a hyperintense signal. The tumor typically shows heterogeneous contrast enhancement on T1-weighted sequences after contrast administration . LocalizOrbital metastases are reported in the literature with an incidence of up to 13% of all orbital tumors . In 2 toThe mean age is over 60 years when orbital metastatic masses are diagnosed . ClinicaA lesion without any symptoms and empty tumor anamnesis suggests a benign lesion. In case of missing anamnesis, a biopsy by fine-needle aspiration is preferred over an open biopsy. Metastatic lesions are more common in the anterior orbit than posterior orbit ; howeverRhabdomyosarcoma (RMS) is a very rare malignant tumor of the childhood. Approximately 90% of orbital RMS occur before the age of 16 years . The heaFor the diagnosis and grading of RMS, a biopsy is the reference standard . CT and Benign and malignant orbital tumors can be challenging for image interpretation. Apart from their typical clinical presentation, the radiologist should know characteristic appearances and key features on cross-sectional images (CT an MRI) to determine the correct diagnosis and distinguish between similar entities. Moreover, the precise description of lesion location, involved orbital compartments, and the extent is crucial for further treatment. This pictorial review should help the radiologist in the diagnosis and evaluation of orbital tumors.


Dieulafoy’s lesion are relatively rare and can cause severe gastrointestinal bleeding. A Dieulafoy’s lesion is defined as an artery that erodes the overlying epithelium without the presence of an ulcer. Bleeding in Dieulafoy’s lesion predominantly involves the proximal stomach and upper gastrointestinal tract and is rarely observed in the lower gastrointestinal tract.Case 1 was a 58-year-old woman complaining of sudden headache and vomiting who was diagnosed with subarachnoid hemorrhage. She underwent transcatheter embolization for intracranial aneurysm treatment but had an acute profuse hematochezia on the 11th day of admission. Case 2 was a 63-year-old man admitted to the respiratory intensive care unit because of fever with altered consciousness level for a week. He was diagnosed with advanced lung cancer that had metastasized to multiple organs one month prior. On the third day of admission, he had an attack of profuse hematochezia, and quickly developed shock and apathy.Both patients were diagnosed with actively bleeding rectal Dieulafoy’s lesion by bedside emergency colonoscopy.Endoscopic hemostatic clipping was performed in 2 patients.Hemostasis was successfully achieved in these 2 patients, and there was no recurrence of symptoms during follow-up.We propose that hemostatic clipping is one of the options in the treatment of rectal Dieulafoy’s lesions. In the gastrointestinal tract, Dieulafoy’s lesion refers to the persistence of an abnormally large and tortuous primary arterial branch that extends in the submucosa without caliber loss, protruding through a small mucosal defect. Dieulafoy’s lesion accounts for 5% of all causes of acute gastrointestinal bleeding, and over 70% of the lesions occur in the proximal stomach, especially in the lesser curvature of the gastroesophageal junction. However, these lesions have also been reported in other parts of the gastrointestinal tract, such as the esophagus, duodenum, jejunum, and colon.Dieulafoy’s lesion is an uncommon but well-recognized cause of significant gastrointestinal bleeding. This lesion was initially described as a gastric aneurysm by Gallard in 1884 Currently, limited studies report that rectal lesions account for < 2% of all Dieulafoy’s lesions, making it an extremely rare cause of gastrointestinal bleeding.The etiology of rectal Dieulafoy’s lesion is unknown, and major underlying disorders include hypertension, diabetes mellitus, and chronic kidney disease. Surgery or selective arterial embolization have been the main treatment methods for rectal Dieulafoy’s lesion in the past. However, these patients frequently experience serious complications and are at high risk for surgery or respiratory or circulatory failure, and cannot be transferred to the intervention room. Bedside colonoscopy is currently considered the first option for the management of gastrointestinal Dieulafoy’s lesions. Although the effectiveness of different emergent endoscopic treatments, including injection, coagulation, clipping, and band ligation, in the management of rectal Dieulafoy’s lesions has been demonstrated, there is no consensus on the optimal endoscopic hemostasis technique for rectal Dieulafoy’s lesions owing to the rarity of the disease. We report 2 cases of acute hemorrhage due to rectal Dieulafoy’s lesion that were both successfully treated by endoscopic hemostatic clipping. We also present a review of literature on rectal Dieulafoy’s lesion and the recent trends in its clinical features, pathogenesis, and endoscopic therapies.Rectal Dieulafoy’s lesion was first reported by Franko et al.This study was approved by the Ethics Committee of Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology. Informed written consent was obtained from the patient for publication of this case report and accompanying images.A 58-year-old woman complaining of sudden headache and vomiting was transferred to the neurosurgery intensive care unit of our hospital. She was diagnosed with subarachnoid hemorrhage by computed tomography. She had a history of hypertension and belonged to the Rh-negative blood group. Transcatheter embolization of intracranial aneurysms was performed on the day of admission. She received oral aspirin and clopidogrel bisulfate after the operation. The patient was repeatedly administered diclofenac sodium suppositories and intravenous injection of dexamethasone due to postoperative persistent fever. On the 11th day of admission, the patient had an acute profuse hematochezia and a decreased blood pressure of 74/54 mm Hg. Her hemoglobin level decreased from 11 g/dL at admission to 9 g/dL , accompanied by a decrease in serum albumin to 29.4g/L .Other laboratory test results, such as plasma concentrations of urea, creatinine, amylase, platelet counts, and coagulation, were within the normal range. Bedside colonoscopy found a large amount of bright red blood and blood clots in the left colon, and yellow-colored stool in the right colon. After careful washing, pulsatile fresh bleeding from an exposed vessel without a mucosal defect or ulceration was found 3 cm proximal to the anal verge . The patient improved and was discharged 2 days later. There was no further bleeding after 1 month of follow-up.A 63-year-old man was admitted to the respiratory intensive care unit of our hospital due to fever with altered conscious level for a week. This patient was diagnosed with advanced lung cancer that had metastasized to multiple organs one month prior and had been administered durvalumab in combination with etoposide and carboplatin. He had a medical history of hypertension and constipation. After admission, the patient was administered noninvasive ventilator assisted ventilation and antibiotics, as well as diclofenac sodium suppositories and intravenous methylprednisolone sodium succinate injection. On the third day of admission, he had an attack of profuse hematochezia, his blood pressure dropped to 86/56 mmHg, and his hemoglobin level decreased from 13.1 to 9.1 g/dL . Bedside colonoscopy revealed rectal Dieulafoy’s lesion 5 cm from the anal verge with active pulsating bleeding without surrounding ulceration Fig. A and B.  This lesion is majorly detected in the proximal stomach and is rarely reported in the rectum. Due to its rarity, the understanding of rectal Dieulafoy’s lesions mostly depends on case reports and small case series: there is a lack of systematic studies on its etiology, patient characteristics, clinical features, and therapeutic modalities.Dieulafoy’s lesions are characterized by aberrantly enlarged submucosal arterioles protruding through a small mucosal defect, which is an uncommon but well-recognized cause of acute life-threatening gastrointestinal hemorrhage. rectal Dieulafoy’s lesions frequently involve patients over the age of 60 , chronic renal disease (22%), diabetes mellitus (20%), cardiovascular and cerebrovascular diseases (19%), and cancer (9%) were among the most common comorbid conditions  and a lower rebleeding rate (18% vs 22%) than those of endoscopic monotherapy (Table There is no consensus on the treatment of rectal Dieulafoy’s lesion. Therapeutic endoscopy has emerged to be a feasible, safe, and effective modality with a hemostatic success rate above 90%.es Table . Thermoces Table . Endoscoes Table . Therefoe 18% vs 2% than tIn conclusion, rectal Dieulafoy’s lesion represents a rare cause of lower gastrointestinal bleeding. Massive hemorrhage could increase the morbidity and mortality of these patients. We report 2 cases of acute hemorrhages due to rectal Dieulafoy’s lesion in 2 critically ill patients hospitalized for other conditions. Both patients were treated successfully by endoscopic hemostatic clipping, without rebleeding during follow-up. We propose that hemostatic clipping is one of the options in the treatment of rectal Dieulafoy’s lesions.Conceptualization: Ping Han, Wei Yan.Data curation: Yu Lei.Investigation: Ping Han, Wei Hou, Jingmei Liu.Methodology: Yu Lei, Nianjun Chen.Resources: Dean Tian, Qiaozhen Guo.Visualization: Jingmei Liu, Dean Tian.Writing—original draft: Ping Han, Yu Lei.Writing—review and editing: Qiaozhen Guo, Wei Yan.


Thoracic SMARCA4‐deficient undifferentiated tumours are ordinarily found as a huge mass with systemic metastasis, and the prognosis is poor. The potential of immunotherapy for these unresectable tumours has been reported. An asymptomatic 68‐year‐old man with a smoking history had a left lung mass without distant metastasis and underwent complete resection. Two months after surgery, with no adjuvant therapy, he developed multiple distant metastases with aphasia and died 4 months after surgery. Adjuvant treatment may be necessary with immune checkpoint inhibitors, with a closer follow‐up to detect recurrence without symptoms. Thoracic SMARCA4‐deficient undifferentiated tumours are ordinarily found as a huge mass with systemic metastasis, and the prognosis is poor. Although a small number of operable cases have been reported, it is unclear whether complete resection contributes to a longer prognosis. Here, we present a case of SMARCA4‐UT in which distant metastasis occurred shortly after complete resection, leading to an aggressive course. Thoracic SMARCA4‐deficient undifferentiated tumour (SMARCA4‐UT) is a high‐grade malignant neoplasm caused by the loss of function of SMARCA4, a key member of the SWItch/Sucrose‐NonFermentable (SWI/SNF) adenosine triphosphatase (ATPase)‐dependent chromatin remodelling complex.A 68‐year‐old man with a 50‐pack‐year smoking history was referred to our hospital for evaluation of an abnormal chest radiograph. Computed tomography revealed a 3.5‐cm mass in the left upper lung lobe and an enlarged hilar lymph node Figure . The swoSMARCA4‐UT is histopathologically characterized by an undifferentiated or rhabdoid phenotype and SMARCA4 deficiency.The tumour often appears in the fourth and fifth decades of life, usually with a smoking history, and shows a strong male predominance .SMARCA4 and often have additional mutation in TP53, KRAS, STK11 and KEAP1, which are common drivers of smoking‐related lung cancer. SMARCA4 is a key member of the SWI/SNF ATPase‐dependent chromatin remodelling complex, but the details of the relationship between SMARCA4 deficiency and cancer development remain unclear. SMARCA4 deficiency is observed in about 5% of conventional non‐small cell lung cancers, which should be distinguished by epithelial architecture, diffuse strong keratin expression and ancillary marker including SMARCA2.The tumours genetically have inactivating mutations in Owing to the aggressive behaviour of these tumours, most cases present at an inoperable stage with symptoms, and are associated with a short survival time.SMARCA4‐UT is generally insensitive to cytotoxic chemotherapy; therefore, in this case, we did not administer adjuvant chemotherapy. Although three recent cases of unresectable SMARCA4‐UTs receiving combined immunotherapy, including atezolizumab, showed a strong effect ,Takafumi Iguchi, Kyukwang Chung and Ryu Nakajima were involved in the conception or design of the work, and the acquisition, analysis or interpretation of data for the work. Shinjiro Mizuguchi drafted the work and revised it critically for important intellectual content. Makoto Takahama was involved in the final approval of the version to be published.None declared.The authors declare that appropriate written informed consent was obtained for the publication of this manuscript and accompanying images.