Sclerosing cholangitis secondary to bleomycin-iodinated embolization for liver hemangioma

Sclerosing cholangitis(SC)is a rarely reported morbidity secondary to transcatheter arterial chemoembolization(TACE)with bleomycin-iodinated oil(BIO)for liver cavernous hemangioma(LCH).This report retrospectively evaluated the diagnostic and therapeutic course of a patient with LDH who presented obstructive jaundice 6 years after TACE with BIO.Preoperative imaging identified a suspected malignant biliary stricture located at the convergence of the left and right hepatic ducts.Operative exploration demonstrated a full-thickness sclerosis of the hilar bile duct with right hepatic duct stricture and right lobe atrophy.Radical hepatic hilar resection with right-side hemihepatectomy and Roux-en-Y hepaticojejunostomy was performed because hilar cancer could not be excluded on frozen biopsy.Pathological results showed chronic pyogenic inflammation of the common and right hepatic ducts with SC in the portal area.Secondary SC is a long-term complication that may occur in LCH patients after TACE with BIO and must be differentiated from hilar malignancy.Hepatic duct plasty is a definitive but technically challenging treatment modality for secondary SC.

CT image morphology features of pulmonary sclerosing hemangiomas

Objective: The aim of the study was to analyze the CT morphology features of pulmonary sclerosing hemangiomas (PSHs) and improve the diagnosis ability of this disease.Methods: The 18 cases of pulmonary sclerosing hemangioma (PSH) confirmed by operation and histopathology from August 2002 to May 2009 were collected,including 17 females and 2 males,aged from 19 to 60 years old,with an average age of 43 years.All the cases underwent plain CT scan,among them,16 cases received enhanced CT scan.Results: The 18 cases had isolated mass.Mean long-axis diameter of these lesions was (2.7 ± 1.3) cm (range,1.9–4.2 cm).Of all cases,5 cases (27.8%) were round in shape,9 cases (50%) were oval,4 cases (22.2%) were lobulated,and 14 cases (77.8%) were smooth margin.The air meniscus sign was in 2 cases (11.1%),and the halo sign in 3 cases (16.7%).Two cases (11.1%) contained small nodular calcification,the remaining 16 cases (70%) were homogeneous density,the CT density of the masses ranged from 24–47 HU,and the mean value was 35 HU.Sixteen cases received enhanced scan,the welt vessel sign was in 8 cases (44.4%),1 case showed less enhancement,5 cases showed marked homogeneous enhancement and 10 cases showed intense and patchy heterogeneous enhanced.The CT density of the enhancing masses ranged from 60–110 HU,the mean value was 35 HU,and the net enhancement value was 14–80 HU,the mean value was 55 HU.Conclusion: PSH should be considered in middle-aged female whose CT found that single round or oval pulmonary nodules,with smooth margin,or associated with the air meniscus sign,the halo sign,or the marked enhancement.

A study of histogenesis of pulmonary sclerosing hemangioma

Objective： To investigate the histogenesis and differential diagnosis of pulmonary sclerosing hemangioma （PSH）. Methods： Thyroid transcription factor-1 （TTF-1）, surfactant proteins A, B （SP-A, SP-B）, epithelial membrane antigen （EMA）, vimentin, pancytokeratin, cytokeratin7 （CK7）, CK5/6, calretinin, S-100, neurospecific enolase （NSE）, synaptophysin （Syn）, chromogranin A （CgA）, CD34, factor-Ⅷ-related antigen （F-Ⅷ） and smooth muscle actin （SMA）in 55 patients with PSH were examined with immunohistochemistry, while samples from 19 patients were also observed by electron microscope. Follow-up information were reviewed. Results： Pathologically, PSH mainly consisted of both surface lining cuboidal cells and pale polygonal cells. Immunohistochemitry revealed that coexpression of TTF-1, EMA and vimentin in both cuboidal and polygonal cells was 94.5 % （52/55）, 90.5% （50/55） and 58.2% （32/55）, respectively. In cuboidal cells, the expression of SP-A and SP-B was 92.7% （51/55） and 81.8 % （45/55）, respectively. Whereas pancytokeratin and CK7 immunostained the cuboidal cells in all cases. The polygonal cells were difussed immunostained with Syn in 13 PSHs （24%） and NSE in 8 PSHs （15%）, while immunoreactions with S-100 and CgA were separated detected in 7 PSHs （13%）. There was no significant difference of TTF-1 and EMA expression between these two cells （χ^2 value was 1.371 and 3.352, respectively. P 〉 0.05）. In contrast, there was a significant difference of vimentin between them （χ^2 value was 17.720, P 〈 0.001）. Electron microcopy observation showed that ultrastructure may not differ from each other sometime. The follow-up was obtained for 49 cases ranged from 3 months to 14 years. 48 of them had no recurrence or metastasis except one case had recurrence. Conclusion： PSH may origin from the primitive respiratory epithelial. Polygonal and cuboidal cells are all parenchymal neoplasm cells. The concomitant examination of TTF-1, SPA, SP-B, EMA, virnentin and pancytokeratin may favor the diagnosis and differential diagnosis of PSH.

MSC-derived exosomes attenuate hepatic fibrosis in primary sclerosing cholangitis through inhibition of Th17 differentiation

Primary sclerosing cholangitis(PSC)is an autoimmune cholangiopathy characterized by chronic inflammation of the biliary epithelium and periductal fibrosis,with no curative treatment available,and liver transplantation is inevitable for end-stage patients.Human placentalmesenchymal stem cell(hpMSC)-derived exosomes have demonstrated the ability to prevent fibrosis,inhibit collagen production and possess immunomodulatory properties in autoimmune liver disease.Here,we prepared hpMSC-derived exosomes(Exo^(MSC))and further investigated the anti-fibrotic effects and detailed mechanism on PSC based on Mdr2^(−/−)mice and multicellular organoids established from PSC patients.The results showed that Exo^(MSC) ameliorated liver fibrosis in Mdr2^(−/−)mice with significant collagen reduction in the preductal area where Th17 differentiation was inhibited as demonstrated by RNAseq analysis,and the percentage of CD4+IL-17A+T cells was reduced both in Exo^(MSC)-treated Mdr2^(−/−)mice(Mdr2^(−/−)-Exo)in vivo and Exo^(MSC)-treated Th17 differentiation progressed in vitro.Furthermore,Exo^(MSC) improved the hypersecretory phenotype and intercellular interactions in the hepatic Th17 microenvironment by regulating PERK/CHOP signaling as supported by multicellular organoids.Thus,our data demonstrate the antifibrosis effect of Exo^(MSC) in PSC disease by inhibiting Th17 differentiation,and ameliorating the Th17-induced microenvironment,indicating the promising potential therapeutic role of Exo^(MSC) in liver fibrosis of PSC or Th17-related diseases.

Renal anastomosing hemangioma following partial nephrectomy for renal cell carcinoma:A case report

BACKGROUND Renal anastomosing hemangioma(AH)is a rare benign vascular tumor characterized by unique histopathological features.CASE SUMMARY We report a highly unusual case of renal AH.A male patient had undergone partial nephrectomy for clear cell carcinoma of the kidney four years prior.A follow-up computed tomography scan in the third postoperative year revealed a new mass near the surgical site on the same side of the kidney,raising suspicions of tumor recurrence.However,the characteristics on contrast-enhanced magnetic resonance imaging and ultrasonography were more consistent with those of a benign lesion.The patient strongly insisted on undergoing surgery due to concerns about the possibility of renal cancer recurrence.Postoperative pathology confirmed the diagnosis of renal AH.CONCLUSION This case report presents the imaging features of a patient with rare renal AH and a history of renal clear cell carcinoma,providing broader insights into the differential diagnosis of new lesions after surgery for renal cell carcinoma.

Autoimmune hepatitis and primary sclerosing cholangitis after direct-acting antiviral treatment for hepatitis C virus:A case report

BACKGROUND Chronic hepatitis C virus(HCV)infection is a major global health concern that leads to liver fibrosis,cirrhosis,and cancer.Regimens containing direct-acting antivirals(DAAs)have become the mainstay of HCV treatment,achieving a high sustained virological response(SVR)with minimal adverse events.CASE SUMMARY A 74-year-old woman with chronic HCV infection was treated with the DAAs ledipasvir,and sofosbuvir for 12 wk and achieved SVR.Twenty-four weeks after treatment completion,the liver enzyme and serum IgG levels increased,and antinuclear antibody became positive without HCV viremia,suggesting the development of autoimmune hepatitis(AIH).After liver biopsy indicated AIH,a definite AIH diagnosis was made and prednisolone was initiated.The treatment was effective,and the liver enzyme and serum IgG levels normalized.However,multiple strictures of the intrahepatic and extrahepatic bile ducts with dilatation of the peripheral bile ducts appeared on magnetic resonance cholangiopancreatography after 3 years of achieving SVR,which were consistent with primary sclerosing cholangitis.CONCLUSION The potential risk of developing autoimmune liver diseases after DAA treatment should be considered.

Misdiagnosis of hemangioma of left triangular ligament of the liver as gastric submucosal stromal tumor:Two case reports

BACKGROUND Extragastric lesions are typically not misdiagnosed as gastric submucosal tumor(SMT).However,we encountered two rare cases where extrinsic lesions were misdiagnosed as gastric SMTs.CASE SUMMARY We describe two cases of gastric SMT-like protrusions initially misdiagnosed as gastric SMTs by the abdominal contrast-enhanced computed tomography(CT)and endoscopic ultrasound(EUS).Based on the CT and EUS findings,the patients underwent gastroscopy;however,no tumor was identified after incising the gastric wall.Subsequent surgical exploration revealed no gastric lesions in both patients,but a mass was found in the left triangular ligament of the liver.The patients underwent laparoscopic tumor resection,and the postoperative diagnosis was hepatic hemangiomas.CONCLUSION During EUS procedures,scanning across different layers and at varying degrees of gastric cavity distension,coupled with meticulous image analysis,has the potential to mitigate the likelihood of such misdiagnoses.

Portal Venous Thrombosis and Splenic Hemangioma, Secondary to Acute Pancreatitis: Case Report

We present an unusual case of portal vein thrombosis with a splanchnic hemangioma secondary to acute biliary pancreatitis. We report a 45-year-old patient, who has systemic arterial hypertension in treatment, was admitted for abdominal pain in the epigastrium, with irradiation to the right hypochondrium, accompanied by nausea and vomiting of 10 occasions of bile content, physical examination with pain in the right hypochondrium, Murphy positive. We have laboratory studies with a lipase of 788, so a diagnosis of pancreatitis is made with an etiology to be determined. The laboratories suggestive of acute biliary pancreatitis (lipase 788.71);an imaging study was subsequently performed (ultrasonography) with the result of stone in the common bile duct. A laparoscopy was performed with relative improvement, so he was discharged and returned 20 days after surgery due to abdominal pain of the same intensity in the left hypochondrium. Ending his hospitalization with a splenectomy for splenic hemangioma with portal vein thrombosis.

Bilateral circumscribed choroidal hemangioma:a case report

Dear Editor,Bilateral circumscribed choroidal hemangioma(CCH)is mostly associated with Sturge-Weber syndrome and rarely occurs in healthy individuals.So,we present the case about bilateral CCH that occurred in a healthy person and to evaluate the efficacy of 0.05 mL of anti-vascular endothelial growth factor(anti-VEGF)medication and laser photocoagulation in the treatment of CCH.We obtained the written informed consent from the patient,and this case study was in accordance with the tenets of the Declaration of Helsinki.

A rare complication of measles infection presented with subacute sclerosing panencephalitis: Report of two cases in India

Rationale:Subacute sclerosing panencephalitis(SSPE)is a progressive neurological disorder caused by persistent measles virus infection.SSPE predominantly affects children and adolescents.The symptoms usually develop 6-15 years after measles infection and ultimately leading to death in many cases.Patient concerns:Patient 1 presented with cognitive decline and myoclonus and the Patient 2 presented with diminution of vision with myoclonic jerks.Diagnosis:Based on the clinical features with a characteristic electroencephalogram pattern and the presence of a high titer of anti-measles IgG in serum and cerebrospinal fluid,these patients were diagnosed as SSPE.Interventions:Antiepileptics were started for controlling myoclonus along with supportive treatment.Outcomes:Both patients were discharged on antiepileptics and supportive care.Lessons:Whenever there are unusual clinical manifestations with unknown vaccination status,SSPE can be suspected and the cerebrospinal fluid should be examined for anti-measles antibodies.Our case study also highlights the importance of universal coverage of measles vaccination.To reduce the incidence of measles and associated deaths,it is important to maintain a high level of immunization coverage for the measles vaccine and to strengthen all the integral components of the national immunization program.

Sclerosing Mucoepidermoid Cancer: A Unique Entity

Sclerosing mucoepidermoid thyroid cancer (SMECE) is a rare entity with less than 100 cases reported in the literature. Previously considered to have an indolent course, however, recent evidence has reported an aggressive nature ranging from local invasion to distant metastasis. We present a 66-year-old Caucasian female with SMECE who initially presented neck compressive symptoms. A thyroid ultrasound (US) revealed a solid hypoechoic mass replacing the left thyroid lobe. Fine needle aspiration cytology (FNAC) of the nodule resulted in suspicion of Papillary Thyroid Cancer, Bethesda category 5. The patient underwent total thyroidectomy and surgical pathology showed SMECE. Post-therapy whole-body scan following treatment with 150 mCi I-131 showed no residual or metastatic disease. SMECE is more common in females, between the third to eighth decade of life. Preoperative diagnosis may not be accurate given variable cytopathologic features. Differential diagnoses include primary squamous cell carcinoma of the thyroid, squamous differentiation of other thyroid malignancies, anaplastic thyroid cancer and nodular sclerosing variety of Hodgkin’s lymphoma. Due to its rarity, treatment of SMECE has ranged from thyroid surgery without or with radioactive iodine therapy, to surgery and external beam radiation and even chemotherapy.

One Case of Right Posterior Mediastinum Intraneural Hemangioma Misdiagnosed as Neurilemmoma

Although rare, intraneural hemangiomas should be considered in the differential diagnosis of peripheral nerve lesions. We report on a 59-year-old female patient, who was admitted to the hospital due to the discovery of bilateral breast masses for 3 months, there was no paresthesia or dyskinesia. The patient accidentally found a mass in the right upper mediastinum while completing a plain chest X-ray, initially suspected as a benign neurilemmoma on CT. Surgical resection and pathological analysis confirmed an intraneural hemangioma. Unexpectedly, the patient developed new-onset right upper limb numbness and paresthesia 3 months post-operatively, probably related to surgical nerve injury. This case underscores the importance of maintaining a broad differential for mediastinal masses, and the potential for iatrogenic neurological complications when managing these rare, yet vascular lesions.

Clinical characteristics of renal anastomotic hemangioma

In this editorial,we comment on the article by Chen and Cai.We focus on renal anastomotic hemangioma,which is a rare benign hemangiomatous disease.This disease has unique clinical characteristics.Its biological behavior is benign,but its imaging results are similar to those of renal cancer.Renal anastomotic hemangioma is easy to misdiagnose and can lead to unnecessary radical nephrectomy.Therefore,urologists need a better understanding of this disease.We believe that patients with renal anastomotic hemangioma should receive individualized diagnosis and treatment to avoid overtreatment.

Reconsideration of the clinical management of hepatic hemangioma

In this letter,we comment on the article by Zhou et al that was published in the recent issue of the World Journal of Gastrointestinal Surgery.This article proposes a new clinical grading system based on a multidisciplinary team,which prompts us to rethink the clinical management of hepatic hemangioma.Hepatic hemangioma is the most common benign solid liver tumor.In general,follow-up and obser-vation for the vast majority of hepatic hemangioma is reasonable.For those pa-tients with symptoms and severe complications,surgical intervention is ne-cessary.Specific surgical indications,however,are still not clear.An effective grading system is helpful in further guiding the clinical management of hepatic hemangioma.In this article,we review the recent literature,summarize the sur-gical indications and treatment of hepatic hemangioma,and evaluate the potential of this new clinical grading system.

Retrospective analysis based on a clinical grading system for patients with hepatic hemangioma:A single center experience

BACKGROUND The optimal approach for managing hepatic hemangioma is controversial.AIM To evaluate a clinical grading system for management of hepatic hemangioma based on our 17-year of single institution experience.METHODS A clinical grading system was retrospectively applied to 1171 patients with hepatic hemangioma from January 2002 to December 2018.Patients were classified into four groups based on the clinical grading system and treatment:(1)Observation group with score<4(Obs score<4);(2)Surgical group with score<4(Sur score<4);(3)Observation group with score≥4(Obs score≥4);and(4)Surgical group with score≥4(Sur score≥4).The clinico-pathological index and outcomes were evaluated.RESULTS There were significantly fewer symptomatic patients in surgical groups(Sur score≥4 vs Obs score≥4,P<0.001;Sur score<4 vs Obs score<4,χ^(2)=8.60,P=0.004;Sur score≥4 vs Obs score<4,P<0.001).The patients in Sur score≥4 had a lower rate of in need for intervention and total patients with adverse event than in Obs score≥4(P<0.001;P<0.001).Nevertheless,there was no significant difference in need for intervention and total patients with adverse event between the Sur score<4 and Obs score<4(P>0.05;χ^(2)=1.68,P>0.05).CONCLUSION This clinical grading system appeared as a practical tool for hepatic hemangioma.Surgery can be suggested for patients with a score≥4.For those with<4,follow-up should be proposed.

Endoscopic ultrasound-guided lauromacrogol injection for treatment of colorectal cavernous hemangioma:Two case reports

BACKGROUND Colorectal cavernous hemangioma is a rare vascular malformation resulting in recurrent lower gastrointestinal hemorrhage,and can be misinterpreted as colitis.Surgical resection is currently the mainstay of treatment,with an emphasis on sphincter preservation.CASE SUMMARY We present details of two young patients with a history of persistent hematochezia diagnosed with colorectal cavernous hemangioma by endoscopic ultrasound(EUS).Cavernous hemangioma was relieved by several EUS-guided lauromacrogol injections and the patients achieved favorable clinical prognosis.CONCLUSION Multiple sequential EUS-guided injections of lauromacrogol is a safe,effective,cost-efficient,and minimally invasive alternative for colorectal cavernous hemangioma.

Recombinant adeno-associated virus 8-mediated inhibition of microRNA let-7a ameliorates sclerosing cholangitis in a clinically relevant mouse model

BACKGROUND Primary sclerosing cholangitis(PSC)is characterized by chronic inflammation and it predisposes to cholangiocarcinoma due to lack of effective treatment options.Recombinant adeno-associated virus(rAAV)provides a promising platform for gene therapy on such kinds of diseases.A microRNA(miRNA)let-7a has been reported to be associated with the progress of PSC but the potential therapeutic implication of inhibition of let-7a on PSC has not been evaluated.AIM To investigate the therapeutic effects of inhibition of a miRNA let-7a transferred by recombinant adeno-associated virus 8(rAAV8)on a xenobiotic-induced mouse model of sclerosing cholangitis.METHODS A xenobiotic-induced mouse model of sclerosing cholangitis was induced by 0.1% 3,5-Diethoxycarbonyl-1,4-Dihydrocollidine(DDC)feeding for 2 wk or 6 wk.A single dose of rAAV8-mediated anti-let-7a-5p sponges or scramble control was injected in vivo into mice onset of DDC feeding.Upon sacrifice,the liver and the serum were collected from each mouse.The hepatobiliary injuries,hepatic inflammation and fibrosis were evaluated.The targets of let-7a-5p and downstream molecule NF-κB were detected using Western blot.RESULTS rAAV8-mediated anti-let-7a-5p sponges can depress the expression of let-7a-5p in mice after DDC feeding for 2 wk or 6 wk.The reduced expression of let-7a-5p can alleviate hepato-biliary injuries indicated by serum markers,and prevent the proliferation of cholangiocytes and biliary fibrosis.Furthermore,inhibition of let-7a mediated by rAAV8 can increase the expression of potential target molecules such as suppressor of cytokine signaling 1 and Dectin1,which consequently inhibit of NF-κB-mediated hepatic inflammation.CONCLUSION Our study demonstrates that a rAAV8 vector designed for liver-specific inhibition of let-7a-5p can potently ameliorate symptoms in a xenobiotic-induced mouse model of sclerosing cholangitis,which provides a possible clinical translation of PSC of human.

Anorectal hemangioma, a rare cause of lower gastrointestinal bleeding, treated with selective embolization: A case report

BACKGROUND Anorectal hemangioma is a rare and frequently misdiagnosed cause of lower gastrointestinal(GI)bleeding.Here,we present a minimally invasive therapy with selective embolization.CASE SUMMARY A 21-year-old male patient experienced painless rectal bleeding since childhood and was treated for ulcerative colitis.Diagnostic studies later revealed specific characteristics for vascular lesions-anorectal hemangiomas.The severity of rectal bleeding caused symptomatic anemia and possible surgical treatment was asso-ciated with a high risk of fecal incontinence.Here,we present selective emboli-zation,a minimally invasive therapeutic approach that is proven as an alternative therapeutic method of choice.The patient significantly improved temporarily and had a small ischemic ulcer,which healed with a control colonoscopy and deve-loped no stenosis.CONCLUSION Awareness of the clinical and radiological features of GI hemangiomas may help improve diagnostics and avoid inappropriate therapeutic procedures.

Limited validity of Mayo endoscopic subscore in ulcerative colitis with concomitant primary sclerosing cholangitis

BACKGROUND Ulcerative colitis(UC)with concomitant primary sclerosing cholangitis(PSC)represents a distinct disease entity(PSC-UC).Mayo endoscopic subscore(MES)is a standard tool for assessing disease activity in UC but its relevance in PSC-UC remains unclear.AIM To assess the accuracy of MES in UC and PSC-UC patients,we performed histological scoring using Nancy histological index(NHI).METHODS MES was assessed in 30 PSC-UC and 29 UC adult patients during endoscopy.NHI and inflammation were evaluated in biopsies from the cecum,rectum,and terminal ileum.In addition,perinuclear anti-neutrophil cytoplasmic antibodies,fecal calprotectin,body mass index,and other relevant clinical characteristics were collected.RESULTS The median MES and NHI were similar for UC patients(MES grade 2 and NHI grade 2 in the rectum)but were different for PSC-UC patients(MES grade 0 and NHI grade 2 in the cecum).There was a correlation between MES and NHI for UC patients(Spearman's r=0.40,P=0.029)but not for PSC-UC patients.Histopathological examination revealed persistent microscopic inflammation in 88%of PSC-UC patients with MES grade 0(46%of all PSC-UC patients).Moreover,MES overestimated the severity of active inflammation in an additional 11%of PSCUC patients.CONCLUSION MES insufficiently identifies microscopic inflammation in PSC-UC.This indicates that histological evaluation should become a routine procedure of the diagnostic and grading system in both PSC-UC and PSC.

IgG4-related sclerosing cholangitis associated with essential thrombocythemia:A case report

BACKGROUND The complexity of immunoglobulin G4(IgG4)-related diseases and their potential connection to hematologic malignancies remains unclear.This article provided a review of the diagnosis and treatment of a patient with IgG4-related sclerosing cholangitis(SC)and essential thrombocythemia(ET),along with an analysis of relevant literature to enhance comprehension of this disease.CASE SUMMARY A 56-year-old male was admitted to two hospitals with deteriorating jaundice and pruritus prior to hospitalization.Beyond our expectations,the patient was first diagnosed with IgG4-SC and ET with the Janus kinase 2 V617F mutation.Interestingly,the administration of acetate prednisone significantly resulted in improvements in both IgG4-SC and ET.Clinicians need to pay attention to immune disorders and inflammation as they contribute to the development of various disease phenotypes.CONCLUSION When IgG4-SC is suspected without histopathological evidence,diagnostic therapy and long-term regular follow-up can lead to positive treatment outcomes.Clinicians should be mindful of the potential presence of concurrent hematologic diseases in patients with immune disorders.