Generalized prurigo nodularis with dramatic response to dupilumab treatment: A case report

BACKGROUND Prurigo nodularis(PN)is a chronic condition characterized by a papulonodular pruriginous eruption of unknown aetiology.Currently,there are no medications for PN that the United States Food and Drug Administration has approved,which leads to very variable practices in the prescription of off-label treatments.Treatment of PN is based on clinical experience rather than controlled trials.We present our case of generalized PN,in which we had a dramatic response with dupilumab.CASE SUMMARY A 58-year-old female patient was admitted to our clinic with severe itchy,erythematous nodular lesions that were widespread all over her body,especially on the legs and back.It was learned that the patient's complaints started 4 years ago,and there was a significant increase in the lesions in the last period.Dermatological examination revealed diffuse firm erythematous excoriated nodular lesions all over the body.In the blood tests of the patient,serum Immunoglobulin E(IgE)was measured at 9330 IU/mL.The patient was diagnosed with generalized prurigo nodularis together with clinical and histopathological findings.Due to severe clinical findings and the presence of comorbidities,dupilumab treatment was planned for the patient.In the follow-up 4 mo later,it was observed that all nodular lesions healed with postinflammatory hypopigmentation.The IgE value decreased to 1500 IU/mL after 4 mo of dupilumab treatment.CONCLUSION Dupilumab treatment stands out as an effective and safe systemic treatment agent among existing systemic treatments.

Primary pulmonary meningioma and minute pulmonary meningothelial-like nodules: Rare pulmonary nodular lesions requiring more awareness in clinical practice

In this editorial,we comment on an article by Ruan et al published in a recent issue of the World Journal of Clinical Case.Pulmonary meningothelial proliferative lesions,including primary pulmonary meningiomas,minute pulmonary meningothelial-like nodules,and metastatic pulmonary meningiomas are rare pulmonary lesions.These lesions are difficult to differentiate from lung cancers based on clinical and imaging manifestations.Herein,we briefly introduce the clinical,imaging,and pathological characteristics of these lesions and discuss their pathogenesis to strengthen the current understanding of pulmonary meningothelial proliferative lesions in clinical diagnosis and therapy.

Development of preoperative prognostic models including radiological features for survival of singular nodular HCC patients

Background:Early singular nodular hepatocellular carcinoma(HCC)is an ideal surgical indication in clinical practice.However,almost half of the patients have tumor recurrence,and there is no reliable prognostic prediction tool.Besides,it is unclear whether preoperative neoadjuvant therapy is necessary for patients with early singular nodular HCC and which patient needs it.It is critical to identify the patients with high risk of recurrence and to treat these patients preoperatively with neoadjuvant therapy and thus,to improve the outcomes of these patients.The present study aimed to develop two prognostic models to preoperatively predict the recurrence-free survival(RFS)and overall survival(OS)in patients with singular nodular HCC by integrating the clinical data and radiological features.Methods:We retrospective recruited 211 patients with singular nodular HCC from December 2009 to January 2019 at Eastern Hepatobiliary Surgery Hospital(EHBH).They all met the surgical indications and underwent radical resection.We randomly divided the patients into the training cohort(n=132)and the validation cohort(n=79).We established and validated multivariate Cox proportional hazard models by the preoperative clinicopathologic factors and radiological features for association with RFS and OS.By analyzing the receiver operating characteristic(ROC)curve,the discrimination accuracy of the models was compared with that of the traditional predictive models.Results:Our RFS model was based on HBV-DNA score,cirrhosis,tumor diameter and tumor capsule in imaging.RFS nomogram had fine calibration and discrimination capabilities,with a C-index of 0.74(95%CI:0.68-0.80).The OS nomogram,based on cirrhosis,tumor diameter and tumor capsule in imaging,had fine calibration and discrimination capabilities,with a C-index of 0.81(95%CI:0.74-0.87).The area under the receiver operating characteristic curve(AUC)of our model was larger than that of traditional liver cancer staging system,Korea model and Nomograms in Hepatectomy Patients with Hepatitis B VirusRelated Hepatocellular Carcinoma,indicating better discrimination capability.According to the models,we fitted the linear prediction equations.These results were validated in the validation cohort.Conclusions:Compared with previous radiography model,the new-developed predictive model was concise and applicable to predict the postoperative survival of patients with singular nodular HCC.Our models may preoperatively identify patients with high risk of recurrence.These patients may benefit from neoadjuvant therapy which may improve the patients’outcomes.

Advances in the Intervention of Prunella Spica Capsules on Postoperative Recurrence of Nodular Goiter

As important drugs for the treatment of nodular goiter(NG),Prunella Spica preparations are widely used clinically,and have a significant effect on NG.Various active ingredients in the preparations intervene in the formation of NG by inhibiting the proliferation of thyroid follicular cells,promoting cell apoptosis,regulating immunity,improving the microcirculation of thyroid tissue and other mechanisms,and can reduce the postoperative recurrence of NG.

Papillary thyroid carcinoma with nodular fasciitis-like stroma-an unusual variant with distinctive histopathology:A case report

BACKGROUND Papillary thyroid carcinoma(PTC)is regarded as a fairly common endocrine malignancy,which can be divided into different multiple variants due to wide morphologic differences.The majority of PTC variants have been reported,but PTC with nodular fasciitis-like stroma(NFS)is a rare pathological variant and has been infrequently reported in the relevant literature.This condition involves abundant reactive stromal components rich in spindle cells,which may account for 60%-80%of the tumor along with a typical papillary carcinoma.CASE SUMMARY A 44-year-old man presented with a 4-mo history of a palpable mass over the anterior aspect of the left neck,the tumor demonstrated gradual enlargement but was painless during the 4 mo prior to discovery.Thyroid function test results were normal.Physical examination showed an enormous and firm nodular mass in the left lobe of the thyroid gland extending to the level of the hyoid bone.Ultrasonography of the neck revealed a well-defined heterogeneous lesion measuring around 5.0 cm×4.0 cm with a hypoechoic complex nodule,decreased vascularity and speckles of microcalcification.The patient underwent left thyroidectomy with central compartment lymph node dissection.Final histopathological examination confirmed the diagnosis of PTC with extensive fibromatosis-like stroma combined with typical PTC.The patient was asymptomatic at the 3-mo follow-up.CONCLUSION PTC-NFS is a rare pathological variant and its diagnosis and prognosis may be similar to typical papillary carcinoma.

Prof. Gui-rong DONG's experience in treatment of prurigo nodularis with acupuncture

The patient with prurigo nodularis were treated by needling at Bǎihuì（百会 GV 20）, Fēngchi（风池 GB 20）,Fēngfǔ（风府 GV 16）, Dàzhuī（大椎 GV 14）,Fēngmén（风门 BL 12）, Xīnshù（心俞 BL 15）, Gānshù（肝俞 BL18）, Qìhǎi（气海 CV 6）,Guānyuán（关元 CV 4）, Zhōngwǎn（中脘 CV12）, Xuèhǎi（血海 SP 10）, Zúsānlǐ（足三里ST 36）, Sānyīnjiāo（三阴交 SP 6）, Nèiting（内庭 ST 44）,Qūchí（曲池 LI 11）, Wàiguān（外关 TE 5） and Hégǔ（合谷 LI 4）. The treatment was given 4 times a week. After 2 weeks of treatment, the subjective pruritus was alleviated and tolerable. The patients could fall into sleep. After 2 months of treatment, no new skin rashes appear and pruritus disappeared basically, the old rashes started fading. The red color was getting pale and the bumpy rashes were absorbed. After 5 months of treatment, the itching symptoms were gone and skin rashes disappeared basically. The lesion skin was pigmented in dark grey. After 6 months of treatment, the dark grey pigmentation disappeared and skin was normal in color. The disease was not recurred in 1 year of follow-up.

Large focal nodular hyperplasia is unresponsive to arterial embolization: A case report

BACKGROUND Focal nodular hyperplasia(FNH)commonly occurs in women;it is usually asymptomatic and sometimes difficult to differentiate from hepatocellular carcinoma(HCC).CASE SUMMARY A large space-occupying lesion in the right lobe of the liver was incidentally detected in an adult man and diagnosed as HCC.Transcatheter arterial chemoembolization was applied once monthly for 2 years,but the lesion did not decrease in size.It was revealed by biopsy to be FNH.Eleven years later,the patient underwent liver resection due to hemorrhage and the pathological examination confirmed FNH.CONCLUSION For a space-occupying lesion,it is prerequisite to pathologically confirm the diagnosis and the corresponding intervention can be effective.

Focal nodular hyperplasia of the liver in 86 patients

BACKGROUND: Focal nodular hyperplasia (FNH), the second most common benign hepatic tumor after hemangioma, is characterized by a stellate central scar and hyperplastic nodules. Although some large FNH may be associated with significant symptoms, more frequently they are discovered incidentally on physical examination or the work-up of unrelated symptoms. Since its nature and pathogenesis are still controversial, accurate diagnosis of FNH based on clinical presentation and radiographic studies is difficult. The purpose of this study was to explore the diagnosis and treatment of FNH. METHODS: Eighty-six FNH patients confirmed pathologically were treated at the Liver Cancer Institute in our hospital from 1996 to 2006. Their clinical manifestions, imaging presentation, pathological findings, and surgical results were analyzed retrospectively. RESULTS: Of the 86 patients with 99 foci, 54 were male and 32 female, with a mean age of 37 years. Eighty patients had a single solitary focus and 6 had multiple foci. Tumor diameter was less than 5 cm in 69 patients, 5-10 cm in 15, and more than 10 cm in 2. The overall rate of correct preoperative diagnosis was 59.3% (51/86) including 32.9% (26/79) by color Doppler flow imaging (CDFI), 60.3% (35/58) by CT, and 77.4% (24/31) by MRI. All the 86 patients underwent resection with good curative effect. CONCLUSIONS: CT and MRI are important diagnostic methods for FNH but it is difficult to make a definite preoperative diagnosis for partial classical and all non-classical FNH patients. We suggest that patients with clinical symptoms or with indefinite diagnosis should accept surgical removal.

Ultrasonography of sclerosing angiomatoid nodular transformation in the spleen

We report three rare cases of sclerosing angiomatoid nodular transformation(SANT) in the spleen.We compared the conventional and contrast-enhanced ultrasonographic appearance.The conventional sonographic examinations exhibited solitary lesions without common respects,while contrast-enhanced ultrasonography(CEUS) revealed nodular appearance mimicking its pathologic characteristics.It suggests that CEUS can provide morphologic information for diagnosing SANT.

Effect of fatty liver background on contrast-enhanced ultrasonographic appearance of focal nodular hyperplasia

BACKGROUND: Focal nodular hyperplasia (FNH) is increasingly diagnosed as a result of the advances in imaging studies such as contrast-enhanced ultrasonography (CEUS), enhanced computed tomography and magnetic resonance imaging. However, FNH with atypical features can be difficult to differentiate from other benign and malignant tumors. The aim of this study was to investigate the influence of fatty liver background on the CEUS characteristics of FNH. METHODS: Twenty-six patients with FNH were divided into two groups: group A included 14 patients with fatty liver and group B included 12 patients with normal liver background. Conventional two-dimensional ultrasonography and color Doppler flow imaging (CDFI) were conducted and followed by real-time dual-frame CEUS. RESULTS: On two-dimensional ultrasonography, hypoechoic nodules were present in most of the patients in group A (12/14) and hyperechoic nodules in most of those in group B (7/12). The difference in the nodule echotextures between the two groups was statistically significant (P<0.05). Nodules with centrifugal blood flow signals on CDFI were found in 6 of the 14 patients in group A and 5 of the 12 in group B (P>0.05). On CEUS, nodules with a central spoked-wheel-like enhancement pattern in the early arterial phase were observed in 8 patients in group A and those with an eccentric enhancement pattern in the remaining 6 patients. In this group, 3 patients had hypoechoic nodules in the delayed phase. Eleven of the 14 patients in this group were diagnosed accurately with CEUS. In group B, nodules with a rapid central spoked-wheel-like enhancement pattern in the early arterial phase were found in 8 patients by CEUS and those with rapid an eccentric enhancement pattern in 4. The nodules were found to be continuously enhanced in the delayed phase. All of the patients in group B were accurately diagnosed with CEUS. CONCLUSIONS: A FNH nodule on a background of fatty liver may present a hypoechoic pattern on two-dimensional ultrasonography and a hypoechoic wash-out pattern in the delayed phase on CEUS. At this time, punch biopsy is needed for the diagnosis or differential diagnosis of FNH.

Focal nodular hyperplasia of the liver: pathological analysis of 11 cases

BACKGROUND: Focal nodular hyperplasia (FNH) is a benign tumor-like lesion of the liver, predominantly affect- ing women. Its etiology is obscure and its pathogenesis is poorly understood. FNH should be differentiated from oth- er benign and malignant hepatic lesions. The aim of this study was to explore the pathological characteristics of FNH of the liver. METHODS: Eleven patients with FNH were studied retro- spectively by using hematoxylin and eosin, immunohisto- chemical and histochemical staining. RESULTS: In 8 female and 3 male FNH patients aged 19 to 54 years (mean 32), most of lesions showed central scars macroscopically. Microscopically 8 patients were found of classical type, 2 were of telangiectic type, and 1 was of mixed type. CONCLUSION: FNH is an uncommon benign hyperplastic lesion of the liver. It should be differentiated from hepato- cellular adenoma, alpha-fetoprotein negative hepatocellular carcinoma, and fibrolamellar carcinoma.

A case of hepatocellular carcinoma arising within large focal nodular hyperplasia with review of the literature

Focal nodular hyperplasia （FNH） is a relatively rare benign hepatic tumor, usually presenting as a solitary lesion; however, multiple Iocalizations have also been described. The association of FNH with other hepatic lesions, such as adenomas and haemangiomas has been reported by various authors. We herein report a case of a hepatocellular carcinoma arising within a large focal nodular hyperplasia, in a young female patient.

Hepatic focal nodular hyperplasia in children:Imaging features on multi-slice computed tomography

AIM:To retrospectively analyze the imaging features of hepatic focal nodular hyperplasia(FNH) in children on dynamic contrast-enhanced multi-slice computed tomography(MSCT) and computed tomography angiography(CTA) images.METHODS:From September 1999 to April 2012,a total of 218 cases of hepatic FNH were confirmed by either surgical resection or biopsy in the Sun Yat-sen Memorial Hospital of Sun Yat-sen University and the Cancer center of Sun Yat-sen University,including 12 cases(5.5%) of FNH in children(age ≤ 18 years old).All the 12 pediatric patients underwent MSCT.We retrospectively analyzed the imaging features of FNH lesions,including the number,location,size,margin,density of FNH demonstrated on pre-contrast and contrastenhanced computed tomography(CT) scanning,central scar,fibrous septa,pseudocapsule,the morphology of the feeding arteries and the presence of draining vessels(portal vein or hepatic vein).RESULTS:All the 12 pediatric cases of FNH had solitary lesion.The maximum diameter of the lesions was 4.0-12.9 cm,with an average diameter of 5.5 ± 2.5 cm.The majority of the FNH lesions(10/12,83.3%) had well-defined margins.Central scar(10/12,83.3%) and fibrous septa(11/12,91.7%) were commonly found in children with FNH.Central scar was either isodense(n = 7) or hypodense(n = 3) on pre-contrast CT images and showed progressive enhancement in 8 cases in the equilibrium phase.Fibrous septa were linear hypodense areas in the arterial phase and isodense in the portal and equilibrium phases.Pseudocapsule was very rare(1/12,8.3%) in pediatric FNH.With the exception of central scars and fibrous septa within the lesions,all 12 cases of pediatric FNH were homogenously enhanced on the contrast-enhanced CT images,significantly hyperdense in the arterial phase(12/12,100.0%),and isodense in the portal venous phase(7/12,58.3%) and equilibrium phase(11/12,91.7%).Central feeding arteries inside the tumors were observed on CTA images for all 12 cases of FNH,whereas no neovascularization of malignant tumors was noted.In 9 cases(75.0%),there was a spoke-wheel shaped centrifugal blood supply inside the tumors.The draining hepatic vein was detected in 8 cases of pediatric FNH.However,the draining vessels in the other 4 cases could not be detected.No associated hepatic adenoma or hemangioma was observed in the livers of the 12 pediatric cases.CONCLUSION:The characteristic imaging appearances of MSCT and CTA may reflect the pathological and hemodynamic features of pediatric FNH.Dynamic multi-phase MSCT and CTA imaging is an effective method for diagnosing FNH in children.

Complete spontaneous regression of giant focal nodular hyperplasia of the liver: Magnetic resonance imaging evaluation with hepatobiliary contrast media

Focal nodular hyperplasia(FNH) of the liver is a benign lesion occurring in 0.6%-3% of the general population that probably reflects a local hyperplastic response of hepatocytes to a vascular abnormality. Most lesions are diagnosed incidentally and the natural history of the disease remains largely unknown. It has been shown that most FNH remain stable, or even regress, over a long follow-up period. We present a patient with FNH of the liver who was followed up for 7 years. A 26-yearold woman with a 5-year history of oral contraceptive use was referred to our hospital in February 2005 for further examination of a liver tumour. The diagnosis of FNH was made using magnetic resonance(MR) imaging with hepatospecific contrast media; this technique allows a correct diagnosis, in particular distinguishing FNH from hepatic adenoma, avoiding an invasive procedure such as the lesion biopsy. After 7-year from the diagnosis, we observed the complete spontaneous regression of the lesion by enhanced MR scanning. In this patient, discontinuation of oral contraceptive use and two childbirths may have influenced the natural history of FNH. To our knowledge, in the English literature there is no report illustrating a complete regression of giant FNH but only studies of decreasing in size. The present case suggests that a young patient with giant FNH should be managed conservatively rather than by resection, because FNH has the potential for spontaneous regression.

Actinic prurigo of the lip: Two case reports

Actinic prurigo is a photodermatosis that can affect the skin, conjunctiva and lips. It is caused by an abnormal reaction to sunlight and is more common in high-altitude living people, mainly in indigenous descendants. The diagnosis of actinic prurigo can be challenging, mainly when lip lesions are the only manifestation, which is not a common clinical presentation. The aim of this article is to report two cases of actinic prurigo showing only lip lesions. The patients were Afro-American and were unaware of possible Indian ancestry. Clinical exam, photographs, videoroscopy examination and biopsy were performed, and the diagnosis of actinic prurigo was established. Topical corticosteroid and lip balm with ultraviolet protection were prescribed with excellent results. The relevance of this report is to show that although some patients may not demonstrate the classical clinical presentation of actinic prurigo, the associated clinical and histological exams are determinants for the correct diagnosis and successful treatment of this disease.

Sclerosing angiomatoid nodular transformation of the spleen, a rare cause for splenectomy: Two case reports

BACKGROUND Sclerosing angiomatoid nodular transformation(SANT)is a rare benign disease of the spleen with unknown origin.Clinical symptoms are inhomogeneous,and suspicious splenic lesion often found incidentally,leading to splenectomy,as malignancy cannot securely be ruled out.Diagnosis is made histologically after resection.CASE SUMMARY Two cases of German,white,non-smoking,and non-drinking patients of normal weight are presented.The first one is a 26-year-old man without medical history who was exhibiting an undesired weight loss of 10 kg and recurring vomiting for about 18 mo.The second one is a 65-year-old woman with hypertension who had previously undergone gynecological surgery,suffering from a lasting feeling of abdominal fullness.Both showed radiologically an inhomogeneous splenic lesion leading to splenectomy approximately 6 and 9 wk after surgical presentation.Both diagnoses of SANT were made histologically.Follow-up went well,and both were treated according to the recommendation for asplenic patients.CONCLUSION SANT is a rare cause of splenectomy and an incidental histological finding.Further research should focus on clinical and radiological diagnosis of SANT as well as on treatment of patients with asymptomatic and small findings.

Clonality and allelotype analyses of focal nodular hyperplasia compared with hepatocellular adenoma and carcinoma

AIM： To identify clonality and genetic alterations in focal nodular hyperplasia （FNH） and the nodules derived from it. METHODS： Twelve FNH lesions were examined. Twelve hepatocellular adenomas （HCAs） and 22 hepatocellular carcinomas （HCCs） were used as references. Nodules of different types were identified and isolated from FNH by microdissection. An X-chromosome inactivation assay was employed to describe their clonality status. Loss of heterozygosity （LOH） was detected, using 57 markers, for genetic alterations.RESULTS： Nodules of altered hepatocytes （NAH）, the putative precursors of HCA and HCC, were found in all the FNH lesions. Polyclonality was revealed in 10 FNH lesions from female patients, and LOH was not detected in any of the six FNH lesions examined, the results apparently showing their polyclonal nature. In contrast, monoclonality was demonstrated in all the eight HCAs and in four of the HCCs from females, and allelic imbalances were found in the HCAs （9/9） and HCCs （15/18）, with chromosomal arms 11p, 13q and 17p affected in the former, and 6q, 8p, 11p, 16q and 17p affected in the latter lesions in high frequencies （≥ 30%）. Monodonality was revealed in 21 （40%） of the 52 microdissected NAH, but was not found in any of the five ordinary nodules. LOH was found in all of the 13 NAH tested, being highly frequent at six loci on 8p, 11p, 13q and 17p. CONCLUSION： FNH, as a whole, is polyclonal, but some of the NAH lesions derived from it are already neoplastic and harbor similar allelic imbalances as HCAs.

Research on focal nodular hyperplasia with MSCT and postprocessing

AIM： To investigate and evaluate the pathological features and diagnostic value of focal nodular hyperplasia （FNH） with multiection spiral computed tomography （MSCT） and postprocessing. METHODS： A total of 25 patients with FNH who had undergone MSCT and postprocessing were included in the investigation. All patients had been pathologically or clinically confirmed with FNH. A number of 75 cases of hepatic carcinomas, hemangiomas and adenomas were randomly selected at a same period for a comparative study. RESULTS： There was a single focus in 22 cases and multiple foci in 3 cases. On the plain scan, 17 lesions showed hypodensity, 7 isodensity and 4 hyperdensity （the case with fatty liver）. With contrast, 28 lesions were enhanced evenly or in the nodules in the arterial phase; 13 lesions still showed hyperdensity, 11 lesions isodensity and 4 lesions hypodensity in the parenchymatous phase; in the delayed phase only 5 lesions showed hyperdensity but 9 lesions showed isodensity or slight hypodensity and 14 lesions showed hypodensity. Twelve lesions of 28 had central asteroid scars. Thickened feeding arteries in postprocessing were seen in 24 lesions, and were integrated into the parenchymatous lesions with a gradual and smooth course. On the contrary, there were no artery penetrated into the lesion found in any of comparative hepatic tumors. CONCLUSION： Doctors could make a correct diagnosis and differentiation of FNH on evaluation of the characteristic appearance on MSCT with postprocessing,

Sclerosing angiomatoid nodular transformation of the spleen: A case report and literature review

BACKGROUND Sclerosing angiomatoid nodular transformation(SANT)is a rare disease of the spleen.It has unique pathological features and mimics splenic tumor on radiological imaging.CASE SUMMARY A 47-year-old woman was incidentally found to have a splenic mass on abdominal ultrasound.She had a 10-cm postoperative scar in the lower abdomen due to previous cesarean sections.The patient had a past history of anemia of unknown etiology for 20 years.The patient underwent laparoscopic splenectomy.The postoperative course was uneventful,with a hospital stay of 7 d.The histopathological examination of the spleen revealed SANT.At the 6-mo followup,the patient remained disease-free.CONCLUSION SANT is a rare benign disease mimicking a malignant tumor.A definitive diagnosis can be made only on histopathology.

Regression of gastric endoscopic submucosal dissection induced polypoid nodular scar after Helicobacter pylori eradication:A case report

BACKGROUND Endoscopic submucosal dissection(ESD)is the treatment of choice for early gastric cancer and premalignant gastric dysplasia.In some cases,ESD induced ulcer heals as a polypoid nodular scar(PNS).These scars may make the physicians raise several clinical implications such as post-ESD neoplastic recurrence.CASE SUMMARY We described a case of gastric ESD induced PNS which is regressed after Helicobacter pylori(H.pylori)eradication.A 58-year-old male patient was referred to the outpatient clinic for evaluation and treatment of gastric low-grade dysplasia(LGD).ESD was performed.A PNS was developed at the ESD site.An endoscopic biopsy was done and there was no histological evidence of remnant tumor or recurrence but a hyperplastic mucosal change.The PNS showed increase in size in follow-up endoscopy,and the biopsy specimen demonstrated H.pylori infestation.H.pylori eradication was done and the PNS was regressed.CONCLUSION H.pylori eradication is considerable for the regression of PNS if H.pylori infesta tion is confirmed.