Systemic lupus erythematosus with multicentric reticulohistiocytosis: A case report

BACKGROUND Multicentric reticulohistiocytosis(MRH)/systemic lupus erythematosus(SLE) overlap syndrome is an uncommon disease in the clinic and is diagnosed through characteristic clinical manifestations, histopathology, and immunopathology. Here, we report the case of a 30-year-old woman with SLE who developed MRH.CASE SUMMARY A 30-year-old woman with a history of polyarthritis for the past 12 years had multiple skin nodules on her body for 10 years, including the sacrococcygeal area, dorsum of the hands, interphalangeal joint of the feet and sternoclavicular joint. The histopathology of a biopsy of the distal interphalangeal joint of the hands revealed granulomatous inflammation, fibrous hyperplasia with ground-glass degeneration, inflammatory cell exudation and focal necrosis. The immunohistochemical stains showed positive staining for CD68 and negative staining for S100 and acid-fast staining. The patient was diagnosed with SLE with MRH. Her symptoms were improved after a combined treatment of prednisone, hydroxychloroquine and cyclophosphamide.CONCLUSION MRH/SLE overlap syndrome is difficult to diagnose and treat. Cyclophosphamide may be an alternative choice for the treatment of MRH.

Is breast conservative surgery a reasonable option in multifocal or multicentric tumors?

The incidence of multifocal(MF) and multicentric(MC) carcinomas varies widely among clinical studies,depending on definitions and methods for pathological sampling.Magnetic resonance imaging is increasingly used because it can help identify additional and conventionally occult tumors with high sensitivity.However,false positive lesions might incorrectly influence treatment decisions.Therefore,preoperative biopsies must be performed to avoid unnecessary surgery.Most studies have shown higher lymph node involvement rates in MF/MC tumors than in unifocal tumors.However,the rate of local recurrences is usually low after breast conservative treatment(BCT) of MC/MF tumors.It has been suggested that BCT is a reasonable option for MC/MF tumors in women aged 50-69 years,with small tumors and absence of extensive ductal carcinoma in situ.A metaanalysis showed an apparent decreased overall survival in MC/MF tumors but data are controversial.Surgery should achieve both acceptable cosmetic results and negative margins,which requires thorough preoperative radiological workup and localization of lesions.Boost radiotherapy techniques must be evaluated since double boosts might result in increased toxicity,namely fibrosis.In conclusion,BCT is feasible in selected patients with MC/MF but the choice of surgery must be discussed in a multidisciplinary team comprising at least radiologists,surgeons and radiotherapists.

Idiopathic multicentric Castleman disease with pulmonary and cutaneous lesions treated with tocilizumab:A case report

BACKGROUND Human herpes virus-8(HHV-8)-negative,idiopathic multicentric Castleman disease(iMCD)is a rare and life-threatening disorder driven by proinflammatory cytokines,which is still poorly understood.Pulmonary parenchyma lesion is a rare condition in iMCD,which mainly manifests as lymphocytic interstitial pneumonia and is an indicator of severe iMCD.Cutaneous lesion is also very rare and mainly occurs in Asians.There have been few reports of iMCD patients with both skin and lung parenchyma involvement.CASE SUMMARY We present a Chinese man who complained about a 3-year history of intermittent dry cough and a 2-year history of diffuse reddish-brown maculopapules.Laboratory examination revealed polyclonal hypergammaglobulinemia and hypercytokinemia including interleukin 6.Chest computed tomography revealed small patchy shadows with ground-glass nodules scattered in two lobes and mediastinal lymphadenopathy.The pathological result of the lymph node was consistent with the plasma cell type of Castleman disease.As serum human immunodeficiency virus test and HHV-8 staining of the lymph node were negative,the patient was finally diagnosed with HHV-8 negative i MCD.He was treated with tocilizumab at an intravenous(i.v.)dose of 8 mg/kg every 2 wk combined with methylprednisolone at an i.v.dose of 80 mg/d initially with gradual dose tapering.Partial remission was achieved 9 mo later.CONCLUSION i MCD with lung parenchyma and skin involvement is a rare condition that requires clinicians'attention and awareness for early diagnosis.

Multicentric Gliomas Misdiagnosed as Metastatic Tumors： One Case Report and Literature Review

Multicentric gliomas are considered to be well recognized but uncommon; often scatter widely in different lobes or hemispheres; and cannot be attributed to a definite pathwayEll. A patient diagnosed as multicentric gliomas is presented in this paper. He was firstly misdiagnosed as cerebral metastatic tumors, but later the histopathological examination revealed them to be glioblastoma （WHO grade IV）. Additionally, the aim of the paper was to describe the case history of the patient and the problems encountered in the pathogenesis, pathophysiology, diagnosis and treatment.

Multicentric occurrence of hepatocellular carcinoma with nonalcoholic steatohepatitis

AIM:To reveal the manner of hepatocellular carcinoma (HCC) development in patients with nonalcoholic steatohepatitis(NASH) focusing on multicentric occurrence (MO) of HCC.METHODS:We compared clinicopathological characteristics between patients with and without MO of HCC arising from NASH background.The clinical features were implicated with reference to the literature available.RESULTS:MO of HCC was identified with histological proof in 4 out of 12 patients with NASH-related HCC(2 males and 2 females).One patient had synchronous MO;an advanced HCC,two well-differentiated HCCs and a dysplastic nodule,followed by the development of metachronous MO of HCC.The other three patients had multiple advanced HCCs accompanied by a well-differentiated HCC or a dysplastic nodule.Of these three patients,one had synchronous MO,one had metachronous MO and the other had both synchronous and metachronous MO.There were no obvious differences between the patients with or without MO in terms of liver function tests,tumor markers and anatomical extent of HCC.On the other hand,all four patients with MO of HCC were older than 70 years old and had the comorbidities of obesity,type 2 diabetes mellitus(T2DM),hypertension and cirrhosis.Although these conditions were not limited to MO of HCC,all the conditions were met in only one of eight patients without MO of HCC.Thus,concurrence of these conditions may be a predisposing situation to synchronous MO of HCC.In particular,old age,T2DM and cirrhosis were suggested to be prerequisite for MO because these factors were depicted in common among two other cases with MO of HCC under NASH in the literature.CONCLUSION:The putative predisposing factors and necessary preconditions for synchronous MO of HCC in NASH were suggested in this study.Further investigations are required to clarify the accurate prevalence and predictors of MO to establish better strategies for treatment and prevention leading to the prognostic improvement in NASH.

Multicentric reticulohistiocytosis with prominent skin lesions and arthritis:A case report

BACKGROUND Multicentric reticulohistiocytosis(MRH)is a rare non-Langerhans histiocytosis of unknown etiology characterized by papulonodular skin lesions and progressive,erosive arthritis.To date,there have been approximately 300 cases of MRH reported worldwide.The majority of patients are Caucasian from western countries,and Asian patients are rare.Here,we report a case of MRH in a Chinese patient.CASE SUMMARY A 38-year-old male was admitted to the hospital with a rash that had persisted for over 2 years and bilateral knee pain for over 1 year.The patient’s symptoms had previously been misdiagnosed as eczema when there were only skin symptoms and was finally diagnosed as MRH after a skin biopsy of the left upper back.The patient was treated with glucocorticoids combined with an immunosuppressive regimen.While the skin lesions on both arms,abdomen,and back subsided,the skin lesions on the rest of the body did not increase.The interphalangeal joints of both thumbs and bilateral knee joints remained swollen and painful.CONCLUSION The case will help clinicians better identify and treat this disease in the absence of epidemiological studies or randomized controlled data.

DUCK HEPATITIS B VIRUS DNA WITHIN HEPATIC MULTICENTRIC CANCER AND/OR METASTATIC CANCER

Duck hepatitis B vims (DHBV) DNA was detected in different tumorous nodules of ducks with hepatic multicentric cancer or intrahepatic metastasis by Southern blot technique. Among 7 ducks with hepatocellular carcinoma of multiple tumor nodules, the hybridization pattern of Integrated DHBV DNA In different tumorous nodules was identical in 3 cases and different in 2 cases. One case showed a similar hybridization pattern in two tumorous nodules and other one was negative tor DHBV DNA. Integrated DHBV DNA was also identified in a metastatic lung cancer of ducks with hepatocellular carcinoma. The hybridization pattern of metastasis of lungs was as the some as that in primary hepatocellular carcinoma. The same discrete hybridization bands In the different tumorous nodules indicate that these nodules might arise from one transformed cell. The different hybridization patterns In various tumorous nodules show that these tumorous nodules might arise from various transformed cells. The results suggest that the hybridization pattern of different nodules of hepatocellular carcinoma with viral DNA probe could make a cell clone origin marker of tumor nodule to differentiate hepatic multlcentric cancer from Intrahepatic metastatic cancer.

I Do Biotech Dental Implants: Prospective Multicentric Study after 5 Years of Functional Loading

Introduction: I Do Biotech’s implants were developed starting in 2014. Since then, they obtained GMP and KFDA licenses for distribution in 2015. The main objective of this paper is to determine the survival rate of I Do Biotech implants five years after the first surgery. Material and Methods: 1000 implants were used on 480 prostheses across 10 clinics on 320 healthy, non-smoker and non-diabetic patients, chosen at random, of which 160 are male and 160 female, all in the age range of 30 to 50 years old. The failure rate was studied related to the patient’s gender, the length and diameter of the implant, anatomical location, the percentage of peri-implantitis, prosthodontic failures and the patient’s quality of life. Discussion: The results obtained are similar to those of Van Steenberghe D. Dieter-Busenlechner, E. Serrano Catauria and far superior to those of Sáenz Guzmán. Failure rates vary greatly from study to study due to the heterogeneity of the samples in the other research papers. Conclusion: The overall implant failure rate at 5 years is 1.7%. The factors affecting significantly the survival rate are: the implant diameter, its length and the anatomic area. Failure ratios increase significantly when the diameter or the length of the implant decreases, and when they are placed in the posterior maxilla (up to 4.3%). The rate of peri-implantitis is 5.1%. The prosthodontic failure rate is 2.91%. The improvement in quality of life and satisfaction increases with the years.

A case of Sjogren's syndrome evolving into multicentric reticulohistiocytosis

Introduction:Multicentric reticulohistiocytosis(MRH)is a rare disease that is known to affect the skin and joints,primarily.It is considered a rare form of non-Langerhans cell histiocytosis(Group C)that can cause destructive inflammatory arthritis involving both the small and large joints.Cutaneous eruptions of periungual,“coral beads”and nodules appearing over the distal fingers are considered pathognomonic clues for identifying this disorder.Histology evaluation of the cutaneous papules typically shows infiltrative histiocytes and multinucleated giant cells.Although no well-established therapies exist to date,a variety of immunosuppressants have been used with varying degrees of success.Case Description:A 53-year-old Caucasian female patient with a family history of rheumatoid arthritis and a personal history of Sjogren's syndrome presented to the rheumatology clinic complaining of pain in her bilateral hands and fingers.There were several small,papulo-nodular lesions ranging from 1 to 2mm in size noted at the base of her nails.A 4mm punch biopsy of one of the papules from the neck showed dermal infiltration of eosinophilic mononucleated and multinucleated giant cells with“ground glass”appearing cytoplasm consistent with MRH.X-ray of her hands showed periarticular demineralization and erosions surrounding several bilateral proximal and distal interphalangeal joints,and thus tofacitinib in addition to methotrexate,hydroxychloroquine,and dexamethasone(dosed weekly)was started to help control any further articular damage.Conclusion:Our aim is to further support the relation of MRH with autoimmune diseases,including Sjogren's syndrome.Autoimmune diseases have been reported in association with MRH,although a clear association has yet to be made.

Transanal total mesorectal excision for rectal cancer:a multicentric cohort study

Background:Transanal total mesorectal excision(taTME)has recently emerged as a promising novel surgical procedure for rectal cancer.It is believed to hold the potential advantage of providing better access to mobilize the distal rectum and achieving better pathologic results.This study aimed to evaluate the feasibility of taTME for rectal cancer and summarize the preliminary experience in 10 Chinese hospitals.Methods:A total of 211 patients were enrolled in this study.Variables for evaluation of safety,feasibility,and oncologic outcomes were retrospectively collected and analysed.Results:The median distance between the tumor and the anal verge was 5.9cm(range,1.5–12 cm).The median operating time was 280 min(range,70–600 min)and the median estimated intra-operative blood loss was 50mL(range,10–1,500 mL).The overall rate of complication was 27.9%.Among the 211 patients,175(82.9%)had complete TME and 33(15.6%)had near complete TME.The circumferential resection margin was negative in 97.7%of patients.The patients were followed for a median of 35months(range,2–86months).There was 7.6%(16)mortality,6.2%(13)had local recurrence,and 12.8%(27)had systemic recurrence.Kaplan–Meier survival analysis showed that 1-,2-,and 3-year disease-free survival rates were 94.8%,89.3%,and 80.2%,respectively,and 1-,2-,and 3-year OS rates were 97.4%,95.7%,and 92.9%,respectively.Conclusions:Although limited by its retrospective nature,taTME was safe and feasible in selected patients.Future work with rigorous data recording is warranted.

Evaluation of the GATIS score for predicting prognosis in rectal neuroendocrine neoplasms

The GATIS score,developed by Zeng et al,represents a significant advancement in predicting the prognosis of patients with rectal neuroendocrine neoplasms(RNENs).This study,which included 1408 patients from 17 major medical centres in China over 12 years,introduces a novel prognostic model based on the tumour grade,T stage,tumour size,age,and the prognostic nutritional index.Compared with traditional methods such as the World Health Organization classification and TNM staging systems,the GATIS score has superior predictive power for overall survival and progression-free survival.With a C-index of 0.915 in the training set and 0.812 in the external validation set,the GATIS score’s robustness and reliability are evident.The study’s use of a large,multi-centre cohort and rigorous validation processes underscore its significance.The GATIS score offers clinicians a powerful tool to accurately predict patient outcomes,guide treatment decisions,and improve follow-up strategies.This development represents a crucial step forwards in the management of R-NENs,addressing the complexity and variability of these tumours and setting a new benchmark for future research and clinical practice.

New strategy to distinguish clonal origin of RHCC/MHCC between intrahepatic metastasis and multicentric occurrence

Hepatocellular carcinoma (HCC) is a kind of malignancy with high potential of metastasis and multicentric occurrence. The treatment of recurrent hepatocellular carcinoma (RHCC) and multinodular hepatocellular carcinoma (MHCC) is always a nodus because of the diverse clonal origin of RHCC/MHCC. Theoretically, the RHCC/MHCC can originate from intrahepatic metastasis (IM type) or multicentric occurrence (MO type). Our previous study proposed that there are at least 6 subtypes of clonal origin patterns in RHCC. RHCC and MHCC with different clonal origins have variant biological behaviors, clinical prognosis as well as treatment strategy. Generally speaking, patients with IM type HCC have a poorer prognosis compared with those with MO type HCC. Therefore, it is essential to emphasize the distribution of the clonal origin in HCC in order to determine the choice of clinical treatment. Undoubtedly, the detection of clonal origin pattern will become a promising breakthrough in the molecular pathological diagnosis of HCC. We should attach more attention to the establishment of a standardized molecular pathological clonal origin detection method and a new stratification of clinical treatment choice for RHCC/MHCC in future.

Intertumoral heterogeneity of molecular phenotype and analysis of prognosis in multifocal and multicentric breast cancer

Objective This study was designed to investigate the prognostic implications of the intertumoral heterogeneity of molecular phenotype in multifocal and multicentric breast cancer(MMBC).Methods The clinical and follow-up data of 146 patients with MMBC from Jan.2009to Dec.2009 treated in Tumor Hospital Affiliated to Zhengzhou University were retrospectively analyzed.

Glucocorticoids combined with tofacitinib in the treatment of Castleman’s disease:A case report

BACKGROUND Castleman’s disease(CD),also known as vascular follicular lymphadenopathy is a rare proliferative disease of lymphoid tissue of unknown etiology that is clinically classified as unicentric CD(UCD) or multicentric CD(MCD) depending on lymph node involvement.At present,idiopathic MCD(iMCD) is treated with interleukin-6 inhibitors,but some patients have poor clinical outcomes.This paper reports on a case of iMCD that achieved a good therapeutic effect after treatment with glucocorticoids combined with tofacitinib.The relevant data are summarized and reported below.CASE SUMMARY This paper reports on a case of MCD in a 49-year-old female with persistent peritoneal effusion as the first manifestation and combined with multiple lymphadenopathies.Lymph node biopsy showed Castleman’s disease-like changes.The ascites subsided after treatment with glucocorticoids and tofacitinib,indicating that the treatment was effective.CONCLUSION The combination of glucocorticoids with tofacitinib is an effective regimen for the treatment of CD.

Strategy for improving survival and reducing recurrence of HCV-related hepatocellular carcinoma

Hepatocellular carcinoma(HCC)is the sixth most common cancer and the third leading cause of cancerrelated death in the world.With advances in imaging diagnostics,accompanied by better understanding of high-risk patients,HCC is now frequently detected at an early stage;however,the prognosis remains poor.The recurrence rate after treatment of HCC is higher than that associated with cancers of other organs.This may be because of the high incidence of intrahepatic distant recurrence and multicentric recurrence,especially with hepatitis C virus(HCV)-related hepatocellular carcinoma.The Barcelona Clinic Liver Cancer(BCLC)classification has recently emerged as the standard classification system for the clinical management of patients with HCC.According to the BCLC staging system,curative therapies(resection,transplantation,transcatheter arterial chemoembolization,percutaneous ethanol injection therapy,percutaneous microwave coagulation therapy and percutaneous radiofrequency ablation)can improve survival in HCC patients diagnosed at an early stage and offer a potential long-term cure.However,treatment strategies for recurrent disease are not mentioned in the BCLC classsification.The strategy for recurrence may differ according to the recurrence pattern,i.e.,intrahepatic distant recurrence vs multicentricrecurrence.In this article,we review recurrent HCC and the therapeutic strategies for reducing recurrent HCC,especially HCV-related HCC.

Unicentric Castleman disease was misdiagnosed as pancreatic mass:A case report

BACKGROUND Castleman’s disease(CD)is a lymphatic proliferative disorder of unknown cause and is rarely seen clinically.It has been divided into unicentric and multicentric types.Unicentric CD(UCD)occurs as a solitary enlarged mass and mediastinal lymph nodes are the most common site.Surgical excision has proven to be curative for UCD.Multicentric CD(MCD)appears as a systemic disease with peripheral lymphadenopathy.MCD had a poor response to surgery and monoclonal antibodies with rituximab have become a research hotspot.CASE SUMMARY A 44-year-old woman presented with a pancreatic mass during routine physical examination.She had no obvious symptoms,such as fever,abdominal pain,abdominal distension,or jaundice.Ultrasound examination indicated a hypoechoic mass between the body of the pancreas,left lobe of the liver and stomach.It had a clear boundary,irregular shape,uneven echo,and no obvious blood flow signals.To clarify the diagnosis,contrast-enhanced ultrasound examination was performed,which showed a benign pancreatic lesion.Neuroendocrine or solid pseudopapillary tumor was a possible diagnosis.The patient underwent further contrast-enhanced computed tomography and contrastenhanced magnetic resonance imaging,which were suggestive of solid pseudopapillary tumor or neuroendocrine tumor.All the examinations failed to give a definitive diagnosis,and the patient underwent surgery.The final pathological and immunohistochemical results showed that the mass was CD.CONCLUSION This case highlights when lymphadenopathy is encountered clinically,CD should be considered and a biopsy should be performed.

Prevention and Treatment of KSHV-associated Diseases with Antiviral Drugs

Kaposi’s sarcoma-associated herpesvirus (KSHV) was first identified as the etiologic agent of Kaposi’s sarcoma (KS) in 1994. KSHV infection is necessary,but not sufficient for the development of Kaposi sarcoma (KS),primary effusion lymphoma (PEL),and multicentric Castleman disease (MCD). Advances in the prevention and treatment of KSHV-associated Diseases have been achieved,even though current treatment options are ineffective,or toxic to many affected persons. The identification of new targets for potential future therapies and the randomized trial to evaluate the efficacy of new antivirals are required.

Liver resection vs radiofrequency ablation in single hepatocellular carcinoma of posterosuperior segments in elderly patients

BACKGROUND Liver resection and radiofrequency ablation are considered curative options for hepatocellular carcinoma.The choice between these techniques is still controversial especially in cases of hepatocellular carcinoma affecting posterosuperior segments in elderly patients.AIM To compare post-operative outcomes between liver resection and radiofrequency ablation in elderly with single hepatocellular carcinoma located in posterosuperior segments.METHODS A retrospective multicentric study was performed enrolling 77 patients age≥70-years-old with single hepatocellular carcinoma(≤30 mm),located in posterosuperior segments(4a,7,8).Patients were divided into liver resection and radiofrequency ablation groups and preoperative,peri-operative and long-term outcomes were retrospectively analyzed and compared using a 1:1 propensity score matching.RESULTS After propensity score matching,twenty-six patients were included in each group.Operative time and overall postoperative complications were higher in the resection group compared to the ablation group(165 min vs 20 min,P<0.01;54%vs 19%P=0.02 respectively).A median hospital stay was significantly longer in the resection group than in the ablation group(7.5 d vs 3 d,P<0.01).Ninety-day mortality was comparable between the two groups.There were no significant differences between resection and ablation group in terms of overall survival and disease free survival at 1,3,and 5 years.CONCLUSION Radiofrequency ablation in posterosuperior segments in elderly is safe and feasible and ensures a short hospital stay,better quality of life and does not modify the overall and disease-free survival.

Paget's Disease is Associated with Eleven Cancerous Regions:a Case Report and Therapeutic Strategy

Paget＇s disease of the breast is an uncommon disorder that accounts＇for 1% to 3% of all mammary tumors. The incidence of underlying carcinoma associated with Paget＇s disease has been reported in 82% to 100% of cases. The finding of underlying carcinoma reaches almost 100% when a palpable lump is also present. In this rare case, we described a patient presenting with Paget＇s disease but no palpable lump. However, we found 11 independent regions which were all invasive ductal carcinoma after the operation. Considering this patient, we should pay more attention to a multifocal and multicentric breast carcinoma associated with Paget＇s disease. Furthermore, we believe the mammography examination and a modified radical mastectomy are the most appropriate treatments for this population in clinical practice.

Castleman’s Disease of the Pleura: Case Presentation and Review of Literature

Purpose. Castleman’s Disease is a very rare medical disease and the presence of this condition in the pleura is even rarer and there are only few reported cases. The presence of symptoms is also unusual and has been mostly described in multicentric cases of castleman’s disease instead of solitary lesions (unicentric castleman). We report a case of unicentric pleural castlema’s disease with presence of constitutional symptoms. Methods. A 36-year-old male without any significant past medical history who presented to the emergency due to an incidental large pleural effusion seen in chest radiograph, but referring chronic constitutional symptoms consistent of significant weight loss and chronic cough. Results. Chest computed tomography revealed a mass and several attemps with minimally invase approach failed to identify the lesion. The mass was excised through VATS but due to its significant vascularity, it had to be converted to open thoracotomy. Conclusion. The treatment of unicentric lesions is mainly complete surgical excision and the prognosis is excellent with some chances of recurrence. There are less than one dozen of reported pleural castleman reported in the literature. This case evidence than unicentric lesion may exhibit constitutional symptoms and confirmed than lesions above 5 cm can be removed by VATS but the chances for conversion to an open thoracotomy are high.