Extramammary Paget’s Disease Manifested by Intraepithelial Adenocarcinoma of the Vulva and Anus Combined with Invasive Adenocarcinoma of the Ampullary Part of the Rectum

The Object of the Study: The author of the given paper describes an unusual combination of two diseases: extramammary Paget’s disease manifested by intraepithelial adenocarcinoma of the vulva and anus combined with invasive adenocarcinoma of the ampullary part of the rectum and describes the atypical manifestations of these diseases. The Content: The content of this research paper includes a description of the patient, an analysis of the clinical picture, diagnostic methods and therapeutic interventions used, a report of the following disease, and the result of the presented case. The Result of the Research Work: The result of the research work is the analysis of a clinical case with two different tumors, where such a combination of tumors is rarely described in the literature. Moreover, no large specific sample with this combination of diseases is available. Patient Characteristics: The given case report describes a patient of the Palliative Care Unit of the Gerontology Clinic with a primary diagnosis of C20-rectal adenocarcinoma in the background of the anal canal, perineal skin Paget’s disease, stage IV. The presented complications of the patient’s primary diagnosis are multiple metastases in the liver;status post palliative chemotherapy;hepatomegaly;metastases to abdominal lymph nodes, inguinal lymph nodes;metastases at Th12, L4 level;pain syndrome. The presented above combination of diagnosed diseases is very rare. Applied Diagnostics: In October 2021, it was performed diagnostic manipulation: biopsy and the pathologist have provided a microscopic description. The first tissue fragment had a pronounced electrothermal lesion and the epithelial structures were not valuable. The second skin tissue fragment was covered with hyperplastic and acanthotic epithelium;its basal and middle layers contained multiple large cells proliferates extending into the medial epidermis, and the cytoplasm of these cells reacted positively with PAS (Periodic Acid Schiff reaction). It needs to be noted that the patient had previously had several years of biopsies from the perineal and anal epidermis, where Paget’s disease had also been diagnosed. The performed immunohistochemistry showed these cells to be CK20 positive, CK7 rare positive and p16 negative. The following pathohistological findings were made: morphological and immunohistochemical picture is consistent with Paget’s disease. According to the ICD-10, the patient was diagnosed with C51 malignant neoplasm of the female external genitalia. Using imaging diagnostics, it became clear that the patient’s rectal adenocarcinoma had progressed to metastatic stage with distant liver metastases in the background of anal canal, perineal skin Paget’s disease. Therapeutic Plan of the Patient: Based on the patient’s main diagnoses, the complications of the principal diagnosis, the patient’s overall severe condition, pain syndrome, age and comorbidities, palliative chemotherapy was approved as a therapeutic option in council of doctors. Monitoring and Outcome of the Patient: The patient’s general condition was becoming worse over time, and she was diagnosed with exitus latalis in December 2022. At that time, the patient was discharged from hospital and was on palliative care at home under the control of her family physician.

Obstructive ileus due to a giant fibroepithelial polyp of the anus

Fibroepithelial polyps or hypertrophied anal papillae are essentially skin tags that project up from the dentate line and the junction between the skin and the epithelial lining of the anus. They are usually small in size, but sometimes they become enlarged, causing unexpected medical conditions. An extremely rare case of a giant hypertrophied anal papilla complicated by obstructive ileus is reported. Fibroepithelial anal polyp, despite its size, should be included in the differential diagnosis of a smooth mass located near the anal verge, especially in a patient with a history of chronic anal irritation or infection.

Education of Parents When a Child Born with an Imperforate Anus;Does It Improve the Health of the Child?

Objectives: To describe and investigate the value of an education program for parents of children born with an imperforate anus in order to help them cope with the new situation of having a stoma. A comparison is made with a group of parents following routine hospital. A secondary aim was to illuminate the parents’ feelings and concerns in the first month after the birth of the child. Subjects and methods: The program was tested in 20 Vietnamese mothers of babies born with an imperforate anus;10 followed an intervention comprising an education program and 10 the ordinary routine hospital. The study design is both qualitative and quantitative. The mothers were interviewed, using open-ended questions, within a week of their child’s birth and then repeatedly for up to one month. Finally, the conditions of children were accessed on their return to the hospital for the second operation after one month of care at home. The qualitative data were subjected to content analysis. Results: All mothers felt sad and worried in the beginning, but this quickly changed to confidence, particularly among mothers in the intervention group who received education. While at home, mothers in both groups had financial concerns, as they were unable to work as much as expected and also had to buy equipment for colostomy care. The mothers in the control group complained about a lack of knowledge and how it affected the care of their child. The mothers in the intervention group, however, felt confident in their caring even at home. When the families returned for the second operation, the children in the intervention group were significantly healthier, had increased more in weight, and had fewer complications and emergency return visits to hospital compared to the control group. In the control group skin problems around the stoma, diarrhea, bleeding or constipation while at home were reported (p < 0.01). Conclusion: The education improved the care at home resulting in healthier children and more confident parents.

OBSERVATION ON THERAPEUTIC EFFECTS OF ACUPUNCTURE IN 206 CASES WITH POSTOPERATIVE COMPLICATION OF ANUS AND INTESTINE

The authors have treated postoperative complication of anus and intestine by usingacupuncture at main point Chengshan(BL 57) with cool-producing needling manipulation. Therapeuticeffects of the treatment on postoperative severe pain, retention of urine, constipation, and bleedingwere observed clinically. Results indicated that the cure rate was 97. 6 % and the total effective ratewas 100%. Strong stimulation of Chengshan(BL 57) point improved mainly postoperative edema,spasm, local edema. The improvement of hyperemia and spasm is a ma jor factor of curing various postoperative complication of the anus and intestine. The therapeutic method has advantages of using lesspoints, rapidly producing effects, shorter therapeutic course, suffering little for patients, and no sideeffect.

Neurostimulation for fecal incontinence after correction of repair of imperforate anus

We are reporting the case of a 32-year-old female who had suffered from fecal incontinence(FI). She was born with an imperforate anus and a recto-vaginal fistula; she underwent repair at 6 mo of age. At 29 years of age, she was still fecally incontinent despite extensive pelvic floor reeducation. A magnetic resonance imaging and an anal electromyography were performed. Because her symptoms were considered to be probably due to extra-sphincteric implantation of the neo-anus, a redo was performed of the recto-neo-anal intra-sphincteric anastomosis. A neurostimulator device was subsequently implanted for persistent incontinence. Solid and liquid FI resolved, and her quality of life improved markedly. Combining surgery to correct the position of the neo-anus within the anal sphincter complex and neurostimulation could thus become a new approach in cases of refractory FI for patients with imperforate anus as a newborn. Follow-up into adulthood after pediatric imperforate anus surgery should be recommended for adult patients with persistent FI.

THE FACTORS AND TREATMENT OF LOCAL RECURRENCE AFTER RADICAL RESECTION RESERVING THE ANUS IN THE PATIENTS WITH RECTAL CANCER

Of seventy-one patients with rectal cancer after radical resection retaining the anus, 15 developed local recurrence with a recurrence rate of 21.1%. Local recurrence was correlated with improper safety margin from the lower edge of cancer to the anal end. There was statistical significant difference between 3 cm or more and 2 cm or less. The local recurrence was also related to the pathologic stage, histologic differentiation and implant of free cancer cells. It is suggested that the surgical indication of saving the anus be strict and without stretching, the safety margin from the lower edge of cancer to the anal end should not be less than 2 cm in early rectal cancer and not less than 4 cm in advanced lesions. During the operation, no touching tumor technique, thorough rinsing of the peritoneal cavity and pre- or post-operative radiotherapy are important for prevention of local recurrence. Early local recurrent rectal cancer can be detected by periodic examinations.

Non-Operative Management of Pilonidal Sinuses Located Around Anus

Aim: Pilonidal disease is generally located at sacrococcygeal region whereas it is rarely located near anus. The aim of this study is to discuss the results of crystallized phenol application that we performed for patients with sinus pilonidalis located near anus with 95% success rate. Patients and Methods: Patients admitted between 2005 to 2011 with sinuses located in 2 cm range of anus or were primarily located up to 2 cm to the anal verge were enrolled in the study. Patients’ demographic features, Body Mass Index (BMI), family history, skin color, hair thickness, number of sinus openings, and the status of the sinus (acute vs. chronic) were recorded. Crystallized phenol was applied into the sinus. The pa tients were followed-up after recovery during the first 6 months and annually afterwards. Results: A total of 25 sinus pilonidalis cases located near anus were encountered. All patients were male;crystallized phenol application was per formed on all patients a total of 115 times. The mean number of applications was 5.6 (between 4 and 8 times). The mean recovery period was 74.5 days (range: 31 - 154) and the mean follow-up period was 36.16 months (range: 18 - 48). No surgical intervention was required. Conclusion: Sinus pilonidalis cases located in perianal region can be successfully treated with the crystallized phenol application which is a simple and inexpensive method, that can easily be performed in an outpatient setting.

Perianal disease in inflammatory bowel disease:Broadening treatment and surveillance strategies for anal cancer

The perianal disease affects up to one-third of individuals with Crohn's disease(CD),causing disabling symptoms and significant impairment in quality of life,particularly for those with perianal fistulising CD(PFCD).The collaborative effort between gastroenterologists and surgeons is essential for addressing PFCD to achieve fistula closure and promote luminal healing.Limited fistula healing rates with conventional therapies have prompted the emergence of new biological agents,endoscopic procedures and surgical techniques that show promising results.Among these,mesenchymal stem cells injection is a particularly hopeful therapy.In addition to the burden of fistulas,individuals with perianal CD may face an increased risk of developing anal cancer.This underscores the importance of surveillance programmes and timely interventions to prevent late diagnoses and poor outcomes.Currently,there is no established formal anal screening programme.In this review,we provide an overview of the current state of the art in managing PFCD,including novel medical,endoscopic and surgical approaches.The discussion also focuses on the relevance of establishing an anal cancer screening programme in CD,intending to propose a risk-based surveillance algorithm.The validation of this surveillance programme would be a significant step forward in improving patient care and outcomes.

Long-term outcome of stem cell transplantation with and without anti-tumor necrotic factor therapy in perianal fistula with Crohn’s disease

BACKGROUND Stem cell transplantation is a promising therapeutic option for curing perianal fistula in Crohn’s disease(CD).Anti-tumor necrotic factor(TNF)therapy combined with drainage procedure is effective as well.However,previous studies are limited to proving whether the combination treatment of biologics and stem cell transplantation improves the effect of fistula closure.AIM This study aimed to evaluate the long-term outcomes of stem cell transplantation and compare Crohn’s perianal fistula(CPF)closure rates after stem cell transplantation with and without anti-TNF therapy,and to identify the factors affecting CPF closure and recurrence.METHODS The patients with CD who underwent stem cell transplantation for treating perianal fistula in our institution between Jun 2014 and December 2022 were enrolled.Clinical data were compared according to anti-TNF therapy and CPF closure.RESULTS A total of 65 patients were included.The median age of females was 26 years(range:21-31)and that of males was 29(44.6%).The mean follow-up duration was 65.88±32.65 months,and complete closure was observed in 50(76.9%)patients.The closure rates were similar after stem cell transplantation with and without anti-TNF therapy(66.7%vs 81.6%at 3 year,P=0.098).The patients with fistula closure had short fistulous tract and infrequent proctitis and anorectal stricture(P=0.027,0.002,and 0.008,respectively).Clinical factors such as complexity,number of fistulas,presence of concurrent abscess,and medication were not significant for closure.The cumulative 1-,2-,and 3-year closure rates were 66.2%,73.8%,and 75.4%,respectively.CONCLUSION Anti-TNF therapy does not increase CPF closure rates in patients with stem cell transplantation.However,both refractory and non-refractory CPF have similar closure rates after additional anti-TNF therapy.Fistulous tract length,proctitis,and anal stricture are risk factors for non-closure in patients with CPF after stem cell transplantation.

Anal metastasis in esophageal cancer:A case report

BACKGROUND Esophageal cancer is the sixth leading cause of cancer-related death and eighth most common cancer,affecting>450000 people worldwide.Esophageal squamous cell carcinoma is the most common histological type,whereas esophageal adenoid cystic carcinoma(EACC)is rare.The liver is the most common distant metastatic site in esophageal cancer.Anal metastasis is rare and has not been reported in clinical practice before.Here,we report anal metastases in a patient with EACC after regular chemotherapy and surgical resection.CASE SUMMARY A 61-year-old esophageal cancer patient was found to have lung and brain metastases during standardized treatment.The patient’s treatment plan was continuously adjusted according to the latest treatment guidelines.However,the patient subsequently noticed rectal bleeding and itching,and after obtaining pathology results at the local hospital,anal metastasis of esophageal cancer was diagnosed.CONCLUSION Postoperative pathology and immunohistochemistry confirmed EACC with rare anal metastasis.More exploration of EACC diagnosis and treatment is needed.

Hailey-Hailey disease with lichenoid lesions around the anus

To the Editor: Hailey-Hailey disease (HHD), first discovered by the brothers Howard and Hugh Hailey,[1]is a genodermatosis at intertriginous sites. Mutation of ATP2C1 on chromosome 3q21-2 coding a calciumdependent ATPase gives rise to calcium dysfunction within keratinocytes, resulting in acantholysis due to a signal transduction disorder.[2] It has been suggested that this gene mutation combined with irritation such as frequent friction, cold, and ultraviolet exposure leads to the development of HHD.[3]

Earliest-known deuterostomes with big mouth but no anus

Subject Code:D02With the support by the National Natural Science Foundation of China,the research team led by Prof.Shu Degan(舒德干)at the State Key Laboratory of Continental Dynamics,Department of Geology,Northwest University,Xi’an,recently reported the finding of microscopic deuterostomes from the basal Cambrian of Shaanxi,China in Nature(2017,542:228—231)as a highlighted paper on front cover.

Transcutaneous perianal sonography:A sensitive method for the detection of perianal inflammatory lesions in Crohn's disease

AIM:Pelvic magnetic resonance imaging(MRI)and endoanal ultrasound which are established imaging methods for perianal inflammatory lesions in patients with Crohn's disease require expensive specialized equipments and expertise.We investigated the feasibility and sensitivity of transcutaneous perianal ultrasound(PAUS)using regular ultrasound probes in the imaging of perianal inflammatory lesions.The sonographic findings were correlated to pelvic MR]-scans. METHODS:We performed PAUS in 25 patients with Crohn's disease and clinical signs of perianal inflammatory disease. Wibhin a median of 10 d(range 0-75)these patients underwent MRI of the pelvis.Regular convex and linear high resolution probes were used for PAUS.The sonographic findings were correlated to the MRI findings by blinded investigators. RESULTS:The sonographic investigations were well tolerated by all patients.Fistulae typically presented as hypoechoic tracks.Twenty-nine fistulae were detected in 22 patients.Abscesses were detected in 7 patients and presented as hypo-or anechoic formations.Twenty-six of 29 fistulae and 6 of 7 abscesses could be confirmed by MRI.Kappa statistics showed an excellent agreement (kappa>0.83)between the two imaging methods. CONCLUSION:PAUS is a simple,painless,feasible,real- time method that can be performed without specific patient preparation which is comparable in its sensitivity to pelvic MRI in the detection of perianal fistulae and/or abscesses. PAUS can especially be recommended as a screening tool in acute perianal disorders such as perianal abscess and for follow-up studies of perianal inflammatory disease.

Pancreatitis-associated protein:From a lectin to an anti-inflammatory cytokine

Pancreatitis-associated protein (PAP) was discovered in the pancreatic juice of rats with acute pancreatitis. PAP is a 16 kDa secretory protein structurally related to the C-type lectins although classical lectin-related function has not been reported yet. Then, it was demonstrated that PAP expression may be activated in some tissues in a constitutive or injury- and inflammation-induced manner. More recently, it has been found that PAP acts as an anti-inflammatory factor in vitro and in vivo. PAP expression can be induced by several pro- and anti-inflammatory cytokines and by itself through a JAK/STAT3-dependent pathway. PAP is able to activate the expression of the anti-inflammatory factor SOCS3 through the JAK/STAT3-dependent pathway. The JAK/STAT3/SOCS3 pathway seems to be a common point between PAP and several cytokines. Therefore, it is reasonable to propose that PAP is a new anti- inflammatory cytokine.

Magnetic resonance imaging may predict deep remission in patients with perianal fistulizing Crohn's disease

To evaluate the imaging course of Crohn’s disease (CD) patients with perianal fistulas on long-term maintenance anti-tumor necrosis factor (TNF)-α therapy and identify predictors of deep remission.METHODSAll patients with perianal CD treated with anti-TNF-α therapy at our tertiary care center were evaluated by magnetic resonance imaging (MRI) and clinical assessment. Two MR examinations were performed: at initiation of anti-TNF-α treatment and then at least 2 years after. Clinical assessment (remission, response and non-response) was based on Present’s criteria. Rectoscopic patterns, MRI Van Assche score, and MRI fistula activity signs (T2 signal and contrast enhancement) were collected for the two MR examinations. Fistula healing was defined as the absence of T2 hyperintensity and contrast enhancement on MRI. Deep remission was defined as the association of both clinical remission, absence of anal canal ulcers and healing on MRI. Characteristics and imaging patterns of patients with and without deep remission were compared by univariate and multivariate analyses.RESULTSForty-nine consecutive patients (31 females and 18 males) were included. They ranged in age from 14-70 years (mean, 33 years). MRI and clinical assessment were performed after a mean period of exposure to anti-TNF-α therapy of 40 ± 3.7 mo. Clinical remission, response and non-response were observed in 53.1%, 20.4%, and 26.5% of patients, respectively. Deep remission was observed in 32.7% of patients. Among the 26 patients in clinical remission, 10 had persisting inflammation of fistulas on MRI (T2 hyperintensity, n = 7; contrast enhancement, n = 10). Univariate analysis showed that deep remission was associated with the absence of rectal involvement and the absence of switch of anti-TNF-α treatment or surgery requirement. Multivariate analysis demonstrated that only the absence of rectal involvement (OR = 4.6; 95%CI: 1.03-20.5) was associated with deep remission.CONCLUSIONDeep remission is achieved in approximately one third of patients on maintenance anti-TNF-α therapy. Absence of rectal involvement is predictive of deep remission.

Neonatal colon perforation due to anorectal malformations: Can it be avoided?

Anorectal malformations (ARM) are common anomalies in neonates. Diagnostic and therapeutic delays in the management of ARM may lead to colonic perforation, and even death. Physical examination of the perineum is often sufficient to diagnose ARM in neonates. Notwithstanding, delayed diagnosis of ARM has become increasingly familiar to surgeons, as evidenced by the number of recent publications on this topic in the literature. In this commentary, we discuss spontaneous colonic perforation due to delayed diagnosis of ARM in neonates, and highlight the importance of early diagnosis in assuring good outcomes with surgical management. At this point, a thorough examination of the perineum during the initial newborn assessment is mandatory, particularly in those patients presenting with abdominal signs or symptoms.

Anorectal emergencies

Anorectal emergencies refer to anorectal disorders presenting with some alarming symptoms such as acute anal pain and bleeding which might require an immediate management. This article deals with the diagnosis and management of common anorectal emergencies such as acutely thrombosed external hemorrhoid, thrombosed or strangulated internal hemorrhoid, bleeding hemorrhoid, bleeding anorectal varices, anal fissure, irreducible or strangulated rectal prolapse, anorectal abscess, perineal necrotizing fasciitis(Fournier gangrene), retained anorectal foreign bodies and obstructing rectal cancer. Sexually transmitted diseases as anorectal non-surgical emergencies and some anorectal emergencies in neonates are also discussed. The last part of this review dedicates to the management of early complications following common anorectal procedures that may present as an emergency including acute urinary retention, bleeding, fecal impaction and anorectal sepsis. Although many of anorectal disorders presenting in an emergency setting are not life-threatening and may be successfully treated in an outpatient clinic, an accurate diagnosis and proper management remains a challenging problem for clinicians. A detailed history taking and a careful physical examination, including digital rectal examination and anoscopy, is essential for correct diagnosis and plan of treatment. In some cases, some imaging examinations, such as endoanal ultrasonography and computerized tomography scan of whole abdomen, are required. If in doubt, the attending physicians should not hesitate to consult an expert e.g., colorectal surgeon about the diagnosis, proper management and appropriate follow-up.

Anal carcinoma in giant anal condyloma, multidisciplinary approach necessary for optimal outcome:Two case reports and review of literature

BACKGROUND Anal cancers are caused by human papilloma virus(HPV). Buschke-Lowenstein tumor also known as giant anal condyloma(GCA) is a variant of giant neglected anal tumors arising from warts caused by HPV infection. HPV are a family of double-stranded DNA viruses and primarily cause sexually transmitted disease of the genitalia and oropharyngeal mucosa. These tumors are slow growing;locally destructive large verrucous masses.CASE SUMMARY We present a series of two cases with large anal tumors harboring invasive cancers and highlight their presentation and management. Tumors with high risk HPV subtypes(HPV 16, 18, 31, 33) may progress into invasive squamous cell carcinoma(SCC). Untreated GCA can attain enormous size and extend into the pelvic organs and bony structures. Some tumors show malignant degeneration into SCC and are often difficult to diagnose given the large size of the tumors.Complete surgical excision with negative margins is the treatment of choice and necessary to prevent recurrence. This is often not feasible and leaves large surgical wounds with tissue defects with delay in healing and increases postoperative morbidity. Pelvic reconstructive techniques including muscle flaps and grafts are often necessary to close the defects. Human immunodeficiency virus and immunocompromised patients generally do poorly with standard treatments.CONCLUSION A multidisciplinary team of colorectal and plastic surgeons, medical and radiation oncologists along with combination treatment modalities are necessary when malignant transformation occurs in GCA, for optimal outcomes.

澳大利亚国立大学教学管理模式及其启示

澳大利亚国立大学(ANU)的教学管理模式可以概括为把大学建设成一种学习型社区。澳大利亚国立大学的教学模式启示我们:要建设高水平大学,培养创造性人才,应反思教育教学本质,定位大学人才培养目标;更新教育教学观念,由以教师为主体转向以学生为主体;扩大对外开放和交流的力度,提高国际化办学程度;加快技术改造,提高教学手段的现代化和信息化。

Surgical salvage therapy of anal cancer

AIM:To evaluate the results of salvage resection in the management of persistent or locally recurrent anal canal cancer.METHODS: Details of all patients with anal canal cancer treated from 1978 to 1994 at Cancer Hospital of Chinese Academy of Medical Sciences (CAMS) were reviewed retrospectively.Sixteen patients who presented with persistent or locally recurrent anal canal cancer received salvage surgery. Before surgery all of the patients had received radiotherapy alone as their primary treatments.RESULTS:Of the 16 patients, 14 received salvage abdominoperineal resection (APR) and two had transanal local excision. There were no deaths attributable to operation.Delayed healing of the perineal wound occurred in eight patients. Complications unrelated to the perineal wound were found in five patients. The median follow-up time was 120 (range 5-245) months after salvage surgery. Nine patients died of disease progression, with a median survival time of 16 (range 5-27) months. Six patients had a long-term survival.CONCLUSION:Salvage resection after radiotherapy can yield a long-time survival in selected patients with anal canal cancer. However it offers little hope to patients with T4 and/or N2-3 tumors.