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Journal of Clinical Medicine Jan 2023Perianal fistulas are defined as pathological connections between the anorectal canal and the perianal skin. Most perianal fistulas are cryptoglandular fistulas, which... (Review)
Review
Perianal fistulas are defined as pathological connections between the anorectal canal and the perianal skin. Most perianal fistulas are cryptoglandular fistulas, which are thought to originate from infected anal glands. The remainder of the fistulas mainly arises as complications of Crohn's disease (CD), trauma, or as a result of malignancies. Fistulas in CD are considered as a consequence of a chronic and transmural inflammatory process in the distal bowel and can, in some cases, even precede the diagnosis of CD. Although both cryptoglandular and CD-associated fistulas might look similar macroscopically, they differ considerably in their complexity, treatment options, and healing rate. Therefore, it is of crucial importance to differentiate between these two types of fistulas. In this review, the differences between CD-associated and cryptoglandular perianal fistulas in epidemiology, pathogenesis, and clinical management are discussed. Finally, a flow chart is provided for physicians to guide them when dealing with patients displaying their first episode of perianal fistulas.
PubMed: 36675403
DOI: 10.3390/jcm12020466 -
Radiographics : a Review Publication of... 2022Normal Cowper glands and ducts are imperceptible at imaging. However, abnormalities of the Cowper glands and ducts are increasingly seen owing to increasing use of... (Review)
Review
Normal Cowper glands and ducts are imperceptible at imaging. However, abnormalities of the Cowper glands and ducts are increasingly seen owing to increasing use of cross-sectional imaging. In this article, the authors present a comprehensive review of the normal anatomy of the Cowper glands and ducts and the clinical and imaging findings of conditions that affect them in an effort to help lead to appropriate diagnosis and management. A Cowper duct syringocele is a congenital or acquired dilatation of the Cowper duct in the bulb of the corpus spongiosum. Retrograde urethrography is the standard test to diagnose a communicating (open) Cowper duct syringocele, and MRI is used for further morphologic assessment. Cowperitis refers to inflammation of the gland and/or duct, and recurrent cowperitis is usually associated with an underlying syringocele, a urethral fistulous connection to the Cowper gland, or a perianal fistula. MRI allows detection of the underlying structural abnormality and the superimposed inflammation. Cowper stones are typically radiopaque and are found in both the Cowper gland and duct. A Cowper gland hemorrhagic cyst may be incidentally detected at MRI of the prostate, and follow-up MRI may be required to ensure evolution of hemorrhage and exclude an underlying neoplasm. Cowper gland carcinoma is rare and includes adenocarcinoma and adenoid cystic carcinoma subtypes. Transperineal US may be used for initial evaluation and CT and/or MRI are necessary for further characterization and staging. Cowper gland hyperplasia is a rare entity with few previous reports that may be incidentally detected at imaging and may mimic malignancy. RSNA, 2022.
Topics: Male; Humans; Bulbourethral Glands; Diagnosis, Differential; Urethral Diseases; Cysts; Inflammation
PubMed: 36149823
DOI: 10.1148/rg.220099 -
Journal of Ultrasonography Nov 2020The presence of ectopic functional endometrial glands and struma anywhere except in the lining of the uterine cavity is considered as endometriosis. Extrapelvic...
The presence of ectopic functional endometrial glands and struma anywhere except in the lining of the uterine cavity is considered as endometriosis. Extrapelvic endometriosis involving the abdominal wall cesarean section scar is uncommonly seen, and it rarely involves the perineum, umbilicus, pleura, kidneys, lungs and liver. The purpose of the present study is to highlight rare ectopic sites, explain the pathogenesis of extrapelvic endometriosis, and evaluate the diagnostic significance of clinical findings, serum CA 125 level, and ultrasonography. 24 female patients with extrapelvic endometriomas in whom the final diagnosis was based on the surgical results and histopathological reports of the excised specimens. The patients underwent a clinical examination, an ultrasound scan, and evaluation of the serum CA 125 level. They were also examined by transvaginal ultrasound to rule out ovarian endometriosis or uterine adenomyosis. They were further subjected to abdominal wall ultrasound in cases of cesarean section scar or umbilical region swellings, and transperineal ultrasound for perianal lesions. Transvaginal ultrasound was performed in patients with perineal endometrioma to assess the relation between the lesion and the external anal sphincter. In 19 patients, abdominal wall cesarean section scar endometrioma was detected. Three patients had perianal endometriomas, and two patients - umbilical endometriomas. Ultrasound scanning was a useful diagnostic tool to evaluate extrapelvic endometriosis and its extension, especially in cases without typical clinical features that can be suggestive of endometrioma, low diagnostic sensitivity of serum CA 125, and low incidence of concomitant intrapelvic disease.
PubMed: 33365153
DOI: 10.15557/JoU.2020.0029 -
Clinical and Experimental Dermatology Jul 2015Extramammary Paget disease (EMPD) is an uncommon intraepithelial adenocarcinoma that involves body sites with apocrine glands such as the genital, perineal and perianal... (Review)
Review
Extramammary Paget disease (EMPD) is an uncommon intraepithelial adenocarcinoma that involves body sites with apocrine glands such as the genital, perineal and perianal regions. Risk stratification and treatment planning for EMPD can be challenging. This review presents important prognostic information in EMPD to assist physicians with risk stratification of patients with EMPD. The best-understood prognostic factors are depth of invasion and involvement of extracutaneous sites. Tumours that invade into the reticular dermis or have a depth of > 1 mm are associated with poorer prognosis. Additionally, tumours spreading outside the skin into lymph nodes or other tissues are higher risk. There is an emerging understanding of the importance of tumour genetics in risk stratification, and we review the data on Ki-67, cyclin D1, Mucin 5AC and E-cadherin. There is less evidence supporting the importance of lesion site and patient age in risk stratification. This succinct review will be helpful in clinical practice and in EMPD research.
Topics: Humans; Neoplasm Grading; Neoplasm Invasiveness; Paget Disease, Extramammary; Predictive Value of Tests; Prognosis; Skin Neoplasms
PubMed: 26011765
DOI: 10.1111/ced.12690 -
Current Treatment Options in Oncology Jun 2022Extramammary Paget's disease (EMPD) is a rare neoplastic disease affecting areas rich in apocrine glands in the elderly. EMPD clinically resembles a benign inflammatory... (Review)
Review
Extramammary Paget's disease (EMPD) is a rare neoplastic disease affecting areas rich in apocrine glands in the elderly. EMPD clinically resembles a benign inflammatory skin disease, and ill-defined tumor borders can lead to misdiagnosis and incomplete excision. Several prognostic factors have been reported, including nodule formation, tumor thickness, tumor invasion, lymphovascular invasion, and a perianal location, which are characteristic of primary tumors. EMPD typically presents as an in situ tumor spreading horizontally within the epidermis and then invading into the dermis as it transitions to a vertical growth phase. For this reason, tumor thickness, rather than tumor size, is correlated with patient prognosis. The best treatment for resectable EMPD is complete surgical removal of the tumor. EMPD sometimes has unclear tumor borders, and it can unexpectedly spread beyond its clinical boundaries. Surgical resection in such cases is often associated with tumor-positive margins, which can result in recurrence. However, surgical excision with wide margins may deteriorate patients' organ functions and quality of life. Mohs micrographic surgery may be ideal for controlling the surgical margins and minimizing the sacrifice of normal tissue, but this technique is not always feasible because of constraints associated with the medical environment. No standard treatment for unresectable or metastatic EMPD has been established. Although conventional chemotherapy has been used as the first-line treatment, it frequently causes adverse events, and consequently, targeted therapy will become more valuable in the near future.
Topics: Aged; Humans; Margins of Excision; Mohs Surgery; Paget Disease, Extramammary; Prognosis; Quality of Life
PubMed: 35377101
DOI: 10.1007/s11864-021-00923-3 -
Anais Brasileiros de Dermatologia 2015Paget's disease, described by Sir James Paget in 1874, is classified as mammary and extramammary. The mammary type is rare and often associated with intraductal cancer... (Review)
Review
Paget's disease, described by Sir James Paget in 1874, is classified as mammary and extramammary. The mammary type is rare and often associated with intraductal cancer (93-100% of cases). It is more prevalent in postmenopausal women and it appears as an eczematoid, erythematous, moist or crusted lesion, with or without fine scaling, infiltration and inversion of the nipple. It must be distinguished from erosive adenomatosis of the nipple, cutaneous extension of breast carcinoma, psoriasis, atopic dermatitis, contact dermatitis, chronic eczema, lactiferous ducts ectasia, Bowen's disease, basal cell carcinoma, melanoma and intraductal papilloma. Diagnosis is histological and prognosis and treatment depend on the type of underlying breast cancer. Extramammary Paget's disease is considered an adenocarcinoma originating from the skin or skin appendages in areas with apocrine glands. The primary location is the vulvar area, followed by the perianal region, scrotum, penis and axillae. It starts as an erythematous plaque of indolent growth, with well-defined edges, fine scaling, excoriations, exulcerations and lichenification. In most cases it is not associated with cancer, although there are publications linking it to tumors of the vulva, vagina, cervix and corpus uteri, bladder, ovary, gallbladder, liver, breast, colon and rectum. Differential diagnoses are candidiasis, psoriasis and chronic lichen simplex. Histopathology confirms the diagnosis. Before treatment begins, associated malignancies should be investigated. Surgical excision and micrographic surgery are the best treatment options, although recurrences are frequent.
Topics: Breast Neoplasms; Carcinoma; Diagnosis, Differential; Female; Genital Neoplasms, Male; Humans; Male; Nipples; Paget Disease, Extramammary; Paget's Disease, Mammary; Skin Neoplasms; Vulvar Neoplasms
PubMed: 25830993
DOI: 10.1590/abd1806-4841.20153189 -
The Journal of International Medical... Feb 2021Cryptoglandular perianal fistula is a common benign anorectal disorder that is managed mainly with surgery. A fistula is typically defined as a pathological... (Review)
Review
Cryptoglandular perianal fistula is a common benign anorectal disorder that is managed mainly with surgery. A fistula is typically defined as a pathological communication between two epithelialized surfaces. More specifically, perianal fistula manifests as an abnormal tract between the anorectal canal and the perianal skin. Perianal fistulas are often characterized by significantly decreased patient quality of life. The cryptoglandular theory of perianal fistulas suggests their development from the proctodeal glands, which originate from the intersphincteric plane and perforate the internal sphincter with their ducts. Involvement of proctodeal glands in the inflammatory process could play a primary role in the formation of cryptoglandular perianal fistula. The objective of this narrative review was to investigate the current knowledge of the pathogenesis of cryptoglandular perianal fistula with the specific aims of characterizing the potential role of proinflammatory factors responsible for the development of chronic inflammation. Further studies are crucial to improve the therapeutic management of cryptoglandular perianal fistulas.
Topics: Anal Canal; Humans; Quality of Life; Rectal Fistula; Treatment Outcome
PubMed: 33595349
DOI: 10.1177/0300060520986669 -
Journal of Comparative Pathology Jan 2023c-KIT and its ligand stem cell factor (SCF) play a direct role in the oncogenesis of various cancers by regulating the cell fate. Recent evidence indicates that an...
c-KIT and its ligand stem cell factor (SCF) play a direct role in the oncogenesis of various cancers by regulating the cell fate. Recent evidence indicates that an increased expression of c-KIT/SCF, driven by hormonal imbalances, is an important step in the development of hormone-dependent cancers. We investigated the possible role of the c-KIT/SCF system in the carcinogenesis in 44 perianal gland tumours (16 adenomas, 15 epitheliomas and 13 carcinomas) and 10 normal perianal gland tissues by assessing the percentage and type of cells that expressed c-KIT and SCF as well as the cellular localization of immunoreactivity. No differences in immunolabelling of SCF were found between normal glands and neoplastic cells of any histotype. The highest expression of c-KIT was seen in carcinomas and a positive correlation was found between c-KIT labelling score and mitotic index (R = 0.876; P <0.01). c-KIT labelling patterns in hepatoid cells varied among the tumour histotypes with adenomas having only membranous labelling. Three labelling patterns (membranous only, membranous and cytosolic, and cytosolic only) were seen in the other tumour histotypes. Cytosolic labelling was statistically more frequent in carcinomas than in adenomas (P <0.001). These findings suggest that c-KIT expression and its cellular localization may play a role in the development and progression of perianal gland tumours by influencing cell proliferation.
Topics: Animals; Dogs; Stem Cell Factor; Anal Gland Neoplasms; Proto-Oncogene Proteins c-kit; Carcinoma; Adenoma; Dog Diseases
PubMed: 36652776
DOI: 10.1016/j.jcpa.2022.11.007 -
Rozhledy V Chirurgii : Mesicnik... 2020Perianal hidradenitis suppurativa is a chronic recurrent inflammatory, suppurative, and fistulising disease of apocrine glands, adjacent anal canal and soft tissues....
INTRODUCTION
Perianal hidradenitis suppurativa is a chronic recurrent inflammatory, suppurative, and fistulising disease of apocrine glands, adjacent anal canal and soft tissues. Perianal area is the second most common affected area after axilla. There are three grades of the disease. Hidradenitis suppurativa represents a chronic, recurrent, deep-seated folliculitis resulting in abscesses, followed by the formation of sinus tracts and subsequent scarring. Perianal hidradenitis suppurativa is the last and the most serious grade of the disease and a specific access is needed for patient preparation and surgical treatment alone. The currently preferred method of treatment for patients with extensive perianal hidradentitis is excision and closure with combination of skin flaps, primary suture and skin graft in one or two stages.
CASE REPORTS
There are three case reports of perianal hidradenitis suppurativa in this article. The local and overall initial treatment of patients followed by a radical excision and closure with a rotation skin flaps and skin grafts is described. The final results were satisfactory, with no recurrence or serious complication.
Topics: Axilla; Hidradenitis Suppurativa; Humans; Perineum; Skin Transplantation; Surgical Flaps
PubMed: 33242970
DOI: 10.33699/PIS.2020.99.9.409-413 -
International Archives of Allergy and... 2019Recurrent severe bacterial and fungal infections are characteristic features of the rare genetic immunodeficiency disorder chronic granulomatous disease (CGD). The... (Review)
Review
Recurrent severe bacterial and fungal infections are characteristic features of the rare genetic immunodeficiency disorder chronic granulomatous disease (CGD). The disease usually manifests within the first years of life with an incidence of 1 in approximately 200,000 live births. The incidence is higher in Iran and Morocco where it reaches 1.5 per 100,000 live births. Mutations have been described in the 5 subunits of NADPH oxidase, mostly in gp91phox and p47phox, with fewer mutations reported in p67phox, p22phox, and p40phox. These mutations cause loss of superoxide production in phagocytic cells. CYBB, the gene encoding the large gp91phox subunit of the transmembrane component cytochrome b558 of the NADPH oxidase complex, is localized on the X-chromosome. Genetic defects in CYBB are responsible for the disease in the majority of male CGD patients. CGD is associated with the development of granulomatous reactions in the skin, lungs, bones, and lymph nodes, and chronic infections may be seen in the liver, gastrointestinal tract, brain, and eyes. There is usually a history of repeated infections, including inflammation of the lymph glands, skin infections, and pneumonia. There may also be a persistent runny nose, inflammation of the skin, and inflammation of the mucous membranes of the mouth. Gastrointestinal problems can also occur, including diarrhea, abdominal pain, and perianal abscesses. Infection of the bones, brain abscesses, obstruction of the genitourinary tract and/or gastrointestinal tract due to the formation of granulomatous tissue, and delayed growth are also symptomatic of CGD. The prevention of infectious complications in patients with CGD involves targeted prophylaxis against opportunistic microorganisms such as Staphylococcus aureus, Klebsiella spp., Salmonella spp. and Aspergillus spp. In this review, we provide an update on organ involvement and the association with specific isolated microorganisms in CGD patients.
Topics: Autoimmunity; Bacterial Infections; Child; Child, Preschool; Female; Granulomatous Disease, Chronic; Humans; Infant; Liver Abscess; Lung Diseases; Male; Mycoses; NADPH Oxidases; Skin Diseases
PubMed: 30904913
DOI: 10.1159/000496181