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Dermatology Online Journal Apr 2019In 1874, Sir James Paget first described Paget disease of the nipple, also known as mammary Paget disease. In 1889, extramammary Paget disease (EMPD) of the scrotum and... (Review)
Review
In 1874, Sir James Paget first described Paget disease of the nipple, also known as mammary Paget disease. In 1889, extramammary Paget disease (EMPD) of the scrotum and penis was identified. Although mammary and extramammary Paget disease are both characterized by epidermal Paget cells and share a similar clinical presentation, their uniqueness lies in anatomical location and histogenesis. EMPD presents as an erythematous plaque on apocrine gland bearing areas (i.e. vulva, perineum, perianal region, scrotum, and penis) in older men and women. It can be a focal, multifocal, or an ectopic process. Immunohistochemical staining allows for differentiation between primary and secondary EMPD in addition to the many other disease entities that clinically resemble this malignancy. When diagnosing a patient with EMPD, a full history and physical should be performed given the possibility of an underlying malignancy. Surgical excision currently is first line therapy and the prognosis is often favorable. Recent advances within the field have examined the expression of chemokine receptors within tumors, which may be applicable in determining prognosis. This review addresses the history, epidemiology, pathogenesis, clinical presentation, histopathology, differential diagnosis, diagnosis, management, and new observations with respect to extramammary Paget disease.
Topics: Anus Neoplasms; Female; Humans; Male; Paget Disease, Extramammary; Penile Neoplasms; Perineum; Prognosis; Scrotum; Skin Neoplasms; Vulvar Neoplasms
PubMed: 31046904
DOI: No ID Found -
European Urology Nov 2022The fifth edition of the World Health Organization (WHO) classification of urogenital tumours (WHO "Blue Book"), published in 2022, contains significant revisions. This... (Review)
Review
The fifth edition of the World Health Organization (WHO) classification of urogenital tumours (WHO "Blue Book"), published in 2022, contains significant revisions. This review summarises the most relevant changes for renal, penile, and testicular tumours. In keeping with other volumes in the fifth edition series, the WHO classification of urogenital tumours follows a hierarchical classification and lists tumours by site, category, family, and type. The section "essential and desirable diagnostic criteria" included in the WHO fifth edition represents morphologic diagnostic criteria, combined with immunohistochemistry and relevant molecular tests. The global introduction of massive parallel sequencing will result in a diagnostic shift from morphology to molecular analyses. Therefore, a molecular-driven renal tumour classification has been introduced, taking recent discoveries in renal tumour genomics into account. Such novel molecularly defined epithelial renal tumours include SMARCB1-deficient medullary renal cell carcinoma (RCC), TFEB-altered RCC, Alk-rearranged RCC, and ELOC-mutated RCC. Eosinophilic solid and cystic RCC is a novel morphologically defined RCC entity. The diverse morphologic patterns of penile squamous cell carcinomas are grouped as human papillomavirus (HPV) associated and HPV independent, and there is an attempt to simplify the morphologic classification. A new chapter with tumours of the scrotum has been introduced. The main nomenclature of testicular tumours is retained, including the use of the term "germ cell neoplasia in situ" (GCNIS) for the preneoplastic lesion of most germ cell tumours and division from those not derived from GCNIS. Nomenclature changes include replacement of the term "primitive neuroectodermal tumour" by "embryonic neuroectodermal tumour" to separate these tumours clearly from Ewing sarcoma. The term "carcinoid" has been changed to "neuroendocrine tumour", with most examples in the testis now classified as "prepubertal type testicular neuroendocrine tumour".
Topics: Carcinoma, Renal Cell; Genitalia, Male; Humans; Kidney Neoplasms; Male; Neoplasms, Germ Cell and Embryonal; Neuroectodermal Tumors; Papillomavirus Infections; Receptor Protein-Tyrosine Kinases; Testicular Neoplasms; World Health Organization
PubMed: 35853783
DOI: 10.1016/j.eururo.2022.06.016 -
Current Oncology (Toronto, Ont.) Aug 2021Extramammary Paget's disease (EMPD) is a rare neoplasm that usually develops in apocrine gland-bearing areas, such as the vulva, scrotum, and penis. EMPD may present... (Review)
Review
Extramammary Paget's disease (EMPD) is a rare neoplasm that usually develops in apocrine gland-bearing areas, such as the vulva, scrotum, and penis. EMPD may present with a focal, multifocal, or an ectopic lesion. Clinically, EMPD lesions often exhibit infiltrative erythema, which is sometimes similar to other skin disorders such as eczema. While primary EMPD arises as intraepithelial neoplasm of the epidermis, EMPD-like lesions may occur from epidermotropic spread of malignant cells or direct extension from an underlying internal neoplasm, known as secondary EMPD. Because treatment strategies differ for primary EMPD and secondary EMPD, accurate diagnosis based on detailed histopathological evaluation is required. In the early stages, EMPD usually shows indolent growth, and most cases are diagnosed as carcinoma in situ. However, invasive lesions may result in metastases, and deep invasion is associated with high incidence of metastases. Conventional chemotherapies have been used for EMPD treatment in patients with distant metastases, but the efficacy is not satisfactory, and the prognosis for such patients remains poor. Recent studies have provided various insights into the molecular pathogenesis of the development and advancement of EMPD, which may lead to novel treatment approaches for metastatic EMPD. This review addresses the diagnosis, pathogenesis, and treatment of EMPD with focus on recent progress in understanding this disease.
Topics: Female; Humans; Male; Paget Disease, Extramammary; Prognosis; Scrotum
PubMed: 34436026
DOI: 10.3390/curroncol28040260 -
Disease Markers 2007While much is known about the natural history of cervical human papillomavirus (HPV) infection and its consequences, including cervical intraepithelial neoplasia and... (Review)
Review
While much is known about the natural history of cervical human papillomavirus (HPV) infection and its consequences, including cervical intraepithelial neoplasia and cervical cancer, relatively little is known about the natural history of anogenital HPV infection and diseases in men. In part this reflects difficulties in penile sampling and visual assessment of penile lesions. Anal HPV infection and disease also remain poorly understood. Although HPV is transmitted sexually and infects the genitals of both sexes, the cervix remains biologically more vulnerable to malignant transformation than does the penis or anus in men. An understanding of male HPV infection is therefore important in terms of reducing transmission of HPV to women and improving women's health. However, it is also important due to the burden of disease in men, who may develop both penile and anal cancer, particularly among HIV-positive men who have sex with men. Improved sampling techniques of the male genitalia and cohort studies in progress should provide important information on the natural history of anogenital HPV infection and disease in men, including risk factors for HPV acquisition and transmission. The impact of HPV vaccination in women on male anogenital HPV infection will also need to be assessed.
Topics: Anus Neoplasms; Carcinoma in Situ; Condylomata Acuminata; Female; Genital Diseases, Male; Humans; Male; Papillomaviridae; Papillomavirus Infections; Penile Neoplasms; Sexual Partners
PubMed: 17627061
DOI: 10.1155/2007/159137 -
Current Oncology (Toronto, Ont.) Apr 2023Carcinoma penis is a rare neoplasm, and the literature is scarce on long-term survival and its predictors. The aim of the study was to determine the clinical profile and...
BACKGROUND
Carcinoma penis is a rare neoplasm, and the literature is scarce on long-term survival and its predictors. The aim of the study was to determine the clinical profile and management patterns, identify predictors of survival, and the impact of education and rural/urban dwelling on survival.
METHODS
Patients with a histological diagnosis of carcinoma penis from January 2015 to December 2019 were included in the study. Demographics, clinical profile, education status, primary residence address, and outcomes were obtained from the case records. Distance from the treatment centre was obtained from the postal code. The primary objectives were to assess relapse-free survival (RFS) and overall survival (OS). The secondary objectives were to identify the predictors of RFS and OS and to determine the clinical profile and treatment patterns in patients with carcinoma penis in India. Time-to-event was calculated by Kaplan-Meir analysis and survival was compared by the log-rank test. Univariate and multivariable Cox regression analyses were used to find independent predictors of relapse and mortality. Logistic regression analyses to examine the associations of rural residence, education status, and distance from the treatment centre with the relapse adjusting for measured confounding variables.
RESULTS
Case records of 102 patients treated during the above period were retrieved. The median age was 55.5 (interquartile range [IQR] 42-65 years). Ulcero-proliferative growth (65%), pain (57%), and dysuria (36%) were the most common presenting features. Clinical examination or imaging revealed inguinal lymphadenopathy in 70.6% of patients, however, only 42% of these lesions were pathologically involved. A total of 58.8% of patients were from rural areas, 46.9% had no formal education, and 50.9% had a primary residence ≥100 km from the hospital. Patients with lower education and rural households had higher TNM stages and nodal involvement. Median RFS and OS were 57.6 months (15.8 months to not reached) and 83.9 months (32.5 months to not reached), respectively. On univariate analysis tumor stage, involvement of lymph nodes, T stage, performance status, and albumin was predictive for relapse and survival. However, on multivariate analysis, the stage remained the only predictor of RFS and nodal involvement, and metastatic disease was a predictor of OS. Education status, rural habitation, and distance from the treatment centre were not predictors for relapse or survival.
CONCLUSIONS
Patients with carcinoma have locally advanced disease at presentation. Rural dwellings and lower education were associated with the advanced stage but did not have a significant bearing on the survival outcomes. The stage at diagnosis and nodal involvement is the most important predictor of RFS and OS.
Topics: Male; Humans; Adult; Middle Aged; Aged; Prognosis; Neoplasm Recurrence, Local; Lymph Nodes; Carcinoma; Penis
PubMed: 37232803
DOI: 10.3390/curroncol30050345 -
Human Pathology Feb 2018Primary carcinomas of the urethra are rare and poorly understood lesions; hence, their clinical and pathologic spectrum is not completely defined. We analyzed a series...
Primary carcinomas of the urethra are rare and poorly understood lesions; hence, their clinical and pathologic spectrum is not completely defined. We analyzed a series of 130 primary urethral tumors and classified 106 of them as primary urethral carcinomas. The age at diagnosis of patients with primary urethral carcinomas ranged from 42 to 97 years (mean, 69.4 years; median, 70 years). There were 73 male and 33 female patients with a ratio of 2.2:1. In male patients, the tumors most frequently developed in the bulbous-membranous segment of the urethra. In female patients, the entire length of the urethra was typically involved. Microscopically, they were poorly differentiated carcinomas with hybrid squamous and urothelial features and developed from precursor intraepithelial conditions such as dysplasia and carcinoma in situ, which were frequently present in the adjacent urethral mucosa. High-risk human papilloma virus infection could be documented in 31.6% of these tumors. Follow-up information was available for 95 patients. Twenty-three patients died of the disease with a mean and median survival of 39 and 21 months, respectively. Urethral carcinomas are aggressive tumors with a high propensity for regional and distant metastases with mean and median survival of 39 and 21 months, respectively. Our observations have important implications for the management of patients with primary carcinoma of the urethra by defining them as a unique entity linked to human papilloma virus infection.
Topics: Adult; Aged; Aged, 80 and over; Carcinoma in Situ; Carcinoma, Squamous Cell; Female; Humans; Male; Middle Aged; Papillomaviridae; Papillomavirus Infections; Urethra; Urethral Neoplasms; Urothelium
PubMed: 28827100
DOI: 10.1016/j.humpath.2017.08.006 -
Journal of Nanobiotechnology Aug 2022Injuries to the urethra can be caused by malformations, trauma, inflammation, or carcinoma, and reconstruction of the injured urethra is still a significant challenge in... (Review)
Review
Injuries to the urethra can be caused by malformations, trauma, inflammation, or carcinoma, and reconstruction of the injured urethra is still a significant challenge in clinical urology. Implanting grafts for urethroplasty and end-to-end anastomosis are typical clinical interventions for urethral injury. However, complications and high recurrence rates remain unsatisfactory. To address this, urethral tissue engineering provides a promising modality for urethral repair. Additionally, developing tailor-made biomimetic natural and synthetic grafts is of great significance for urethral reconstruction. In this work, tailor-made biomimetic natural and synthetic grafts are divided into scaffold-free and scaffolded grafts according to their structures, and the influence of different graft structures on urethral reconstruction is discussed. In addition, future development and potential clinical application strategies of future urethral reconstruction grafts are predicted.
Topics: Humans; Plastic Surgery Procedures; Tissue Engineering; Urethra; Urethral Stricture
PubMed: 36045428
DOI: 10.1186/s12951-022-01599-z -
American Family Physician Jan 2018Noninfectious penile lesions are classified by clinical presentation as papulosquamous (e.g., psoriasis), inflammatory (e.g., lichen sclerosus, lichen nitidus, lichen...
Noninfectious penile lesions are classified by clinical presentation as papulosquamous (e.g., psoriasis), inflammatory (e.g., lichen sclerosus, lichen nitidus, lichen planus), vascular (e.g., angiokeratomas), or neoplastic (e.g., carcinoma in situ, invasive squamous cell carcinoma). Psoriasis presents as red or salmon-colored plaques with overlying silvery scales, often with extragenital cutaneous lesions. Lichen sclerosus presents as a phimotic, hypopigmented prepuce or glans penis with a cellophane-like texture. Lichen nitidus usually produces asymptomatic pinhead-sized, hypopigmented papules. The lesions of lichen planus are pruritic, violaceous, polygonal papules that are typically systemic. Angiokeratomas are typically asymptomatic, well-circumscribed, red or blue papules, often with annular or figurate configurations. Carcinoma in situ should be suspected if there are velvety red or keratotic plaques on the glans penis or prepuce, whereas invasive squamous cell carcinoma presents as a painless lump, ulcer, or fungating mass. Some benign lesions, such as psoriasis and lichen planus, may mimic carcinoma in situ or invasive squamous cell carcinoma. Biopsy is indicated if the diagnosis is in doubt or neoplasm cannot be excluded. The management of benign noninfectious penile lesions usually involves observation, topical corticosteroids, or topical calcineurin inhibitors. Neoplastic lesions generally warrant organ-sparing surgery.
Topics: Diagnosis, Differential; Humans; Male; Penile Diseases; Penis
PubMed: 29365226
DOI: No ID Found -
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 -
Dermatology Online Journal Jul 2018Intimate body piercings involving the nipple and genitalia have increased in prevalence in both men and women. Despite this increase, there is a deficiency in the... (Review)
Review
Intimate body piercings involving the nipple and genitalia have increased in prevalence in both men and women. Despite this increase, there is a deficiency in the literature regarding the short and long-term complications of body piercings, including an increased risk of infection, malignancy, and structural damage to the associated tissue. Breast abscesses associated with nipple piercing can be mistaken as inflammatory carcinoma. Male genital piercings have been associated with urethral rupture, paraphimosis, urethral obstruction, scar formation, and squamous cell carcinoma, whereas female genital piercings may lead to a higher risk of pregnancy and sexually transmitted infections. There are additional problems related to piercings during pregnancy and thereafter. Nipple piercings can hinder breast feeding by inhibiting the milk letdown reflex, increasing nipple sensitivity, and causing discomfort to the infant. Removal of genital piercings during pregnancy could introduce bacteria into the piercing tract, but retaining the piercings could theoretically hinder childbirth. Prevention of complications is critical. Patients must understand the risks of piercings and disclose relevant medical conditions to the practitioner before the procedure. The piercings should be carried out in a hygienic and sterile manner. Finally, physicians should maintain a non-judgmental attitude to encourage patients to seek medical care for complications.
Topics: Body Piercing; Breast Diseases; Female; Genital Diseases, Female; Genital Diseases, Male; Genitalia, Female; Humans; Male; Nipples; Penis; Umbilicus
PubMed: 30261561
DOI: No ID Found