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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 -
Histopathology Mar 2023Squamous cell carcinoma (SCC) is the most common malignant tumour of the penis. The 2022 WHO classification reinforces the 2016 classification and subclassifies... (Review)
Review
Squamous cell carcinoma (SCC) is the most common malignant tumour of the penis. The 2022 WHO classification reinforces the 2016 classification and subclassifies precursor lesions and tumours into human papillomavirus (HPV)-associated and HPV-independent types. HPV-associated penile intraepithelial neoplasia (PeIN) is a precursor lesion of invasive HPV- associated SCC, whereas differentiated PeIN is a precursor lesion of HPV-independent SCC. Block-type positivity of p16 immunohistochemistry is the most practical daily utilised method to separate HPVassociated from HPVindependent penile SCC. If this is not feasible, the term SCC, not otherwise specified (NOS) is appropriate. Certain histologies that were previously classified as "subtypes" are now grouped, and coalesced as "patterns", under the rubric of usual type SCC and verrucous carcinoma (e.g. usual-type SCC includes pseudohyperplastic and acantholytic/pseudoglandular carcinoma, and carcinoma cuniculatum is included as a pattern of verrucous carcinoma). If there is an additional component of the usual type of invasive SCC (formerly termed hybrid histology), the tumour would be a mixed carcinoma (e.g. carcinoma cuniculatum or verrucous carcinoma with usual invasive SCC); in such cases, reporting of the relative percentages in mixed tumours may be useful. The consistent use of uniform nomenclature and reporting of percentages will inform the refinement of future reporting classification schemes and guidelines/recommendations. The classification of scrotal tumours is provided for the first time in the fifth edition of the WHO Blue book, and it follows the schema of penile cancer classification for both precursor lesions and the common SCC of the scrotum. Basal cell carcinoma of the scrotum may have a variable clinical course and finds a separate mention.
Topics: Male; Humans; Papillomavirus Infections; Scrotum; Genital Neoplasms, Male; Skin Neoplasms; Carcinoma, Squamous Cell; Penile Neoplasms; Carcinoma, Verrucous; Human Papillomavirus Viruses; World Health Organization; Papillomaviridae
PubMed: 36221864
DOI: 10.1111/his.14824 -
Human Pathology Jun 2023The fifth edition of the World Health Organization (WHO) classification of urinary and male genital organ tumors has been recently published in 2022. The application of... (Review)
Review
The fifth edition of the World Health Organization (WHO) classification of urinary and male genital organ tumors has been recently published in 2022. The application of molecular profiling has made a substantial impact on the classification of urologic tumors. The new WHO classification introduces a group of molecularly well-defined renal tumor subtypes. The significant changes include addition of a category of "other oncocytic tumors" with oncocytoma/chromophobe renal cell carcinoma (chRCC)-like features, elimination of the subcategorization of type 1/2 papillary RCC, and inclusion of eosinophilic solid and cystic RCC as an independent tumor entity. The WHO/ISUP grading now has been recommended for all RCCs. Major nomenclature changes include replacement of histologic "variants" by "subtypes," "clear cell papillary renal cell carcinoma" to "clear cell renal cell tumor," "TCEB1-mutated RCC" to "ELOC-mutated RCC," "hereditary leiomyomatosis and renal cell carcinoma" to "fumarate hydratase-deficient RCC," "RCC-Unclassified" to "RCC-NOS," "primitive neuroectodermal tumor" to "embryonic neuroectodermal tumor," "testicular carcinoid" to "testicular neuroendocrine tumor," and "basal cell carcinoma of the prostate" to "adenoid-cystic (basal-cell) carcinoma of the prostate." Metastatic, hematolymphoid, mesenchymal, melanocytic, soft tissue, and neuroendocrine tumors are collectively discussed in separate chapters. It has been suggested that the morphological classification of urothelial cancer be replaced with a new molecular taxonomic classification system.
Topics: Humans; Male; Carcinoma, Renal Cell; Kidney Neoplasms; Genitalia, Male; Carcinoma, Basal Cell; World Health Organization; Neuroendocrine Tumors; Skin Neoplasms
PubMed: 36084769
DOI: 10.1016/j.humpath.2022.08.006 -
Obstetrics and Gynecology Clinics of... Sep 2021Urethral and periurethral masses in women include both benign and malignant entities that can be difficult to clinically differentiate. Primary urethral carcinoma is... (Review)
Review
Urethral and periurethral masses in women include both benign and malignant entities that can be difficult to clinically differentiate. Primary urethral carcinoma is rare and the optimal treatment modality may vary depending on the stage at presentation. Because cancer-free survival is poor, clinicians shouldhave a high index of suspicion when evaluating a urethral mass. Some benign-appearing urethral masses may be safely observed. Surgical resection is an effective option that should be used based on patient preference and symptoms, and for suspicious lesions.
Topics: Affect; Diverticulum; Female; Humans; Male; Urethra; Urethral Diseases; Urethral Neoplasms
PubMed: 34416940
DOI: 10.1016/j.ogc.2021.05.011 -
Abdominal Radiology (New York) Jul 2020This article reviews the anatomy and magnetic resonance imaging (MRI) appearance of the penis. The recommended technique for MRI of the penis is outlined, with... (Review)
Review
This article reviews the anatomy and magnetic resonance imaging (MRI) appearance of the penis. The recommended technique for MRI of the penis is outlined, with discussion of the benefits and drawbacks to imaging the penis after artificial erection. Common penile pathologies are reviewed, with a summary of their clinical features, MRI appearance, and treatment. The ability to demonstrate the erectile bodies and vasculature helps to evaluate vascular pathologies such as priapism, Mondor disease, and cavernosal thrombosis. MRI is also a useful tool to assess for traumatic abnormalities of the penis, with the depiction of soft tissue anatomy and fascial layers allowing for evaluation of penile fracture and penile contusion. Malignant disease of the penis is also reviewed, including squamous cell carcinoma, urethral carcinoma, metastases, and rare neoplasms. Staging of these neoplasms is addressed, with discussion of how MRI can be used to assess the extent of tumor involvement. MRI also clearly demonstrates the tunica albuginea, which allows for evaluation of Peyronie's disease.
Topics: Fascia; Humans; Magnetic Resonance Imaging; Male; Penile Induration; Penis
PubMed: 31701192
DOI: 10.1007/s00261-019-02301-y -
The Journal of Dermatological Treatment Mar 2022Penile intraepithelial neoplasia (PeIN) is a histological term for precancerous penile lesions. PeIN is important due to the high morbidity and mortality associated with... (Review)
Review
INTRODUCTION
Penile intraepithelial neoplasia (PeIN) is a histological term for precancerous penile lesions. PeIN is important due to the high morbidity and mortality associated with progression to penile squamous cell carcinoma (PSSC). But PeIN is rare, contributing to a limited evidence-base for the relative efficacy of available treatment options.
OBJECTIVES & METHODS
To consolidate and expand knowledge about PeIN and its treatment, we describe the clinical and histological characteristics, treatments and outcomes of 345 patients with PeIN, managed by our multidisciplinary team. Our results are compared and contrasted with those in the literature, following comprehensive review.
RESULTS
8.7% of patients had concomitant, invasive PSCC, whilst 91.3% demonstrated PeIN alone. 84% had PeIN, and 10.7% PeIN (5.2%, not specified). Clinical or histological evidence of HPV alone was present in 58%; features of lichen sclerosus alone in 12%; features of both in 29.4%. Only 14.4% of patients could be treated solely with topical agents or cryotherapy, whereas the remaining 85.6% underwent some form of surgical intervention, circumcision being the mainstay. Just 2.6% progressed to PSCC.
CONCLUSIONS
Clinical management of PeIN can be rationally optimized with excellent outcomes. Circumcision is important. Topical treatments alone are disappointing.
Topics: Carcinoma in Situ; Humans; Lichen Sclerosus et Atrophicus; Male; Penile Neoplasms; Penis; Skin Neoplasms
PubMed: 32705920
DOI: 10.1080/09546634.2020.1800574 -
Urology Jan 2020To elucidate current understanding on the pathophysiological mechanism of genital lichen sclerosus (LS), urologic manifestations, and treatment options.
OBJECTIVE
To elucidate current understanding on the pathophysiological mechanism of genital lichen sclerosus (LS), urologic manifestations, and treatment options.
MATERIALS AND METHODS
The Medline/PubMed and Embase databases were systematically reviewed for publications pertaining to LS. After applying inclusion and exclusion criteria, references were assessed for relevance to the pathophysiology, presentation, and treatment of LS by title and abstract review by 2 independent reviewers, yielding 186 articles for assessment.
RESULTS
The contemporary understanding of the epidemiology and histology of LS is reviewed herein. Additionally, we explore in detail the 3 hypotheses regarding the pathophysiological mechanism contributing to disease presentation: infectious etiology, primary immune dysregulation, and the isotraumatopic response. We summarize the available biological evidence supporting each hypothesis. This discussion provides context for understanding LS morbidity and may spur new avenues of research. For the clinician, we review the clinical presentation of disease, including the risk of progression to squamous cell carcinoma. The current medical and surgical treatment options are also detailed.
CONCLUSION
LS remains a potentially insidious disease which may lead to debilitating urinary and sexual dysfunction. Cross disciplinary research should aim for earlier detection, as well as more effective and durable treatment. The exact cause of LS remains unknown.
Topics: Administration, Topical; Balanitis Xerotica Obliterans; Biopsy; Circumcision, Male; Diagnosis, Differential; Disease Progression; Female; Glucocorticoids; Humans; Lichen Sclerosus et Atrophicus; Male; Penis; Prevalence; Treatment Outcome; Vulva; Vulvar Lichen Sclerosus
PubMed: 31605681
DOI: 10.1016/j.urology.2019.09.034 -
Journal of Endocrinological... Feb 2024Human papillomavirus (HPV) infection is the most common sexually transmitted disease, in males and females worldwide. While the role of HPV in female diseases is well... (Review)
Review
PURPOSE
Human papillomavirus (HPV) infection is the most common sexually transmitted disease, in males and females worldwide. While the role of HPV in female diseases is well known and largely studied, males have negligibly been included in these programs, also because the proportion of women suffering and dying from HPV-related diseases is much larger than men. The aim of this review is to focus on HPV-related diseases in male patients.
METHODS
We performed a literature analysis on the electronic database PubMed. We considered randomized trials, observational and retrospective studies, original articles having as topic the relationship between HPV male infection and the following items: oral, anal penile cancers, warts, condylomas, male infertility, altered sperm parameters, anti-sperm antibodies (ASA). We also included experimental in vitro studies focused on the effects of HPV infection on oocyte fertilization, blastocyst development, and trophoblastic cell invasiveness. In addition, studies describing the adjuvant administration of the HPV vaccination as a possible strategy to promote HPV clearance from semen in infected males were included.
RESULTS
Regarding head and neck HPV-related diseases, the most important non-neoplastic disease is recurrent respiratory papillomatosis (RRP). Regarding neoplastic diseases, the proportion of head and neck cancers attributable to HPV has increased dramatically worldwide. In addition, nowadays, it is thought that half of head and neck squamous cell carcinomas (HNSCCs) cases in the United States are caused by infection with high-risk HPV. HPV is noteworthy in andrological practice too. It was described as having a high HPV prevalence, ranging between 50 and 70%, in male penile shaft, glans penis/coronal sulcus, semen as well as in scrotal, perianal, and anal regions. Moreover, in male patients, HPV infection has been associated, among other diseases, with penile cancers. HPV semen infection has been reported in about 10% in men from the general population and about 16% in men with unexplained infertility, although these data seem widely underestimated according to clinical experience. In particular, HPV semen infection seems to be most related to asthenozoospermia and to anti-sperm antibodies (ASAs).
CONCLUSIONS
HPV infection represents a health problem with a detrimental social and public impact. Despite this evidence, little has been done to date to widely promote vaccination among young males.
Topics: Humans; Male; Female; Papillomavirus Infections; Penile Neoplasms; Semen; Retrospective Studies; Spermatozoa; Antibodies
PubMed: 37770654
DOI: 10.1007/s40618-023-02192-3 -
Wiener Medizinische Wochenschrift (1946) Feb 2021Melanomas of the glans penis, prepuce, and the corpus of the penis account for less than 1% of all melanoma cases. Due to this rarity, there is currently no standard...
BACKGROUND
Melanomas of the glans penis, prepuce, and the corpus of the penis account for less than 1% of all melanoma cases. Due to this rarity, there is currently no standard treatment approach.
CASE DESCRIPTION
We present the case of a 59-year-old patient with tumorous formations in the prepuce area. Histology was lacking at presentation. Medical history contained excision of a single formation with the character of a viral wart in the same area 1 year previously, with electrodesiccation for relapse a few months later. Due to the appearance of new bumps, the patient presented to the Onkoderma polyclinic. Dermatological examination revealed two nodular tumor formations: one with an erosive surface in the area of the prepuce and one subcutaneous lesion in the area of the right cavernous body. Differential diagnosis included Merkel carcinoma, dermatofibrosarcoma protuberans, anaplastic T/B cell lymphoma, and achromatic melanoma. Histology showed epithelioid cell infiltration and immunohistochemistry was positive for S100 protein, HMB 45, and melan A. Achromatic preputial malignant melanoma was diagnosed. The subcutaneous formation in the area of the cavernous body was defined as an in-transit metastasis. Penectomy under spinal anesthesia was planned.
CONCLUSION
The prognosis for penile melanoma patients is currently poor, probably because of delays in diagnosis. Histopathologic evaluation after excision should be standard to prevent possible severe consequences such as penectomy and partial penile amputation.
Topics: Humans; Male; Melanoma; Middle Aged; Neoplasm Recurrence, Local; Penile Neoplasms; Prognosis; Skin Neoplasms
PubMed: 32382854
DOI: 10.1007/s10354-020-00751-4