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Pathology - Penile disorders
Lichen sclerosus • Penile lichen sclerosus (balanitis xerotica obliterans) is an inflammatory condition mostly affecting the foreskin or glans penis. • The majority of cases manifest in adulthood with phimosis. • Macroscopically, the impacted regions have a whitish and atrophic appearance. • Histologically, there is epidermal atrophy and hyperkeratosis accompanied by underlying bands of hyalinized collagen and a persistent inflammatory cell infiltrate.
Lichen planus • Penile involvement frequently occurs in patients with widespread lichen planus (see p. 288). • The lesions frequently affect the glans penis. • Histological examination reveals a band-like inflammatory infiltrate next to the epithelium. The infiltrate frequently comprises plasma cells, whereas cutaneous lesions are predominantly characterized by lymphocytes and macrophages.
Zoon's balanitis typically manifests as a singular erythematous lesion in uncircumcised older males. It clinically resembles penile Bowen's disease. Histological examination reveals epidermal thinning, spongiosis, lozenge-shaped keratinocytes, and a dense band-like inflammatory infiltrate abundant in plasma cells.
Condylomas are induced by an infection with the human papillomavirus (HPV), typically types 6 and 11. Predominantly observed in sexually active young males. • Macroscopically, condylomas manifest as either flat or frond-like papillary excrescences. • Histologically, they exhibit a papillomatous squamous proliferation characterized by koilocytes (keratinocytes exhibiting HPV-induced cytopathic alterations).
Peyronie's disease • Commonly referred to as penile fibromatosis, albeit likely not associated with other kinds of fibromatosis (see p. 318). • Individuals aged 40–60 have thickening of the corpus cavernosa, resulting in penile discomfort and curvature during erection. • Histological analysis of excised tissue reveals hypocellular collagenous scar tissue accompanied by clusters of chronic inflammatory cells.
Penile cancer • Uncommon neoplasm that typically develops on the glans penis of older males. Risk factors including HPV infection, tobacco use, phimosis, and chronic lichen sclerosus. Circumcision correlates with a decreased risk. Macroscopically, they present as exophytic lumps that may undergo ulceration. The majority are histologically classified as squamous cell carcinomas, originating from regions of squamous dysplasia, occasionally referred to as penile intraepithelial neoplasia.
Lichen sclerosus • Penile lichen sclerosus (balanitis xerotica obliterans) is an inflammatory condition mostly affecting the foreskin or glans penis. • The majority of cases manifest in adulthood with phimosis. • Macroscopically, the impacted regions have a whitish and atrophic appearance. • Histologically, there is epidermal atrophy and hyperkeratosis accompanied by underlying bands of hyalinized collagen and a persistent inflammatory cell infiltrate.
Lichen planus • Penile involvement frequently occurs in patients with widespread lichen planus (see p. 288). • The lesions frequently affect the glans penis. • Histological examination reveals a band-like inflammatory infiltrate next to the epithelium. The infiltrate frequently comprises plasma cells, whereas cutaneous lesions are predominantly characterized by lymphocytes and macrophages.
Zoon's balanitis typically manifests as a singular erythematous lesion in uncircumcised older males. It clinically resembles penile Bowen's disease. Histological examination reveals epidermal thinning, spongiosis, lozenge-shaped keratinocytes, and a dense band-like inflammatory infiltrate abundant in plasma cells.
Condylomas are induced by an infection with the human papillomavirus (HPV), typically types 6 and 11. Predominantly observed in sexually active young males. • Macroscopically, condylomas manifest as either flat or frond-like papillary excrescences. • Histologically, they exhibit a papillomatous squamous proliferation characterized by koilocytes (keratinocytes exhibiting HPV-induced cytopathic alterations).
Peyronie's disease • Commonly referred to as penile fibromatosis, albeit likely not associated with other kinds of fibromatosis (see p. 318). • Individuals aged 40–60 have thickening of the corpus cavernosa, resulting in penile discomfort and curvature during erection. • Histological analysis of excised tissue reveals hypocellular collagenous scar tissue accompanied by clusters of chronic inflammatory cells.
Penile cancer • Uncommon neoplasm that typically develops on the glans penis of older males. Risk factors including HPV infection, tobacco use, phimosis, and chronic lichen sclerosus. Circumcision correlates with a decreased risk. Macroscopically, they present as exophytic lumps that may undergo ulceration. The majority are histologically classified as squamous cell carcinomas, originating from regions of squamous dysplasia, occasionally referred to as penile intraepithelial neoplasia.
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Pathology - Urethral disorders
Urethritis • Typically induced by sexually transmitted infections. • Categorized as gonococcal and non-gonococcal urethritis. • Non-gonococcal urethritis is more prevalent, with the majority of cases attributed to C. trachomatis. Patients commonly report a sense of urethral pruritus. Gonococcal urethritis results from infection with Neisseria gonorrhoeae. Patients typically exhibit a more purulent exudate and dysuria. Gram staining of urethral discharge can identify N. gonorrhoeae as intracellular Gram-negative diplococci. If these organisms remain undetected, however a significant presence of neutrophils confirms urethritis, non-gonococcal urethritis is inferred. Detection of C. trachomatis is typically accomplished by molecular techniques, as culture is both slow and inaccurate.
Prostatic urethral polyp • Lesion of the prostatic urethra comprising prostatic epithelium. • Commonly manifests as haematuria. • Morphologically, they are papillary lesions protruding into the prostatic urethra. • Histologically, they consist of densely packed prostatic-type glands enveloped by urothelium.
Urethral caruncle • A rather prevalent polypoid lesion located in the distal urethra of women. • Exhibits dysuria and intermittent bleeding. • The caruncle appears as a polypoid mass at the urethral meatus. • Histological examination reveals a dense infiltrate of inflammatory cells abundant in blood vessels, covered by hyperplastic epithelium.
Urethral carcinomas • These neoplasms are infrequent, yet more prevalent in females. • Typically diagnosed at an advanced stage with a dismal prognosis. • The majority are squamous cell carcinomas (70%) originating in the distal urethra near the meatus. • The remaining cases consist of urothelial carcinomas (20%) or adenocarcinomas (10%), typically originating in the proximal urethra.
Malignant melanoma is uncommon, however thoroughly documented in the urethra. • They manifest as polypoid or ulcerated masses in the urethra. Histologically, they consist of aberrant epithelioid or spindle-shaped cells. Often amelanotic, which may result in diagnostic challenges. The immunohistochemical reactivity of malignant cells for melanocytic markers (S100, HMB-45, Melan-A) aids in confirming the diagnosis.
Urethritis • Typically induced by sexually transmitted infections. • Categorized as gonococcal and non-gonococcal urethritis. • Non-gonococcal urethritis is more prevalent, with the majority of cases attributed to C. trachomatis. Patients commonly report a sense of urethral pruritus. Gonococcal urethritis results from infection with Neisseria gonorrhoeae. Patients typically exhibit a more purulent exudate and dysuria. Gram staining of urethral discharge can identify N. gonorrhoeae as intracellular Gram-negative diplococci. If these organisms remain undetected, however a significant presence of neutrophils confirms urethritis, non-gonococcal urethritis is inferred. Detection of C. trachomatis is typically accomplished by molecular techniques, as culture is both slow and inaccurate.
Prostatic urethral polyp • Lesion of the prostatic urethra comprising prostatic epithelium. • Commonly manifests as haematuria. • Morphologically, they are papillary lesions protruding into the prostatic urethra. • Histologically, they consist of densely packed prostatic-type glands enveloped by urothelium.
Urethral caruncle • A rather prevalent polypoid lesion located in the distal urethra of women. • Exhibits dysuria and intermittent bleeding. • The caruncle appears as a polypoid mass at the urethral meatus. • Histological examination reveals a dense infiltrate of inflammatory cells abundant in blood vessels, covered by hyperplastic epithelium.
Urethral carcinomas • These neoplasms are infrequent, yet more prevalent in females. • Typically diagnosed at an advanced stage with a dismal prognosis. • The majority are squamous cell carcinomas (70%) originating in the distal urethra near the meatus. • The remaining cases consist of urothelial carcinomas (20%) or adenocarcinomas (10%), typically originating in the proximal urethra.
Malignant melanoma is uncommon, however thoroughly documented in the urethra. • They manifest as polypoid or ulcerated masses in the urethra. Histologically, they consist of aberrant epithelioid or spindle-shaped cells. Often amelanotic, which may result in diagnostic challenges. The immunohistochemical reactivity of malignant cells for melanocytic markers (S100, HMB-45, Melan-A) aids in confirming the diagnosis.
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Pathology - Vaginal neoplasms
Vaginal carcinoma • Rare relative to cervical and vulvar carcinomas. • Predominantly squamous cell carcinomas originating from a precursor dysplastic lesion termed vaginal intraepithelial neoplasia (VAIN).
• Risk factors encompass HPV infection, tobacco use, and immunosuppression. • Prognosis is typically unfavorable, with a 5-year survival rate of approximately 60%. Fibroepithelial stromal polyp • Benign lesion of the distal female genital system, primarily affecting the vagina, but may also occur in the vulva. • Hormonal-responsive lesions manifesting in women of reproductive age as tiny polypoid masses.
• Histologically, they consist of a central fibrovascular core enveloped by hyperplastic squamous epithelium. Stellate and multinucleate stromal cells are generally observed within the core next to the epithelial surface.
Genital rhabdomyoma • A benign tumor exhibiting skeletal muscle differentiation, predominantly found in the vagina. • Typically manifests in middle-aged women with symptoms associated with a mass lesion. • Histologically characterized by a disorganized proliferation of spindle cells featuring abundant, brightly eosinophilic cytoplasm with cross-striations.
Embryonal rhabdomyosarcoma is a malignant tumor exhibiting skeletal muscle differentiation, which may develop in the vagina of children. The majority of cases occur in children under 5 years of age, presenting with vaginal hemorrhage. A tumor may be observed protruding through the vaginal entrance. • Macroscopically, the tumor consists of edematous polypoid nodules protruding from the vaginal wall. • Histologically, the tumor comprises tiny round and spindle-shaped cells aggregated beneath the squamous epithelium of the vaginal wall. Certain tumor cells have prominently eosinophilic cytoplasm, with potential visibility of cytoplasmic cross-striations. The prognosis post-treatment is typically outstanding, with 10-year survival rates above 90%.
Vaginal carcinoma • Rare relative to cervical and vulvar carcinomas. • Predominantly squamous cell carcinomas originating from a precursor dysplastic lesion termed vaginal intraepithelial neoplasia (VAIN).
• Risk factors encompass HPV infection, tobacco use, and immunosuppression. • Prognosis is typically unfavorable, with a 5-year survival rate of approximately 60%. Fibroepithelial stromal polyp • Benign lesion of the distal female genital system, primarily affecting the vagina, but may also occur in the vulva. • Hormonal-responsive lesions manifesting in women of reproductive age as tiny polypoid masses.
• Histologically, they consist of a central fibrovascular core enveloped by hyperplastic squamous epithelium. Stellate and multinucleate stromal cells are generally observed within the core next to the epithelial surface.
Genital rhabdomyoma • A benign tumor exhibiting skeletal muscle differentiation, predominantly found in the vagina. • Typically manifests in middle-aged women with symptoms associated with a mass lesion. • Histologically characterized by a disorganized proliferation of spindle cells featuring abundant, brightly eosinophilic cytoplasm with cross-striations.
Embryonal rhabdomyosarcoma is a malignant tumor exhibiting skeletal muscle differentiation, which may develop in the vagina of children. The majority of cases occur in children under 5 years of age, presenting with vaginal hemorrhage. A tumor may be observed protruding through the vaginal entrance. • Macroscopically, the tumor consists of edematous polypoid nodules protruding from the vaginal wall. • Histologically, the tumor comprises tiny round and spindle-shaped cells aggregated beneath the squamous epithelium of the vaginal wall. Certain tumor cells have prominently eosinophilic cytoplasm, with potential visibility of cytoplasmic cross-striations. The prognosis post-treatment is typically outstanding, with 10-year survival rates above 90%.
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Pathology -Vaginal infections
Bacterial vaginosis is the most prevalent cause of abnormal vaginal discharge, resulting from the proliferation of anaerobic bacteria, including Gardnerella vaginalis and Bacteroides species. • The metabolic byproducts of these bacteria comprise volatile amines that provide a characteristic fishy odor to the discharge. • There is an absence of true inflammation in the vaginal wall, so the term vaginosis is utilized instead of vaginitis.
Vulvovaginal candidiasis, commonly referred to as 'thrush.'• A prevalent infection in young women caused by Candida albicans. The usual manifestation is vulvovaginal pruritus and burning, dyspareunia, and dysuria. A copious white discharge is prevalent. • Wet mount microscopy of the specimen reveals fungal pseudohyphae. The organism can additionally be cultivated in the microbiology laboratory. • Persistent Candida infections may indicate underlying diabetes mellitus or immunosuppression.
Trichomoniasis • A sexually transmitted infection caused by the flagellate protozoan, Trichomonas vaginalis. • The male partner is often asymptomatic, and around fifty percent of infected women are likewise asymptomatic. Women exhibiting symptoms typically report vaginal pruritus and a thin, frothy, malodorous discharge. Dyspareunia and dysuria may also manifest. • Wet mount microscopy of the discharge reveals motile trichomonads
Bacterial vaginosis is the most prevalent cause of abnormal vaginal discharge, resulting from the proliferation of anaerobic bacteria, including Gardnerella vaginalis and Bacteroides species. • The metabolic byproducts of these bacteria comprise volatile amines that provide a characteristic fishy odor to the discharge. • There is an absence of true inflammation in the vaginal wall, so the term vaginosis is utilized instead of vaginitis.
Vulvovaginal candidiasis, commonly referred to as 'thrush.'• A prevalent infection in young women caused by Candida albicans. The usual manifestation is vulvovaginal pruritus and burning, dyspareunia, and dysuria. A copious white discharge is prevalent. • Wet mount microscopy of the specimen reveals fungal pseudohyphae. The organism can additionally be cultivated in the microbiology laboratory. • Persistent Candida infections may indicate underlying diabetes mellitus or immunosuppression.
Trichomoniasis • A sexually transmitted infection caused by the flagellate protozoan, Trichomonas vaginalis. • The male partner is often asymptomatic, and around fifty percent of infected women are likewise asymptomatic. Women exhibiting symptoms typically report vaginal pruritus and a thin, frothy, malodorous discharge. Dyspareunia and dysuria may also manifest. • Wet mount microscopy of the discharge reveals motile trichomonads
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Pathology - Vaginal neoplasms
Vaginal carcinoma • Rare relative to cervical and vulvar carcinomas. • Predominantly squamous cell carcinomas originating from a precursor dysplastic lesion termed vaginal intraepithelial neoplasia (VAIN).
• Risk factors encompass HPV infection, tobacco use, and immunosuppression. • Prognosis is typically unfavorable, with a 5-year survival rate of approximately 60%. Fibroepithelial stromal polyp • Benign lesion of the distal female genital system, primarily affecting the vagina, but may also occur in the vulva. • Hormonal-responsive lesions manifesting in women of reproductive age as tiny polypoid masses.
• Histologically, they consist of a central fibrovascular core enveloped by hyperplastic squamous epithelium. Stellate and multinucleate stromal cells are generally observed within the core next to the epithelial surface.
Genital rhabdomyoma • A benign tumor exhibiting skeletal muscle differentiation, predominantly found in the vagina. • Typically manifests in middle-aged women with symptoms associated with a mass lesion. • Histologically characterized by a disorganized proliferation of spindle cells featuring abundant, brightly eosinophilic cytoplasm with cross-striations.
Embryonal rhabdomyosarcoma is a malignant tumor exhibiting skeletal muscle differentiation, which may develop in the vagina of children. The majority of cases occur in children under 5 years of age, presenting with vaginal hemorrhage. A tumor may be observed protruding through the vaginal entrance. • Macroscopically, the tumor consists of edematous polypoid nodules protruding from the vaginal wall. • Histologically, the tumor comprises tiny round and spindle-shaped cells aggregated beneath the squamous epithelium of the vaginal wall. Certain tumor cells have prominently eosinophilic cytoplasm, with potential visibility of cytoplasmic cross-striations. The prognosis post-treatment is typically outstanding, with 10-year survival rates above 90%.
Vaginal carcinoma • Rare relative to cervical and vulvar carcinomas. • Predominantly squamous cell carcinomas originating from a precursor dysplastic lesion termed vaginal intraepithelial neoplasia (VAIN).
• Risk factors encompass HPV infection, tobacco use, and immunosuppression. • Prognosis is typically unfavorable, with a 5-year survival rate of approximately 60%. Fibroepithelial stromal polyp • Benign lesion of the distal female genital system, primarily affecting the vagina, but may also occur in the vulva. • Hormonal-responsive lesions manifesting in women of reproductive age as tiny polypoid masses.
• Histologically, they consist of a central fibrovascular core enveloped by hyperplastic squamous epithelium. Stellate and multinucleate stromal cells are generally observed within the core next to the epithelial surface.
Genital rhabdomyoma • A benign tumor exhibiting skeletal muscle differentiation, predominantly found in the vagina. • Typically manifests in middle-aged women with symptoms associated with a mass lesion. • Histologically characterized by a disorganized proliferation of spindle cells featuring abundant, brightly eosinophilic cytoplasm with cross-striations.
Embryonal rhabdomyosarcoma is a malignant tumor exhibiting skeletal muscle differentiation, which may develop in the vagina of children. The majority of cases occur in children under 5 years of age, presenting with vaginal hemorrhage. A tumor may be observed protruding through the vaginal entrance. • Macroscopically, the tumor consists of edematous polypoid nodules protruding from the vaginal wall. • Histologically, the tumor comprises tiny round and spindle-shaped cells aggregated beneath the squamous epithelium of the vaginal wall. Certain tumor cells have prominently eosinophilic cytoplasm, with potential visibility of cytoplasmic cross-striations. The prognosis post-treatment is typically outstanding, with 10-year survival rates above 90%.
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Pathology - Urothelial carcinomas
Definition • A category of neoplasms originating in the urothelial tract. Equivalent term • Transitional cell carcinomas.
Epidemiology • Prevalent with more than 250,000 new cases globally. • Can manifest throughout the urothelial system, with the predominant occurrence in the bladder, followed by the renal pelvis and ureters (50:3:1, respectively).
Bladder tumors are likely more prevalent because to carcinogens traversing the urine tract residing in the bladder for extended periods.
Aetiology • Tobacco consumption through cigarettes. • Professional exposure to aromatic amines. Genetics • Non-invasive low-grade papillary urothelial carcinomas exhibit little genetic modifications, with the predominant change being deletions of chromosome 9. Non-invasive high-grade papillary urothelial carcinomas, urothelial carcinoma in situ, and infiltrating urothelial carcinomas exhibit genetic instability characterized by several chromosomal aberrations, including mutations in TP53 and RB genes.
Presentation: • The majority present with hematuria.
Macroscopy Non-invasive papillary tumors often develop as frond-like projections from the urothelial mucosa. Infiltrating urothelial carcinomas generally present as solid masses that invade adjacent tissues. Urothelial carcinoma in situ is a flat lesion that may be macroscopically undetectable or present as an erythematous mucosal region.
Urinary cytopathology • Insufficient sensitivity in detecting low-grade urothelial carcinomas. • Proficient in identifying high-grade lesions characterized by significantly abnormal urothelial cells exhibiting big pleomorphic nuclei with black, coarsely granular chromatin. Histopathology Non-invasive low-grade papillary urothelial carcinoma consists of slender papillae bordered by urothelium with minor disorganization and low-grade nuclear atypia. Non-invasive high-grade papillary urothelial carcinoma consists of branching, fused papillae bordered by urothelium exhibiting significant disorganization and pronounced nuclear atypia.
Urothelial carcinoma in situ is a planar lesion characterized by distinctly high-grade nuclear atypia in the urothelium. Infiltrating urothelial carcinoma is a urothelial tumor that has breached the basement membrane. This group is not officially categorized into low and high grade, however the majority have high-grade nuclear atypia.
Prognosis: All have a propensity for multifocality and recurrence. Non-invasive low-grade papillary urothelial carcinomas present a minimal risk of progression to invasion and mortality (<5%). non-invasive high-grade papillary urothelial carcinomas and carcinoma in situ possess a significantly elevated risk of progression. the prognosis infiltrating primarily depends on stage disease. lymphovascular invasion is an unfavorable prognostic indicator.< />pan>
TNM 7 pathological staging of bladder carcinomas
Primary tumour (T)
pTa: non-invasive tumour.
pT1: tumour invades the lamina propria.
pT2: tumour invades the muscularis propria.
pT3: tumour invades perivesical tissue.
pT4: tumour invades adjacent organs (prostate, uterus, vagina, pelvic wall).
Regional lymph nodes (N)
pN1: single positive node in the primary drainage region.
pN2: multiple positive nodes in the primary drainage region.
pN3: common iliac node involvement.
TNM 7 pathological staging of renal pelvis and ureter
carcinomas
Primary tumour (T)
pTa: non-invasive tumour.
Definition • A category of neoplasms originating in the urothelial tract. Equivalent term • Transitional cell carcinomas.
Epidemiology • Prevalent with more than 250,000 new cases globally. • Can manifest throughout the urothelial system, with the predominant occurrence in the bladder, followed by the renal pelvis and ureters (50:3:1, respectively).
Bladder tumors are likely more prevalent because to carcinogens traversing the urine tract residing in the bladder for extended periods.
Aetiology • Tobacco consumption through cigarettes. • Professional exposure to aromatic amines. Genetics • Non-invasive low-grade papillary urothelial carcinomas exhibit little genetic modifications, with the predominant change being deletions of chromosome 9. Non-invasive high-grade papillary urothelial carcinomas, urothelial carcinoma in situ, and infiltrating urothelial carcinomas exhibit genetic instability characterized by several chromosomal aberrations, including mutations in TP53 and RB genes.
Presentation: • The majority present with hematuria.
Macroscopy Non-invasive papillary tumors often develop as frond-like projections from the urothelial mucosa. Infiltrating urothelial carcinomas generally present as solid masses that invade adjacent tissues. Urothelial carcinoma in situ is a flat lesion that may be macroscopically undetectable or present as an erythematous mucosal region.
Urinary cytopathology • Insufficient sensitivity in detecting low-grade urothelial carcinomas. • Proficient in identifying high-grade lesions characterized by significantly abnormal urothelial cells exhibiting big pleomorphic nuclei with black, coarsely granular chromatin. Histopathology Non-invasive low-grade papillary urothelial carcinoma consists of slender papillae bordered by urothelium with minor disorganization and low-grade nuclear atypia. Non-invasive high-grade papillary urothelial carcinoma consists of branching, fused papillae bordered by urothelium exhibiting significant disorganization and pronounced nuclear atypia.
Urothelial carcinoma in situ is a planar lesion characterized by distinctly high-grade nuclear atypia in the urothelium. Infiltrating urothelial carcinoma is a urothelial tumor that has breached the basement membrane. This group is not officially categorized into low and high grade, however the majority have high-grade nuclear atypia.
Prognosis: All have a propensity for multifocality and recurrence. Non-invasive low-grade papillary urothelial carcinomas present a minimal risk of progression to invasion and mortality (<5%). non-invasive high-grade papillary urothelial carcinomas and carcinoma in situ possess a significantly elevated risk of progression. the prognosis infiltrating primarily depends on stage disease. lymphovascular invasion is an unfavorable prognostic indicator.< />pan>
TNM 7 pathological staging of bladder carcinomas
Primary tumour (T)
pTa: non-invasive tumour.
pT1: tumour invades the lamina propria.
pT2: tumour invades the muscularis propria.
pT3: tumour invades perivesical tissue.
pT4: tumour invades adjacent organs (prostate, uterus, vagina, pelvic wall).
Regional lymph nodes (N)
pN1: single positive node in the primary drainage region.
pN2: multiple positive nodes in the primary drainage region.
pN3: common iliac node involvement.
TNM 7 pathological staging of renal pelvis and ureter
carcinomas
Primary tumour (T)
pTa: non-invasive tumour.
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Pathology - Pediatric kidney neoplasms
Nephroblastoma (Wilms' tumor) is a malignant kidney neoplasm occurring in children. • The second most prevalent pediatric cancer, occurring at an incidence of approximately 1 in 8,000. • The majority of children aged 2 to 5 years exhibit an abdominal mass. Macroscopically, they are distinctly defined tumors with a grey or tan hue. Histologically, the majority of nephroblastomas comprise a combination of undifferentiated small round blue cells (blastema) and regions of highly differentiated epithelial and stromal elements, referred to as 'triphasic' tumors. Most nephroblastomas are classified as low stage with favorable histology, resulting in an excellent prognosis following treatment. Approximately 5% of cases have unfavorable histology, marked by nuclear anaplasia or the presence of multipolar mitotic figures; these instances are linked to a negative prognosis.
Clear cell sarcoma -A rare pediatric renal sarcoma with a significant tendency to metastasis to bone. • The majority of youngsters present between the ages of 1 and 2 years. Macroscopically, they are generally substantial tumors located in the renal medulla. • Histologically, the typical pattern consists of nests or cords of cells interspersed with fibrovascular septa.
Rhabdoid tumor: A rare, extremely aggressive kidney neoplasm occurring in young children. • Typically manifests at one year of age with either hematuria or symptoms indicative of disseminated illness. The tumors are big and infiltrative, with patches of necrosis. The malignant cells exhibit vesicular chromatin, large cherry-red nucleoli, and hyaline pink intracytoplasmic inclusions histologically. Significant vascular invasion is typically apparent. • The prognosis is exceedingly unfavorable, with fatality rates exceeding 80% within two years following diagnosis.
Congenital mesoblastic nephroma • A low-grade fibrolastic renal sarcoma occurring in pediatric patients. • It may be identified via prenatal ultrasound or manifest within the first year of life as an abdominal tumor. • The tumor is predominantly located in the renal sinus and exhibits either a firm whorled morphology or a softer cystic cut surface. Histologically, two forms are identified: a 'classic' type consisting of fascicles of benign spindle cells and a 'cellular' type including sheets of densely packed rounded cells. • The prognosis is often outstanding when the tumor is entirely removed with nephrectomy.
Nephroblastoma (Wilms' tumor) is a malignant kidney neoplasm occurring in children. • The second most prevalent pediatric cancer, occurring at an incidence of approximately 1 in 8,000. • The majority of children aged 2 to 5 years exhibit an abdominal mass. Macroscopically, they are distinctly defined tumors with a grey or tan hue. Histologically, the majority of nephroblastomas comprise a combination of undifferentiated small round blue cells (blastema) and regions of highly differentiated epithelial and stromal elements, referred to as 'triphasic' tumors. Most nephroblastomas are classified as low stage with favorable histology, resulting in an excellent prognosis following treatment. Approximately 5% of cases have unfavorable histology, marked by nuclear anaplasia or the presence of multipolar mitotic figures; these instances are linked to a negative prognosis.
Clear cell sarcoma -A rare pediatric renal sarcoma with a significant tendency to metastasis to bone. • The majority of youngsters present between the ages of 1 and 2 years. Macroscopically, they are generally substantial tumors located in the renal medulla. • Histologically, the typical pattern consists of nests or cords of cells interspersed with fibrovascular septa.
Rhabdoid tumor: A rare, extremely aggressive kidney neoplasm occurring in young children. • Typically manifests at one year of age with either hematuria or symptoms indicative of disseminated illness. The tumors are big and infiltrative, with patches of necrosis. The malignant cells exhibit vesicular chromatin, large cherry-red nucleoli, and hyaline pink intracytoplasmic inclusions histologically. Significant vascular invasion is typically apparent. • The prognosis is exceedingly unfavorable, with fatality rates exceeding 80% within two years following diagnosis.
Congenital mesoblastic nephroma • A low-grade fibrolastic renal sarcoma occurring in pediatric patients. • It may be identified via prenatal ultrasound or manifest within the first year of life as an abdominal tumor. • The tumor is predominantly located in the renal sinus and exhibits either a firm whorled morphology or a softer cystic cut surface. Histologically, two forms are identified: a 'classic' type consisting of fascicles of benign spindle cells and a 'cellular' type including sheets of densely packed rounded cells. • The prognosis is often outstanding when the tumor is entirely removed with nephrectomy.
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Pathology - Renal cell carcinoma
Definition: A malignant epithelial tumor originating in the kidney.
Epidemiology • Represents approximately 2% of all cancers globally. • Prevalent in developed nations, with an average incidence of approximately 10 per 100,000 in males and 3 per 100,000 in females.
Aetiology • Identified risk factors encompass smoking, hypertension, obesity, environmental toxins, and prolonged dialysis. Certain genetic disorders, such as von Hippel-Lindau and tuberous sclerosis, are linked to renal cell carcinoma (RCC). Genetics Clear cell renal cell carcinomas exhibit deletions on chromosome 3p. Papillary RCCs exhibit trisomy of chromosomes 7 and 17, together with the deletion of chromosomal Y in males. Chromophobe RCCs exhibit significant chromosomal deletions.
Presentation • Approximately fifty percent of all cases manifest as painless hematuria. • The majority of the remaining cases are detected incidentally by imaging. A minor percentage exhibits metastatic illness.
Macroscopy The tumors are typically located in the renal cortex. • The clear cell types exhibit heterogeneity, characterized by golden yellow regions. • Papillary tumors may exhibit friability. • Chromophobe tumors are often solid and brown-hued. Histopathology transparent cell RCC (70%) consists of cells exhibiting transparent or eosinophilic cytoplasm arranged within a fine vascular framework. • Papillary RCC (15%) exhibits a papillary or tubulopapillary development pattern and measures greater than 5 mm in size. Chromophobe RCC (5%) consists of layers of big cells characterized by well-defined cell boundaries. The tumor's vasculature is characterized by thick walls. The remaining components consist of a combination of uncommon subtypes, including collecting duct carcinoma and mucinous tubular and spindle cell carcinoma.
Prognosis • The overall five-year survival rate is around 60%. • Stage and grade are the primary prognostic determinants. • The prevalent grading system utilized is the four-tiered Fuhrman system, which assesses nuclear size, shape, chromatin structure, and nucleolar prominence. Grade 1 exhibits the most favorable outlook, while grade 4 demonstrates the least favorable.
TNM 7 pathological staging of renal cell carcinomas
Primary tumour (T)
pT1a: tumour d 4cm, limited to the kidney.
pT1b: tumour > 4cm, but d 7cm, limited to the kidney.
pT2a: tumour > 7cm, but d 10cm, limited to the kidney.
pT2b: tumour > 10cm, limited to the kidney.
pT3a: tumour extends into perinephric fat or renal veins.
pT3b: tumour extends into the vena cava below the diaphragm.
pT3c: tumour extends into the vena cava above the diaphragm.
pT4: tumour directly invades the adrenal gland or beyond the Gerota fascia.
Regional lymph nodes (N)
pN0: no regional lymph node metastasis.
pN1: metastasis in regional lymph node
Definition: A malignant epithelial tumor originating in the kidney.
Epidemiology • Represents approximately 2% of all cancers globally. • Prevalent in developed nations, with an average incidence of approximately 10 per 100,000 in males and 3 per 100,000 in females.
Aetiology • Identified risk factors encompass smoking, hypertension, obesity, environmental toxins, and prolonged dialysis. Certain genetic disorders, such as von Hippel-Lindau and tuberous sclerosis, are linked to renal cell carcinoma (RCC). Genetics Clear cell renal cell carcinomas exhibit deletions on chromosome 3p. Papillary RCCs exhibit trisomy of chromosomes 7 and 17, together with the deletion of chromosomal Y in males. Chromophobe RCCs exhibit significant chromosomal deletions.
Presentation • Approximately fifty percent of all cases manifest as painless hematuria. • The majority of the remaining cases are detected incidentally by imaging. A minor percentage exhibits metastatic illness.
Macroscopy The tumors are typically located in the renal cortex. • The clear cell types exhibit heterogeneity, characterized by golden yellow regions. • Papillary tumors may exhibit friability. • Chromophobe tumors are often solid and brown-hued. Histopathology transparent cell RCC (70%) consists of cells exhibiting transparent or eosinophilic cytoplasm arranged within a fine vascular framework. • Papillary RCC (15%) exhibits a papillary or tubulopapillary development pattern and measures greater than 5 mm in size. Chromophobe RCC (5%) consists of layers of big cells characterized by well-defined cell boundaries. The tumor's vasculature is characterized by thick walls. The remaining components consist of a combination of uncommon subtypes, including collecting duct carcinoma and mucinous tubular and spindle cell carcinoma.
Prognosis • The overall five-year survival rate is around 60%. • Stage and grade are the primary prognostic determinants. • The prevalent grading system utilized is the four-tiered Fuhrman system, which assesses nuclear size, shape, chromatin structure, and nucleolar prominence. Grade 1 exhibits the most favorable outlook, while grade 4 demonstrates the least favorable.
TNM 7 pathological staging of renal cell carcinomas
Primary tumour (T)
pT1a: tumour d 4cm, limited to the kidney.
pT1b: tumour > 4cm, but d 7cm, limited to the kidney.
pT2a: tumour > 7cm, but d 10cm, limited to the kidney.
pT2b: tumour > 10cm, limited to the kidney.
pT3a: tumour extends into perinephric fat or renal veins.
pT3b: tumour extends into the vena cava below the diaphragm.
pT3c: tumour extends into the vena cava above the diaphragm.
pT4: tumour directly invades the adrenal gland or beyond the Gerota fascia.
Regional lymph nodes (N)
pN0: no regional lymph node metastasis.
pN1: metastasis in regional lymph node
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Pathology - Non-malignant kidney neoplasms
Papillary adenoma • Benign renal epithelial neoplasm exhibiting a papillary or tubulopapillary structure, measuring 5mm or less. • Commonly identified accidentally in nephrectomy specimens or during autopsy. Macroscopically, they are well-defined cortical nodules measuring 5 mm or smaller. Histologically, they consist of non-atypical epithelial cells exhibiting papillary or tubulopapillary arrangements.
Oncocytoma • Benign oncocytic renal epithelial tumor typically identified incidentally in adults. • The majority of instances are sporadic; nevertheless, certain occurrences are linked to hereditary abnormalities (e.g., Birt–Hogg–Dubé syndrome). Macroscopically, they are well-defined tumors exhibiting a mahogany brown hue, frequently accompanied by core scarring. Histologically, they are distinguished by cells possessing rich granular eosinophilic cytoplasm, proliferating in nests inside an edematous stroma. Oncocytomas are benign tumors that lack the ability for metastatic dissemination. Nonetheless, substantial tumors are frequently resected. • The infiltration of tumor cells into perinephric fat may occur, and this has no detrimental effect.
Angiomyolipoma • A benign mesenchymal tumor of the kidney consisting of varying quantities of adipose tissue, smooth muscle, and robust blood arteries. • Predominantly occurring sporadically in adults, a minor percentage is linked to tuberous sclerosis. These are more likely to be many and bilateral. • While most are discovered inadvertently, they may occasionally manifest with flank pain resulting from hemorrhage into the tumor. Macroscopically, they present as lobulated kidney masses that may exhibit a yellow appearance if their fat content is elevated. Histologically, they consist of a combination of adipose tissue, smooth muscle bundles, and thick-walled blood arteries in varying proportions.
Cystic nephroma • Benign cystic renal tumor with a significant preference for women. • Macroscopically, they are enclosed multicystic lesions devoid of a solid component. The cysts are microscopically bordered by a singular layer of flattened cuboidal epithelial cells. The septa may exhibit cellular regions that resemble ovarian stroma, and notably, the nuclei of the cells within the septa frequently respond to antibodies targeting estrogen and progesterone receptors
Leiomyoma • Benign leiomyomas typically originating from the renal capsule. • The majority manifest in adults as incidental, tiny, well-defined capsular tumors. • Histologically, they exhibit bundles of unremarkable smooth muscle cells.
Renomedullary interstitial cell tumor • Frequently observed benign renal tumors that are sometimes discovered inadvertently during autopsy examinations of the kidneys. Macroscopically, they are little (1–5mm) white nodules located at the center of a medullary pyramid. Histologically, they consist of tiny stellate or polygonal cells embedded in a loose stroma. Entrapped tubules may be located near the periphery of the lesion.
Papillary adenoma • Benign renal epithelial neoplasm exhibiting a papillary or tubulopapillary structure, measuring 5mm or less. • Commonly identified accidentally in nephrectomy specimens or during autopsy. Macroscopically, they are well-defined cortical nodules measuring 5 mm or smaller. Histologically, they consist of non-atypical epithelial cells exhibiting papillary or tubulopapillary arrangements.
Oncocytoma • Benign oncocytic renal epithelial tumor typically identified incidentally in adults. • The majority of instances are sporadic; nevertheless, certain occurrences are linked to hereditary abnormalities (e.g., Birt–Hogg–Dubé syndrome). Macroscopically, they are well-defined tumors exhibiting a mahogany brown hue, frequently accompanied by core scarring. Histologically, they are distinguished by cells possessing rich granular eosinophilic cytoplasm, proliferating in nests inside an edematous stroma. Oncocytomas are benign tumors that lack the ability for metastatic dissemination. Nonetheless, substantial tumors are frequently resected. • The infiltration of tumor cells into perinephric fat may occur, and this has no detrimental effect.
Angiomyolipoma • A benign mesenchymal tumor of the kidney consisting of varying quantities of adipose tissue, smooth muscle, and robust blood arteries. • Predominantly occurring sporadically in adults, a minor percentage is linked to tuberous sclerosis. These are more likely to be many and bilateral. • While most are discovered inadvertently, they may occasionally manifest with flank pain resulting from hemorrhage into the tumor. Macroscopically, they present as lobulated kidney masses that may exhibit a yellow appearance if their fat content is elevated. Histologically, they consist of a combination of adipose tissue, smooth muscle bundles, and thick-walled blood arteries in varying proportions.
Cystic nephroma • Benign cystic renal tumor with a significant preference for women. • Macroscopically, they are enclosed multicystic lesions devoid of a solid component. The cysts are microscopically bordered by a singular layer of flattened cuboidal epithelial cells. The septa may exhibit cellular regions that resemble ovarian stroma, and notably, the nuclei of the cells within the septa frequently respond to antibodies targeting estrogen and progesterone receptors
Leiomyoma • Benign leiomyomas typically originating from the renal capsule. • The majority manifest in adults as incidental, tiny, well-defined capsular tumors. • Histologically, they exhibit bundles of unremarkable smooth muscle cells.
Renomedullary interstitial cell tumor • Frequently observed benign renal tumors that are sometimes discovered inadvertently during autopsy examinations of the kidneys. Macroscopically, they are little (1–5mm) white nodules located at the center of a medullary pyramid. Histologically, they consist of tiny stellate or polygonal cells embedded in a loose stroma. Entrapped tubules may be located near the periphery of the lesion.
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Pathology - Cystic renal disorders
Autosomal dominant polycystic kidney disease The predominant cystic renal disease, occurring in approximately 1 in 500 individuals, is a primary contributor to end-stage renal failure. • Ninety percent of cases result from a hereditary mutation in the PKD1 gene located on chromosome 16. Deficiencies in the functionality of the PKD1 protein result in cystic alterations in renal tubules and the degeneration of normal renal tissue. Most patients often present in adulthood, generally between the ages of 30 and 40, exhibiting hypertension, flank pain, and hematuria. The kidneys are significantly enlarged, exceeding 2 kg in weight, and are entirely substituted by cysts. Histologically, both kidneys have many cysts lined by flattened cuboidal epithelium, with minimal intervening normal renal parenchyma. Extra-renal symptoms encompass hepatic cysts and berry aneurysms. Subarachnoid hemorrhage resulting from a ruptured berry aneurysm is a severe consequence of adult polycystic kidney disease (APKD) and a prevalent cause of abrupt mortality.
Autosomal recessive polycystic kidney disease • A rare, hereditary condition resulting in bilateral polycystic kidneys and congenital hepatic fibrosis. • Induced by mutations in the PKHD1 gene located on chromosome 6p, which encodes the protein fibrocystin, a constituent of the cilia on collecting duct epithelial cells. • Morphologically, the kidneys are enlarged and exhibit numerous cysts. • Histologically, the cysts are bordered by flattened cuboidal epithelium. • Severe cases result in neonatal mortality due to pulmonary hypoplasia. • Patients with milder renal illness who survive into childhood experience congenital hepatic fibrosis and problems associated with portal hypertension.
Medullary cystic disease • Congenital occurrence of several cysts at the corticomedullary junction, ranging in size from less than 1 mm to 2 cm. The pediatric condition, juvenile nephronophthisis, is inherited in an autosomal recessive manner and is linked to mutations in the NPH1, NPH2, or NPH3 genes. The adult condition, uraemic medullary cystic disease, is inherited in an autosomal dominant manner and is linked to mutations in the MCDK1 or MCDK2 genes.
Medullary sponge kidney • Characterized by an abnormal expansion of the collecting ducts, resulting in microcystic alterations of the renal medullae and papillae accompanied by calcification. • This condition typically manifests in adulthood with recurrent infections. Acquired renal cystic disease • Formation of many bilateral cortical and medullary cysts in individuals with end-stage renal disease undergoing dialysis. An essential supplementary characteristic is the heightened prevalence of kidney tumors, which are frequently of the papillary variety and may be many.
Autosomal dominant polycystic kidney disease The predominant cystic renal disease, occurring in approximately 1 in 500 individuals, is a primary contributor to end-stage renal failure. • Ninety percent of cases result from a hereditary mutation in the PKD1 gene located on chromosome 16. Deficiencies in the functionality of the PKD1 protein result in cystic alterations in renal tubules and the degeneration of normal renal tissue. Most patients often present in adulthood, generally between the ages of 30 and 40, exhibiting hypertension, flank pain, and hematuria. The kidneys are significantly enlarged, exceeding 2 kg in weight, and are entirely substituted by cysts. Histologically, both kidneys have many cysts lined by flattened cuboidal epithelium, with minimal intervening normal renal parenchyma. Extra-renal symptoms encompass hepatic cysts and berry aneurysms. Subarachnoid hemorrhage resulting from a ruptured berry aneurysm is a severe consequence of adult polycystic kidney disease (APKD) and a prevalent cause of abrupt mortality.
Autosomal recessive polycystic kidney disease • A rare, hereditary condition resulting in bilateral polycystic kidneys and congenital hepatic fibrosis. • Induced by mutations in the PKHD1 gene located on chromosome 6p, which encodes the protein fibrocystin, a constituent of the cilia on collecting duct epithelial cells. • Morphologically, the kidneys are enlarged and exhibit numerous cysts. • Histologically, the cysts are bordered by flattened cuboidal epithelium. • Severe cases result in neonatal mortality due to pulmonary hypoplasia. • Patients with milder renal illness who survive into childhood experience congenital hepatic fibrosis and problems associated with portal hypertension.
Medullary cystic disease • Congenital occurrence of several cysts at the corticomedullary junction, ranging in size from less than 1 mm to 2 cm. The pediatric condition, juvenile nephronophthisis, is inherited in an autosomal recessive manner and is linked to mutations in the NPH1, NPH2, or NPH3 genes. The adult condition, uraemic medullary cystic disease, is inherited in an autosomal dominant manner and is linked to mutations in the MCDK1 or MCDK2 genes.
Medullary sponge kidney • Characterized by an abnormal expansion of the collecting ducts, resulting in microcystic alterations of the renal medullae and papillae accompanied by calcification. • This condition typically manifests in adulthood with recurrent infections. Acquired renal cystic disease • Formation of many bilateral cortical and medullary cysts in individuals with end-stage renal disease undergoing dialysis. An essential supplementary characteristic is the heightened prevalence of kidney tumors, which are frequently of the papillary variety and may be many.
