A Review of the Literature on Extrarenal Retroperitoneal Angiomyolipoma.

BACKGROUND: Extrarenal retroperitoneal angiomyolipomas are rare. AIM: To review the literature. RESULTS: Angiomyolipomas, previously classified as hamartomas, are now classified as benign tumours. Thirty cases of primary retroperitoneal angiomyolipomas have been reported. Diagnosis of the disease upon is based radiological and pathological findings of triphasic features of (a) fat and (b) blood vessels and myoid tissue. Immunohistochemistry tends to be positive for HMB45, MART1, HHF35, calponin, NKI-C3, and CD117. The lesion is common in women. Treatment options have included the following: (a) radical surgical excision of the lesion with renal sparing surgery or radical nephrectomy in cases where malignant tumours could not be excluded and (b) selective embolization of the lesion alone or prior to surgical excision. One case of retroperitoneal angiomyolipoma was reported in a patient 15 years after undergoing radical nephrectomy for angiomyolipoma of kidney and two cases of distant metastases of angiomyolipoma have been reported following radical resection of the tumour. CONCLUSIONS: With the report of two cases of metastases ensuing surgical resection of the primary lesions there is need for academic pathologists to debate and review angiomyolipomas to decide whether to reclassify angiomyolipomas as slow-growing malignant tumours or whether the reported cases of metastases were de novo tumours or metastatic lesions.

Primary Lymphoepithelioma-Like Carcinoma of the Prostate Gland: A Review of the Literature.

Background. Primary lymphoepithelioma-like carcinoma of the prostate gland (PLELCP) is rare with hardly any information on its diagnostic features and biological behaviour. Aim. To review the literature. Method. Various Internet data bases were searched. Literature Review. PLELCP is extremely rare and there are hardly any pictures of the tumour involving the prostate; hence it would appear that clinicians would need to use their knowledge of the microscopic and immunohistochemical characteristics of the tumour in the nasopharynx and urinary bladder as diagnostic aid. PLELCP on microscopy mimics nasopharyngeal LELC. The LELC component of the tumour is characterized by indistinct cytoplasmic borders and a syncytial growth pattern. The stroma may be densely infiltrated by lymphoid cells admixed with some plasma cells and neutrophils and at times prominent infiltration of eosinophils. PLELCPs tend to have adenocarcinoma, either as the only pattern or with additional ductal components or adenosquamous carcinoma. PLELCPs stain positively with PSA, PSAP, AMACR/P504S, EMA, and cytokeratins AE1/AE3, 7, 8, and 20. There is no consensus on treatment of PLECP. The reported prognosis has been poor. Conclusions. PLELCPs should be entered into a multicenter trial to determine the biological behaviour and to find the best treatment option that would improve the prognosis.

A Review of the Literature on Primary Leiomyosarcoma of the Prostate Gland.

Primary leiomyosarcoma of the prostate (PLSOP) is rare, with less than 200 cases reported so far. PLSOPs present with lower urinary tract symptoms, haematuria, and perineal pain; may or may not be associated with a history of previous treatment for adenocarcinoma of prostate by means of radiotherapy and or hormonal treatment; may afflict children and adult male. Examination may reveal benign enlarged prostate and hard enlarged mass. PLSOPs may be diagnosed by histological examination findings of spindle-shaped carcinoma cells in prostate specimens. Immunohistochemical staining tends to be positive for vimentin, CD44, smooth muscle actin, and calponin, focally positive for desmin, and at times positive for keratin. They stain negatively for PSA, S-100, CD34, CD117, and cytokeratin. Cytogenetic study on primary leiomyosarcoma of the prostate gland may show clonal chromosomal rearrangement involving Chromosomes 2, 3, 9, 11, and 19. On the whole the prognosis is poor. Surgery with or without chemotherapy would appear to be the mainstay of treatment for PLSOPs that are operable, but generally there is no consensus opinion on the best therapeutic approach. Most cases of PLSOPs are diagnosed in an advanced stage of the disease. A global multicenter trial is required to find therapies that would improve the prognosis.

Tuberculosis of the Penis: A Review of the Literature.

Background. Tuberculosis of the penis (TBP) is rare. Aim. To review the literature. Method. Various internet data bases were searched. Literature Review. TBP could be primary or secondary, may develop following circumcision performed by a person who had pulmonary Tb, and may be transmitted to the penis from ejaculation, contamination from clothing, or from contact with endometrial secretions, following an earlier pulmonary Tb or Tb elsewhere. TBP presents with a painless/painful small nodule, ulcer, mass on penis which gradually enlarges, and induration/swelling of penis, with or without erectile dysfunction. Inguinal lymph nodes may or may not be palpable. The patient's voiding is normal. There may or may not be history of circumcision, pulmonary Tb, and BCG immunization. TBP mimics penile carcinoma, granulomatous syphilis penile ulcer, genital herpes simplex, granuloma inguinale, and HIV infection. Diagnosis is established by microscopic examination finding of granulomas +/-AFB in penile discharge or biopsy of lesion or culture of Tb organism from discharge or biopsy specimens or positive Elisa serology/PCR for Tb. PTBs respond to first- or 2nd-line anti-Tb 6-month treatment. Close contacts should be screened. Extrapulmonary Tb should be excluded. Conclusions. Clinicians should consider possibility of PTB in cases of penile lesions and erectile failure.

Clear cell adenocarcinoma of the urethra: review of the literature.

BACKGROUND: Clear cell adenocarcinoma of the urethra (CCAU) is extremely rare and a number of clinicians may be unfamiliar with its diagnosis and biological behaviour. AIMS: To review the literature on CCAU. METHODS: Various internet databases were used. RESULTS/LITERATURE REVIEW: (i) CCAU occurs in adults and in women in the great majority of cases. (ii) It has a particular association with urethral diverticulum, which has been present in 56% of the patients; is indistinguishable from clear cell adenocarcinoma of the female genital tract but is not associated with endometriosis; and probably does not arise by malignant transformation of nephrogenic adenoma. (iii) It is usually, readily distinguished from nephrogenic adenoma because of greater cytological a-typicality and mitotic activity and does not stain for prostate-specific antigen or prostatic acid phosphatase. (iv) It has been treated by anterior exenteration in women and cystoprostatectomy in men and at times by radiotherapy; chemotherapy has rarely been given. (v) CCAU is aggressive with low 5-year survival rates. (vi) There is no consensus opinion of treatment options that would improve the prognosis. CONCLUSIONS: Few cases of CCAU have been reported. Urologists, gynaecologists, pathologists, and oncologists should report cases of CCAU they encounter and enter them into a multicentric trial to determine the best treatment options that would improve the prognosis.

Nephrogenic Adenoma of the Urinary Bladder: A Review of the Literature.

Background. Nephrogenic adenoma of the urinary bladder (NAUB) is a rare lesion associated with nonspecific symptoms and could inadvertently be misdiagnosed. Aim. To review the literature. Methods. Various internet search engines were used. Results. NAUB is a benign tubular and papillary lesion of the bladder, is more common in men and adults, and has been associated with chronic inflammation/irritation, previous bladder surgery, diverticula, renal transplantation, and intravesical BCG; recurrences and malignant transformations have been reported. Differential diagnoses include clear cell adenocarcinoma, endocervicosis, papillary urothelial carcinoma, prostatic adenocarcinoma of bladder, and nested variant of urothelial carcinoma; most NAUBs have both surface papillary and submucosal tubular components; both the papillae and tubules tend to be lined by a single layer of mitotically inactive bland cells which have pale to clear cytoplasm. Diagnosis may be established by using immunohistochemistry (positive staining with racemase; PAX2; keratins stain positive with fibromyxoid variant), electron microscopy, DNA analysis, and cytological studies. Treatment. Endoscopic resection is the treatment but recurrences including sporadic malignant transformation have been reported. Conclusions. There is no consensus on best treatment. A multicentre study is required to identify the treatment that would reduce the recurrence rate, taking into consideration that intravesical BCG is associated with NAUB.

Melanoma of the urinary bladder: a review of the literature.

Background. Melanomas of the urinary bladder and urethra are rare. Aims. To review the literature on the disease. Methods. Various Internet databases were used to identify reported cases of the disease. Results. Less than 30 cases of primary melanoma of the urinary bladder and urethra have been reported in the literature and they have been associated with melanosis and commonly with metastases. The lesions may be primary or metastatic with no gender preference. The diagnostic features include pigmented raised lesions which histologically exhibit spindled or epitheliod cells, necrosis, mitotic figures, and atypical melanocytes. Immunohistochemically they stain positively with S100; HMB45; and other melanocyte markers, but negatively with Keratin and Vimentin. The treatment involves excision and possibly IL-2. The prognostic factors include size and depth of invasion as well as metastatic lesions. Conclusions. Less than 30 cases (about 24 cases) of the disease have been reported. There are also reports of metastatic melanomas of the urinary bladder emanating from primary melanomas originating elsewhere. Diagnosis of the primary disease is based upon the histological appearance of the lesion, positive staining with S100 and HMB45, and evidence of absence of melanoma elsewhere. Primary melanoma of the bladder is usually a fatal lesion.

Nested variant of urothelial carcinoma.

Background. Nested variant of urothelial carcinoma was added to the WHO's classification in 2004. Aims. To review the literature on nested variant of urothelial carcinoma. Results. About 200 cases of the tumour have been reported so far and it has the ensuing morphological features: large numbers of small confluent irregular nests of bland-appearing, closely packed, haphazardly arranged, and poorly defined urothelial cells infiltrating the lamina propria and the muscularis propria. The tumour has a bland histomorphologic appearance, has an aggressive biological behaviour, and has at times been misdiagnosed as a benign lesion which had led to a significant delay in the establishment of the correct diagnosis and contributing to the advanced stage of the disease. Immunohistochemically, the tumour shares some characteristic features with high-risk conventional urothelial carcinomas such as high proliferation index and loss of p27 expression. However, p53, bcl-2, or EGF-r immunoreactivity is not frequently seen. The tumour must be differentiated from a number of proliferative lesions of the urothelium. Conclusions. Correct and early diagnosis of this tumour is essential to provide early curative treatment to avoid diagnosis at an advanced stage. A multicentre trial is required to identify treatment options that would improve the outcome of this tumour.

Sarcomatoid variant of urothelial carcinoma (carcinosarcoma, spindle cell carcinoma): a review of the literature.

Background. Sarcomatoid variant of urothelial carcinoma (SVUC) was added to the WHO classification in 2004. Aims. To review the literature. Materials and Method. Various internet databases were used. Result. SVUCs are rare biphasic malignant neoplasms exhibiting morphologic/immunohistochemical evidence of epithelial and mesenchymal differentiation with the presence or absence of heterologous elements. Some cases of SVUC have been associated with radiation therapy and cyclophosphamide treatment. Patients' ages range from 50 to 77 years (mean age 66). Patients tend to be younger and they more commonly presented with high-grade histology and advanced stage disease, in comparison with patients who had conventional urothelial carcinoma (CUC). Results of molecular/genetic studies strongly argue for a common monoclonal cell origin of both the epithelial and mesenchymal components in SUVC. The cancer specific survival of SVUC is poor in comparison with CUC. Radical surgical excision and chemoradiation may be associated with improved prognosis; chemoradiation as an organ preserving alternative to radical excision may be associated with improved outcome. There is no consensus opinion on the best treatment modalities for SUVC. Conclusions. SVUC is rare and is associated with inferior outcome compared with CUC. A multicentre trial of various treatment options is required. Cases of SVUC should be reported.

Lymphoma of the urinary bladder.

Background. Lymphoma of the urinary bladder (LUB) is rare. Aims. To review the literature on LUB. Methods. Various internet databases were used. Results. LUB can be either primary or secondary. The tumour has female predominance; most cases occur in middle-age women. Secondary LUB occurs in 10% to 25% of leukemias/lymphomas and in advanced-stage systemic lymphoma. Less than 100 cases have been reported. MALT typically affects adults older than 60 years; 75% are female. Diffuse large B-cell lymphoma is also common and may arise from transformation of MALT. LUB presents with haematuria, dysuria, urinary frequency, nocturia, and abdominal or back pain. Macroscopic examination of LUBs show large discrete tumours centred in the dome or lateral walls of the bladder. Positive staining of LUB varies by the subtype of lymphoma; B-cell lymphomas are CD20 positive. MALT lymphoma is positively stained for CD20, CD19, and FMC7 and negatively stained for CD5, CD10, and CD11c. LUB stains negatively with Pan-keratin, vimentin, CK20, and CK7. MALT lymphoma exhibits t(11; 18)(q21: 21). Radiotherapy is an effective treatment for the MALT type of LUB with no recurrence. Conclusions. LUB is diagnosed by its characteristic morphology and immunohistochemical characteristics. Radiotherapy is a useful treatment.

Primary Clear Cell Carcinoma of the Urinary Bladder.

Primary clear cell carcinoma of the urinary bladder (PCCUB) is rare. Literature review has revealed 47 cases of PCCUB which commonly affects women. The histogenesis of PCCUB is not certain and Müllerian origin and urotheilal origin have been postulated. The microscopic characteristics of PCCUB include cells with abundant clear cytoplasm, arranged in a solid, glandular, tubulocystic, or papillary pattern. The cells may be flat or cuboidal with abundant clear eosinophilic cytoplasm. Hobnailing may be evident. PCCUB, on immunohistochemistry, stain positively with pan-cytokeratin, cytokeratin 7, and CA 125. PCCUB may manifest with visible haematuria, lower urinary tract symptoms, and discharge. There is no consensus opinion regarding the best treatment option for PCCUBs and patient outcomes are not very clear. Surgery has been the adopted treatment of choice. Differential diagnoses of PCCUB include nephrogenic metaplasia, urothelial carcinoma with clear cell cytoplasm, diffuse large B-cell lymphoma, and metastatic clear cell carcinoma with the primary originating elsewhere. Conclusions. A thorough radiological imaging assessment is required in cases of PCCUB to exclude a primary tumour elsewhere. Urologists and oncologists should report cases of PCCUB they encounter and should enter them into a multicentric trial to ascertain the best management option.

Microcystic variant of urothelial carcinoma.

Background. Microcystic variant of urothelial carcinoma is one of the new variants of urothelial carcinoma that was added to the WHO classification in 2004. Aims. To review the literature on microcystic variant of urothelial carcinoma. Methods. Various internet search engines were used to identify reported cases of the tumour. Results. Microscopic features of the tumour include: (i) Conspicuous intracellular and intercellular lumina/microcysts encompassed by malignant urothelial or squamous cells. (ii) The lumina are usually empty; may contain granular eosinophilic debris, mucin, or necrotic cells. (iii) The cysts may be variable in size; round, or oval, up to 2 mm; lined by urothelium which are either flattened cells or low columnar cells however, they do not contain colonic epithelium or goblet cells; are infiltrative; invade the muscularis propria; mimic cystitis cystica and cystitis glandularis; occasionally exhibit neuroendocrine differentiation. (iv) Elongated and irregular branching spaces are usually seen. About 17 cases of the tumour have been reported with only 2 patients who have survived. The tumour tends to be of high-grade and high-stage. There is no consensus opinion on the best option of treatment of the tumour. Conclusions. It would prove difficult at the moment to be dogmatic regarding its prognosis but it is a highly aggressive tumour. New cases of the tumour should be reported in order to document its biological behaviour.

Primary carcinoid tumour of the kidney: a review of the literature.

Context. Primary renal carcinoid tumours are rare. Their pathogenesis is unknown and the clinical presentation is similar to other renal tumours thus posing diagnostic dilemmas for clinicians. Objectives. To review the literature for case reports of primary renal carcinoids. Methods. Literature was extensively searched for case reports for primary renal carcinoids. Reports of metastatic carcinoids to the kidneys were excluded. Results. Approximately less than 90 cases of primary carcinoid tumours of the kidney have been reported in the literature. A total of 29 cases of primary renal carcinoids were reviewed. The mean age of presentation was 48 years (range 29-75) with both right kidney (48.3%) and left kidney (44.8%) being equally affected. 28.6% of the cases reviewed were diagnosed as an incidental finding. The mean followup time was 20 months with 73.1% of patients without evidence of disease after surgical treatment (radical or partial nephrectomy). Primary carcinoid tumours of the kidney are often well differentiated tumours. They are often misdiagnosed because of their rarity and similar presentation with other renal tumours. Conclusions. Primary carcinoid tumours of the kidney are rare tumours with an indolent course with frequent metastasis. Metastatic work up and followup is required in their management.

The expression of beta human chorionic gonadotrophin (ß-HCG) in human urothelial carcinoma.

INTRODUCTION: Studies regarding the immuno-histological expression and relevance of Beta-Human Chorionic Gonadotrophin (=-HCG) in urothelial carcinoma are few. There is also no clear cut way of predicting exactly which superficial urothelial carcinomas would subsequently recur or progress and which muscle-invasive urothelial tumours would progress. The objective of the study was to study the immunohistological expression of =-HCG in urothelial carcinoma with regards to grade, category and outcome following treatment METHODS: The expression of =-HCG in urothelial carcinomas of 86 patients was studied with regards to grade, stage and outcome using an immunohistological (ABC) method and formalin fixed/paraffin embedded tumours. RESULTS: Of the 86 tumours (55 superficial and 31 muscle-invasive) studied 45, 16 and 26 were graded as G1, G2, and G3 respectively. Thirteen of the 55 superficial tumours were positively stained for ß=-HCG and 42 negatively stained. Twenty of the 31 muscle-invasive tumours studied were positively stained for ß=-HCG and 11 were negative. Of the 13 ß=-HCG positive superficial tumours only one did not recur at follow up and 12 subsequently recurred, of the 42 ß=-HCG negative superficial tumours 19 did not recur and 23 recurred. Only one of twenty patients with ß=-HCG positive muscle-invasive tumours survived; 6 of 11 patients with ß=-HCG negative muscle-invasive tumours survived. The results indicate that positive staining of the tumours was more commonly associated with tumours of higher grade, higher stage and inferior outcome. CONCLUSION: The Immunohistological expression of ß=-HCG would likely predict superficial tumours that would recur and muscle-invasive tumours with inferior outcome.