The micropapillary variant of urothelial carcinoma is one of the variants of urothelial carcinoma that was added to the World Health Organization (WHO) classification in 2004. This variant of urothelial carcinoma is uncommon and, therefore, a number of practitioners have not yet encountered the tumour in their clinical practice. In view of this, the diagnostic features and the biological behaviour of this tumour are not well known by practitioners. This review documents the diagnostic features, management and outcome of the micropapillary variant of urothelial carcinoma. Various internet search engines were used to identify literature on the micropapillary variant of urothelial carcinoma, including case reports and case series, which formed the basis of the literature review. At least 500 cases of the micropapillary variant of urothelial carcinoma have been reported since it was first described in 1994. The gross morphology is variable and there are no special features to differentiate this variant from other variants or from conventional urothelial carcinoma. The micropapillary variant may be sessile, papillary, ulcerative or polypoid or may manifest as an infiltrative mass of variable size, ranging from microscopic to > 10 cm. The microscopic features of this variant of urothelial carcinoma exhibit an architecture that is reminiscent of the papillary configuration of ovarian papillary serous tumours. The nuclei of the micropapillary variant cells are commonly of high grade and show reverse polarity to the external surface of the tumour nests. A minority of the tumour-containing spaces represent actual lymphovascular invasion, which is supported by their characteristic immunohistochemical staining with endothelial markers. Lymphovascular invasion can be found in most cases of the invasive micropapillary variant of urothelial carcinoma if the specimens are adequately sampled, but a majority of the tumour-containing lacunae do not have endothelial lining and, therefore, do not represent true lymphovascular invasion. Psammoma bodies, which are common in ovarian papillary serous tumours, are rare in the micropapillary variant of urothelial carcinoma, and most of these tumours exhibit deep muscle invasion. Urine cytology exhibits papillary/spheroid clusters of tumour cells that have a high nuclear grade. The micropapillary variant of urothelial carcinoma is a rare and aggressive variant and may be under-reported as some pathologists may not be aware of its diagnostic features. Reports so far would indicate that this variant is associated with poor prognosis and adjuvant chemotherapy may have a questionable efficacy. Because of the rarity of this variant of urothelial carcinoma, there is no consensus opinion regarding the optimal treatment. Therefore, there is a need for urologists and oncologists throughout the world to report cases of the micropapillary variant of urothelial carcinoma so that the biological behaviour of the tumour can be ascertained. There is also a need for multicentre trials of treatment of this variant of urothelial carcinoma so that lessons can be learnt regarding the biological behaviour of the tumour and a consensus opinion can be established regarding the best treatment modality for such an apparently aggressive tumour.

Synovial cell sarcomas of the kidney are rare tumours that most urologists and pathologists throughout the world have not encountered. Because of this, most practitioners may not be aware of the presentation, diagnostic features or biological behaviour of such tumours. This article reviews the literature on synovial cell sarcomas of the kidney in order to document the presentation, investigation, management as well as outcome following treatment of these tumours. Synovial sarcoma of the kidney is a rare tumour that was first reported in 1999 and, since then, 64 cases have been reported in the literature. Synovial sarcomas of the kidney tend to be found in patients who are aged between 17 and 61 years, and the clinical presentation and radiography findings are similar to those of other renal tumours. There are three main types of primary synovial sarcomas of the kidney: the biphasic, the monophasic and the poorly differentiated varieties. Primary biphasic synovial sarcomas contain both glandular elements and spindle epithelial cells, whereas primary monophasic synovial sarcomas are composed of spindle cells only and poorly differentiated synovial sarcomas (PDSSs) exhibit three histological variants: large cell, small cell and high-grade spindle cell variant. PDSSs are composed of sheets of undifferentiated round cells with hyperchromatic nuclei and frequent mitoses, and such tumours are often associated with a rich vascular pattern with dilated and thin-walled vascular spaces that resemble haemangiopericytoma. The differential diagnoses include adult Wilms' tumour, transitional cell carcinoma, renal cell carcinoma, haemangiopericytoma, congenital ectoblastic nephroma and primitive neuroectodermal tumour (PNET).Diagnosis of renal synovial sarcoma is based on microscopic findings of (a) spindle cells (monophasic) or (b) glandular cells and spindle cells (biphasic) supported by immunohistochemical staining characteristics [positivity for CD99, CD56, Bcl-2, epithelial membrane antigen (EMA) and cytokeratin (CK)] and molecular genetic studies of the tumour showing characteristic chromosomal translocation t(X;18)(p11.2;q11.2), which is seen in cases of synovial sarcoma. There is no consensus regarding the treatment of synovial sarcoma of the kidney; however, surgical excision including a portion of surrounding normal tissue (radical nephrectomy) has so far been the main treatment. In addition, response to ifosfamide and doxorubicin (adriamycin) chemotherapy has been reported. Recurrence following treatment for non-metastatic synovial sarcoma is approximately 36% and prognosis for advanced disease is poor. Diagnosis of synovial sarcoma of the kidney is based upon characteristic microscopic findings that must be supported by specific diagnostic immunohistochemistry characteristics and molecular genetic evidence of chromosomal translocation. Because of the rarity of synovial sarcomas of the kidney, urologists and oncologists need to report all cases they encounter, together with reports of long periods of follow-up, to enable practitioners to fully understand the biological behaviour of such tumours.

Chronic pelvic pain (CPP) can be a debilitating and sometimes life-changing condition. It affects both sexes, but is more common in women. The socioeconomic cost of CPP is huge and treatment requires a multidisciplinary approach. Treatment of CPP may be lengthy with disappointing results and the management of the condition is an ever-evolving field with some options still in their infancy. We have reviewed the literature on CPP, identified using various internet databases, focusing on the management of CPP in women over the last 15 years and examining the available evidence for the efficacy of the management options of CPP. A review of the relevant literature indicates that the causes of CPP in women may be both gynaecological and non-gynaecological. CPP may be a result of multiple factors and the treatment of this condition can be medical or surgical; other treatment modalities include psychotherapy, physiotherapy and lifestyle modification, and treatment may involve the use of more than one modality. Medical treatment includes the prolonged use of hormonal preparations for specific causes or the non-specific control of pain using analgesics whereas surgical management of CPP targets the specific tissues involved and may include the partial or complete excision of pathological tissues. Physical therapy was found to be helpful in managing symptoms of pelvic floor pain and abdominal myofascial pain and psychological therapy modifies symptoms in CPP, especially if a significant organic cause is present. Further randomized controlled studies are required to determine the efficacy of some of these treatment methods.

Lymphoepithelioma tumours are commonly found in the nasopharynx, and histologically similar lymphoepitheliomas have been found elsewhere in the body, including the urinary bladder. Because of the rarity of lymphoepithelioma-like carcinoma (LELC) of the urinary bladder, most practitioners will be unfamiliar with its presentation, management and outcome. LELC of the urinary tract is just one of the variants of urothelial carcinoma that were added to the World Health Organization (WHO) classification in 2004, characterized by their morphological features and immunohistochemical staining characteristics, and including also the nested, microcystic, micropapillary, plasmacytoid variants. The aim of this article is to briefly outline the features of LELC based on the results of various internet searches. LELC of the urinary bladder is a rare tumour, accounting for 0.4–1.3% of all carcinomas of the urinary bladder, and occurs mainly in adults, with a reported mean age at diagnosis of 67–69 years (range 52–84 years). The majority of patients with LELC of the urinary bladder are men (75%), and fewer than 100 cases have been reported in the literature. Patients with LELC of the urinary bladder mostly present with haematuria and a definitive diagnosis is based on accurate histological interpretation. The morphological features of the tumours and the characteristic immunohistochemical staining are key to the diagnosis. Microscopically, they resemble lymphoepithelioma of nasopharynx and are Epstein–Barr virus negative. The lymphoepithelioma-like component should be > 50% and microscopic examination depicts undifferentiated tumour cells in syncytial sheets with minimal cytoplasm, prominent nucleoli, numerous mitoses and lymphocytes. LELC of the urinary bladder tends to exhibit positive staining for cytokeratin (CK) 7, AE1/AE3, epithelial membrane antigen, and often p53 and B- and T-cell markers. In addition, they exhibit negative staining for CD45/leucocyte common antigen. The differential diagnosis of LELC of urinary bladder includes florid cystitis, large cell undifferentiated carcinoma, lymphoma and small cell carcinoma. These tumours are usually muscle-invasive and often coexist with transitional cell carcinoma and carcinoma in situ. Chronic inflammation may obscure the tumour and cystectomy is usually employed as a treatment method in view of the fact that the tumour may be multifocal with synchronous urothelial carcinoma. There is no consensus on the optimum treatment in view of the rarity of the disease. Nevertheless, there is evidence to suggest that patients with pure or predominant LELC of the urinary bladder should undergo transurethral resection with or without adjuvant cisplatin-based systemic chemotherapy and focal LELC should be treated by radical cystectomy with adjuvant cisplatin-based systemic chemotherapy. After bladder-preserving treatment, the patient should undergo regular follow-up cystoscopies.

Inverted papilloma of the genitourinary tract is an uncommon neoplasm. Some urothelial carcinomas exhibit prominent inverted growth patterns that may pose a diagnostic dilemma. The presence of multiple inverted papillomas, their frequent recurrence and association with transitional cell carcinoma may lead to conflicting clinical conclusions regarding their biological behaviour and hence there is a need to review the literature. Internet databases including PubMed, Google Scholar, Google and Educus were searched to identify publications on inverted papilloma. Thirty-four publications formed the foundation for this review of the literature. The results revealed that inverted papilloma is a rare endophytic urothelial neoplasm that is most commonly found in the urinary bladder and less commonly in the upper urinary tract and urethra. Inverted papillomas are more common in men than in women and may present with obstructive or irritating voiding symptoms or haematuria. Inverted papilloma of the prostatic urethra may present with perineal pain whereas inverted papilloma of the upper renal tract may present with haematuria and loin pain. Inverted papillomas may be difficult to differentiate from inverted urothelial carcinomas but exhibit an absence of, or only few, atypical cells whereas inverted urothelial carcinomas exhibit atypia. Immunohistochemistry can be of assistance as urothelial carcinomas are positive for Ki-67 and p53, fluorescence in situ hybridization (FISH) and cytokeratin 20; however, inverted papillomas generally do not express these markers. FISH analysis can often detect chromosomal anomalies in urothelial carcinomas, but chromosomal abnormalities are absent in inverted papillomas. Inverted papillomas are endophytic and, when they are completely resected or excised, they do not initially recur; however, urothelial carcinomas recur and may progress. Inverted papillomas rarely coexist with urothelial carcinomas and malignant transformation has rarely been reported in inverted papillomas. Microscopic examination may enable inverted papillomas to be classified as the more common classic trabecular subtype or the glandular subtype.

Background. Acquired immunodeficiency syndrome (AIDS) affects all organs of the genitourinary tract. In view of this, clinicians need to be aware of the ways in which AIDS affects each of these organs. Aim. The aim of this article is to review the literature regarding human immunodeficiency virus (HIV) infections and/or AIDS and its impact on the genitourinary tract. Methods. An internet search was carried out to document the reported associations between HIV infection or AIDS and the urinary tract. The search engines used were Google, Google Scholar and PubMed. Scrutiny of manuscripts which discussed in detail the association between HIV infection or AIDS and the organs of the genitourinary tract formed the basis for the review. Results. The organs of the genitourinary tract may be affected by AIDS in a number of ways, as follows: Urethra A number of patients infected with HIV or suffering from AIDS develop dysuria and urinary frequency as a result of cytomegalovirus infection. Other patients may develop B- or T-cell lymphoma involving the urethra. Prostate Opportunistic infections of the prostate gland may be encountered. Scrotum Atypical organisms associated with Fournier's gangrene may be encountered. Epididymis Opportunistic infections of the epididymis may occur. Testis Opportunistic infections of the testis, testicular atrophy and hypogonadism have been reported. Penis Kaposi's sarcoma and squamous cell carcinoma of the penis have been reported. Bladder Voiding dysfunction and acute retention of urine may develop. Urodynamics may show areflexia, hyporeflexia or hyperreflexia. Ureter Renal colic and ureteric obstruction may result from the deposition of concrements of antiretroviral drugs in the ureter. Kidney Structural renal problems including opportunistic infections, tumours and nephropathy may develop. Functional renal problems may include hyponatraemia, hypokalaemia and acute renal failure. Sexual function Primary or secondary gonadal failure in the HIV-infected patient may result in impotence. Malignancies Kaposi's sarcoma, non-Hodgkin's lymphoma and squamous cell carcinoma may develop. Obstruction of the upper urinary tract may necessitate creation of a nephrostomy. Conclusions A number of genitourinary problems have documented associations with HIV infection and AIDS, including opportunistic infections of genitourinary tract organs; functional and structural renal problems; tumours of the penis, bladder and other organs of the genitourinary tract; voiding problems including urinary retention; ureteric obstruction; and impotence.

Plasmacytoid urothelial carcinoma is a new variant of urothelial carcinomas that was added to the World Health Organization (WHO) classification list in 2004; therefore, in view of its rarity, most practitioners may not be familiar with its biological behaviour. Various internet databases were searched to identify cases of plasmacytoid urothelial carcinoma of the urinary bladder in order to document the presentation, investigation, management, treatment and outcome of the disease. In addition, the only reported case of plasmacytoid urothelial carcinoma of the ureter is included. Plasmacytoid urothelial carcinoma of the urinary bladder is a rare variant of urothelial carcinoma and fewer than 100 cases have been reported in the literature. It is an aggressive subtype of urothelial carcinoma with plasmacytoid features and the diagnostic features include mimicking haematolymphoid malignancy, a dyscohesive invasive pattern, eccentric nuclei, evidence of prominent eosinophilic cytoplasm (amphophilic cytoplasm may also be observed) and intracytoplasmic mucin-positive lumina may be present. Patients often present with plasmacytoid urothelial carcinoma at a late stage and exhibit peritoneal surface spread. Mucin stains may reveal intracytoplasmic lumina and plasmacytoid urothelial carcinomas tend to depict immunohistochemical staining characteristics such as keratin positivity [cytokeratin (CK)7 and, at times, CK20], CD138 positivity (many carcinomas, not only plasmacytoid urothelial carcinoma, stain positive for CD138), MUM1 and kappa/lambda light chain negativity, E-cadherin negativity (conventional and micropapillary types of urothelial carcinoma tend to stain positive for E-cadherin and signet-ring cell carcinomas of the bladder tend to stain negative). In the differential diagnosis of plasmacytoid urothelial carcinoma, plasmacytoma must be considered, and immunostaining characteristics can be helpful in the confirmation of the diagnosis in that p63-positive staining and strong keratin would indicate carcinoma. Kappa and lambda in situ hybridization or immunoperoxidase would identify most plasmacytomas. CD138 positivity is expressed by many carcinomas and is, therefore, not helpful, whereas MUM1 preferentially stains plasmacytoma. Plasmacytoid urothelial carcinoma of the urinary bladder has characteristic plasmacytoid morphological features and immunohistochemical staining is helpful in differentiating this tumour from other differential diagnoses. Plasmacytoid urothelial carcinomas are aggressive variants of urothelial carcinomas of the urinary bladder that have been associated with poor prognosis and death of patients following various treatment options including transurethral resection of bladder tumour and/or radical cystectomy, transurethral resection biopsy and/or cystectomy and/or chemotherapy, transurethral resection of bladder tumour, and chemotherapy and/or radiotherapy. Most patients do not survive post treatment; however, there are reports of survival following treatments that have included combination systemic chemotherapy.

Small cell carcinoma of the urinary bladder is a rare tumour that resembles small cell carcinoma in the lung and elsewhere. Because of its rarity, practitioners may not be familiar with the biological behaviour of this tumour. The aim of this article is to review the literature on small cell carcinoma of the urinary bladder. Various internet search engines were used to explore the literature on small cell carcinoma of the urinary bladder including Google, Google Scholar, UpToDate, Pub Med and Educus. About 42 references were selected as the basis for the literature review. Small cell carcinoma of the urinary bladder is an aggressive tumour that typically presents with advanced or disseminated disease. This is a rare disease, accounting for only 0.5–1.0% of all bladder malignancies. Affected individuals show no age, sex or clinical differences from those with the typical type of urothelial carcinoma. Some cases of small cell carcinoma of the urinary bladder arise from urothelial carcinoma in situ while others arise from totipotent stem cells in the submucosa. The tumours are usually large polypoid masses that can occur anywhere in the bladder. Microscopically, the tumours appear as loosely cohesive sheets, or nests, of small to intermediate-sized cells with minimal cytoplasm, hyperchromatic nuclei, stippled or coarsely granular chromatin, indistinct nucleoli and no nuclear overlapping. Mitotic activity and necrosis are common and the tumours may co-exist with other forms of in situ, or invasive, carcinoma. Radical cystectomy is the main treatment for small cell carcinoma of the urinary bladder, unless metastatic disease is present, followed by systemic treatment. Response to chemotherapy is good, similar to treatment for small cell carcinoma of the lung, but the overall prognosis remains poor. There is no consensus regarding treatment of small cell carcinoma of the urinary bladder although general recommendations for the approach to treatment of this aggressive disease have been suggested.