Management of Renal Malignancies in Von Hippel-Lindau Syndrome: Lessons Learned from a Series of Six Patients from Sri Lanka.

Management of renal malignancies in Von Hippel-Lindau (VHL) is challenging. We present six patients [mean age = 35.1 years (range: 24-54), males = 5] with VHL syndrome with multiple bilateral renal malignancies and the lessons learned during their management. The number of tumors at the time of presentation ranged from 1 to 6, while the number of new lesions varied from 1 to 3. Different combinations of radical nephrectomy (n = 2), partial nephrectomy (n = 7), and focal therapy (n = 6) were used appropriately. Median follow-up was 36 months (range: 12-72). Two patients developed new lesions which were managed with focal therapy. Nephron-sparing approaches are successful even in bilateral, multifocal, large, and recurring renal tumors associated with VHL. Awareness about the availability of efficacious surgical and minimally invasive measures would reduce psychosocial problems faced by patients and their families due to the social stigma associated with malignancies running in a family and burden of renal replacement therapy.

Cancer services in Sri Lanka: current status and future directions.

BACKGROUND: The burden of cancer in Sri Lanka is on the rise. The overall incidence of cancer in Sri Lanka has doubled over the past 25 years with a parallel rise in cancer-related mortality. Cancer has become the second commonest cause of hospital mortality in Sri Lanka. In this review, we aim to provide an overview of the current status and future direction of cancer care in Sri Lanka. MAIN BODY: In Sri Lanka, cancer services are predominantly provided by the state sector free of charge to the general public. With the establishment of national cancer policy on cancer prevention and control, there has been a commendable improvement in the cancer services provided island-wide. An increasing number of breast, oropharyngeal, thyroid, oesophageal, colorectal, lung, and gastric cancers are being diagnosed and treated annually. Primary prevention measures include restrictions in tobacco and HPV vaccination. Screening programs for selected cancers such as breast, oral and cervical cancers are delivered. Medical oncology units with facilities for systemic therapy and adequately supported by surgical, pathology, and radiology departments have been established in each district general hospital island-wide. Although the current progress is commendable, future changes are necessary to overcome the current limitations and to cater the ever increasing burden of cancer. Measures are necessary to enhance the coverage of Sri Lanka Cancer Registry. Timely high-quality research and audits are essential. Community participation in planning strategies for cancer prevention and treatment is minimal. Community-based palliative care facilities and radiation and other systemic therapy should be made available in all provinces. A culture of multi-disciplinary care with proper referral pathways would help to improve the current setting. CONCLUSION: In conclusion, Sri Lanka has a reasonably balanced and continuously expanding program for prevention, screening, and treatment of cancers. Emphasis on preventive strategies related to reducing tobacco smoking, chewing betel, and obesity, making cancers a notifiable disease, involving the community in planning cancer care and prevention strategies, conducting research to evaluate cost-effectiveness of existing treatment and increasing radiotherapy facilities would further improve the cancer services in Sri Lanka.

Clinical characteristics, etiology, management and outcome of hematospermia: a systematic review.

INTRODUCTION: Hematospermia is an uncommon symptom but can cause significant anxiety among the patient and his partner. The available data on the underlying etiology, management and outcome are variable and inconsistent. This systematic review was aimed to describe the clinical characteristics, etiology, treatment and outcomes of hematospermia. METHODS: Keywords were searched in PubMed, Scopus, LILACS and Google Scholar. Relevant articles were manually added from the list of references of eligible articles. Studies with a considerable assessment of patients with hematospermia were included. Qualitative analysis was performed using the available data. RESULTS: Twenty studies (Fifteen prospective and five retrospective, n=2079 patients, mean age =46.2 (range: 15-89) years) were eligible. Community screening reported a 0.5% prevalence of hematospermia (one study). Majority had hematospermia as the main/only symptom while dysuria (n=38/232, 16.4%), lower urinary tract symptoms (n=113/833, 13.6%), Hematuria (65/566, 11.5%) and testicular pain (n=68/631, 10.7%), were associated in some patients. Suspicious rectal examination (one study) and elevated PSA (Prostate Specific Antigen) levels (four studies) were indicative of sinister pathologies. Common etiologies were urogenital infections/inflammatory conditions followed by prostatic, seminal vesicular or urethral calculi. Malignancies were detected in 5.4% (n=74/1362, 11 studies) of patients >40 years old and the majority had prostate cancers (67/74, 90.5%). Etiology was unknown in 51.8% (n=603/1163). Definitive treatment of the underlying etiology (n=260/347, 74.9%) resolved the symptoms while spontaneous resolution occurred in the vast majority 88.9% (n=168/189) with unknown etiology. CONCLUSIONS: Hematospermia is relatively an innocent symptom. Malignancies are rare and occurred in men over 40 years. Clinical assessment including a rectal examination and a PSA level would be sufficient to identify most causes. Urogenital infections/inflammation and prostatic calculi are the commonly found etiologies. There was no identifiable cause in almost half of those with hematospermia. The majority has a benign course.

Diagnostic yield and outcome of transrectal ultrasound-guided prostate biopsy in Sri Lanka.

Abstract: The study was aimed to determine the diagnostic yield and outcome of transrectal ultrasound-guided prostate biopsy (TRUSPB) in a cohort of Sri Lankan men. A prospective study was conducted among 333 (median age: 70 years, range:48-88) men from a single urology unit. All patients underwent TRUSPB for persistently elevated serum prostate specific antigen (PSA)≥4.00 ng/mL or suspicious rectal examination. The prostate cancer (PCa) detection rate was 57.7%. The PCa detection rate for PSA levels of 4.00 to <10, 10.00 to <20, 20.00 to <40, 40.00 to <100 and ≥100.00 ng/mL were 15/43(34.9%), 23/88(26.1%), 41/72(56.9%), 51/63(81.0%) and 60/62(96.8%) respectively. Ten patients required further biopsies for rising PSA despite a first benign biopsy and three had PCa. Mild complications were identified in 6.9%(n=23). The high PCa detection rate probably reflects the difference in our policy to perform biopsies only when serum PSA level is persistently elevated. TRUSPB appears to have a satisfactory yield with acceptable level of complications in the Sri Lankan resource limited setting.

Risk factors of renal cell carcinoma in a cohort of Sri Lankan patients: A case-control study.

BACKGROUND: Associated risk factors for renal cell carcinoma (RCC) include smoking, obesity, hypertension, and diabetes mellitus (DM). Studies on their role in Sri Lankan patients are sparse. The aims were to determine the risk factors for RCC in a cohort of Sri Lankan patients. METHODS: A hospital-based case-control study was done in a teaching hospital from January 1, 2009, to December 31, 2015. The prospectively collected data included history of smoking, hypertension, DM, and body mass index (BMI). The controls were patients who were admitted after trauma and who required abdominal ultrasonography as part of routine assessment. The controls were accrued at a ratio of 1:2 and were age- and gender-matched. Chi-squared test was used, and P < 0.05 was considered statistically significant. RESULTS: There were 178 patients with RCC. The mean age of patients with RCC was 56.9 years. Male to female ratio was 3.5:1. Forty-nine (27.5%) patients were < 50-year-old. 36.5% were asymptomatic and diagnosed incidentally by ultrasound scanning. Obesity (BMI > 30) (P < 0.01), DM (P < 0.05), and hypertension (P < 0.01) were significantly more common among the cases than in controls. There was no difference in the prevalence of smoking between the two groups (P > 0.5). CONCLUSIONS: Average age at the time of diagnosis of RCC in Sri Lankan patients is lower than the developed world, with a large proportion of patients being under 50 years. Obesity, hypertension, and DM are associated risk factors for RCC in Sri Lankan patients while smoking is not.

Mineralogical, compositional and isotope characterization of human kidney stones (urolithiasis) in a Sri Lankan population.

In order to understand the processes of stone formation, compositional, spectroscopic, mineralogical and crystallographic characteristics of human urinary stones collected from patients in Sri Lanka were investigated in detail. The data showed that the majority of urinary calculi were calcium oxalate, either whewellite or weddellite. Other solid phases of stones were composed of struvite, uricite and hydroxylapatite. However, mixed compositions were common except for whewellite stones which occur frequently in pure form. Scanning electron microscope observations and associated energy-dispersive X-ray analyses revealed that whewellite or weddellite was well crystalized compared to other stones types, while phosphate stones were mostly cryptocrystalline. The average δ13C and δ18O of stones were - 32.2‰ (- 37.3 to - 17.4‰) and - 24.2‰ (- 26.7‰ to - 8.9‰), respectively. The δ13C values were highly depleted compared to North American and European urinary stones. This may be due to food habits of Asians who consume rice as the staple food.

Outcome of uncomplicated ureteric calculi managed with medical expulsive therapy in the outpatient clinic of a urology unit in Sri Lanka.

OBJECTIVE: Although medical expulsive therapy (MET) is shown to be effective for ureteric calculi, the optimum duration and the stone size suitable for MET are not well established yet. The objectives of the study were to determine the optimum duration and maximum stone size suitable for MET. RESULTS: All patients with radiologically confirmed uncomplicated ureteric calculi treated with MET using tamsulosin over a period of 6 months in the outpatient setting were followed up. There were 213 patients. 165 were men. Mean age was 42 years. At presentation 42 stones were in upper ureter (19.7%), 51 in mid ureter (23.9%), 120 in lower ureter (56.3%). The majority (82.7%) of stones were less than 10 mm. Seven stones (3.3%) were over 15 mm. Ninety-two (43.2%) patients had spontaneous passage of stones within 6-weeks of MET. Another 38.9% passed the stone within the next 6-weeks. Thirty-eight patients (17.8%) required surgery. Uncomplicated ureteric stones up to 10 mm can be given a trial of MET using tamsulosin which can be extended up to 12-weeks with a success rate over 92%. This may have substantial clinical and fiscal benefits by reducing the number of interventional procedures especially in resource-poor settings.

New developments in chyluria after global programs to eliminate lymphatic filariasis.

Chyluria, commonly seen in south Asian countries, is mainly a manifestation of lymphatic filariasis as a result of infestation with Wuchereria bancrofti, although many other causes can contribute. Many patients can be effectively treated with dietary modifications and drug therapy. The most widely used drug is diethyl carbamazine. The recurrences are common after such treatment. Such patients would benefit from sclerotherapy to obliterate the lympatico-renal fistulae located mainly in the renal pelvicalyceal system. The commonly used sclerosing agent is a combination of 5% povidone-iodine and 50% dextrose instilled through a ureteric catheter. A small percentage of patients who recur after sclerotherapy and those with systemic complications, such as hypoproteinemia and edema, might require surgery in the form of renal hilar lymphatic disconnection. Although it is a major operation, the success rates are >90%. Laparoscopic and robotic techniques have minimized the morbidity related to such surgery. With the advent of the global program for eradication of filariasis initiated by the World Health Organization, the incidence of the disease is decreasing. Mass chemotherapy with diethyl carbamazine is the mainstay of this global program. Many years after eliminating filariasis, chyluria continue to occur in such populations, though in dwindling numbers. Future research should aim at finding more efficacious sclerosing agents with minimal recurrences.

Metachronous bladder metastases of a type 2 papillary renal cell carcinoma: a case report and review of the literature.

BACKGROUND: Renal cell carcinoma developing metastases in the bladder is rare. Bladder metastasis due to a papillary type of renal cell carcinoma is rarer. Such metastases could be synchronous or metachronous. CASE PRESENTATION: Here we present a 55-year-old female patient with haematuria who underwent left nephro-ureterectomy for a suspected urothelial tumour. Histopathology revealed it to be a type 2 papillary renal cell carcinoma. Eighteen months later, she developed metachronous bladder metastasis of the papillary renal cell carcinoma which was treated with total cystectomy. Currently, she is on interferon therapy. CONCLUSIONS: These bladder metastases from renal cell carcinoma could be due to drop metastases, lymphatic spread or haematogenous spread. The exact mechanism in a given case appears to be unpredictable.

Pathological Characteristics of Primary Bladder Carcinoma Treated at a Tertiary Care Hospital and Changing Demographics of Bladder Cancer in Sri Lanka.

Objectives. The aim was to compare demographics and pathological features of bladder carcinoma treated in a urology unit with findings of previous studies done in Sri Lanka. Materials and Methods. Data of newly diagnosed patients with bladder cancer in a tertiary referral centre from 2011 to 2014 were analysed. Data on bladder cancers diagnosed from 1993 to 2014 were obtained from previous publications and Sri Lanka Cancer Registry. Results. There were 148 patients and mean age was 65 years. Male to female ratio was 4.1 : 1. Urothelial carcinoma (UC) was found in 89.2% of patients. Muscle invasion was noted in 35% of patients compared to 48.4% two decades ago. In patients with UC, 16.5% were found to have pT1 high grade tumour. It was 5.3% from 1993 to 2000. Pure squamous cell carcinoma was found in 8.1% of patients while primary or de novo carcinoma in situ (not associated with high grade pT1 tumours) was seen in one patient only. Conclusions. The percentage of squamous carcinoma is higher among Sri Lankan patients while primary carcinoma in situ is a rarity. The percentage of muscle invasive disease has decreased while the percentage of pT1 high grade tumours has increased during the last two decades in Sri Lanka.

A solitary fibrous tumor of the kidney.

A solitary fibrous tumor (SFT) is an uncommon spindle cell neoplasm that usually occurs in the pleura, but may occur in extrapleural sites. Its occurrence in the kidney is rare. We report a SFT, clinically thought to be a renal cell carcinoma arising in the kidney of a 68-year-old female. The tumor was well-circumscribed and composed of a mixture of spindle cells and dense collagenous bands. Immunohistochemical studies revealed reactivity for CD34, CD99, and Bcl-2 protein, with no staining for keratin or muscle markers, confirming the diagnosis. The immunohistochemical study was the key to diagnosis. Several younger members of her family had colorectal and lung cancers suggesting the possibility of a familial or genetic susceptibility.

Clinicopathological characteristics and primary treatment of prostate cancer in a urology unit of Sri Lanka.

AIMS: The aim was to describe the clinicopathological characteristics of prostate cancer and the primary treatment modality in a cohort of patients seen in a urology unit of Sri Lanka. MATERIALS AND METHODS: Data were collected prospectively from all patients with newly diagnosed prostate cancer and managed in a urology unit in Sri Lanka from January 2010 to December 2013. Patient's age, clinical presentation, prostate specific antigen (PSA) level, mode of diagnosis, Gleason sum score, stage of the disease and main modality of treatment were recorded and analyzed. RESULTS: There were 278 cases of histologically proven prostate cancer. Mean patient age was 70.5 years. About 50% presented with lower urinary tract symptoms. Only 2% were screening detected cases. Five (2%) patients had a first-degree relative who had prostate cancer. About 81% of patients had a serum PSA above 20 ng/ml. Gleason sum score was 8 or more in 44% of patients. Metastases were found at the time of diagnosis in 60% of patients. Forty-eight patients underwent radical radiotherapy, while seven patients had radical prostatectomy. Most (94%) of the 203 patients who required androgen deprivation therapy had surgical orchiectomy. CONCLUSIONS: Sri Lankans appear to be having a low incidence of prostate cancer, but a larger proportion of high-grade cancers in comparison to the UK and USA. Although genetic differences may exist, a dietary or an environmental factor is more likely to be the cause for these changes. The protective effect of this factor appears to wane as South Asians emigrate and live in UK and USA.

Sudden unexpected death probably due to Eagle's syndrome: a case report.

An abnormally long styloid process producing autonomic effects gives rise to Eagle's syndrome. Sudden deaths have not been previously reported. We present the case of a 39-year-old female who died of probable vagus mediated cardiac inhibition due to Eagle's syndrome. The diagnosis was established only after the autopsy examination, which revealed the elongation of the styloid processes in the form of fibrous membranes, compressing both carotid sinuses. The anatomical changes were not visible in the radiological films.

The operational cost of a urology unit.

OBJECTIVES: To assess the operational cost of a urology unit, individual cost for certain index operations in urology, and to develop a framework to audit finances of a unit. DESIGN: A financial audit. SETTING: Urology unit in a teaching hospital. METHODS: Data of cost in providing urology services during one month were collected. It included three main areas: ward, operating theatre and outpatient clinic. Direct costs included staff wages, drugs, consumables, investigations and food. Indirect expenses such as administration, water, electricity and cleaning services were also calculated. For each type of operation a relative value was assigned depending on the nature of the operation. When direct expenses were not available, the hospital was divided into different cost centres and apportioning of the cost was done accordingly. RESULTS: The monthly operational cost of running a 19 bed urology unit with three operating sessions a week was Rs. 1 294 259. Staff wages constituted 61.2% of the cost. The cost of performing a pyelolithotomy was Rs. 18 669. Transurethral resection of the prostate (TURP) was done at a cost of Rs. 21 271. CONCLUSION: When the basic principles and the framework are understood, clinicians can perform financial audit and cost analysis of their units.

A case of bladder and colonic duplication.

Duplication of the bladder is a rare congenital anomaly with an unknown embryological basis. A 12-year-old boy with dysuria was found to have a tri-radiate external urethral meatus instead of a vertical slit. Cystourethroscopy revealed two bladders with bifid proximal urethra. Magnetic resonance imaging (MRI) revealed duplication of colon and bladder. He did not have any symptoms related to the urinary tract.