Tuberous sclerosis is a disease with a well known association with renal masses, both cysts and angiomyolipomas. Reported here is a case of a 26 year old woman who had, in addition to angiomyolipomas of the kidneys, adenoma sebaceum, paraungual fibromas, shagreen patches and cerebral tubers.
With maturing functional and oncologic outcomes data, open partial nephrectomy (OPN) has become the standard of care for T1a renal tumor. Laparoscopic approach can provide a speedier recovery with less blood loss and postoperative pain. Presuming adequate laparoscopic expertise, laparoscopic partial nephrectomy can provide equivalent oncologic outcome as for OPN albeit with higher urologic complications rate and longer warm ischemia time. With refinement of technique and use of robotic assistant, the shortcomings of laparoscopic approach can be further reduced. This article is a mini-review on the current status of laparoscopic approach to partial nephrectomy in the management of small renal mass.
Numerous immunohistochemical (IHC) biomarkers have been employed to aid in the difficult differentiation between chromophobe renal cell carcinoma (chRCC) and renal oncocytoma (RO). A systematic review and meta-analysis of the published literature was carried out to summarise and analyse the evidence for discriminatory IHC biomarkers to differentiate the two entities.
Metastatic renal cell carcinoma (RCC) presenting with multiple deposits in the head and neck region is unusual. It is not uncommon for a RCC to metastasise to a distant site after years of a tumour-free period, but most of it would be expected to have a single site of deposit. We report a rare case of a patient who had a nephrectomy 10 years earlier for RCC and presented with tumours in the frontal sinus and posterior pharyngeal wall. Radiological imaging and histology confirmed metastatic RCC at both sites.
Oral sodium phosphate (OSP), an effective bowel purgative, is available over the counter (OTC) and requires a substantially lower volume than polyethylene glycol-based preparative agents. Rarely, OSP consumption has been associated with acute hypocalcemia and hyperphosphatemia. We describe a case of chronic kidney disease patient developing symptomatic hypocalcemia following OTC OSP.
Angiomyolipomas are rare tumours of the kidney which have a tendency to bleed. We studied the clinical and radiological features of 11 patients from our institution to identify features which may be predictive of a massive haemorrhage. Tumours more than 6 cm in diameter were found to bleed and the demonstration of pseudoaneurysms on contrast CT scanning was associated with massive haemorrhage. We conclude that large angiomyolipomas should be treated to avoid haemorrhage and the detection of pseudoaneurysms should prompt urgent treatment.
Five cases of renal angiomyolipoma which underwent spontaneous rupture are described. These patients presented as an "acute abdomen" for which the diagnosis was not initially apparent. A high index of suspicion is required to make the diagnosis even with modern imaging techniques. The treatment of these tumours is discussed.
The propensity of choriocarcinoma to metastasize to lungs, liver and brain is well known. Though theoretically metastases are possible to anywhere in the body, renal metastases are rare. A 56 year old Malay woman who had total abdominal hysterectomy in 1985 for molar pregnancy presented with haemoptysis and dyspnea in 1990. Examination showed she had choriocarcinoma with pulmonary and renal metastases.
Angiomyolipomas (AML) are the most common mesenchymal renal neoplasms arising in the cortex or medulla. Intra-renal and retroperitoneal hemorrhages have been frequently reported. AML can exceptionally involve the renal vein and inferior vena cava. We report a case with extension into the inferior vena cava.
Diffusely-infiltrating mucinous adenocarcinoma of the renal pelvis associated with lithiasis and chronic gout is reported in a 61-year-old Malay man. The patient underwent left nephrectomy and vesiculo-lithotomy. This tumour is postulated to arise in response to chronic irritation of the urothelium.
Eight histologically-confirmed cases of clear cell sarcoma of the kidney (CCSK) were studied for possible mutations in the p53 tumor suppressor gene by the immunohistochemical demonstration of mutant p53 proteins using a monoclonal (DO7: Dako) and a polyclonal (AB565: Chemicon) antibody to p53 protein. All cases exhibited p53 protein nuclear immunopositivity, although in varying numbers of tumor cells and with different staining intensities. p53 protein (DO7 or AB565) was expressed in < 25% of the tumor cells in four (50%) of the cases, including the one case with a known long term survival of 13 years from the time of diagnosis. The other tumors showed p53 protein immunopositivity in > 25% of the tumor cells when stained with either DO7 or AB565 or both. The intensity of staining, graded on visual impression into weak, moderate or strong, did not correlate well with the ratio of positive staining tumor cells. While this study is unable to clarify the relative prevalence and importance of p53 mutational events in the pathogenesis of this aggressive renal tumor of childhood, it is reasonably suggestive that alterations in the p53 tumor suppressor gene do occur in CCSK.
Wilms' tumour (nephroblastoma) has been associated with chromosomal abnormalities at the 11p13, 11p15 and 16q regions. A study into the possibility of mutations occurring within p53, the ubiquitous adult tumour suppressor gene, in Wilms' tumour was carried out. Thirty-eight cases were studied. Of these 36 were categorised into the favourable histology group and two into the unfavourable histology group based on the National Wilms' Tumour Study criteria. Archival formalin-fixed, paraffin-embedded tissue sections from each case were stained with a polyclonal (AB565:Chemicon) and a monoclonal (DO7:Dako) antibody raised against p53 protein using a peroxidase-labelled streptavidin biotin kit (Dako). 'Cure' (disease-free survival of 60 months or longer) was documented in 39% of cases with favourable histology tumours. Eleven percent in this group succumbed to the disease. Both cases with unfavourable histology died. Four out of 36 (11%) tumours with favourable histology demonstrated weak to moderate staining with both AB565 and DO7 in more than 75% of tumour cells. In contrast, p53 protein expression in unfavourable histology tumours was significantly increased compared with the favourable histology group (P = 0.021) with both cases demonstrating immunopositivity in > 75% of tumour cells when stained with AB565 and DO7. The intensity of staining ranged from moderate to strong in both cases. It appears from this preliminary study that the immunohistochemical expression of p53 protein in Wilms' tumour, presumably a result of mutation in the p53 tumour suppressor gene, correlates with histological classification, histological categorisation being one of the useful features in the prognostic assessment of Wilms' tumours.
Clear cell sarcoma of kidney (CCSK) is a rare but distinct tumor of childhood frequently confused with Wilms' tumor (nephroblastoma). It has a characteristic histology, a marked predilection for metastasis to bone, and an aggressive clinical course with a high relapse rate in spite of surgical excision, chemotherapy and radiotherapy. We report the first histologically proven CCSK in a Malaysian patient. This was an 8-mth-old Malay boy who was clinically diagnosed to have stage I Wilms' tumor. Despite treatment, he developed multiple metastases 10 mths after initial presentation and died soon after. Emphasis is placed on recognizing this entity in view of (1) its naturally aggressive behaviour and (2) the prospect of improving prognosis with currently recommended intensified chemotherapeutic regimes. Its immunohistochemical profile of vimentin-positivity and negativity for epithelial membrane antigen, cytokeratin and Factor-8 related antigen is more in favour of a mesenchymal or glomerular origin than a tubular or vascular origin.
A five-month-old male baby presented with an abdominal mass which was found on computerised tomography (CT) to be involving the left kidney. Nephrectomy and histopathological study showed morphological featues of a malignant rhabdoid tumour. The tumour cells stained strongly for cytokeratin and epithelial membrane antigen and less intensely for vimentin. Electron microscopy revealed concentric whorled arrays of intermediate filaments within the tumour cell cytoplasm. The child was put on post-operative chemotherapy and radiotherapy but developed bilateral lung metastases and died three months after surgery.
The clinical experience of the novel drug temsirolimus on eight patients with metastatic renal cell carcinoma and who were refractory to other forms of treatment is reported. Although none of the patients showed complete or partial response, three patients had stable disease. One patient was prematurely withdrawn due to pneumonitis. Five patients died during the period of observation of twenty months and the median survival time from start of treatment was ten months. Three patients showed no evidence of adverse events (AE). Five patients showed dyslipidemia and two had pneumonitis for which, the drug had to be withdrawn in one of them. None had significant leucopenia. We conclude that temsirolimus has activity even in heavily pretreated patients in advanced renal cell carcinoma and in addition, has the benefits of ease of administration and good tolerability.