"The overwhelming majority of cases occur in individuals with sickle cell trait (HbAS) and, less commonly, with the haemoglobin SC (HbSC) variant of sickle cell disease. This prompted the authors who first defined it to name it the "seventh sickle cell nephropathy" 2. As such, a central renal mass in a young ("
"It exhibits SMARCB1/INI1 loss at immunohistochemistry, found in several other types of childhood malignancies, such as malignant rhabdoid tumour of the kidney and atypical teratoid/rhabdoid tumour (AT/RT) 6."
"It exhibits SMARCB1/INI1 loss at immunohistochemistry, found in several other types of childhood malignancies, such as malignant rhabdoid tumour of the kidney and atypical teratoid/rhabdoid tumour (AT/RT) 6."
"Presenting symptoms include frank haematuria, abdominal or flank pain, a palpable mass, and possibly, signs of metastatic disease. The latter are due to the fact that at the time of diagnosis, most patients will have developed metastatic disease. Similarly to other types of renal cancer, the lungs are the most common location for distant metastases and virtually every patient with liver metastases has lung metastases."
"There is no recommended treatment. Treatment protocols used in other types of renal carcinoma have all been tried but response to any treatment is very poor. The accepted treatment for patients without metastatic disease is total or radical nephrectomy. Patients with metastases at presentation undergo chemotherapy and/or radiotherapy but again, response to either is poor. Overall prognosis is dismal: depending on the cohort the publication is based on, mean survival time is between 4 and 12 months; a good estimate is 7.7 months 3."
"There is no recommended treatment. Treatment protocols used in other types of renal carcinoma have all been tried but response to any treatment is very poor. The accepted treatment for patients without metastatic disease is total or radical nephrectomy. Patients with metastases at presentation undergo chemotherapy and/or radiotherapy but again, response to either is poor. Overall prognosis is dismal: depending on the cohort the publication is based on, mean survival time is between 4 and 12 months; a good estimate is 7.7 months 3."