PURPOSE: Malignant rhabdoid tumor (MRT) is a rare and highly aggressive tumor that affects young children. Due to its extreme rarity, most of the available data are based on retrospective case series. To add to the current knowledge of this disease, we reviewed the patients treated for extra-cranial MRT in our institute. MATERIALS AND METHODS: A retrospective medical record review was conducted on children treated for pathologically confirmed extra-cranial MRT at Seoul National University Children's Hospital between January 2003 and May 2013. RESULTS: Eleven patients (7 boys, 4 girls) were diagnosed with extra-cranial MRT at a median age of 9 months old. INI1 staining was important in the pathological confirmation. Six patients (55%) had renal MRT and five (45%) had soft tissue MRT. Five patients (45%) had metastases at diagnosis. All patients underwent chemotherapy, eight patients (73%) underwent surgery, six patients (55%) received therapeutic radiotherapy, and four patients (36%) underwent high dose chemotherapy with autologous stem cell rescue (HDCT/ASCR) with melphalan, etoposide, and carboplatin. Five patients (45%) died of disease following progression (n=3) or relapse (n=2), however, there was no treatment related mortality. The overall survival of the cohort was 53.0% and the event-free survival was 54.5% with a median follow-up duration of 17.8 months (range, 2.3 to 112.3 months). CONCLUSION: Extra-cranial MRT is still a highly aggressive tumor in young children. However, the improved survival of our cohort is promising and HDCT/ASCR with melphalan, etoposide, and carboplatin may be a promising treatment option.
Carboplatin ; Child* ; Cohort Studies ; Diagnosis ; Disease-Free Survival ; Drug Therapy ; Etoposide ; Follow-Up Studies ; Humans ; Kidney Neoplasms ; Medical Records ; Melphalan ; Mortality ; Neoplasm Metastasis ; Radiotherapy ; Recurrence ; Retrospective Studies ; Rhabdoid Tumor* ; Seoul ; Soft Tissue Neoplasms ; Stem Cells

Tumor lysis syndrome is rare in hepatocellular carcinoma (HCC), but it has been reported more frequently recently in response to treatments such as transcatheter arterial chemoembolization (TACE), radiofrequency thermal ablation (RFTA), and sorafenib. Tumor lysis syndrome induced by low-dose steroid appears to be very unusual in HCC. We report a patient with hepatitis-C-related liver cirrhosis and HCC in whom tumor lysis syndrome occurred due to low-dose steroid (10 mg of prednisolone). The patient was a 90-year-old male who presented at the emergency room of our hospital with general weakness and poor oral intake. He had started to take prednisolone to treat adrenal insufficiency 2 days previously. Laboratory results revealed hyperuricemia, hyperphosphatemia, and increased creatinine. These abnormalities fulfilled the criteria in the Cairo-Bishop definition of tumor lysis syndrome. Although the patient received adequate hydration, severe metabolic acidosis and acute kidney injury progressed unabated. He finally developed multiple organ failure, and died 3 days after admission. This was a case of tumor lysis syndrome caused by administration of low-dose steroid in a patient with HCC.
Acute Kidney Injury/pathology ; Aged, 80 and over ; Antineoplastic Agents/therapeutic use ; Carcinoma, Hepatocellular/*pathology/therapy ; Chemoembolization, Therapeutic ; Creatinine/blood ; Humans ; Liver Neoplasms/*pathology/*therapy ; Male ; Niacinamide/analogs & derivatives/therapeutic use ; Phenylurea Compounds/therapeutic use ; Steroids/adverse effects/therapeutic use ; Tomography, X-Ray Computed ; Tumor Lysis Syndrome/*diagnosis/drug therapy

Metastatic renal cell carcinoma (RCC) in the nose and paranasal sinuses is very rare. We report an unusual case of metastatic RCC that presented as recurrent epistaxis ten years after curative nephrectomy. The purpose of this report is to draw the attention of clinicians to the possibility of metastatic RCC in patients with recurrent epistaxis and nasal mass. We also discuss treatment options and review the relevant literature.
Adult ; Carcinoma, Renal Cell ; diagnosis ; secondary ; therapy ; Chemoradiotherapy ; Diagnosis, Differential ; Epistaxis ; diagnosis ; therapy ; Humans ; Indoles ; therapeutic use ; Kidney Diseases ; diagnosis ; therapy ; Male ; Neoplasm Metastasis ; Nose ; pathology ; Nose Neoplasms ; diagnosis ; secondary ; therapy ; Positron-Emission Tomography ; Pyrroles ; therapeutic use ; Recurrence ; Tomography, X-Ray Computed

PURPOSE: Collecting duct carcinoma (CDC) of the kidney is an aggressive disease with a poor prognosis, accountings for less than 1% of all renal cancers. To date, no standard therapy for CDC has been established. The aim of this study is an investigation of clinicopathologic findings of CDC and correlation of the disease status with a prognosis. MATERIALS AND METHODS: From 1996 to 2009, 35 patients with CDC were treated at eight medical centers. The diagnosis of CDC was made based on nephrectomy in 27 cases and renal biopsy in eight cases. RESULTS: Median PFS and OS for all patients were 5.8 months (95% CI 3.5 to 9.2) and 54.4 months (95% CI 0 to 109.2), respectively. The OS of patients with Stages I-III was 69.9 months (95% CI 54.0 to 85.8), while that of patients with Stage IV was 8.6 months (95% CI 0 to 23.3), which showed a statistically significant difference (p=0.01). In addition, among patients with Stage IV, the OS of patients who received a palliative treatment (immunotherapy, chemotherapy, or targeted therapy) was 18.4 months, which was higher than the OS of patients without treatment of 4.5 months. CONCLUSION: CDC is a highly aggressive form of renal cell carcinoma. Despite most of the treatments, PFS and OS were short, however, there were some long-term survivors, therefore, conduct of additional research on the predictive markers of the several clinical, pathological differences and their treatments will be necessary.
Biopsy ; Carcinoma, Renal Cell* ; Centers for Disease Control and Prevention (U.S.) ; Diagnosis ; Drug Therapy ; Gynecology* ; Humans ; Kidney ; Kidney Neoplasms ; Nephrectomy ; Palliative Care ; Prognosis ; Survivors

Radio-frequency ablation (RFA) is a curative treatment for hepatocellular carcinoma (HCC). Percutaneous RFA has been shown to be beneficial for patients with small renal cell carcinoma (RCC) lacking indications for resection. We experienced the case of a 53-year-old male who had conditions that suggested HCC, RCC, and alcoholic liver cirrhosis. Abdominal contrast-enhanced computed tomography (CT) and magnetic resonance image showed liver cirrhosis with 2.8 cm ill-defined mass in segment 2 of the liver and 1.9 cm hypervascular mass in the left kidney. These findings were compatible with the double primary cancers of HCC and RCC. Transarterial chemoembolization (TACE) was performed to treat the HCC. After the TACE, a focal lipiodol uptake defect was noticed on a follow up CT images and loco-regional treatment was recommended. Therefore, we performed RFAs to treat HCC and RCC. There was no evidence of recurrence in the follow up image after 1 month.
Carcinoma, Hepatocellular/complications/*diagnosis/therapy ; Carcinoma, Renal Cell/complications/*diagnosis/therapy ; Catheter Ablation ; Humans ; Kidney Neoplasms/complications/*diagnosis/therapy ; Liver Cirrhosis/complications/*diagnosis ; Liver Neoplasms/complications/*diagnosis/therapy ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Tomography, X-Ray Computed

Sunitinib as a multitarget tyrosine kinase inhibitor is one of the anti-tumor agents, approved by the United States Food and Drug Administration to use treat gastrointestinal stromal tumor and metastatic renal cell carcinoma. The agent is known to commonly induce adverse reactions such as fatigue, nausea, diarrhea, stomatitis, esophagitis, hypertension, skin toxicity, reduciton in cardiac output of left ventricle, and hypothyroidism. However, it has been reported to rarely induce adverse reactions such as nephrotic syndrome and irreversible reduction in renal functions, and cases of intestinal perforation or pneumatosis interstinalis as such reactions have been consistently reported. In this report, a 66-year old man showing abdominal pain had renal cell carcinoma and history of sunitinib at a dosage of 50 mg/day on a 4-weeks-on, 2-weeks-off schedule. Seven days after the third cycle he was referred to the hospital because of abdominal pain. Computed tomography showed pneumoperitoneum with linear pneumatosis intestinalis in his small bowel. The patient underwent surgical exploration that confirmed the pneumatosis intestinalis at 100 cm distal to Treitz's ligament. We report a rare case of intestinal perforation with pneumatosis intestinalis after administration of sunitinib to a patient with metastatic renal cell carcinoma.
Aged ; Antineoplastic Agents/adverse effects/*therapeutic use ; Carcinoma, Renal Cell/*drug therapy ; Drug Administration Schedule ; Humans ; Indoles/adverse effects/*therapeutic use ; Intestinal Perforation/*diagnosis/etiology/surgery ; Kidney Neoplasms/*drug therapy ; Lung/radiography ; Male ; Pneumatosis Cystoides Intestinalis/*diagnosis/etiology ; Positron-Emission Tomography ; Pyrroles/adverse effects/*therapeutic use ; Tomography, X-Ray Computed

The purpose of this study was to compare the dose distribution of intensity-modulated radiotherapy (IMRT) in 7 and 5 fields as well as 3-D conformal radiotherapy (3D-CRT) plans for gastric cancer using dosimetric analysis. In 15 patients with gastric cancer after D1 resection, dosimetric parameters for IMRT (7 and 5 fields) and 3D-CRT were calculated with a total dose of 45 Gy (1.8 Gy/day). These parameters included the conformal index (CI), homogeneity index (HI), maximum dose spot for the planned target volume (PTV), dose-volume histogram (DVH) and dose distribution in the organs at risk (OAR), mean dose (Dmean), maximal dose (Dmax) in the spinal cord, percentage of the normal liver volume receiving more than 30 Gy (V30) and percentage of the normal kidney volume receiving more than 20 Gy (V20). IMRT (7 and 5 fields) and 3D-CRT achieved the PTV coverage. However, IMRT presented significantly higher CI and HI values and lower maximum dose spot distribution than 3D-CRT (P=0.001). For dose distribution of OAR, IMRT had a significantly lower Dmean and Dmax in spinal cord than 3D-CRT (P=0.009). There was no obvious difference in V30 of liver and V20 of kidney between IMRT and 3D-CRT, but 5-field IMRT showed lower Dmean in the normal liver than other two plans (P=0.001). IMRT revealed favorable tumor coverage as compared to 3D-CRT and IMRT plans. Specifically, 5-field IMRT plan was superior to 3D-CRT in protecting the spinal cord and liver, but this superiority was not observed in the kidney. Further studies are needed to compare differences among the three approaches.
Combined Modality Therapy ; Female ; Humans ; Kidney ; radiation effects ; Liver ; radiation effects ; Male ; Middle Aged ; Postoperative Period ; Radiation Injuries ; diagnosis ; prevention & control ; Radiation Monitoring ; methods ; Radiometry ; methods ; Radiotherapy Dosage ; Radiotherapy Planning, Computer-Assisted ; methods ; Radiotherapy, Conformal ; methods ; Radiotherapy, Intensity-Modulated ; methods ; Spine ; radiation effects ; Stomach Neoplasms ; radiotherapy ; surgery

BACKGROUND/AIMS: Nonselective beta-blockers (NSBBs), such as propranolol, reportedly exert a pleiotropic effect in liver cirrhosis. A previous report suggested that survival was higher in patients receiving adjusted doses of NSBBs than in ligation patients. This study investigated whether low-dose NSBB medication has beneficial effects in patients with liver cirrhosis, especially in terms of overall survival. METHODS: We retrospectively studied 273 cirrhotic patients (199 males; age 53.6+/-10.2 years, mean+/-SD) who visited our institution between March 2003 and December 2007; follow-up data were collected until June 2011. Among them, 138 patients were given a low-dose NSBB (BB group: propranolol, 20-60 mg/day), and the remaining 135 patients were not given an NSBB (NBB group). Both groups were stratified randomly according to Child-Turcotte-Pugh (CTP) classification and age. RESULTS: The causes of liver cirrhosis were alcohol (n=109, 39.9%), hepatitis B virus (n=125, 45.8%), hepatitis C virus (n=20, 7.3%), and cryptogenic (n=19, 7.0%). The CTP classes were distributed as follows: A, n=116, 42.5%; B, n=126, 46.2%; and C, n=31, 11.4%. Neither the overall survival (P=0.133) nor the hepatocellular carcinoma (HCC)-free survival (P=0.910) differed significantly between the BB and NBB groups [probability of overall survival at 4 years: 75.1% (95% CI=67.7-82.5%) and 81.2% (95% CI=74.4-88.0%), respectively; P=0.236]. In addition, the delta CTP score did not differ significantly between the two groups. CONCLUSIONS: Use of low-dose NSBB medication in patients with liver cirrhosis is not indicated in terms of overall and HCC-free survival.
Adrenergic beta-Antagonists/*therapeutic use ; Adult ; Aged ; Alcohol Drinking ; Carcinoma, Hepatocellular/complications/diagnosis ; Female ; Follow-Up Studies ; Humans ; Kaplan-Meier Estimate ; Kidney Failure, Chronic/complications/diagnosis ; Liver Cirrhosis/complications/*drug therapy/mortality ; Liver Neoplasms/complications/diagnosis ; Male ; Middle Aged ; Predictive Value of Tests ; Proportional Hazards Models ; Propranolol/*therapeutic use ; Retrospective Studies ; Severity of Illness Index

Angiotensin II type 1 receptor blocker (ARB), which is frequently prescribed in patients with glomerulonephritis (GN), is suggested to increase the risk of cancer. We registered 3,288 patients with renal biopsy and analyzed the relationship between the use of renin-angiotensin-aldosterone system (RAAS) blockade and the incidence of cancer or cancer mortality. After renal biopsy, cancer developed in 33 patients with an incidence rate of 1.0% (95% of CI for incidence: 0.7%-1.3%). There was no difference in the cancer incidence among the groups according to the use of angiotensin-converting enzyme inhibitors (ACEI) or ARB: 1.2% in the None (23/1960), 0.7% in the ARB-only (5/748), 0.4% in the ACEI-only (1/247), and 1.2% in the ACEI-ARB (4/333) (P = 0.487) groups. The cancer mortality was 2.1%, 0.4%, 0.0%, and 0.3% in None, ACEI-only, ARB-only, and ACEI-ARB group, respectively (P < 0.001). The risk of cancer mortality in patients with ARB was only 0.124 (0.034-0.445) compared to that of non-users of ARB by Cox's hazard proportional analysis. In conclusion, prescription of ACEI or ARB in patients with GN does not increase cancer incidence and recipients of ARB show rather lower rates of all-cause mortality and cancer mortality.
Adult ; Aged ; Angiotensin II Type 1 Receptor Blockers/*therapeutic use ; Angiotensin-Converting Enzyme Inhibitors/*therapeutic use ; Female ; Follow-Up Studies ; Glomerulonephritis/complications/diagnosis/*drug therapy ; Humans ; Incidence ; Kidney/pathology ; Male ; Middle Aged ; Neoplasms/complications/*epidemiology/mortality ; Renin-Angiotensin System/*drug effects ; Retrospective Studies ; Risk Factors

Angiotensin II type 1 receptor blocker (ARB), which is frequently prescribed in patients with glomerulonephritis (GN), is suggested to increase the risk of cancer. We registered 3,288 patients with renal biopsy and analyzed the relationship between the use of renin-angiotensin-aldosterone system (RAAS) blockade and the incidence of cancer or cancer mortality. After renal biopsy, cancer developed in 33 patients with an incidence rate of 1.0% (95% of CI for incidence: 0.7%-1.3%). There was no difference in the cancer incidence among the groups according to the use of angiotensin-converting enzyme inhibitors (ACEI) or ARB: 1.2% in the None (23/1960), 0.7% in the ARB-only (5/748), 0.4% in the ACEI-only (1/247), and 1.2% in the ACEI-ARB (4/333) (P = 0.487) groups. The cancer mortality was 2.1%, 0.4%, 0.0%, and 0.3% in None, ACEI-only, ARB-only, and ACEI-ARB group, respectively (P < 0.001). The risk of cancer mortality in patients with ARB was only 0.124 (0.034-0.445) compared to that of non-users of ARB by Cox's hazard proportional analysis. In conclusion, prescription of ACEI or ARB in patients with GN does not increase cancer incidence and recipients of ARB show rather lower rates of all-cause mortality and cancer mortality.
Adult ; Aged ; Angiotensin II Type 1 Receptor Blockers/*therapeutic use ; Angiotensin-Converting Enzyme Inhibitors/*therapeutic use ; Female ; Follow-Up Studies ; Glomerulonephritis/complications/diagnosis/*drug therapy ; Humans ; Incidence ; Kidney/pathology ; Male ; Middle Aged ; Neoplasms/complications/*epidemiology/mortality ; Renin-Angiotensin System/*drug effects ; Retrospective Studies ; Risk Factors