Renal medullary carcinoma: a potential sickle cell nephropathy of children and adolescents

cDNAs of potyviruses from Allium plants cultivated in different parts of the world were cloned by RT-PCR with a common primer for amplifying the 3' terminal genomic RNAs of onion yellow dwarf virus (OYDV), leek yellow stripe virus (LYSV) and, probably, of closely related potyviruses. Their nucleotide sequences bearing the viral coat protein (CP) gene and the 3' non-coding sequence were determined and compared. The degree of their sequence similarities clearly differentiated the respective viruses into 3 groups, namely OYDV "garlic-type", "wakegi-type" and LYSV group. The "garlic-type" included all the garlic isolates and two Indonesian shallot isolates. The "wakegi-type" group consisted of the isolates from Indonesian shallot and previously reported ones from Japanese Allium plants excluding garlic. The LYSV group was represented by LYSV isolates from garlic and leek. The CP sequences of LYSV group viruses differed from those of OYDV "garlic-type" and "wakegi-type" viruses (less than 60% similarities). In contrast, the sequence similarities between the OYDV "wakegi-type" and "garlic-type" isolates were 73.5 to 76.7%, suggesting they were closely related but should be discriminated as distinct species. These findings indicate that at least three distinct potyviruses, clearly distinguishable by the viral CP sequence, are present in Allium species. Finally, we concluded that the "garlic-type" viruses correspond to the typical OYDV and the "wakegi-type" viruses represent the viruses previously identified as Welsh onion yellow stripe virus (WoYSV) and shallot yellow stripe virus (SYSV). We propose the name wakegi yellow dwarf virus (WYDV) for the "wakegi-type" viruses.     

Renal cell carcinoma

This is a report on two cases of mesomelic dysplasia Langer type. This is a rare kind of dwarfism which combines severe dwarfism with predominant shortening of the forearms and lower legs and other morphological abnormalities. The diagnosis can be made by ultrasonography in the early second trimester of the pregnancy.     

Renal medullary carcinoma

Hyperhomocysteinemia is an independent risk factor for coronary artery and cerebrovascular disease, but its significance in the perioperative period is unknown. Nitrous oxide inhibits methionine synthase, which aids in the conversion of homocysteine to methionine. In this prospective, controlled, randomized study, we determined the effect of intraoperative nitrous oxide exposure on postoperative plasma homocysteine concentrations. Twenty ASA physical status I-III patients, aged >18 yr, presenting for elective craniotomy, were randomized to receive general anesthesia with or without nitrous oxide (inspired nitrous oxide >50%). Plasma was sampled before the induction of anesthesia, on arrival in the postanesthesia care unit (PACU) after discontinuation of nitrous oxide, and 24 h after induction. There was a significant increase (22.6+/-11.4 vs 13.0+/-4.7 micromol/L; P = 0.0038 for postoperative versus preinduction values) in plasma homocysteine concentrations in the nitrous oxide group on arrival in the PACU and for 24 h. In the nonnitrous oxide group, mean plasma homocysteine concentrations did not change (9.5+/-1.9 vs 9.8+/-1.6 micromol/L; P = 0.86 for postoperative versus preinduction values). The change in plasma homocysteine concentrations in the nitrous oxide group was significantly different from that in the nonnitrous group (P = 0.0031). We conclude that the use of intraoperative nitrous oxide leads to significant increases in perioperative plasma homocysteine concentrations. IMPLICATIONS: Short-term exposure to nitrous oxide led to significant increases in plasma homocysteine. Further investigations are required to determine the clinical significance of this change.     

Renal carcinoma in the dog

Thirty-three cases of canine renal carcinoma reported between 1907 and 1972 are reviewed, and clinical and pathological features of nine other cases seen in Bristol between 1966 and 1975 are described. Clinical features frequently include nonspecific signs of anorexia and weight loss. The age range of affected dogs varies from 3 to 15 years, with a mean of 7.1 years. Male dogs appear to be affected more frequently than females, but there is no breed predilection. The clinical behaviour of canine renal carcinoma is variable, with 4 years the longest survival time after nephrectomy. The primary tumours characteristically are large and unilateral and occupy most of one pole of a kidney. Metastatic spread can involve a wide range of organs, the commonest being lymph nodes and lung. Solid, tubular and papillary patterns are common. Clear cell carcinoma is uncommon.     

Renal carcinoma and von Hippel-Lindau disease

We report a case of life-threatening esophageal hemorrhage after metal stent implantation successfully treated by arterial embolization. An 85-year-old woman was admitted in shock secondary to massive hematemesis and melena. Recent medical history revealed esophageal cancer treated 8 weeks previously by endoesophageal radiotherapy (40 Gy) and endoscopic placement of a covered Wall-stent prosthesis. Selective arteriography of the fifth posterior right intercostal artery showed massive contrast extravasation in the esophagus. Embolization was performed with 150-250-micron polyvinyl alcohol particles. Follow-up at 5 months was uneventful. Arteriography and embolization are advised when severe hemorrhage occurs after esophageal implantation of metal stents.     

Renal cell carcinoma in a patient with Beckwith-Wiedemann syndrome

We report the case of a patient with Beckwith-Wiedemann syndrome (BWS) who developed renal cell carcinoma (RCC). At birth, this patient presented with macroglossia, diastasis recti, mild gigantism, hepatomegaly and hypoglycemia, and the diagnosis of BWS was made. At 22 months, an intrapelvic rhabdomyosarcoma was detected and resected. At 37 months, computed tomography (CT) demonstrated a small mass with high attenuation in the right kidney, which was surgically confirmed to be RCC.     

Renal masses simulating primary renal cell carcinoma in patients with advanced malignancies

Most patients who present with a large solid renal mass and evidence of advanced malignancy will have primary renal cell carcinoma but a small subset with similar features have different and more treatable malignancies. We identified 7 patients with clinical and radiological findings suggestive of metastatic renal cell carcinoma who were ultimately diagnosed as have non-Hodgkin's lymphoma (5), germ cell tumor (1) or transitional cell carcinoma (1). Two of these patients presented with abdominal pain, gross hematuria and a flank mass. Computerized tomography was interpreted as showing renal cell carcinoma in all patients, although lymphoma and sarcoma were included in the differential diagnoses in 2. With the correct diagnosis and appropriate therapy, 4 of the 7 patients are currently disease-free. We emphasize the need for histological documentation in such patients in view of curative therapy available for possible underlying neoplasms simulating renal cell carcinoma.     

Renal cell carcinoma in a horseshoe kidney: report of two cases

We report 2 patients with renal cell carcinoma in a horseshoe kidney. A renal tumor was diagnosed during evaluation of right humeral metastatic cancer of unknown origin in case 1 (64-year-old male) and gross hematuria in case 2 (54-year-old male). Both patients underwent radical nephrectomy with division of isthmus and retroperitoneal lymph node dissection. The tumors were staged pathologically as pT2bpN0pV0pM1(oss) and pT3pN0pV1bM0, respectively. Only 27 cases of renal cell carcinoma developed in a horseshoe kidney, including the present two cases, have been reported in the Japanese literature. The blood supply to the horseshoe kidney and its evaluation by preoperative angiography were discussed.     

Renal transplantation in patients with renal cell carcinoma and von Hippel-Lindau disease

This study reviewed the management and outcome of patients with von Hippel-Lindau disease (VHLD) who underwent renal transplantation after being rendered anephric to treat multifocal bilateral renal cell carcinoma (RCC). Five patients with bilateral RCC and VHLD underwent renal transplantation at our hospital. Initial treatment of RCC consisted of bilateral nephrectomy in 2 patients and unilateral nephron-sparing surgery with contralateral nephrectomy in 3 patients. All of the latter 3 patients experienced isolated tumor recurrence in the renal remnant at 48, 64, and 66 months postoperatively; this was managed by complete excision of the renal remnant. Renal transplantation was performed 11 to 24 months after initiation of dialysis. Postoperatively, all of the allografts functioned well with no further requirement for dialysis. Currently, 4 patients are alive at a mean post-transplant follow-up interval of 26 months (range, 7 to 66 months) with excellent graft function and no evidence of malignancy. One patient died 17 months following transplantation due to metastatic disease. Renal transplantation can provide satisfactory replacement therapy for patients with end-stage renal disease with VHLD and treated RCC.     

Post-Chernobyl thyroid carcinoma in Belarus children and adolescents: comparison with naturally occurring thyroid carcinoma in Italy and France

After the Chernobyl nuclear accident (April 26, 1986), childhood thyroid carcinoma had a great increase in Belarus and Ukraine, as a consequence of the exposure to iodine radioactive fallout. The epidemiological and clinical features of the disease were studied in 472 patients less than 21 yr old at diagnosis, with differentiated thyroid carcinoma, representing 97.7% of all thyroid carcinomas diagnosed in Belarus between May, 1986, and December, 1995. The results were compared with those of 369 subjects of the same age group, with naturally occurring thyroid carcinoma, observed in Italy and France. Between 1986 and 1989, the number of thyroid cancer cases per year ranged from 3-8 and increased to 31 in 1990, to 66 in 1991, to 72 in 1992, to 93 in 1993, to 96 in 1994, and to 90 in 1995. The age at diagnosis was 14 yr or less in 78.8% (children group) and more than 14, but less than 21, yr in the remaining subjects (adolescents group). Mean (+/- SD) age at the time of the accident was 4.4 +/- 3.4 yr (3.2 +/- 2.3 in children and 8.9 +/- 2.7 in adolescents), the majority of the patients (62.9%) being 5 yr old or less. The time interval between the accident and the diagnosis (latency period) decreased progressively from 7.5 +/- 1.6 yr in children 0-2 yr old at the time of the accident to 6.0 +/- 1.6 yr in those 9-11 yr old. Since 1993, the yearly distribution of new cases showed a decrease in the subjects 9 yr old or more at the time of the accident but not in those 5 yr old or less. This could not be accounted for by a shift of exposed subjects to an age group at diagnosis not included in this study, because only subjects less than 12 yr of age at the time of the accident were considered in this analysis. Mean age at diagnosis in Belarus patients was 11.3 +/- 3.1 yr (10.1 +/- 2.3 in children and 15.7 +/- 1.4 in adolescents), whereas, among patients with naturally-occurring thyroid carcinomas from Italy and France, the majority of cases were diagnosed after 14 yr of age (mean age at diagnosis: 14.6 +/- 4.2 yr). The female-to-male ratio was significantly higher in Italy and France (2.5/1), compared with the ratio of patients from Belarus (1.6/1). Most of the tumors were papillary in both series, but a relatively high proportion of follicular carcinomas (P = 0.0001) was found in Italy/France (15.2%), as opposed to 5.3% in Belarus. Extrathyroidal extension and lymph node metastases were more frequent in Belarus (49.1%, P = 0.0001; and 64.6%, P = 0.002, respectively) with respect to Italy/France (24.9% and 53.9%, respectively). Thyroid lymphocytic infiltration and circulating antithyroperoxidase antibody were more frequent in Belarus patients. Our analysis of Belarus thyroid cancer patients less than 21 yr old showed that the post-Chernobyl increase in thyroid carcinomas involved both children and, to a much lesser extent, adolescents. Subjects 5 yr old or less at the time of the accident accounted for the majority of the patients. No evidence of a decrease in the number of new cases was observed in this age group, as opposed to older subjects. These data support the concept that subjects who were younger at the time of radiation exposure had, and continue to have, a greater risk of developing thyroid carcinoma and strongly suggest that this age group should be carefully monitored in the future. When compared with naturally occurring thyroid carcinoma of the same age group observed in Italy and France, the post-Chernobyl Belarus thyroid carcinomas affected younger subjects, were less influenced by gender, were virtually always papillary, had a greater aggressiveness at presentation, and were more frequently associated with thyroid autoimmunity.     

Renal cell carcinoma: the Ochsner Medical Institution experience (1945-1978)

We reviewed 161 patients operated upon for renal cell carcinoma between 1945 and 1978. Life table and survival analyses were computed to compare the effects of stage, tumor differentiation, cell type, surgical technique, renal vein involvement and sex on the years of survival. Patients with stage I and well differentiated tumors had the best prognosis. All patients surviving 10 years or more had well differentiated tumors. The type of nephrectomy did not affect survival and lymphadenectomy was only of value in staging the disease. The stage and differentiation of the tumor were more important to outcome than choice of therapy.