1/159. Total parenteral nutrition and home parenteral nutrition: an effective combination to sustain malnourished children with cancer.A patient with Wilms' tumor and severe failure to thrive required total parenteral nutrition (TPN) for "catch-up" growth. This case underscores how TPN might be useful in the management of a child with cancer. Cancer cachexia, chemotherapy, radiation, and infections caused by immune suppression can lead to potentially serious macro- and micronutrient deficiencies.- - - - - - - - - - ranking = 1keywords = cancer (Clic here for more details about this article) |
2/159. Metanephric adenofibroma: report of a case and review of the literature.The recent recognition of a variety of pediatric renal tumors of different biologic behavior places an ever-increasing demand on the surgical pathologist for an accurate diagnosis. Although metanephric adenofibroma is one of the rarest benign renal tumors, the clinical importance of correctly diagnosing it cannot be overemphasized because it can potentially be mistaken as Wilms' tumor. We describe the clinical, radiologic, and pathologic features of a case of metanephric adenofibroma and discuss its differential diagnosis. The neoplasm was composed of two discrete components: a major fibroblastic element and a minor immature epithelial element. The latter formed a small nodule beneath the renal capsule, which could barely be detected by magnetic resonance imaging. This subcapsular nodule, however, was slightly soft and tan and was distinctly different from the white, whorled cut surface of the main tumor. It was formed by closely packed small immature epithelial cells in a slightly edematous background, which was histologically identical to metanephric adenoma and closely resembled epithelial Wilms' tumor. Unlike Wilms' tumor, however, the epithelial cells were very bland with no mitoses. The main bulk of the tumor was formed by spindle fibroblastic cells that were cytologically similar to the spindle cells in congenital mesoblastic nephroma. The tumor, however, was well demarcated without the irregular infiltrating edges of congenital mesoblastic nephroma. In contrast to the randomly distributed epithelial element throughout the stromal component in previous reported cases of metanephric adenofibroma, our finding of the exceedingly small and discrete epithelial component expands the known histologic spectrum of the disease. In addition, the presence of such minute epithelial nodule underscores the importance of diligent pathologic examination and careful sampling of tissue for histologic examination.- - - - - - - - - - ranking = 1.2910263350888keywords = neoplasm (Clic here for more details about this article) |
3/159. syndrome of microcephaly, Dandy-Walker malformation, and wilms tumor caused by mosaic variegated aneuploidy with premature centromere division (PCD): report of a new case and review of the literature.We report a male infant with multiple congenital anomalies and mosaic variegated aneuploidy; a rare cytogenetic abnormality characterized by mosaicism for several different aneuploidies involving many different chromosomes. He had prenatal-onset growth retardation, microcephaly, dysmorphic face, seizures, hypotonia, feeding difficulty, and developmental delay. In addition, he developed bilateral Wilms tumors. Neuroradiological examination revealed Dandy-Walker malformation and hypoplasia of the cerebral hemisphere and pons. cytogenetic analysis revealed various multiple numerical aneuploidies in blood lymphocytes, fibroblasts, and bone marrow cells, together with premature centromere division (PCD). Peripheral blood chromosome analysis from his parents also showed PCD, but no aneuploid cells. The clinical phenotype and multiple aneuploidies of the patient may be a consequence of the homozygous PCD trait inherited from his parents. Comparison with previously reported cases of multiple aneuploidy suggests that mosaic variegated aneuploidy with PCD may be a clinically recognizable syndrome with major phenotypes being mental retardation, microcephaly, structural brain anomalies (including Dandy-Walker malformation), and possible cancer predisposition.- - - - - - - - - - ranking = 0.2keywords = cancer (Clic here for more details about this article) |
4/159. Bilateral renal cancer in children: a difficult, challenging and changing management problem.Bilateral disease occurs in 6% of patients with Wilms' tumour. Bilateral renal involvement is present in 25%-50% of children with B-cell non-Hodgkin's lymphoma. Paediatric oncologists therefore encounter bilateral renal disease relatively frequently. A patient with bilateral Wilms' tumour is presented, in whom at least 16 synchronous tumours in the kidneys were treated successfully by primary chemotherapy and 'nephron-sparing' surgery, without renal radiotherapy. We believe the successful treatment without radiotherapy will allow greater potential for normal growth in the future. The case history of a patient with renal failure due to the infiltration of both kidneys by lymphoma and who was treated successfully by chemotherapy, is also presented. One kidney has completely returned to normal function and growth; the other has completely failed and almost disappeared, demonstrating complete reversibility of the damage up to some critical point, beyond which, failure and atrophy result. These two case histories demonstrate extreme examples of clinical dilemmas involving bilateral renal tumours and allow discussion of modern management aimed at preserving renal function.- - - - - - - - - - ranking = 0.8keywords = cancer (Clic here for more details about this article) |
5/159. Malignant neoplasms after treatment for metachronous bilateral Wilms' tumor: a literature review.Information regarding malignant neoplasms after treatment for metachronous bilateral Wilms' tumor is limited. A medline search was performed of all English-language articles from 1950 to 1997 pertaining to metachronous bilateral Wilms' tumor. A total of 108 different cases were identified and analyzed. Mean follow-up was 5.8 years after initial diagnosis of Wilms' tumor (range, 1 month to 25.6 years). Eleven of 63 evaluable children (17.5%) had a congenital anomaly. Four patients (3.7%) developed a malignant neoplasm after treatment of a metachronous bilateral Wilms' tumor. Three of 18 patients followed for at least 10 years developed a solid tumor, including two sarcomas in the irradiated areas. Two of the 4 children who developed a malignant neoplasm had a congenital anomaly. Malignant neoplasms after treatment for metachronous bilateral Wilms' tumor can occur. health-care professionals caring for these patients should be aware of this late sequelae of treatment.- - - - - - - - - - ranking = 10.32821068071keywords = neoplasm (Clic here for more details about this article) |
6/159. Clinicopathologic and cytologic features of a metanephric adenoma of the kidney: a case report.Metanephric adenoma is a recently described rare and benign renal neoplasm. Our patient, a 37-year-old woman, suffered from flank pain for five months and was found to have a renal mass. Ultrasound, computerized tomography and angiography findings were consistent with a hypovascular renal cyst. Wilms' tumor was the initial misdiagnosis, based on needle biopsy and aspiration cytology. A radical nephrectomy was performed. Histologically, the tumor was well defined and was composed of uniform small cells arranged in a solid, tubular or rosette-like pattern. The prognosis is good for metanephric adenoma. The tumor was first considered a benign counterpart of papillary carcinoma or Wilms' tumor; however, recent cytogenetic evidence suggested that the tumor might be related to papillary adenoma and papillary renal cell carcinoma. The clinical, radiologic, histologic and cytologic features presented here should help to promote the correct preoperative diagnosis and to avoid unnecessary aggressive treatment.- - - - - - - - - - ranking = 1.2910263350888keywords = neoplasm (Clic here for more details about this article) |
7/159. Antithrombin treatment of severe hepatic veno-occlusive disease in children with cancer.hepatic veno-occlusive disease (VOD) is a well-known complication of chemotherapy in wilms tumor patients, particularly young children. Although this complication resolves uneventfully in most patients, fatal cases have been reported. Severe VOD after transplantation has a high mortality rate ranging from 45% to 98%. New hemostatic therapeutic strategies have significantly improved the prognosis of VOD. Chemotherapy-related VOD in wilms tumor usually has a good prognosis. We describe two patients with wilms tumor and one with acute lymphoblastic leukemia, who developed severe veno-occlusive disease of the liver according to the baltimore criteria while undergoing chemotherapy; the symptoms were hepatomegaly, ascites, hyperbilirubinemia, weight gain and, in one patient, short-term lethargy. Elevated LDH levels of 872 to 12,000 U/l were observed in our patients. All patients had thrombocytopenia between 29,000 and 40,000/microl and decreased antithrombin (AT) and protein c levels; two patients had gastrointestinal bleeding. All patients developed a coagulopathy because of severe hepatic dysfunction. Two patients received low-dose heparin at the onset of VOD. The thrombolytic therapy was rapidly changed to AT supplementation (20-80 IU/kg bw 2x per day) without heparin when thrombocytes were very low or gastrointestinal bleeding occurred. Resolution of VOD was observed in all patients receiving AT alone. The chemotherapy was discontinued in a patient with accidental actinomycin D overdosage in view of the severity of symptoms. The remaining two patients received chemotherapy according to the therapy protocol after restitution. All patients survived without sequelae with a median follow-up of 28 months (range 8-48 months). CONCLUSION: hepatic veno-occlusive disease is a rare but increasingly recognized complication in pediatric cancer patients receiving conventional chemotherapy. AT supplementation constitutes a good alternative treatment of severe VOD in comparison with other thrombolytic therapies, particularly in patients at high risk of bleeding.- - - - - - - - - - ranking = 1keywords = cancer (Clic here for more details about this article) |
8/159. Partial nephrectomy in cystic partially differentiated nephroblastoma.Cystic partially differentiated nephroblastoma (CPDN) is an uncommon renal neoplasm in children. It is now recognized as a tumor with low but definite malignant potential. The authors report a patient that was treated with partial nephrectomy and chemotherapy with successful outcome. The literature on CPDN is briefly discussed.- - - - - - - - - - ranking = 1.2910263350888keywords = neoplasm (Clic here for more details about this article) |
9/159. A malignant rhabdoid tumor of the kidney occurring concurrently with a brain tumor: report of a case.Malignant rhabdoid tumor of the kidney (MRTK) is one of the most lethal neoplasms to occur in young infants. Cases of MRTK accompanying an embryonal tumor in the central nervous system have occasionally been described. We present herein an interesting case of MRTK that was clinically diagnosed preoperatively. A male infant aged 6 months with both a midline brain tumor and a renal neoplasm was transferred to our institution. Although roentgenographic evaluation suggested that the renal lesion was a Wilms' tumor, midkine (MK), a growth and differentiation factor characteristically present in the urine of patients with Wilms' tumor, was not detected. A preoperative diagnosis of MRTK was established based on the lack of urinary MK in addition to the typical clinical features of the young age and the concurrent brain tumor.- - - - - - - - - - ranking = 2.5820526701776keywords = neoplasm (Clic here for more details about this article) |
10/159. Synchronous wilms tumor and fibrolamellar hepatocellular carcinoma: report of a case.Fibrolamellar hepatocellular carcinoma (FHCC) is a unique histologic variant of HCC that occurs in a younger subset of patients than classical HCC, and is associated with a better prognosis. wilms tumor (WT) is a malignant embryonal neoplasm of the kidney and is one of the most common solid tumors of childhood, occurring at an estimated frequency of 1 in 8000 to 10,000 births. Although second malignant neoplasms (SMNs) following therapy for WTs have been reported in the liver, the coexistence of HCC and WT is extremely rare. We present the first report of a synchronous anaplastic WT and FHCC in a previously healthy 4-year-old girl. Despite the presence of focal immunohistochemical positivity for p53 in the WT, molecular analysis failed to reveal a germline or somatic p53 mutation, and was inconclusive in establishing a clonal relation between the two tumors.- - - - - - - - - - ranking = 2.5820526701776keywords = neoplasm (Clic here for more details about this article) |
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