1/9. Secondary cardiac tumor in children.We describe our clinical experience of eight cases of secondary cardiac tumor. The pathology of the tumors were lymphoma (three), Wilms' tumor (two), malignant teratoma (one), neuroblastoma (one), and pleuropulmonary blastoma (one). Metastatic sites were the right atrium in Wilms' tumor and neuroblastoma, the left atrium in pleuropulmonary blastoma and malignant teratoma, and multiple sites in lymphoma. Primary masses in the mediastinum extended directly to the heart (three lymphoma, malignant teratoma, pleuropulmonary blastoma). Wilms' tumor and neuroblastoma showed cardiac metastases through the inferior vena cava. Many cases revealed vague abnormal cardiovascular findings (symptoms in six; physical signs in five). In five cases surgery was performed to relieve the possible obstruction to flow and to identify the pathology (lymphoma in three, Wilms' tumor in one, and malignant teratoma in one). Chemotherapy prior to operation resulted in the disappearance of the intracardiac masses in each case of Wilms' tumor and pleuropulmonary blastoma. All three patients with lymphoma died immediately after operation. Four died of multiple metastases or pneumocystis pneumonia several months after operation. This study indicates that suspicion of a secondary cardiac tumor is crucial to early diagnosis. Because of the poor postoperative outcome, surgery for secondary cardiac tumors should be done cautiously only in cases with definite hemodynamic decompensation.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/9. adult Wilms' tumor associated with polycythemia--a case report.Both Wilms' tumor (nephroblastoma) in adults and polycythemia in Wilms' tumor are rare. Herein we report an extremely rare case of adult Wilms' tumor associated with polycythemia. A 41-year-old female was incidentally found to have right renal mass by abdominal sonography in a routine health examination. Laboratory examination revealed polycythemia (hemoglobin 20.2 g/dL). Although physical examination was unremarkable, CT scan revealed an homogeneous mass at the middle pole of right kidney, and chest x-ray revealed no metastatic lesions. Right radical nephrectomy was performed smoothly. Grossly, the tumor of 5 x 4.5 x 4.5 cm in size was well circumscribed, and had no vascular structure or collecting system involvement. Microscopic features were consistent with adult nephroblastoma. The post-operative course was uneventful. At follow-up, the patient was well, showed no evidence of recurrence and her hemoglobin level had returned to normal (hemoglobin 14.5 g/dl). We suggest that the relationship between polycythemia and Wilms' tumor should be carefully evaluated before surgical treatment.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/9. Facilitating care for childhood cancer survivors: integrating children's oncology group long-term follow-up guidelines and health links in clinical practice.Childhood cancer survivors are a growing, vulnerable group with health care needs unique to their cancer treatments. They may experience many late physical and psychological complications (late effects) of treatment including organ dysfunction, infertility, second neoplasms, chronic hepatitis, musculoskeletal problems, alterations in cognitive function, and myriad psychosocial problems. Health care providers may be unaware of actual or potential survivor problems. Until recently, there were no clearly defined, easily accessible risk-based guidelines for cancer survivor follow-up care. This article will use a case-study approach to demonstrate how the newly developed Children's Oncology Group Long-term Follow-up Guidelines and Health Links can be used in clinical practice to improve awareness about late effects and the importance of follow-up care for childhood cancer survivors. The Children's Oncology Group Guidelines and Health Links were created by a multidisciplinary team of health care experts and patient advocates to provide a systematic plan for pediatric cancer survivor follow-up care and health education across the cancer continuum.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
4/9. Passive seeding in metanephric adenoma: a review of pseudometastatic lesions in perinephric lymph nodes.Lymph node involvement derived from a discrete neoplastic process fundamentally implies tumor malignancy. However, rarely, inconsequential passive transport of benign neoplastic cells to the lymph node can occur and may cause confusion as to the nature of the neoplasm (ie, malignant vs benign). We describe a 10-cm right renal metanephric adenoma incidentally discovered in a 30-year-old woman during cesarean section for a triplet pregnancy. Subsequent nephrectomy following an equivocal needle biopsy diagnosis showed histologic features classic for metanephric adenoma, including the lack of cytologic atypia and mitoses. necrosis present in this lesion appeared to be secondary to tumor physical disruption. The tumor cells were positive for wilms tumor 1 (WT1) antigen, pankeratin, and CD57, focally positive for epithelial membrane antigen, and negative for cytokeratin 7, cytokeratin 34betaE12, and CD56. Electron microscopy confirmed the tumor's epithelial nature, and cytogenetics revealed a diploid 46XX karyotype. The tumor proliferation index with Ki-67 was only 3% to 5% and the proliferating cell nuclear antigen index was 0%. A single, concurrently resected hilar lymph node contained scattered subcapsular, sinusoidal, and focally intralymphovascular psammoma bodies along with occasional adherent epithelial cells. These cells were highlighted by pankeratin but were nonreactive to WT1 antigen, similar to the nonviable cells in the primary tumor. Clinical surveillance and follow-up showed no disease recurrence 4 years after nephrectomy. We postulate that the lymph node inclusions found in this case represent passive transport of neoplastic cells to the lymph node following manipulation of the renal mass. We conclude that this phenomenon is understudied and underrecognized and can easily be mistaken for metastasis.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
5/9. rhabdomyosarcoma, wilms tumor, and deletion of the patched gene in Gorlin syndrome.BACKGROUND: A 5-year-old year girl with a medical history of mental retardation, physical abnormalities and a known interstitial deletion of chromosome 9q22-q32 presented with a palpable suprapubic mass. During ultrasound investigation, a left renal mass was also detected. The patient underwent surgical removal of both neoplasms, which were diagnosed as a rhabdomyosarcoma and a wilms tumor. Seven years later, she presented with macroglossia and a benign mandibular cyst. INVESTIGATIONS: physical examination, karyotyping, abdominal and pelvic ultrasound, brain CT scan, anatomic pathology analysis with immunohistochemistry, and typing of polymorphic markers in the patched (PTCH) gene region. DIAGNOSIS: Gorlin syndrome with synchronous rhabdomyosarcoma and wilms tumor. MANAGEMENT: Left nephrectomy, excision of paravesical tumor, excision of mandibular cysts, chemotherapy, and radiotherapy.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
6/9. Wilms' tumor in a multilocular cyst of the kidney (cystic Wilms' tumor).A 2-year-old boy had a Wilms' tumor of the left kidney that appeared during physical examination as an abdominal mass and on gross examination as a multilocular cyst. Multilocular cyst of the kidney is a rare but distinctive congenital lesion. Its true biologic behavior is unknown. Although simple nephrectomy is sufficient for most lesions, more aggressive procedures are recommended for those lesions that contain Wilms' tumor (cystic Wilms' tumor).- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
7/9. adult Wilms' tumor: report of a case and review of the literature.A 48 year old male presented with a one and a half year history of a progressively increasing, asymptomatic lump in the left hypochondrium and no history of hematuria. His general physical examination was unremarkable, and an abdominal examination revealed a large, firm, irregular and tender mass in the left hypochondrium extending into the lumbar region. Chest X-ray was normal. An intravenous urogram revealed a normally functioning right kidney with non-visualization of the left kidney. CT-scan of the abdomen revealed a large, mixed attenuating mass replacing the left kidney. At laparotomy, a large, fleshy, well-encapsulated tumor was found in the left kidney with no surrounding infiltration and a left radical nephrectomy was performed. Microscopic examination revealed a poorly differentiated tumor comprised of small round cells with focal areas of abortive embryonal tubular and glomerular differentiation suggestive of Wilms' tumor. The patient was advised chemotherapy and radiotherapy but he absconded and was lost to follow-up.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
8/9. Cornelia de lange syndrome associated with Wilms' tumour and infantile haemangioendothelioma of the liver: report of two autopsy cases.Two cases of Cornelia de lange syndrome associated with infantile haemangioendothelioma of the liver and Wilms' tumour are reported. The patients showed the characteristic facies of the Cornelia de lange syndrome, with synophrys, long curly eyelashes and small upturned nose, and physical features, including generalized hirsutism, monodactyly, syndactyly and clinodactyly. Post-mortem examination revealed annular pancreas, patency of the foramen ovale, duodenal atresia and evidence of cytomegalic infection. The cases are reported to document a possible association between malformations and neoplasms in this syndrome.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
9/9. Physical localisation of the breakpoints of a constitutional translocation t(5;6)(q21;q21) in a child with bilateral Wilms' tumour.A 6 month old boy presented with bilateral Wilms' tumour. cytogenetic analysis of the lymphocytes from the patient showed a de novo balanced translocation t(5;6)(q21;q21), which was also present in the tumour material as the sole cytogenetic abnormality. To facilitate the identification of the translocation breakpoints, we have established a lymphoblastoid cell line (MA214L) from the patient which maintains the translocation in culture. We have used Genethon microsatellite markers as sequence tagged sites (STSs) to isolate yeast artificial chromosome (YAC) clones to 5q and 6q from human genomic libraries. Using fluorescence in situ hybridisation (FISH) on metaphase preparations of MA214L, we have physically defined the translocation breakpoints between YAC clones on each chromosome arm. The genetic distance separating the flanking YACs on 6q21 is 3 cM, while that on 5q21 is 4 cM. To date this is the first report of these chromosomal regions being implicated in Wilms' tumourigenesis.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |