Summary information; Polski (, pdf). Review article; English () · Italiano (). Clinical practice guidelines; Deutsch (). Clinical genetics review. , bestthing.info l_S1_Nephroblastom_Wilms-. Tumor_pdf uri.  Graf N, Rübe C, Gessler M. SIOP Studies. 7. NWTS Studies. UK Studies. Rationale for SIOP Pre-operative treatment. Risk stratification following.
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Nephroblastoma is one of the most common causes of abdominal-pelvic mass in children. It still raises a diagnostic problem because of the. Neuroblastoma (NBL) is the most common extra-cranial tumour in childhood. It can present as an abdominal mass, but is usually metastatic at diagnosis so the. Available online: bestthing.info, accessed August Mitry E, Ciccolallo L, Coleman MP, et al.
This variability means survival rates also differ.
Associations and risk factors The vast majority of cases are sporadic. NBLs have a variable prognosis; tumour stage, patient age, tumour oncogenes and DNA content are all known to be implicated.
The MYCN oncogene is responsible for providing the code used by proteins in tissue development.
If this mutates, which can be signalled by abnormal amplification, cancerous cells can develop and the resulting mass is more resistant to treatment, thus it has a more unfavourable outcome [ 1 ].
This is thought to be secondary to a reduction in mitosis [ 1 ]. Other markers which can affect management include chromosomes and nerve receptors. Alterations in two chromosomes, namely a deletion on the short arm of chromosome 1 1p seen in around a quarter of NBLs, and deletion of chromosome 11q have a poorer prognosis [ 1 ].
Although 1p deletion is associated with MCYN amplification, 11q is not correlated and appears to have separate negative prognostic factors. TrkA is a neurotrophin receptor that may actually be associated with an improved prognosis. Clinical features Presenting features are diverse and very much dependent on the anatomical location of the tumour. Most commonly, NBLs are located within the adrenal gland, but can be found in sympathetic ganglia of the retroperitoneum, posterior mediastinum, neck or pelvis [ 1 ].
Wilms’ tumor: biology, diagnosis and treatment
The organ of Zuckerkandl is a mass of neural crest tissue adjacent to the mid to distal abdominal aorta and it is another recognized site of disease. Abdominal masses usually cause pain due to their mass effect, as well as abdominal distension [ 6 ]. They often grow to a large size before causing problems, so a palpable mass on presentation is common. Abdominal masses can also compress renal vessels resulting in hypertension being a presenting feature. Thoracic NBL may present with airway compromise, scoliosis or as an incidental finding on chest x-ray.
The biology of thoracic NBL tends to be less aggressive than with abdominal disease and as such the prognosis tends to be more favourable. Paraneoplastic syndromes may be associated with non-metastatic disease.
The other is the excessive production of vasoactive intestinal peptide VIP resulting in watery diarrhoea and failure to thrive [ 1 ]. Common sites of metastases include liver, lymph nodes and bone marrow [ 1 ]. Diagnosis Plain films are non-specific for NBL and are largely unhelpful in the diagnostic pathway. Features that are suggestive of thoracic disease include abnormalities of the normal silhouettes typically seen on chest x-rays.
The right and left paraspinal lines are where the lung or pleura interacts with the mediastinal soft tissues.
Lymph nodes within the abdomenal or pelvic lymph nodes contain the tumor. The tumor has penetrated through the peritoneal surface. Peritoneal surface has tumor implants. Gross or microscopic tumor remains postoperatively. The tumor is not completely resectable due to local infiltration into vital structures.
Either before or during the surgery, tumor spillage occurs.
The tumor was biopsied using tru-cut biopsy, open biopsy, or fine needle aspiration before removal. The tumor is removed in more than one piece. Stage IV: At this stage, lung, liver, bone, brain, etc or lymph node metastases outside the abdominopelvic region are detected.
Stage V: Bilateral involvement is detected. Each side should be staged according to the criteria to determine the extent of the disease. Classic Wilms' tumors are triphasic and composed of epithelial, blastemal, and stromal elements. Diffuse anaplastic changes generally predict a poor outcome. Wilms tumors with anaplastic changes are called unfavorable histology and require more aggressive treatment.
Wilms tumors can also be seen in association with Trisomy Epidemiology Wilms' tumor affects 1 in 10, children in North America.
Therefore it is the most common pediatric abdominal malignancy and the fourth most common childhood malignancy. Clinics There doesn't seem to be a sex preference concerning the incidence. Blastematous tissue with some differentiated glomerular structures associated with mesenchymal tissue and tubules.
Courtesy Pierre Bedossa Treatment Multimodality therapy including nephrectomy is used for the management of all stages of Wilms' tumor. Chemotherapy has proven beneficial in all stages of the disease and radiation therapy is used to improve the outcome of later stage tumors, including stage II malignancies with diffuse anaplastic changes.
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Prognosis Wilms' tumor can be classified into favorable and anaplastic histology groups for prognostic purposes. Favorable histology group does not have anaplastic cells in the tumor.It enables to determine whether there is an evidence of extension into the inferior vena cava or beyond, and to check for the liver metastases.
However combined 1p and 16q loss is only identified in 2. The most common complications are cardiotoxicity 4. Thoracic NBL may present with airway compromise, scoliosis or as an incidental finding on chest x-ray. The tumor has been biopsied prior to removal or there is local spillage of tumor during surgery, confined to the flank.
Either before or during the surgery, tumor spillage occurs. The primary goals were to maximise cure while minimising toxicity.
The group recommends pre-operative chemotherapy which is a logical strategy for patients with large tumors, in a setting where supportive care is restricted and radiotherapy may not be available