Wilms tumor

Synonyms in the broader sense

Nephroblastoma, tumor, cancer

definition

Of the Wilms tumor is a malignant mixed tumor that consists of embryonic Adenosarcoma components With rhabdomyoblastic and heteroblastic, as well as differently differentiated parts of kidney tissues and mostly on one or both Kidneys is demonstrable. At times it happens that the tumor already fills large parts of the abdomen. The size is not necessarily a prognostic criterion, since tumors of this size can be surgically removed under certain circumstances.

Forms and causes of Wilms' tumor

Based on various studies, a classification, the so-called SIOP-Classification (I.nternational S.ociety of Paediatric Oncology).

Is the tumor at stage 1, it is confined to one kidney and the capsule is intact. In stage 2 the tumor already crosses the kidney capsule and grows into fatty tissue or blood vessels, but it can still be completely removed surgically. Is a peritoneal metastasis already detectable, and is one because of the involvement of vital organs surgery not possible, one speaks of stage 3. Stage 4 is reached when distant metastases are already in lung, liver, Bones or brain are detectable. Bilateral kidney involvement would then be referred to as stage 5.

The causes of the emergence of the Wilms tumor are largely unknown to this day.Only a genetic connection could be found. So are carriers with a defective gene 11p13 or 11p15 particularly at risk from a nephroblastoma or a so-called WAGRSyndrome. This syndrome includes in addition to the actual Wilms tumor also a faulty iris system of the eyes (Aniridia) and reduced or delayed growth in the child.

Epidemiology

Overall that is Wilms tumor a rare one tumorwhich occurs around 70-100 times a year in Germany (0,9/100.000/Year). However, it is the most common tumor in childhood and accounts for 6-8% of all childhood tumors. Children between the ages of 2-5 years are particularly affected. After the age of 10, a Nephroblastoma be diagnosed. Tumorous changes can be observed in both kidneys of the child in 5% of cases. Statistics show that boys are more frequently affected than girls.

Symptoms

The Symptoms of Wilms' tumor are mostly quite unspecific. Often the children suffer from anorexia, Vomit, Abdominal pain and fever. Bloody urine is less common (Hematuria), as this already means that the tumor has invaded the urinary system. Under certain circumstances, parents can feel a bulge in the abdomen, which then corresponds to the extent of the tumor. Sometimes the children are constipated (Constipation) or diarrhea, Weight loss, paleness, urinary tract infection or high blood pressure.

therapy

The therapy to be sought is primarily the surgical removal of the tumor or the organs (if possible) that are affected by the tumor. Complete removal of the tumor is usually only possible in stages 1 and 2 (see above). If a tumor is in stage 1, treatment with a Chemotherapeutic agent begin to shrink the tumor as much as possible. After the surgical removal of the tumor and the affected kidney (Nephrectomy) would then be a so-called postoperative chemotherapy carried out to kill any remaining tumor cells that have not been surgically removed.

In stage 2, in addition to tumor-reducing chemotherapy before the operation (preoperative neoadjuvant chemotherapy) and tumor and kidney removal, followed by chemotherapy with parallel radiation.

In stages 3 and 4, in addition to tumor-reducing chemotherapy, radiation should also be carried out before the operation. If the findings have become so small that they are also operable, the tumor and kidney would be removed and chemotherapy followed by radiation. The treatment to reduce the size of the tumor is also referred to as "Down staging“.

As a chemotherapy drug are mostly used Vincristine With Adriamycin (possibly plus Actinomycin D plus ifosfamide / cyclophosphamide) is used. After the operation, chemotherapy is continued for 5-10 months.

The operation takes place in the following steps: First, a central abdominal incision is made. The important kidney vessels are clamped off kidney separated from the kidney bed. Any metastases that may be seen are also removed. The Lymph nodes along the main artery (aorta) and the vena cava (Vena cava) are also removed prophylactically, even if there is no evidence of tumor involvement at this point. If both kidneys are affected, the kidney with the larger findings is completely removed and the other kidney is operated on to preserve the organs. All that is done is to peel the tumor out of its location.

In addition to the known complications of chemotherapy (Hair loss, Nausea, Vomit, Tiredness, inflammation of the mucous membranes and much more), it can also lead to pelvic deformities under radiation Pulmonary fibrosis and myocardial damage come. A complication after the operation is the so-called tumor thrombus Vena cava which occurs in 5% of cases.

prophylaxis

Since the mechanism by which a Wilms tumor develops is largely not understood, no prophylactic measures are known.

Summary

Of the Wilms tumor (Nephroblastoma) is a rare one, but the most common of childhood tumors tumor. It is a malignant mixed tumor made up of different tissues, but usually originates from one or both kidneys. In addition to unspecific complaints such as nausea, vomiting, weight loss, a palpable mass or a blood-colored urine should make you think of the diagnosis nephroblastoma. The spread of the tumor is classified according to the so-called SIOP classification. Stage 1 and 2 are localized and mostly operable, while in stages 3 and 4 the tumor has already spread in the body and cannot be operated on directly.

In addition to the patient interview, the doctor should palpate the abdomen, an ultrasound, a urine examination, possibly CT and X-ray examinations for so-called tumor staging (spread of the tumor). Tumors of all stages must first be treated with a chemotherapy pretreated and treated after the surgical removal of the tumor and the kidney. In some stages, additional radiation may be necessary afterwards or before and afterwards. The gift of various Chemotherapy substances should be passed on 5-10 months after the surgical procedure. At over 75%, the healing rate of Wilms' tumor is quite good, although this is stage-dependent and ranges from 100% (stage 1) to 50-60% (stage 3 and 4).

Further on the topic: Wilms tumor prognosis