Wilms Tumor

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Original Editors - Students from Bellarmine University's Pathophysiology of Complex Patient Problems project.

Top Contributors - Rachel Flaherty, Ali Hafele, Lucinda hampton, Oyemi Sillo, Uchechukwu Chukwuemeka, WikiSysop, Kim Jackson, 127.0.0.1, Elaine Lonnemann and Vidya Acharya Rachel Flaherty and Allison Hafele 

Definition/Description

Wilms’ Tumor, also known as nephroblastoma, is a solid tumor of the kidney that develops from immature kidney cells.¹ It is the most common cancer of the kidneys in children, and the fourth most common type of cancer in children.^1,2 Wilms’ tumors are most often unilateral, affecting only one kidney.³ 5-10% of children with Wilms’ tumors have more than one tumor in the same kidney, and approximately 5% of children with the cancer have bilateral Wilms’ tumors, affecting both kidneys.³

Prevalence

Wilms’ tumor is most common in children ages 3 to 4 and becomes much less common after the age of 5.² Wilms’ tumor affects boys are girls equally, and can also be found in adults, though it is very rare.¹ On average, there are 460 new cases of Wilms’ tumor diagnosed in the United States every year. Approximately 1 in 8,000-10,000 children are affected.¹ About 5% of all cancers in children are Wilms’ tumors.³

Characteristics/Clinical Presentation

Wilms’ tumor is often hard to find early because the tumor can grow large without any signs or symptoms. These children may look and act normally.³ The most common first clinical sign is swelling or a hard mass in the abdomen. It is often firm and large enough to be palpated on both sides of the abdomen, and is usually not painful.³
Some children may also experience:^2,3
-Fever
-Loss of appetite
-Shortness of breath
-Constipation
-Nausea
-Blood in the urine
-Abdominal pain
Wilms’ tumors may also cause high blood pressure. The blood pressure can get high enough to cause bleeding in the eye or a change in consciousness in rare cases.³

Associated Co-morbidities

The types of secondary cancers include bone and soft tissue sarcomas, breast cancer, lymphoma, tumors of the digestive tract, melanoma and acute leukaemias. Certain birth defects and also correlated with Wilms tumor including Aniridia, hemihypertrophy, cryptorchidism, hypospadias. There are also several syndromes that are often associated with Wilms tumor including Sotos syndrome, Perlman syndrome, Simpson-Golabi-Behmel syndrome, Bloom syndrome, Frasier syndrome, Beckwith-Wiedemann Syndrome, Denys -Drash syndrome, and WAGR syndrome which are explained in further detail in differential diagnosis. (CANCER ORG)

Medications

The most effective chemotherapeutics in treatment of nephroblastoma are: actinomycin D (ACT), vincristine (VCR), doxorubicin (ADM), cyclophosphamide (ctx), ifosfamide (IFO), etoposide and carboplatin. (huszno) NWTS recommends polchemotherapy treatment (ACT, VCR, ADM) for a period of 15 weeks in stage III Less aggressive treatment using two medications (VCR and ACT) can be used in cancer stages I and II. (HUZON). It is important to note toxicity of such treatment is highger in adults than in children (HUS).

Toxicity: among vinicristine, dactinomycin and Adriamycin, the main acue toxicity was neuropathy due to vincristine. During chemotherapy with ICE (ifosfamide, carboplatin, etoposide) all patients had hematological toxicity such as neutropenia and thrombocytopenia in stage IV toxicity. The type, timing, and dosage of chemotherapy have been major risk factors in combined therapy. (HU)

Side Effects: Late side effects include cardiotoxicity, reproductive problems, renal dysfunction and the development of benign and malignant second tumors. (HUS) In some cases fertility may be impaired. (HUS) Other side effects of chemotherapy include: hair loss , mouth sores , loss of appetite , nausea and vomiting , diarrhea or constipation , increased chance of infections (from having too few white blood cells), Easy bruising or bleeding (from having too few blood platelets), Fatigue or extreme tiredness (from having too few red blood cells) (CANCer org)

Diagnostic Tests/Lab Tests/Lab Values

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Etiology/Causes

Wilms’ tumor is typically seen in otherwise healthy children, and the exact cause in most children is unknown.^4,5 However, approximately 10% of patients with the tumor have a congenital anomaly, including certain birth defect syndromes and genetic changes.5 Wilms’ tumor, whether hereditary or sporadic, shows evidence of changes in one or more of at least ten different genes.⁴
Common syndromes that are associated with Wilms’ tumor include the following:
WAGR Syndrome: WAGR stands for Wilms tumor, Aniridia (lack of the iris of the eyes), Genitourinary tract abnormalities, and mental retardation. Children who have WAGR syndrome have approximately 30-50% chance of developing a Wilms’ tumor, and often have them bilaterally. In this syndrome, cells are missing part of chromosome 11, which involves the WT1 gene.^3,6
Beckwith-Wiedemann sydrome: These children are at a 5% risk of having a Wilms’ tumor. This syndrome is caused by a malformation of chromosome 11.³
Denys-Drash Syndrome: This syndrome is also caused by mutations to the WT1 gene. The risk for Wilms’ tumor in these patients is greater than 90%.⁶

Systemic Involvement

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Medical Management (current best evidence)

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Physical Therapy Management (current best evidence)

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Alternative/Holistic Management (current best evidence)

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Differential Diagnosis

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Case Reports/ Case Studies

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Resources

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Recent Related Research (from Pubmed)

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References

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