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The most frequent and often the only presenting symptom of Wilms tumor is a palpable abdominal mass. Parents may notice a firm, smooth swelling in the child’s belly during bathing or dressing. Unlike masses caused by infection or inflammation, this tumor is typically painless in its early stages. The mass is usually firm and immobile, located on one side of the abdomen, known as the flank. Because the retroperitoneal space where the kidneys reside is expandable, the tumor can grow to a significant size before it becomes visible or palpable. In some cases, the mass is discovered incidentally during a routine physical examination by a pediatrician or when imaging is performed for an unrelated reason.
While the mass itself may be painless, the rapid growth of the tumor can stretch the capsule of the kidney or press against adjacent organs, leading to abdominal pain. This pain can range from a mild, vague discomfort to severe, acute pain if bleeding occurs within the tumor. Abdominal distension is also common, where the child’s belly appears swollen or larger than usual. Parents might report that the child’s clothes feel tighter around the waist or that the child complains of feeling full in rare cases where the tumor ruptures, the child may present with an acute surgical abdomen, characterized by severe pain, rigidity, and signs of internal bleeding.
Hematuria, or blood in the urine, occurs in a portion of children with Wilms tumor. This can be macroscopic, meaning the urine appears pink, red, or cola-colored to the naked eye, or microscopic, in which blood cells are detected only via urinalysis. The presence of blood indicates that the tumor has invaded the kidney’s collecting system. While not present in every case, hematuria is a significant sign that warrants immediate investigation. Other urinary symptoms may include frequent urination or pain upon urination if the tumor mass compresses the bladder or ureters. However, these presentations are less common than abdominal masses.
High blood pressure is a notable condition associated with Wilms tumor, occurring in a significant number of patients. The mechanism involves the renal vasculature. As the tumor grows, it can compress the renal artery or its branches, reducing blood flow to the healthy kidney tissue. This ischemia triggers the kidney to release excessive amounts of renin, a hormone that regulates blood pressure. The resulting hypertension is often asymptomatic and detected only during vital sign monitoring. However, in severe cases, it can lead to headaches, visual disturbances, or irritability. Managing this hypertension is a key component of the pre-surgical stabilization process.
Wilms tumor is a systemic disease that can produce generalized symptoms affecting the child’s overall well-being.
The tumor’s tendency to invade nearby blood vessels can lead to specific vascular conditions. Wilms tumor has a propensity to grow into the renal vein and can extend into the inferior vena cava, the large vein that carries blood from the lower body to the heart. This vascular obstruction can cause swelling in the legs (edema) or visible collateral veins on the abdomen. In boys, a left-sided varicocele may develop. This is a swelling of the veins in the scrotum caused by the obstruction of the left spermatic vein, which drains into the left renal vein. The sudden appearance of a varicocele in a young child is a clinical red flag that requires ultrasound evaluation of the kidneys.
Although less common at initial presentation, respiratory symptoms can indicate that the disease has spread to the lungs, which is the most common site of metastasis for Wilms tumor. Symptoms may include shortness of breath, cough, or chest pain. However, it is essential to note that many children with lung metastases remain asymptomatic, and the spread is only detected through staging CT scans. The presence of lung nodules defines the disease as Stage IV, but even at this stage, the condition remains highly treatable compared to other metastatic cancers.
Rarely, a Wilms tumor can present with paraneoplastic syndromes, which are systemic conditions caused by hormones or chemical signals secreted by the tumor. Acquired von Willebrand disease, a bleeding disorder, has been described in association with Wilms tumor. This condition resolves once the tumor is removed. Another rare association is erythrocytosis, an increase in red blood cells caused by the tumor secreting erythropoietin. Recognizing these unusual presentations requires a high level of clinical acumen and a multidisciplinary approach at Liv Hospital.
For children with syndromic Wilms tumor, the physical conditions associated with the underlying genetic disorder are often evident. Children with Beckwith-Wiedemann syndrome may present with hemihypertrophy, where one side of the body is noticeably larger than the other. This asymmetry can affect the limbs, trunk, or face. Children with WAGR syndrome will present with aniridia, which can cause significant visual impairment and light sensitivity. Identifying these phenotypic conditions is crucial because it changes the patient’s long-term surveillance plan, extending monitoring beyond cancer treatment to include other aspects of the syndrome.
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In many cases, the swelling is painless, but some children may experience stomach aches or discomfort due to pressure on other organs or if there is bleeding inside the tumor.
The tumor can compress the kidney’s blood vessels, causing the kidney to release renin, a hormone that raises blood pressure throughout the body.
It can make the urine look pink, red, or like tea or cola. Sometimes the amount of blood is so small that it can only be seen with a microscope.
Yes, the large mass can press on the stomach, making the child feel full quickly, leading to a loss of appetite and sometimes weight loss or vomiting.
If the tumor has spread to the lungs, a child might have a cough or trouble breathing, but often there are no symptoms outside the abdomen, even if it has spread.
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