SAEM Clinical Images Series: Wilma, Take a Look at This!

A 2-year, 11-month-old female with a history of constipation was brought to the ED by her mother for abdominal pain. The mother noticed that the patient’s abdomen had been enlarging for months. When they visited the pediatrician several months ago, the pediatrician also noticed a mildly enlarged abdomen but the patient was asymptomatic at that time. She was well during the interval until more recently, the patient began to complain of persistent abdominal pain and would point to the epigastric area. The patient had two episodes of unprovoked, non-bloody, non-bilious vomiting the morning prior to the ED visit. The patient had been tolerating oral intake well, passing adequate urine, having normal bowel movements, and behaving at baseline. No associated fever, diarrhea, bloody stool, dysuria, hematuria, or weight loss.

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Vitals: Temp 97.9 °F (36.6 °C); BP 103/68; Pulse 121; RR 26; SpO2 99% on room air

Constitutional: Active, well-developed, and in no distress.

HEENT: Normocephalic and atraumatic. No scleral icterus. TMs intact, no erythema. No rhinorrhea, no erythema. Moist mucous membranes, oropharynx is clear, no exudates or erythema.

Cardiovascular: Normal rate, regular rhythm and normal heart sounds.

Pulmonary: Breath sounds normal. No wheezing, no stridor, no decreased breath sounds. Normal effort, no acute respiratory distress.

Abdomen: Protuberant, distended abdomen with mild generalized tenderness to palpation. Rigid mass palpated in the upper right quadrant. Normal bowel sounds are heard.

Genitourinary: Normal anatomy. No hernias visualized, no erythema.

Skin: No jaundice or rashes visualized.

Neurological: Awake and alert. No focal deficits present.

CBC: No leukocytosis, leukopenia, anemia, or thrombocytopenia.

CMP: Electrolytes, kidney, and liver function tests were within normal limits.

The most common pediatric renal malignancy is a Wilms tumor, also known as nephroblastoma. It is an embryonal tumor due to disrupted nephrogenesis. It affects approximately 1 in 10,000 children with the median age of onset being 3.5 years (1). The most common chief complaint is abdominal pain, as in this case.

Here a large homogenous mass initially appears to be projecting from the liver, but it can also be seen protruding out of the right kidney. Pediatric abdominal organs commonly overlap so it is essential to note the origination of a mass, primarily for surgical planning. If ultrasound imaging is equivocal, CT is the next best step in differentiating the mass origination. Here, a 12 cm x 9.5 cm x 9 cm mass was noted to originate from the right kidney. If the mass becomes big enough, patients can present with vomiting due to the direct compression of the alimentary tract, such as in this case. Other presenting signs and symptoms may be fever, hypertension, anemia, hematuria, or dysuria (2).

In the US, the National Wilms Tumor Study Group recommends primary nephrectomy followed by a chemotherapy regimen that is tailored to the individual patient and tumor staging. With modern multidisciplinary management, curative therapy is achievable in approximately 90% of affected patients (2). This patient had a successful nephrectomy performed by general surgery and initiated chemotherapy on the medical floor. The patient was eventually discharged home with pediatric oncology follow-up.

Take-Home Points

  • Think of pediatric malignancy if the patient presents with chronic abdominal distention and pain.
  • Pediatric abdominal structures commonly overlap. Knowing the origination of an abdominal mass is essential for surgical planning. If ultrasound is equivocal, CT imaging is the next best step.
  • The definitive management of a Wilms tumor is a multidisciplinary approach, with primary nephrectomy followed by a tailored chemotherapy regimen as the gold-standard treatment in the US.

  1. Spreafico F, Fernandez CV, Brok J, Nakata K, Vujanic G, Geller JI, Gessler M, Maschietto M, Behjati S, Polanco A, Paintsil V, Luna-Fineman S, Pritchard-Jones K. Wilms tumour. Nat Rev Dis Primers. 2021 Oct 14;7(1):75. doi: 10.1038/s41572-021-00308-8. PMID: 34650095.
  2. Sonn G, Shortliffe LM. Management of Wilms tumor: current standard of care. Nat Clin Pract Urol. 2008 Oct;5(10):551-60. doi: 10.1038/ncpuro1218. PMID: 18836464.
  3. Leslie SW, Sajjad H, Murphy PB. Wilms Tumor. 2023 May 30. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan–. PMID: 28723033.

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