Nephrostomy tubes for management of a bladder occupying embryonal rhabdomyosarcoma in a 2-year-old: A case report and review of the literature

Abstract

Local control approaches for bladder/prostate rhabdomyosarcoma (RMS) may include surgery and/or radiation after neoadjuvant chemotherapy, with goal of maximizing long term function. We report a case of bladder RMS presenting with acute renal complications in a 2-year-old female necessitating bilateral nephrostomy tube placement before administration of chemotherapy. As local control for RMS is delayed until after 12 weeks of neoadjuvant chemotherapy has been given, management of medical complications prior to surgery is not protocolized, making case reports and discussion helpful to future patients and providers.

1 Background

Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma of childhood. RMS tumors typically arise within skeletal muscle and occur in almost equally amounts in both young children and teenagers. Risk group assignment is complex, incorporating fusion status, stage, and group designations. Bladder/prostate (B/P) is the primary site in 25 % of RMS pediatric patients, and is considered a favorable primary site of disease, with survival at five years for patients of 84 %. Past local control approaches included radical pelvic surgery along with radiation. However, there are increased risks with radiation and surgery, especially among younger children, due to its association with treatment-related morbidity. Newer approaches that focus on maintaining long term B/P function include initial biopsy, systemic chemotherapy, and a multidisciplinary approach to local control with either or both surgery and radiation therapy.

Local control for patients with B/P RMS occurs after 12 weeks of chemotherapy. Performing surgery at this time known as delayed primary excision (DPE), has allowed for reduction in radiation therapy dose and volume to minimize radiation associated toxicity and complications. However, maintaining long-term function has become of increased emphasis, acute and subacute complications in patients with large B/P masses may arise. Currently, there is scarce literature regarding initial management of acute and subacute complications of large B/P RMS tumors. Adult literature demonstrates the most common complication in patients with malignant urinary obstruction treated with percutaneous nephrostomy tubes was dislodgement, followed by infection. We report on management of a patient presenting with a bladder mass causing acute kidney injury (AKI) that quickly necessitated bilateral nephrostomy tube placement shortly after diagnosis, with administration of subsequent chemotherapy.

2 Case report

A previously healthy 2-year-old girl was admitted to the hospital after presenting with abdominal pain, urinary retention, constipation, AKI (creatinine 8.05mg/dL) and purulent urine. She had previously been diagnosed with a urinary tract infection (UTI) 2 months prior. Genitourinary exam was concerning for a lesion emanating from her urethra and anterior vaginal wall. Ultrasound was concerning for bladder mass. An MRI of the pelvis followed by cystoscopy with biopsy revealed a lobular, heterogeneously enhancing mass involving the bladder neck extending into the urethra, measuring 7.2cm × 4.1cm x 4.6cm ( Fig. 1 ). In addition to her AKI, she was noted to have bilateral hydroureteronephrosis, thus urethral stent placement was attempted but unsuccessful as the ureteral orifices could not be identified given the large mass, therefore a urethral catheter was placed. Creatinine improved to 0.40mg/dL over the next two days. Family declined additional interventions requiring anesthesia pending her biopsy results. The patient was discharged with a Foley catheter in place.

She was readmitted two days later with a creatinine level of 1.48mg/dL ( Fig. 2 ). Renal/bladder ultrasound on admission revealed bilateral hydronephrosis despite foley concerning for obstructive uropathy due to bladder RMS, thus, patient underwent bilateral percutaneous nephrostomy tube placement and removal of Foley catheter once her biopsy results finalized. The next day, the patient received cycle 1 of chemotherapy as per Children’s Oncology Group protocol D9803. After placement of nephrostomy tubes, she developed polyuria and severe symptomatic hypocalcemia, hypomagnesemia, and hypokalemia consistent with post-obstructive diuresis that necessitated a 5-day ICU stay for intensive electrolyte management. One week after nephrostomy tube placement, antegrade placement of internal ureteral stents was attempted but unsuccessful due to extravasation of contrast posterior to the bladder during fluoroscopy. Patient thus continued to have nephrostomy tubes, though was discharged after 41 days in the hospital. During this stay, the patient required prolonged use of antihypertensive, pain and anxiety medications. She subsequently had several unscheduled admissions due to fevers and was found to have a subscapular renal hematoma. Ultimately, after having the nephrostomy tubes in for 61 days, the patient trialed clamping tubes for 48 hours, proceeded by successful removal of the tubes.