SHM Converge Scientific Abstract ePoster Gallery-Hypertensive Urgency and Abdominal Pain in a 7-Year-Old Girl

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This case report describes a previously healthy 7-year-old girl who presented with intermittent severe right-sided abdominal/flank pain, constipation, weight loss, and marked hypertension (161/136). Exam revealed a firm right upper quadrant mass. Imaging showed a large 15 cm right renal mass consistent with Wilms tumor, with suspected renal vein tumor thrombus and metastatic disease to the lungs, liver, and retroperitoneal lymph nodes.<br /><br />Because the tumor was extensive and initially unresectable, the patient underwent exploratory laparotomy and biopsy, which confirmed stage IV Wilms tumor. Molecular testing found WT1 gene alterations, and later testing showed no adverse high-risk chromosomal changes such as 1q gain or LOH in 1p, 11p, or 16p. She received neoadjuvant chemotherapy per Children’s Oncology Group protocol AREN0533 (DD4A: vincristine, doxorubicin, dactinomycin), followed by right nephrectomy after 6 weeks. Final pathology demonstrated favorable histology without anaplasia. Because of lung metastases and slow incomplete response, she then transitioned to Regimen M with cyclophosphamide and etoposide, plus radiation therapy.<br /><br />Her hypertension was thought to be secondary to tumor mass effect and renal vein thrombosis causing renal ischemia and renin-mediated vasoconstriction. Blood pressure was managed with lisinopril and amlodipine, along with rescue agents as needed, and resolved about 3 weeks after nephrectomy.<br /><br />The report highlights that Wilms tumor can present with hypertension even in otherwise healthy children, that CT or MRI is important for assessing tumor extent and vascular/metastatic involvement, and that favorable-histology Wilms tumor with lung metastases is treated with multimodal therapy including chemotherapy, surgery, and radiation.

Asset Subtitle

Sabrina Schundler

Meta Tag

Author List Elizabeth R. Van Opstal, Noel Cabanas, Sabrina Schundler

Category Clinical Vignettes

Concept Wilms Tumor

Concept Abdominal Mass

Concept Imaging

Concept Abdominal Pain

Concept Hypertension

Distinguished Non-Finalist

Presenter Organization Rush Medical College

Presenting Author Sabrina Schundler

Track Pediatric

Keywords

Wilms tumor

pediatric hypertension

renal mass

renal vein thrombus

stage IV cancer

neoadjuvant chemotherapy

nephrectomy

lung metastases

favorable histology

WT1 gene alterations

Wilms Tumor

Abdominal Mass

Imaging

Abdominal Pain

Hypertension