RSNA2018读片会病例挑战（8）：女，2岁，腹胀、肾脏异常，活检病理报“Wilms瘤”

2018 RSNA

Image Interpretation Session

Peds

History

• 2 year old girl who originally presented at an outside practice

• “renal abnormalities”

• abdominal distension

• Followed by renal ultrasound

Renal Ultrasound at 2 Years of Age

History

• 2 year old girl who originally presented at an outside practice

• “renal abnormalities”

• abdominal distension

• Followed by renal ultrasound

• At 26 months of age:

• enlarged right kidney; mass suspected

• MRI performed

MRI at 26 Months of Age

T1+C Arterial Phase T1+C PV Phase

T2-FS T2-FS

Subsequently, US-Guided Right Renal Biopsies:

eventual path diagnosis was “Wilms”

Findings

• Young child with asymmetric nephromegaly and multiple solid masses

replacing normal renal parenchyma with loss of cortico-medullary distinction

↓

• Nephroblastomatosis +/- Wilms tumor >>>>>>>>>> lymphoma, metastases

• Very difficult to distinguish hyperplastic nephrogenic rests from Wilms tumor on biopsy

• It can’t be that straightforward, can it?

Additional Findings

• Fluid-filled locules within left abdominal wall, para-aortic/para-renal retroperitoneum, and right pelvis; ? thoracic and right hip lipomatosis; otherwise, decreased adipose tissue

↓

• Overgrowth syndrome with veno-lymphatic vascular malformations

• Isolated hemihypertrophy, Beckwith-Wiedemann, Perlman, Soto, and Simpson-Golabi-Behmel syndromes are associated with overgrowth, nephrogenic rests and Wilms tumor

• So, what could be the diagnosis in this case?

Deepest Gratitude

Abd GI: Rendon Nelson, MD

MSK: Lee Cothran, MD

Breast: Jay Baker, MD

Neuro: Michael Malinzak, MD

Peds: Gary Schooler, MD

Thoracic: Page McAdams MD

CVI & Abd GU: Daniele Marin, MD

Thank You

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FROM：http://archive.rsna.org/2018/Final%20Unknown%20Cases%2011.12.18.pdf