Date of Presentation:3/25/2021

Attending pathologist: Anil Parwani, MD, PhD

Presented by: Ruhani Sardana, MBBS

Prepared by: Ruhani Sardana, MBBS



A man in late 40s presented with complaints of gross hematuria and flank pain.

Non-contrast CT revealed a 7 cm lobulated mass in the anterior aspect of the right kidney and a nonspecific 2.4 cm hypo-attenuated lesion in the right upper lobe of the liver. No significant enlargement of retroperitoneal lymph nodes.

MRI showed heterogeneous lobulated renal mass as well as a liver mass.


A well-circumscribed, irregularly lobulated mass measuring 7.0 x 6.2 x 5.8 cm located in the middle pole of the kidney

Approximately 30% of the mass was necrotic

Invading the renal vein, adherent to the vessel wall

What is the most likely diagnosis?

  1. Neuroblastoma
  2. Desmoplastic Small Round Cell Tumor
  3. Ewing Sarcoma / Primitive Neuroectodermal Tumor
  4. Lymphoma
Ewing Sarcoma / Primitive Neuroectodermal Tumor