A case of congenital capillary proliferation of the kidney (CCPK)

Ken Yamaguchi1, Takahiko Nakazono2, Shuichi Fukui2

  • 1Department of Radiology, Faculty of Medicine, Saga University, 5-1-1 Nabeshima, Saga, 849-8501, Japan. yamaguk@cc.saga-u.ac.jp.

Insights

Congenital capillary proliferation of the kidney (CCPK) is an extremely rare childhood renal vascular lesion. Multimodality imaging and pathological examination confirmed this rare diagnosis in a neonate.

Area of Science:

  • Pediatric Radiology
  • Vascular Pathology
  • Nephrology

Background:

  • Congenital capillary proliferation of the kidney (CCPK) is an extremely rare renal vascular lesion in children.
  • Diagnosis is challenging due to its rarity and potential mimicry of other renal masses.

Observation:

  • A neonate, born to a mother with gestational diabetes, presented with a right renal mass detected via abdominal ultrasound.
  • Imaging revealed a hyperechoic, lobulated mass with peripheral hypervascularity on Doppler, T2 hyperintensity on MRI, and characteristic enhancement patterns on CT.
  • Differential diagnoses included hemangioma and malignancy, necessitating further investigation.

Findings:

  • Surgical pathology confirmed a proliferation of capillaries positive for vascular endothelial markers and negative for GLUT1.
  • Immunohistochemistry and microscopic examination revealed entrapped renal tubules and glomeruli, supporting the CCPK diagnosis.
  • This represents the sixth reported case of CCPK, underscoring its extreme rarity.

Implications:

  • Multimodality imaging plays a crucial role in characterizing rare pediatric renal vascular lesions like CCPK.
  • Accurate pathological examination, including immunohistochemistry, is essential for definitive diagnosis.
  • Increased awareness and reporting of CCPK cases are vital for understanding its presentation and management.

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