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Published on: September 1, 2015
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.
Abstract:
We report a case of congenital capillary proliferation of the kidney (CCPK) along with the multimodality imaging findings. Four-day-old boy who had managed due to his mother's gestational diabetes underwent abdominal ultrasound and a mass was detected in the right kidney. On gray scale ultrasound, the mass exhibited a hyperechoic, slight lobulated shape and a circumscribed margin. On Doppler mode, the mass showed hypervascularity in its peripheral to central zones. On MRI, the mass was hyperintense on the T2-weighted image, and no diffusion restriction was noted on DWI/ADC. On computed tomography, strong enhancement was shown at center of the mass at the post-contrast early phase; homogeneous enhancement at the entirety of the mass was observed at the delayed phase. We suspected hemangioma but did not rule out the possibility of malignancy. Surgery was performed. Pathologically, the specimen showed a proliferation of capillaries which were positive for vascular endothelial markers and negative for GLUT1 in immunohistochemistry. A small number of entrapped tubules and glomeruli were also observed. After an intensive pathological examination, the diagnosis of CCPK was finally considered. CCPK was recently described as an extremely rare childhood renal vascular lesion, and to our knowledge, only five other cases have been reported. Our patient's multimodality imaging findings well reflected the characteristics of a vascular lesion.
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