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[Renal cystadenoma in children. Apropos of 5 cases]
Insights
Multilocular renal cystadenoma is a rare, benign childhood tumor. It presents as a large, spherical mass and must be differentiated from cystic nephroblastoma.
Area of Science:
- Pediatric Pathology
- Surgical Oncology
Background:
- Multilocular renal cystadenoma is an uncommon benign renal tumor in children.
- It occurs approximately once for every 40 Wilms tumors.
Observation:
- This tumor is characterized by isolated, cubic epithelium-lined cysts embedded in mature mesenchyme.
- Macroscopically, it appears as a large, spherical mass with clear demarcation from renal parenchyma.
- Clinical presentation is often dominated by the mass volume; imaging like angiography and echography can aid diagnosis.
Findings:
- Histologically, it consists of interconnected cysts within a mesenchymal matrix.
- Differential diagnosis from cystic nephroblastoma is crucial, with key distinguishing features discussed.
- The lesion is considered a hamartoma originating from aberrant metanephrogenic tissue.
Implications:
- Accurate differentiation from malignant tumors like Wilms tumor is essential for appropriate patient management.
- The term 'renal cystadenoma' is preferred due to its emphasis on neoplastic nature and macroscopic appearance.
- Understanding its hamartomatous origin provides insight into renal tumor development.
Abstract:
The multilocular renal cystadenoma is a benign, rather uncommon tumor occurring in childhood (one case for 40 Wilms tumors). The clinical pathological study of five cases and the review of cases published in the literature show that this tumor, like all cystadenomata, is constituted by isolated cysts lined with cubic epithelium and inserted into each other. These cysts are included into mature mesenchymatous tissue. The renal cystadenoma appears as a large, regular, spheric mass, sharply demarcated from the pelvic cavities and renal parenchyma. Its volume is the main clinical sign. Occasionally, angiographic and echographic investigations may provide additional useful data. In some cases, the renal cystadenoma may be difficult to distinguish from a well differenciated cystic nephroblastoma. The differential features are emphasized and discussed. The renal cystadenoma must be regarded as a hamartoma developed from some metanephrogenic blastematous areas excluded from the normal embryogenetic process. The term of renal cystadenoma emphasizes the neoplastic nature and the macroscopic appearance of this tumor and must be preferred to designate this lesion.