Extensively cystic renal neoplasms

Cystic nephroma, cystic partially differentiated nephroblastoma, multilocular cystic renal cell carcinoma, and cystic hamartoma of renal pelvis

John Eble, S. M. Bonsib

Research output: Contribution to journalArticle

202 Citations (Scopus)

Abstract

Predominantly cystic renal neoplasms have been the source of diagnostic confusion and controversy. In this review, the authors analyze the clinical and pathological features of four entitities that consistently exhibit a diffusely cystic growth pattern, are strikingly similar in their gross appearances, and are not separable by preoperative imaging studies. Based on the literature, this review concludes that tumors in young children that have been classified as cystic nephroma and cystic partially differentiated nephroblastoma likely represent a single entity, and all should be considered highly cystic Wilms' tumors with little or no capacity for invasion or metastasis and diagnosed as cystic partially differentiated nephroblastoma. Conversely, cystic nephroma in adults has no discernible connection with Wilms' tumor or nephrogenic rests and should be considered a benign composite neoplasm of stroma and epithelium of unknown histogenesis, which may rarely become malignant with secondary development of a sarcoma. Multilocular cystic renal cell carcinoma appears to be unrelated to cystic nephroma and if the following criteria are met, it appears to be a neoplasm with an intrinsically cystic growth pattern, and no, or at most little, malignant potential: (1) an expansile mass is surrounded by a fibrous wall, (2) the interior of the tumor entirely is composed of cysts and septa with no expansile solid nodules, and (3) the septa contain aggregates of epithelial cells with clear cytoplasm. Cystic hamartoma of the renal pelvis is a rare, complex tumor composed of stroma with a prominent smooth muscle component and a variety of epithelial elements.

Original languageEnglish (US)
Pages (from-to)2-20
Number of pages19
JournalSeminars in Diagnostic Pathology
Volume15
Issue number1
StatePublished - 1998
Externally publishedYes

Fingerprint

Kidney Pelvis
Hamartoma
Wilms Tumor
Kidney Neoplasms
Renal Cell Carcinoma
Neoplasms
Growth
Sarcoma
Smooth Muscle
Cysts
Cytoplasm
Epithelium
Epithelial Cells
Neoplasm Metastasis

Keywords

  • Clear cell renal cell carcinoma
  • Cyst
  • Cystic hamartoma of renal pelvis
  • Cystic nephroma
  • Cystic partially differentiated nephroblastoma
  • Kidney
  • Multilocular cystic renal cell carcinoma
  • Wilms' tumor

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Medical Laboratory Technology

Cite this

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title = "Extensively cystic renal neoplasms: Cystic nephroma, cystic partially differentiated nephroblastoma, multilocular cystic renal cell carcinoma, and cystic hamartoma of renal pelvis",
abstract = "Predominantly cystic renal neoplasms have been the source of diagnostic confusion and controversy. In this review, the authors analyze the clinical and pathological features of four entitities that consistently exhibit a diffusely cystic growth pattern, are strikingly similar in their gross appearances, and are not separable by preoperative imaging studies. Based on the literature, this review concludes that tumors in young children that have been classified as cystic nephroma and cystic partially differentiated nephroblastoma likely represent a single entity, and all should be considered highly cystic Wilms' tumors with little or no capacity for invasion or metastasis and diagnosed as cystic partially differentiated nephroblastoma. Conversely, cystic nephroma in adults has no discernible connection with Wilms' tumor or nephrogenic rests and should be considered a benign composite neoplasm of stroma and epithelium of unknown histogenesis, which may rarely become malignant with secondary development of a sarcoma. Multilocular cystic renal cell carcinoma appears to be unrelated to cystic nephroma and if the following criteria are met, it appears to be a neoplasm with an intrinsically cystic growth pattern, and no, or at most little, malignant potential: (1) an expansile mass is surrounded by a fibrous wall, (2) the interior of the tumor entirely is composed of cysts and septa with no expansile solid nodules, and (3) the septa contain aggregates of epithelial cells with clear cytoplasm. Cystic hamartoma of the renal pelvis is a rare, complex tumor composed of stroma with a prominent smooth muscle component and a variety of epithelial elements.",
keywords = "Clear cell renal cell carcinoma, Cyst, Cystic hamartoma of renal pelvis, Cystic nephroma, Cystic partially differentiated nephroblastoma, Kidney, Multilocular cystic renal cell carcinoma, Wilms' tumor",
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AU - Bonsib, S. M.

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N2 - Predominantly cystic renal neoplasms have been the source of diagnostic confusion and controversy. In this review, the authors analyze the clinical and pathological features of four entitities that consistently exhibit a diffusely cystic growth pattern, are strikingly similar in their gross appearances, and are not separable by preoperative imaging studies. Based on the literature, this review concludes that tumors in young children that have been classified as cystic nephroma and cystic partially differentiated nephroblastoma likely represent a single entity, and all should be considered highly cystic Wilms' tumors with little or no capacity for invasion or metastasis and diagnosed as cystic partially differentiated nephroblastoma. Conversely, cystic nephroma in adults has no discernible connection with Wilms' tumor or nephrogenic rests and should be considered a benign composite neoplasm of stroma and epithelium of unknown histogenesis, which may rarely become malignant with secondary development of a sarcoma. Multilocular cystic renal cell carcinoma appears to be unrelated to cystic nephroma and if the following criteria are met, it appears to be a neoplasm with an intrinsically cystic growth pattern, and no, or at most little, malignant potential: (1) an expansile mass is surrounded by a fibrous wall, (2) the interior of the tumor entirely is composed of cysts and septa with no expansile solid nodules, and (3) the septa contain aggregates of epithelial cells with clear cytoplasm. Cystic hamartoma of the renal pelvis is a rare, complex tumor composed of stroma with a prominent smooth muscle component and a variety of epithelial elements.

AB - Predominantly cystic renal neoplasms have been the source of diagnostic confusion and controversy. In this review, the authors analyze the clinical and pathological features of four entitities that consistently exhibit a diffusely cystic growth pattern, are strikingly similar in their gross appearances, and are not separable by preoperative imaging studies. Based on the literature, this review concludes that tumors in young children that have been classified as cystic nephroma and cystic partially differentiated nephroblastoma likely represent a single entity, and all should be considered highly cystic Wilms' tumors with little or no capacity for invasion or metastasis and diagnosed as cystic partially differentiated nephroblastoma. Conversely, cystic nephroma in adults has no discernible connection with Wilms' tumor or nephrogenic rests and should be considered a benign composite neoplasm of stroma and epithelium of unknown histogenesis, which may rarely become malignant with secondary development of a sarcoma. Multilocular cystic renal cell carcinoma appears to be unrelated to cystic nephroma and if the following criteria are met, it appears to be a neoplasm with an intrinsically cystic growth pattern, and no, or at most little, malignant potential: (1) an expansile mass is surrounded by a fibrous wall, (2) the interior of the tumor entirely is composed of cysts and septa with no expansile solid nodules, and (3) the septa contain aggregates of epithelial cells with clear cytoplasm. Cystic hamartoma of the renal pelvis is a rare, complex tumor composed of stroma with a prominent smooth muscle component and a variety of epithelial elements.

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KW - Cystic partially differentiated nephroblastoma

KW - Kidney

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KW - Wilms' tumor

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