Predominantly cystic clear cell renal cell carcinoma and multilocular cystic renal neoplasm of low malignant potential form a low-grade spectrum

Maria Tretiakova*, Vikas Mehta, Masha Kocherginsky, Agata Minor, Steven S. Shen, Sahussapont Joseph Sirintrapun, Jorge L. Yao, Isabel Alvarado-Cabrero, Tatjana Antic, Scott E. Eggener, Maria M. Picken, Gladell P. Paner

*Corresponding author for this work

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Multilocular cystic renal cell carcinoma has been recently excluded from clear cell renal cell carcinoma (CCRCC) category and re-designated as multilocular cystic renal neoplasm of low malignant potential (MCRNLMP) due to its uniformly good outcomes. While strict distinction between MCRNLMP from predominantly cystic CCRCC (pc-CCRCC) is being emphasized, the significance of extensive true cystic component in CCRCC has not been investigated. Herein, we analyzed 57 MCRNLMP, 69 pc-CCRCC, and 46 non-cystic CCRCC. There were no statistically significant differences between the three subtypes in age, gender, and laterality. ISUP grades were 1 (73%) or 2 (27%) for MCRNLMP; for pc-CCRCC were 1 (31%), 2 (60%), and 3 (9%); and for non-cystic CCRCC were 1 (9%), 2 (52%), 3 (26%), and 4 (13%). MCRNLMP were either pT stage 1 (91%) or 2 (9%), pT stages for pc-CCRCC were 1 (92.5%), 2 (1.5%), and 3 (6%) and for non-cystic CCRCC were 1 (58.7%), 2 (6.5%), and 3 (34.8%). None of MCRNLMP patients developed recurrences or metastases, and only 1 contralateral kidney tumor and 1 metastasis developed in pc-CCRCC. In contrast, 19 patients with non-cystic CCRCC developed metastases (5-year PFS 58%, CI 38.3–73.5%), and 1 patient died of disease. Monosomy 3 was common in both MCRNLMP (3/3) and pc-CCRCC (6/7). This large series of MCRNLMP confirms its indolent behavior, shows that pc-CCRCC has significantly better prognosis than non-cystic CCRCC and may define the lower grade spectrum of CCRCC. We recommend that the presence and extent of CCRCC cystic component should be documented in the pathology report.

Original languageEnglish (US)
Pages (from-to)85-93
Number of pages9
JournalVirchows Archiv
Volume473
Issue number1
DOIs
StatePublished - Jul 1 2018

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Kidney Neoplasms
Renal Cell Carcinoma
Neoplasm Metastasis
Monosomy

Keywords

  • Carcinoma
  • Clear cell
  • Cystic
  • Multilocular
  • Neoplasm
  • Renal

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Molecular Biology
  • Cell Biology

Cite this

Tretiakova, Maria ; Mehta, Vikas ; Kocherginsky, Masha ; Minor, Agata ; Shen, Steven S. ; Sirintrapun, Sahussapont Joseph ; Yao, Jorge L. ; Alvarado-Cabrero, Isabel ; Antic, Tatjana ; Eggener, Scott E. ; Picken, Maria M. ; Paner, Gladell P. / Predominantly cystic clear cell renal cell carcinoma and multilocular cystic renal neoplasm of low malignant potential form a low-grade spectrum. In: Virchows Archiv. 2018 ; Vol. 473, No. 1. pp. 85-93.
@article{9de92a0093f640118a0768f041dd3247,
title = "Predominantly cystic clear cell renal cell carcinoma and multilocular cystic renal neoplasm of low malignant potential form a low-grade spectrum",
abstract = "Multilocular cystic renal cell carcinoma has been recently excluded from clear cell renal cell carcinoma (CCRCC) category and re-designated as multilocular cystic renal neoplasm of low malignant potential (MCRNLMP) due to its uniformly good outcomes. While strict distinction between MCRNLMP from predominantly cystic CCRCC (pc-CCRCC) is being emphasized, the significance of extensive true cystic component in CCRCC has not been investigated. Herein, we analyzed 57 MCRNLMP, 69 pc-CCRCC, and 46 non-cystic CCRCC. There were no statistically significant differences between the three subtypes in age, gender, and laterality. ISUP grades were 1 (73{\%}) or 2 (27{\%}) for MCRNLMP; for pc-CCRCC were 1 (31{\%}), 2 (60{\%}), and 3 (9{\%}); and for non-cystic CCRCC were 1 (9{\%}), 2 (52{\%}), 3 (26{\%}), and 4 (13{\%}). MCRNLMP were either pT stage 1 (91{\%}) or 2 (9{\%}), pT stages for pc-CCRCC were 1 (92.5{\%}), 2 (1.5{\%}), and 3 (6{\%}) and for non-cystic CCRCC were 1 (58.7{\%}), 2 (6.5{\%}), and 3 (34.8{\%}). None of MCRNLMP patients developed recurrences or metastases, and only 1 contralateral kidney tumor and 1 metastasis developed in pc-CCRCC. In contrast, 19 patients with non-cystic CCRCC developed metastases (5-year PFS 58{\%}, CI 38.3–73.5{\%}), and 1 patient died of disease. Monosomy 3 was common in both MCRNLMP (3/3) and pc-CCRCC (6/7). This large series of MCRNLMP confirms its indolent behavior, shows that pc-CCRCC has significantly better prognosis than non-cystic CCRCC and may define the lower grade spectrum of CCRCC. We recommend that the presence and extent of CCRCC cystic component should be documented in the pathology report.",
keywords = "Carcinoma, Clear cell, Cystic, Multilocular, Neoplasm, Renal",
author = "Maria Tretiakova and Vikas Mehta and Masha Kocherginsky and Agata Minor and Shen, {Steven S.} and Sirintrapun, {Sahussapont Joseph} and Yao, {Jorge L.} and Isabel Alvarado-Cabrero and Tatjana Antic and Eggener, {Scott E.} and Picken, {Maria M.} and Paner, {Gladell P.}",
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Tretiakova, M, Mehta, V, Kocherginsky, M, Minor, A, Shen, SS, Sirintrapun, SJ, Yao, JL, Alvarado-Cabrero, I, Antic, T, Eggener, SE, Picken, MM & Paner, GP 2018, 'Predominantly cystic clear cell renal cell carcinoma and multilocular cystic renal neoplasm of low malignant potential form a low-grade spectrum', Virchows Archiv, vol. 473, no. 1, pp. 85-93. https://doi.org/10.1007/s00428-018-2371-8

Predominantly cystic clear cell renal cell carcinoma and multilocular cystic renal neoplasm of low malignant potential form a low-grade spectrum. / Tretiakova, Maria; Mehta, Vikas; Kocherginsky, Masha; Minor, Agata; Shen, Steven S.; Sirintrapun, Sahussapont Joseph; Yao, Jorge L.; Alvarado-Cabrero, Isabel; Antic, Tatjana; Eggener, Scott E.; Picken, Maria M.; Paner, Gladell P.

In: Virchows Archiv, Vol. 473, No. 1, 01.07.2018, p. 85-93.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Predominantly cystic clear cell renal cell carcinoma and multilocular cystic renal neoplasm of low malignant potential form a low-grade spectrum

AU - Tretiakova, Maria

AU - Mehta, Vikas

AU - Kocherginsky, Masha

AU - Minor, Agata

AU - Shen, Steven S.

AU - Sirintrapun, Sahussapont Joseph

AU - Yao, Jorge L.

AU - Alvarado-Cabrero, Isabel

AU - Antic, Tatjana

AU - Eggener, Scott E.

AU - Picken, Maria M.

AU - Paner, Gladell P.

PY - 2018/7/1

Y1 - 2018/7/1

N2 - Multilocular cystic renal cell carcinoma has been recently excluded from clear cell renal cell carcinoma (CCRCC) category and re-designated as multilocular cystic renal neoplasm of low malignant potential (MCRNLMP) due to its uniformly good outcomes. While strict distinction between MCRNLMP from predominantly cystic CCRCC (pc-CCRCC) is being emphasized, the significance of extensive true cystic component in CCRCC has not been investigated. Herein, we analyzed 57 MCRNLMP, 69 pc-CCRCC, and 46 non-cystic CCRCC. There were no statistically significant differences between the three subtypes in age, gender, and laterality. ISUP grades were 1 (73%) or 2 (27%) for MCRNLMP; for pc-CCRCC were 1 (31%), 2 (60%), and 3 (9%); and for non-cystic CCRCC were 1 (9%), 2 (52%), 3 (26%), and 4 (13%). MCRNLMP were either pT stage 1 (91%) or 2 (9%), pT stages for pc-CCRCC were 1 (92.5%), 2 (1.5%), and 3 (6%) and for non-cystic CCRCC were 1 (58.7%), 2 (6.5%), and 3 (34.8%). None of MCRNLMP patients developed recurrences or metastases, and only 1 contralateral kidney tumor and 1 metastasis developed in pc-CCRCC. In contrast, 19 patients with non-cystic CCRCC developed metastases (5-year PFS 58%, CI 38.3–73.5%), and 1 patient died of disease. Monosomy 3 was common in both MCRNLMP (3/3) and pc-CCRCC (6/7). This large series of MCRNLMP confirms its indolent behavior, shows that pc-CCRCC has significantly better prognosis than non-cystic CCRCC and may define the lower grade spectrum of CCRCC. We recommend that the presence and extent of CCRCC cystic component should be documented in the pathology report.

AB - Multilocular cystic renal cell carcinoma has been recently excluded from clear cell renal cell carcinoma (CCRCC) category and re-designated as multilocular cystic renal neoplasm of low malignant potential (MCRNLMP) due to its uniformly good outcomes. While strict distinction between MCRNLMP from predominantly cystic CCRCC (pc-CCRCC) is being emphasized, the significance of extensive true cystic component in CCRCC has not been investigated. Herein, we analyzed 57 MCRNLMP, 69 pc-CCRCC, and 46 non-cystic CCRCC. There were no statistically significant differences between the three subtypes in age, gender, and laterality. ISUP grades were 1 (73%) or 2 (27%) for MCRNLMP; for pc-CCRCC were 1 (31%), 2 (60%), and 3 (9%); and for non-cystic CCRCC were 1 (9%), 2 (52%), 3 (26%), and 4 (13%). MCRNLMP were either pT stage 1 (91%) or 2 (9%), pT stages for pc-CCRCC were 1 (92.5%), 2 (1.5%), and 3 (6%) and for non-cystic CCRCC were 1 (58.7%), 2 (6.5%), and 3 (34.8%). None of MCRNLMP patients developed recurrences or metastases, and only 1 contralateral kidney tumor and 1 metastasis developed in pc-CCRCC. In contrast, 19 patients with non-cystic CCRCC developed metastases (5-year PFS 58%, CI 38.3–73.5%), and 1 patient died of disease. Monosomy 3 was common in both MCRNLMP (3/3) and pc-CCRCC (6/7). This large series of MCRNLMP confirms its indolent behavior, shows that pc-CCRCC has significantly better prognosis than non-cystic CCRCC and may define the lower grade spectrum of CCRCC. We recommend that the presence and extent of CCRCC cystic component should be documented in the pathology report.

KW - Carcinoma

KW - Clear cell

KW - Cystic

KW - Multilocular

KW - Neoplasm

KW - Renal

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