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(1)

Patient de 45 ans. ATCD de désarticulation de hanche gauche sur ostéo-arthrite en 2006,

compliquée d’une escarre avec fistule urétrale. Lambeau de couverture en 2006 . Ré-évolution d’une fistule uréthro-cutanée associée à une ostéite du cadre obturateur. Bilan pré thérapeutique

Quels sont les éléments sémiologiques significatifs à retenir sur ces images

Laure. Rivail-Eloy JHN

(2)

-masse tumorale multikystique hypervasculaire du pôle inférieur du rein gauche à contours réguliers , sans effraction capsulaire . Pas de néo vascularisation comme on en voit dans les tumeurs de Grawitz classiques.

-pas d'extension veineuse ni d'adénopathies

-que faire pour progresser dans la caractérisation de cette lésion

(3)

2005

2006

2012

l'étude du dossier permet de retrouver de médiocres examens d'imagerie qui fournissent

néanmoins des éléments

chronologiques précieux puisqu'ils montrent que la lésion existait déjà 7 ans auparavant mais elle avait apparemment été

considérée comme un kyste cortical ….

(4)

la lésion est circonscrite par une pseudo-capsule fibreuse

les kystes souvent multicloisonés , à parois d'épaisseur variable , sont séparés par des septa charnus

(5)

Carcinome rénal multikystique à cellules claires Grade 2 de Führman

cortex rénal en zone non lésionnelle :  tubes rénaux + 2 glomérules rénaux en bas de l'image)

lésion tumorale (cellules claires) associée à zone de nécrose (rose)

nucléole des noyaux visible au

grossissement x 400 : détermine le grade 2 de Führman

(6)

Carcinome rénal multikystique à cellules claires

MC-RCC multicystic renal cell carcinoma Rare sous-type de carcinome rénal

à cellules claires (4% )

adultes d'âge moyen 46-53 ans SR 1,2 H / 2,1 F

constitué de multiples kystes bordés de cellules claires et d'agglomérats de cellules claires dans les septa

Führman 1 ou 2 Délétion 3p

Pas de dissémination régionale ni a distance , avec des suivis

moyens de 6,5 ans

plus de 80 % sont découverts au stade T1

pseudo-capsule fibreuse pratiquement constante

(7)

W20 AJR:198, January 2012 stricter pathologic definition of multilocular cystic RCC for diagnosis, the overlap in path- ologic features between solid clear cell RCC undergoing cystic necrosis (Fuhrman grade 3 or 4) and multilocular cystic RCC, a sub- stantially less aggressive subtype of clear cell RCC (Fuhrman grade 1 or 2), has de- creased, and a better prognosis of multilocu- lar cystic RCC is emerging [7]. Some pathol- ogists consider multilocular cystic RCC to have a uniformly benign behavior [8] be- cause there are no reported cases of progres- sion or metastasis [9–11].

Previous investigators have correlated the imaging features of multilocular cystic RCC with the corresponding pathologic features [3, 12, 13], two previous studies specifical- ly using the Bosniak cyst classification sys- tem [14, 15], a system that has proved use- ful in evaluating cystic renal masses with

Multilocular Cystic Renal Cell

Carcinoma: Comparison of Imaging and Pathologic Findings

Nicole M. Hindman1 Morton A. Bosniak1 Andrew B. Rosenkrantz1 Stephanie Lee-Felker2 Jonathan Melamed3

Hindman NM, Bosniak MA, Melamed J, Rosen- krantz AB, Lee-Felker S

1Department of Radiology, New York University School of Medicine, 550 First Ave, HW 202, New York, NY 10016.

Address correspondence to N. M. Hindman (hindmn01@nyumc.org).

2Department of Medicine, New York University School of Medicine, New York, NY.

3Department of Pathology, New York University School of Medicine, New York, NY.

WEB

This is a Web exclusive article.

AJR 2012; 198:W20–W26

0361–803X/12/1981–W20

© American Roentgen Ray Society

M

ultilocular cystic renal cell carci- noma (RCC) is a low-grade neo- plasm with an excellent progno- sis [1]. The first two descriptions appeared in the pathologic literature in 1928 and 1957. This subtype of cystic RCC is rela- tively uncommon, thought to represent 2.3–

3.1% of all cases of RCC [2–5], and has the best prognosis of all subtypes of RCC, cystic or solid. The true incidence of multilocular cystic RCC is unknown because of the vari- ance in pathologic criteria previously used to diagnose this subtype [2, 4]. Current patho- logic criteria, defined by the World Health Organization (WHO) in 2004, for this be- nign-behaving tumor are stricter, limiting these tumors to those with a malignant clear cell lining and small collections of cells but without formation of a malignant expansile nodule [1, 6]. With the application of this

Keywords: imaging, multilocular cystic renal cell carcinoma, pathologic findings

DOI:10.2214/AJR.11.6762

Received February 26, 2011; accepted after revision May 22, 2011.

OBJECTIVE. The purpose of this study was to retrospectively correlate the imaging and pathologic features of multilocular cystic renal cell carcinoma (RCC), a low-grade neoplasm that has an excellent prognosis.

MATERIALS AND METHODS. Institutional databases were searched for the period between 2001 and 2010 to identify cases of resected renal tumors that had been evaluated with CT or MRI and been analyzed by a uropathologist to confirm the histologic diagnosis of multilocular cystic RCC. The images (nine CT, 14 MRI) were reviewed, and a Bosniak cyst category was assigned.

RESULTS. Of 23 confirmed cases of multilocular cystic RCC, imaging revealed seven lesions were Bosniak category IIF, 13 were category III, and three were category IV. Patho- logic examination of the category IIF lesions revealed 99% fluid, 0.001–1% clear cells lining the septum, and 0% fibrosis. The category III lesions were 98–99% fluid, 1–2% clear cells, and 0% fibrosis. The category IV lesions were 20–40% fluid, 1–5% clear cells, and 60–80%

fibrosis. The patient demographics were similar across groups. Clinical follow-up showed no evidence of recurrent or metastatic disease.

CONCLUSION. Multilocular cystic RCC is a rare cystic lesion of the kidney that is low risk to the patient and benign in behavior. It has a variable imaging pattern, the Bosniak cat- egory ranging from IIF to IV. As multilocular cystic RCC lesions increase in complexity on images (higher Bosniak category), there is a corresponding increase in the volume of malig- nant cells lining the tumor and an increase in the presence of vascularized fibrous tissue. Re- gardless of the imaging appearance, the behavior of these tumors was benign in this study.

Clinicians and radiologists should be aware that when this carcinoma is reported to occur, the patient has an excellent prognosis.

Hindman et al.

Multilocular Cystic Renal Cell Carcinoma Genitourinary Imaging

Original Research

W20 AJR:198, January 2012

stricter pathologic definition of multilocular cystic RCC for diagnosis, the overlap in path- ologic features between solid clear cell RCC undergoing cystic necrosis (Fuhrman grade 3 or 4) and multilocular cystic RCC, a sub- stantially less aggressive subtype of clear cell RCC (Fuhrman grade 1 or 2), has de- creased, and a better prognosis of multilocu- lar cystic RCC is emerging [7]. Some pathol- ogists consider multilocular cystic RCC to have a uniformly benign behavior [8] be- cause there are no reported cases of progres- sion or metastasis [9–11].

Previous investigators have correlated the imaging features of multilocular cystic RCC with the corresponding pathologic features [3, 12, 13], two previous studies specifical- ly using the Bosniak cyst classification sys- tem [14, 15], a system that has proved use- ful in evaluating cystic renal masses with

Multilocular Cystic Renal Cell

Carcinoma: Comparison of Imaging and Pathologic Findings

Nicole M. Hindman1 Morton A. Bosniak1 Andrew B. Rosenkrantz1 Stephanie Lee-Felker2 Jonathan Melamed3

Hindman NM, Bosniak MA, Melamed J, Rosen- krantz AB, Lee-Felker S

1Department of Radiology, New York University School of Medicine, 550 First Ave, HW 202, New York, NY 10016.

Address correspondence to N. M. Hindman (hindmn01@nyumc.org).

2Department of Medicine, New York University School of Medicine, New York, NY.

3Department of Pathology, New York University School of Medicine, New York, NY.

WEB

This is a Web exclusive article.

AJR 2012; 198:W20–W26

0361–803X/12/1981–W20

© American Roentgen Ray Society

M

ultilocular cystic renal cell carci- noma (RCC) is a low-grade neo- plasm with an excellent progno- sis [1]. The first two descriptions appeared in the pathologic literature in 1928 and 1957. This subtype of cystic RCC is rela- tively uncommon, thought to represent 2.3–

3.1% of all cases of RCC [2–5], and has the best prognosis of all subtypes of RCC, cystic or solid. The true incidence of multilocular cystic RCC is unknown because of the vari- ance in pathologic criteria previously used to diagnose this subtype [2, 4]. Current patho- logic criteria, defined by the World Health Organization (WHO) in 2004, for this be- nign-behaving tumor are stricter, limiting these tumors to those with a malignant clear cell lining and small collections of cells but without formation of a malignant expansile nodule [1, 6]. With the application of this

Keywords: imaging, multilocular cystic renal cell carcinoma, pathologic findings

DOI:10.2214/AJR.11.6762

Received February 26, 2011; accepted after revision May 22, 2011.

OBJECTIVE. The purpose of this study was to retrospectively correlate the imaging and pathologic features of multilocular cystic renal cell carcinoma (RCC), a low-grade neoplasm that has an excellent prognosis.

MATERIALS AND METHODS. Institutional databases were searched for the period between 2001 and 2010 to identify cases of resected renal tumors that had been evaluated with CT or MRI and been analyzed by a uropathologist to confirm the histologic diagnosis of multilocular cystic RCC. The images (nine CT, 14 MRI) were reviewed, and a Bosniak cyst category was assigned.

RESULTS. Of 23 confirmed cases of multilocular cystic RCC, imaging revealed seven lesions were Bosniak category IIF, 13 were category III, and three were category IV. Patho- logic examination of the category IIF lesions revealed 99% fluid, 0.001–1% clear cells lining the septum, and 0% fibrosis. The category III lesions were 98–99% fluid, 1–2% clear cells, and 0% fibrosis. The category IV lesions were 20–40% fluid, 1–5% clear cells, and 60–80%

fibrosis. The patient demographics were similar across groups. Clinical follow-up showed no evidence of recurrent or metastatic disease.

CONCLUSION. Multilocular cystic RCC is a rare cystic lesion of the kidney that is low risk to the patient and benign in behavior. It has a variable imaging pattern, the Bosniak cat- egory ranging from IIF to IV. As multilocular cystic RCC lesions increase in complexity on images (higher Bosniak category), there is a corresponding increase in the volume of malig- nant cells lining the tumor and an increase in the presence of vascularized fibrous tissue. Re- gardless of the imaging appearance, the behavior of these tumors was benign in this study.

Clinicians and radiologists should be aware that when this carcinoma is reported to occur, the patient has an excellent prognosis.

Hindman et al.

Multilocular Cystic Renal Cell Carcinoma Genitourinary Imaging

Original Research

W20 AJR:198, January 2012

stricter pathologic definition of multilocular cystic RCC for diagnosis, the overlap in path- ologic features between solid clear cell RCC undergoing cystic necrosis (Fuhrman grade 3 or 4) and multilocular cystic RCC, a sub- stantially less aggressive subtype of clear cell RCC (Fuhrman grade 1 or 2), has de- creased, and a better prognosis of multilocu- lar cystic RCC is emerging [7]. Some pathol- ogists consider multilocular cystic RCC to have a uniformly benign behavior [8] be- cause there are no reported cases of progres- sion or metastasis [9–11].

Previous investigators have correlated the imaging features of multilocular cystic RCC with the corresponding pathologic features [3, 12, 13], two previous studies specifical- ly using the Bosniak cyst classification sys- tem [14, 15], a system that has proved use- ful in evaluating cystic renal masses with

Multilocular Cystic Renal Cell

Carcinoma: Comparison of Imaging and Pathologic Findings

Nicole M. Hindman

1

Morton A. Bosniak

1

Andrew B. Rosenkrantz

1

Stephanie Lee-Felker

2

Jonathan Melamed

3

Hindman NM, Bosniak MA, Melamed J, Rosen- krantz AB, Lee-Felker S

1Department of Radiology, New York University School of Medicine, 550 First Ave, HW 202, New York, NY 10016.

Address correspondence to N. M. Hindman (hindmn01@nyumc.org).

2Department of Medicine, New York University School of Medicine, New York, NY.

3Department of Pathology, New York University School of Medicine, New York, NY.

WEB

This is a Web exclusive article.

AJR 2012; 198:W20–W26

0361–803X/12/1981–W20

© American Roentgen Ray Society

M ultilocular cystic renal cell carci- noma (RCC) is a low-grade neo- plasm with an excellent progno- sis [1]. The first two descriptions appeared in the pathologic literature in 1928 and 1957. This subtype of cystic RCC is rela- tively uncommon, thought to represent 2.3–

3.1% of all cases of RCC [2–5], and has the best prognosis of all subtypes of RCC, cystic or solid. The true incidence of multilocular cystic RCC is unknown because of the vari- ance in pathologic criteria previously used to diagnose this subtype [2, 4]. Current patho- logic criteria, defined by the World Health Organization (WHO) in 2004, for this be- nign-behaving tumor are stricter, limiting these tumors to those with a malignant clear cell lining and small collections of cells but without formation of a malignant expansile nodule [1, 6]. With the application of this

Keywords: imaging, multilocular cystic renal cell carcinoma, pathologic findings

DOI:10.2214/AJR.11.6762

Received February 26, 2011; accepted after revision May 22, 2011.

OBJECTIVE. The purpose of this study was to retrospectively correlate the imaging and pathologic features of multilocular cystic renal cell carcinoma (RCC), a low-grade neoplasm that has an excellent prognosis.

MATERIALS AND METHODS. Institutional databases were searched for the period between 2001 and 2010 to identify cases of resected renal tumors that had been evaluated with CT or MRI and been analyzed by a uropathologist to confirm the histologic diagnosis of multilocular cystic RCC. The images (nine CT, 14 MRI) were reviewed, and a Bosniak cyst category was assigned.

RESULTS. Of 23 confirmed cases of multilocular cystic RCC, imaging revealed seven lesions were Bosniak category IIF, 13 were category III, and three were category IV. Patho- logic examination of the category IIF lesions revealed 99% fluid, 0.001–1% clear cells lining the septum, and 0% fibrosis. The category III lesions were 98–99% fluid, 1–2% clear cells, and 0% fibrosis. The category IV lesions were 20–40% fluid, 1–5% clear cells, and 60–80%

fibrosis. The patient demographics were similar across groups. Clinical follow-up showed no evidence of recurrent or metastatic disease.

CONCLUSION. Multilocular cystic RCC is a rare cystic lesion of the kidney that is low risk to the patient and benign in behavior. It has a variable imaging pattern, the Bosniak cat- egory ranging from IIF to IV. As multilocular cystic RCC lesions increase in complexity on images (higher Bosniak category), there is a corresponding increase in the volume of malig- nant cells lining the tumor and an increase in the presence of vascularized fibrous tissue. Re- gardless of the imaging appearance, the behavior of these tumors was benign in this study.

Clinicians and radiologists should be aware that when this carcinoma is reported to occur, the patient has an excellent prognosis.

Hindman et al.

Multilocular Cystic Renal Cell Carcinoma Genitourinary Imaging

Original Research

diagnostic différentiel

-néphrome kystique (récepteurs aux œstrogènes et à la progestérone +; stroma de type ovarien )

-kystes corticaux multiloculés

-néphroblastome kystique partiellement différencié (enfant) -carcinome papillaire à cellules claires kystique (rare)

(8)

messages à retenir

-le carcinome rénal multikystique à cellules claires est un néoplasme de bas grade , généralement découvert

fortuitement , chez un adulte d'âge moyen , avec une légère prédominance féminine

-l'aspect macroscopique est assez évocateur ; contours réguliers liés à la présence d'une pseudo-capsule fibreuse ; absence de néo vascularisation anarchique absence de signes d'agressivité et de dissémination locale ou à distance

-la néphrectomie partielle en chirurgie mini-invasive guidée par robot est

particuliérement adaptée à la situation , même si cette solution élégante n'a pas pu être employée dans le cas rapporté

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