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ctas Urol Esp. 2011;35(6):368---371

Actas Urológicas Españolas

www.elsevier.es/actasuro

ASUISTRY

ollecting duct renal cell carcinoma�

. Husillos ∗, F. Herranz-Amo, D. Subirá, E. Lledó, R. Molina-Escudero,

. Hernández-Fernández

ervicio de Urología, Hospital General Universitario Gregorio Maranón, Madrid, Spain

KEYWORDSKidney;Tumor;Collecting duct;Bellini

AbstractIntroduction: Bellini’s renal cell collecting duct carcinoma is a rarely prevalent renal tumor,with low cancer-specific survival, although its rate of response to antiangiogenic therapies isunknown.Objectives: We retrospectively revise a series of collecting duct tumors, with special emphasison the indication of target therapies and on their results.Materials and methods: Retrospective analysis of renal cell collecting duct carcinoma treatedat our institution from January 2000 to June 2010, taking into account the patient’s age, sex,reason for the consultation, oncological background, side of the affection, surgical treatment,other anatomopathological characteristics, tumor size, TNM clinical staging (2009), adjuvanttreatment and survival time.Results: Six patients are described, five men and one woman, with a mean age of 75 (±7.7)years. Four of them (66.6%) presented disseminated disease upon diagnosis. Five (83%) weretreated with radical nephrectomy and three (50%) received systemic adjuvant treatment, with-out response. The means survival was 5.5 months (4.75---14.75). Only 2 patients (33.3%), bothwith localized disease upon diagnosis, are in complete remission.Conclusion: Renal cell collecting duct carcinoma is a disease with a bad prognosis, little survivaland bad response to target therapies.© 2011 AEU. Published by Elsevier España, S.L. All rights reserved.

PALABRAS CLAVE Carcinoma de células renales del túbulo colector

Rinón;Tumor;Conductoscolectores;Bellini

ResumenIntroducción: El carcinoma renal de los conductos colectores de Bellini es un tumor renalescasamente prevalente, con baja supervivencia cáncer-específica, aunque realmente sedesconoce su tasa de respuesta a terapias antiangiogénicas.Objetivos: Se revisa de manera retrospectiva una serie de tumores de túbulo colector con

especial énfasis en la indicación de terapias diana y en los resultados de la misma.

� Please cite this article as: Husillos A, et al. Carcinoma de células renales del túbulo colecto. Actas Urol Esp. 2011;35:368---71.∗ Corresponding author.

E-mail address: adrhusillos@hotmail.com (A. Husillos).

173-5786/$ – see front matter © 2011 AEU. Published by Elsevier España, S.L. All rights reserved.

Collecting duct renal cell carcinoma 369

Material y métodos: Análisis retrospectivo del carcinoma renal de túbulo colector tratados ennuestra institución desde enero 2000 a junio 2010, teniendo en cuenta la edad del paciente, elsexo, el motivo de consulta, los antecedentes oncológicos, el lado de afectación, el tratamientoquirúrgico, otras características anatomopatológicas, el tamano tumoral, la estadificación TNM(2009), el tratamiento adyuvante y el tiempo de supervivencia.Resultados: Se describen 6 pacientes, 5 varones y una mujer; con una media de edad de75 (± 7,7) anos. Cuatro de ellos (66,6%) presentaban enfermedad diseminada al diagnóstico.Cinco (83,3%) fueron tratados mediante nefrectomía radical y tres (50%) recibieron tratamientosistémico adyuvante, sin respuesta. La mediana de supervivencia fue 5,5 meses (4,75-14,75).Sólo dos pacientes (33,3%), ambos con enfermedad localizada al diagnóstico, se encuentran enremisión completa.Conclusión: El carcinoma renal de túbulo colector es una enfermedad con mal pronóstico,escasa supervivencia y mala respuesta a terapias diana.© 2011 AEU. Publicado por Elsevier España, S.L. Todos los derechos reservados.

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Introduction

Collecting duct renal carcinoma, also called Bellini’s tumor,is the least prevalent subtype of renal carcinoma. Except forthree major series published in recent years,1---3 only isolatedcases have been reported. The collecting duct carcinoma ischaracterized by being an aggressive entity with an unfortu-nate outcome in most patients. In our country, only isolatedcases or case series have been published. The aim of thisstudy is to evaluate our own case series, with emphasis onthe analysis of the response to systemic therapy and, inparticular, to new target therapies.

Materials and methods

We revised all renal tumors diagnosed in our center fromJanuary 2000 to June 2010, and identified cases of collect-ing duct renal carcinoma. The variables analyzed were: age,sex, reason for consultation, oncological background, sideof the affection, surgical treatment, pathological charac-teristics, tumor size, TNM staging (rating 2009), adjuvanttreatment and survival time.

A descriptive statistical analysis was performed usingMicrosoft Office Excel 2003®. For the analysis of quantita-tive variables the mean with the standard deviation and themedian with the interquartile range were used. In the anal-ysis of qualitative variables, we used the percentage. Aninferential statistical analysis was not performed due to lowsample size.

Results

Of a total of 670 renal tumors, 6 (0.89%) were collecting ductrenal cell carcinoma. Table 1 summarizes the characteris-tics of these patients. The mean age was 75 (±7.71) years.The male:female ratio was 5:1. The average size of tumor,measured as the maximum diameter of the macroscopic

piece for tumors that were operated, or with computerizedaxial tomography (CT)/nuclear magnetic resonance (NMR)for the rest, was 65.5 mm (±39.6) (Figs. 1 and 2). Fivepatients (83%) were treated with radical nephrectomy. In

D

Tt

igure 1 CT radiological imaging of locally advanced collect-ng duct renal carcinoma.

ne patient (16.6%) diagnosis was made by percutaneousiopsy. Four of the 6 cases (66.6%) had lymphatic and/orematogenous dissemination at diagnosis. Only two (33.3%)resented localized disease, which are those that are freef recurrence and alive today, with a follow-up of 120 and7 months, respectively. The remaining patients died ofrogression of their disease, with a median survival of 5.54.75---14.75) months.

Of the 4 patients with lymphatic and/or hematogenousissemination, three (75%) received adjuvant systemic ther-py to surgery. Two received immunotherapy (sunitinib andemsirolimus) and a third patient received a conventionalhemotherapy scheme based on cisplatin and gemcitabine.one of them presented remission of the adenopathy and/oretastasis in the follow-up performed with CT. Surgery on

he metastasis was not carried out either.

iscussion

he collecting duct renal carcinoma is a rare entity that hasraditionally presented an ominous oncologic prognosis. Our

370 A. Husillos et al.

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igure 2 NMR radiological imaging of localized collectinguct renal carcinoma.

xperience with target therapies and chemotherapy is veryimited, but it also confirms its ominous prognosis withoutlinical response.

This type of renal tumor develops from the collectingubules of the renal medulla, thus, presenting an unusualnd distinctive histology (Fig. 3). There are no specificadiological features of this tumor.4 From the pathologicaloint of view, there are a number of microscopic fea-ures that point towards its diagnosis: commitment of theedullary pyramids, irregular tubular architecture, markedesmoplasia and the presence of high degree tack cells.mmunohistochemistry is usually positive for Ulex europeus,igh molecular weight cytokeratin (CK19, CK7, CK8/18)ez1, mucin, lysozyme and lectins/peanut aglutinina.5,6

There are three series published with a significant num-er of cases.1---3 They all observed a predominance of males.he age at diagnosis of this neoplasm is usually from thefth decade of life, although there are also cases in young

atients. The mean size at diagnosis varies depending on theeries, but it ranges between 6 and 8 cm. At diagnosis mostatients have locally advanced disease with lymph node

igure 3 Microscopic appearance of the collecting duct renalarcinoma.

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Collecting duct renal cell carcinoma

and/or metastatic disease. All these data are consistent withour cases, although the age at diagnosis we observed tendsto be higher.

The treatment that was performed in most patientsdescribed above was radical nephrectomy, even in thosewith disseminated disease. However, the benefit of cytore-ductive nephrectomy has not been objectified in patientswith metastatic collecting duct renal carcinoma either.7

Survival described long term is low and is around 0---58%at three years.1,8 In our experience, only patients withlocalized disease at diagnosis, lack of lymph node orhematogenous disease are free of recurrence. Two stud-ies compared cancer-specific survival of this neoplasm withclear cell renal carcinoma. In the series of Karakiewitz et al.no differences in survival are found if adjusted for stage.2

Nevertheless, this finding may be due to a low number ofcases (47 patients) and the inclusion of slants in choosingthe comparison cohort because it includes cases treated inthe 80s. In the series of Wright et al. a comparison betweencases diagnosed in the same period is performed, all from2000, finding statistically significant difference in survival infavor of conventional renal cell carcinoma.1

None of the large series evaluates the response toadjuvant treatments with chemotherapy, immunotherapyor antiangiogenic therapy. From a pathological point ofview, there is some histopathogenetic proximity betweencollecting duct carcinoma and carcinoma of the upperurothelium. That is why, some authors have proposedtherapeutic regimens based on carboplatin-gemcitabine,doxorubicin, gemcitabine and paclitaxel-carboplatin.9,10 Inour series, patients treated systemically have not showna good response to adjuvant treatment with temsirolimus,sunitinib or cisplatin and gemcitabine.

In summary, collecting duct renal cell carcinoma is a rarerenal tumor. In our series, most patients are in a dissem-

inated or locally advanced stage at diagnosis. The rate oflong-term survival is low because the only potentially cura-tive treatment appears to be surgery if it is considered inpatients with localized tumors.

371

onflict of interest

he authors declare that they have no conflict of interest.

eferences

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2. Karakiewicz PI, Trinh QD, Rioux-Leclercq N, de la Traille A,Novara G, Tostain J, et al. Collecting duct renal cell carcinoma:a matched analysis of 41 cases. Eur Urol. 2007;52:1140---6.

3. Tokuda N, Naito S, Matsuzaki O, Nagashima Y, Ozono S, IgarashiT. Collecting duct (Bellini duct) renal cell carcinoma: a nation-wide survey in Japan. J Urol. 2006;176:40---3.

4. Yoon SK, Nam KJ, Rha SH, Kim JK, Cho KS, Kim B, et al.Collecting duct carcinoma of the kidney: CT and pathologiccorrelation. Eur J Radiol. 2006;57:453---60.

5. Kobayashi N, Matsuzaki O, Shirai S, Aoki I, Yao M, NagashimaY. Collecting duct carcinoma of the kidney: an inmunohisto-chemical evaluation of the use of antibodies for differencialdiagnosis. Hum Pathol. 2008;39:1350.

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8. De Diego RE, Pascual SC, Gutiérrez Banos JL, Martín GB,Hernández RR, Portillo Martín JA, et al. Carcinoma de Bellini:nuestra experiencia. Arch Esp Urol. 2000;53:611.

9. Orsola A, Trías A, Raventós CX, Espanol L, Cecchini L, Orsola I.Renal collecting (Bellini) duct carcinoma displays similar char-acteristics to upper tract urothelial cell carcinoma. Urology.2005;65:49---54.

10. Milowsky MI, Rosmarin A, Tickoo SK, Papanicolau N, NanusDM. Active chemotherapy for collecting duct carcinoma of thekidney: a case report and review of the literature. Cancer.2002;94:111---6.