Case Study
Collecting Duct Carcinoma
Collecting Duct Carcinoma of the Kidney
Mimicking a Locally Advanced Urothelial Carcinoma
Kevin Lu1, Victor Chia-Hsiang Lin1, I-Chang Lin2, Jau-Chung Hwang3, Tsan-Jung Yu1, Hua-Pin Wang1,
Chao-Yan Chiang1, Hung-Yu Lin1, Eng-Kian Lim1
Division of Urology, Department of 1Surgery, 2Radiology, 3Pathology, Minimally Invasive Surgical Center,
E-Da Hospital, Department of Nursing, I-Shou University, Kaohsiung, Taiwan
BACKGROUND
A 76-year-old male presented to our emergency
department on September, 2007 with the chief complaint
of piercing intolerable right flank pain for several hours.
Tracing back his history, he had a long history of hypertension with regular medical treatment and previous
pulmonary tuberculosis (PTB). No travel or insect-bite
history was noted. He had experienced intermittent right
flank pain and gross hematuria about 1 month previous.
There was no fever, nausea, or loss of body weight during this period. Sudden onset of piercing intolerable right
flank pain occurred on the morning of September, 2007.
Subsequently, he was sent to our emergency department
because of worsening symptoms. During his stay in the
emergency department, vital signs showed a blood pressure of 155/80 mmHg, a pulse rate of 71 beats/min, and
a body temperature of 37.2 ˚C. On the physical examination, knocking tenderness over the right costovertebral angle was noted. Urine analysis revealed hematuria
(red blood cells (RBCs) of > 100/high-power field
(HPF)) and pyuria (white blood sells (WBCs) of 10~25/
HPF). Elevated C-reactive protein (CRP) was also noted
(43.50 mg/L; normal range, 0~5.0 mg/L). He was admitted to our nephrology ward under the impression of
a urinary tract infection. In the ward, renal ultrasonography revealed decreased bilateral renal size with chronic
renal parenchymal change, bilateral renal cysts, left renal stones, and right renal mass lesions, suspected of
being a malignancy or abscess formation (Fig. 1A). A
urologist was consulted to evaluate his renal condition.
Differential Diagnosis
Abdominal computed tomography (CT) was arranged and demonstrated ill-defined hypo-enhanced infiltration of the right kidney with mild circular wall thickening throughout the right ureter, and bilateral small renal stones (Fig. 1B-D). A renal malignancy such as infiltrative urothelial cancer or renal cell carcinoma was
our impression, but an inflammatory condition such as
a chronic granulomatous infection (TB infection) and
xanthogranulomatous pyelonephritis could not be ruled
out. Urine culture yielded non-specific findings of a
mixture of 4 types of bacteria. The patient denied any
past history of urinary tract infection or urolithiasis, and
there were no complaints of fever or chills. The possibility of xanthogranulomatous pyelonephritis was thus
decreased. Although this patient had a past history of
PTB, the acid-fast stain was negative, and TB culture
yielded no growth. He had no night fever or loss of body
weight. A chest x-ray film showed no active lung lesions.
A CT scan revealed no extensive calfications. A diagnosis of TB of the kidney is rare. The patient had been
bothered by right flank pain and gross hematuria for 1
month. While voiding urine cytology was negative for
malignant cells, there were many RBCs, and some polymorphonuclear neutrophil leukocytes (PMNs) and
lymphocytes, so the possibility of infiltrative renal pelvic transitional cell carcinoma was higher than that of
renal cell carcinoma.
Management
On account of a high suspicion of a urothelial
malignancy, he underwent a pure laparoscopic transperitoneal right nephroureterectomy with bladder cuff
excision under general anesthesia with endotracheal
intubation. The distal ureter and bladder cuff were man-
Address reprint requests and correspondence to:
Victor Chia-Hsiang Lin, MD.
Division of Urology, Department of Surgery, E-Da Hospital,
Department of Nursing, I-Shou University, 1 Yi-Da Rd., Jiau-Shu,
Yan-Chau Township, Kaohsiung 824, Taiwan, R.O.C.
Tel: 886-7-6150011 Fax: 886-7-6150913
E-mail: [email protected]
This article has one study questionnaire in page 131
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K. Lu, et al
aged by open resection through a modified Gibson's incision (7 cm long). The postoperative course was
uneventful. The Foley catheter was removed on the 5th
postoperative day, and the Jackson-Pratt drain was removed on the 7th postoperative day. He was discharged
on the 8th postoperative day.
Pathology
The bisected kidney specimens measured 10.0 ×
5.5 × 4.5 cm and weighed 210 g with a grayish-brown
discoloration and smooth outer surface. Yellowish-white
infiltrative tumors of the middle and upper part of the
renal parenchyma measured 7.5 × 4.2 × 4.0 cm. On serial cuts, the tumor had grossly invaded through the renal parenchyma into the perirenal soft tissues and renal
pelvis. Microscopically, the tumors were composed of
an adenocarcinoma with tubular and focal
tubulopapillary growth patterns (Fig. 2A-D). Immunohistochemical studies of the tumor revealed that they
were focally positive for CK20 (an epithelial marker
mainly found in the intestinal epithelium, gastric foveolar epithelium, urothelium, and Merkel cells),1 positive
for vimentin (a 15-kDa protein, one of the intermediate
filaments, normally present in stromal cells but not epithelial cells)2 (Fig. 2E), and positive for high-molecu-
A
B
C
D
Fig. 1.
(A) Renal ultrasonography revealing a heterogeneous echoic mass, measuring 5.09 x 5.06 cm with internal blood flow at
the upper pole of the right kidney. (B) Pre-contrast images. (C) Contrast-enhanced images. (D) Delayed-phase computed
tomographic images demonstrating an ill-defined hypo-enhanced infiltrative mass occupying the upper and middle pole of
the right kidney.
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Collecting Duct Carcinoma
lar-weight (HMW) cytokeratin (characteristic of basal
cells) (Fig. 2F), but negative for CD10 (a common acute
lymphoblastic leukemia antigen (CALLA) and
metallomembrane endopeptidase expressed in a variety
of normal cell types, including lymphoid precursor cells,
germinal center B lymphocytes, and some epithelial and
A
B
C
D
E
F
Fig. 2.
VO
Histopathology. (A) Hematoxylin and eosin (H&E) staining (reduced from 200x). (B) H&E staining (reduced from 400x).
(C) H&E staining (reduced from 200x) of the renal pelvic portion. (D) H&E staining (reduced from 100x) of the perirenal
portion. A-D show an admixture of dilated tubules and papillary structures lined by a single layer of cuboid cells, with
hyperplastic collecting cells adjacent to the tumors. (E) Immunohistochemical staining with vimentin. (F) Immunohistochemical staining with high-molecular-weight cytokeratin.
JTUA 2008 19 No. 2
K. Lu, et al
neoplastic cells).3 Thus, the final diagnosis of collecting
duct carcinoma, T3N0M0, was favored on account of
the microscopic appearance and panel immunohistochemical study results (positive for vimentin and
HMW cytokeratin staining, and negative for CD-10
staining).
DISCUSSION
Collecting duct carcinomas, so-called Bellini's duct
carcinomas, are a relatively rare disease entity and a distinctive subtype of renal cell carcinomas (RCCs), representing fewer than 1% of all renal neoplasms. They are
more common in younger patients, often in the third,
fourth, or fifth decades of life. They arise in the renal
medulla with atypical hyperplastic change in the adjacent collecting ducts, and frequently extend into the renal cortex due to their aggressive infiltrative biological
behavior. On imaging evaluations, they can mimic renal pelvic transitional cell carcinomas. An affinity for
Ulex europaeus agglutinin 1 lectin and expression of
HMW cytokeratin (34ßE21) are reminiscent of collecting duct origin in distal nephrons.4 Cystogenetic studies
of collecting duct carcinomas have shown that the tumors consist of a variety of heterogeneous chromosomal
alternations unrelated to other variants of RCC, without
consistent association with the loss of heterzygosity of
chromosome 3p, typical for clear-cell RCCs or trisomies of chromosomes 7, 12, 16, 17, and 20, which are
typical of papillary RCCs.2 Most patients are symptomatic and are diagnosed as having high-grade and highstage disease with a poor prognosis at the clinic visit.
Generally, collecting duct carcinomas have metastasized
to regional lymph nodes in around 80% of patients, to
the lungs or adrenal glands in 25%, and to the liver in
20% at presentation. The median survival time after a
nephrectomy has been reported to be approximately 22
months. Various treatment strategies have been
attempted, but with only limited efficacies. These tumors usually responded poorly to conventional therapies in the published literature.4,6-8 However, Milowsky
et al. and Peyromaure et al. respectively reported responses to cisplatin and gemcitabine-based chemotherapy in some patients with advanced collecting-duct
carcinoma.6-9
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The treatment of choice for collecting-duct carcinomas is currently restricted to surgical excision.
Whether this tumor should be managed in a similar
manner to urothelial or renal carcinomas remains elusive,
or possibly other completely different therapeutic strategies are warranted. Many chemotherapeutic and/or
immunotherapeutic regimens have been proposed to
manage collecting-duct carcinomas, but they have produced only limited benefits in selected groups of
cases.8,9 With wide acceptance of minimally invasive surgeries and refinement of surgical skills, the laparoscopic
approach is another alternative option. It is comparable
to its open counterpart in oncological efficacy and
perioperative outcomes in short-term follow-up.
However, long-term studies need to be conducted to
confirm these results.
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filament typing: a novel tool for surgical pathology. Lab
Invest 1983;48:372-94.
3. Abdou AG.. CD10 expression in tumour and stromal
cells of bladder carcinoma: an association with bilharziasis APMIS. 2007;115(11):1206-18.
4. Wein AJ, Kavoussi LR, Novick AC, Partin AW, Peters
CA. Campbell-Walsh Urology. 9th ed. Philadephia, PA:
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5. Antonelli A, Portesi E, Cozzoli A, et al. The collecting
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