|Year : 2013 | Volume
| Issue : 2 | Page : 331-333
Renal collecting duct carcinoma: Report of a case with unusual imaging findings regarding renal function
Longwang Wang1, Li Peng2, Teng Hou3, Ying Shi1
1 Department of Urology, Union Hospital, Tongji Medical College, Huazhong University of Science & Technology, Wuhan, Hubei, China
2 Department of Pathology, Union Hospital, Tongji Medical College, Huazhong University of Science & Technology, Wuhan, Hubei, China
3 Department of Urology, Shenzhen People's Hospital, Second Clinical Medical College, Jinan University, Guangzhou, Guangdong, China
|Date of Web Publication||13-Jun-2013|
Jiefang Avenue 1277#, Wuhan, 430022
Source of Support: None, Conflict of Interest: None
Renal collecting duct carcinoma (CDC) is a rare and aggressive type of renal cell cancer (RCC), which is difficult to confirm before surgery. We present a case of CDC presenting a hypovascular mass on renal CTA and deteriorated renal function of the affected kidney on single photon emission computed tomography (SPECT), which are different from the most common RCC, clear cell RCC. Considering these findings, it would be worthwhile investigating the role of CTA and SPECT in CDC diagnosis.
Keywords: Collecting duct carcinoma, CT angiography, Single photon emission computed tomography
|How to cite this article:|
Wang L, Peng L, Hou T, Shi Y. Renal collecting duct carcinoma: Report of a case with unusual imaging findings regarding renal function. J Can Res Ther 2013;9:331-3
|How to cite this URL:|
Wang L, Peng L, Hou T, Shi Y. Renal collecting duct carcinoma: Report of a case with unusual imaging findings regarding renal function. J Can Res Ther [serial online] 2013 [cited 2019 Sep 20];9:331-3. Available from: http://www.cancerjournal.net/text.asp?2013/9/2/331/113425
| > Introduction|| |
Renal collecting duct carcinoma (CDC) is an extremely rare and aggressive renal tumor that derives from the distal segment of the collecting ducts (Bellini duct) in the renal medulla, unlike the common variants of renal cell carcinoma (RCC), which derive from the convoluted tubules. CDC accounts for less than 1% of all the RCCs, but the diagnosis is difficult to confirm before surgery, because the symptoms of CDC, such as hematuria, flank mass and the abdominal CT findings, are almost same as the most common RCC, clear cell RCC, while their prognosis is distinctively different. Here, we present a case of a female patient with a hypovascular renal mass accompanying deteriorated renal function, which was pathologically confirmed as CDC finally, and discuss possible clues to tell CDC before surgery.
| > Case Report|| |
The patient was a 42-year-old woman who had a history of intermittent painless gross hematuria for 6 months and left flank soreness for 2 months. Physical examination revealed a palpable left flank mass about 7 × 6 cm in size, fixed, and non-tender. Renal artery CTA [Figure 1]a found a 6 × 6 × 4.2 cm mass with poor perfusion in the upper pole of the left kidney, which has no clear border with renal pelvis, renal sinus and adjacent renal parenchyma, distal left renal artery and its branches were apparently compressed [Figure 1]b, and there was no evidence of hydronephrosis and retroperitoneal lymph nodes enlargement [Figure 1]c. Left renal calices, renal pelvis and ureter didn't develop in intravenous pyelography [Figure 1]d. Single photon emission computed tomography (SPECT) showed a moderate reduction of left renal function (GFR 22.5 ml/min), while the other was normal (GFR 71.7 ml/min). The distant metastasis was excluded by abdominal CT scan and X-ray of chest. A diagnosis of RCC was made based on these findings and a radical nephrectomy was performed soon.
|Figure 1: Imaging findings before surgery. (a) Three-dimensional reconstruction of CTA revealed a hypovascular mass in the upper pole of left kidney. (b, c) CT findings revealed no significant enhancements|
and no clear border between the mass and renal pelvis, renal sinus and lower pole of normal renal arenchyma. (d) Left renal calices, pelvis and ureter didn't develop in intravenous pyelography and the other one was normal
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Gross examination showed a round-shaped, yellow tumor occupying almost the entire upper pole of left kidney measuring 7 × 6 × 5.5 cm and representing an infiltrating growth pattern with radiating manner from the deep medulla. The cut surface represented multifocal necrosis and hemorrhage. Microscopic examination revealed that the tumor displayed a papillary-like pattern [Figure 2]a and marked glandular tubular structure with mild stromal desmoplasia, as well as some tubules were surrounded by a thick layer of intercellular mucoid material and lined by a single layer of hobnail cells [Figure 2]b. Immunohistochemical studies demonstrated negative staining for cytokeratins (CK5/6, CK7, CK20), CD10/117, p63 and 34BE12, but positive staining for vimentin [Figure 2]c and p504s, and partly positive staining for pax-2 [Figure 2]d, E-cadherin and CK19. The malignancy involved renal pelvis, ureter and capsule, but surgical margin, renal vessels, adrenal and para-aortic lymph nodes were negative. The final pathologic diagnosis was left renal CDC with Fuhrman grade IV and the stage was T1bN0M0 according to the 2009 AJCC TNM staging.
|Figure 2: Microscopic findings of tissue sections from the nephrectomy specimens demonstrating CDC. (a, b) Cancer cells displayed a papillary-like pattern and marked glandular tubular structure with mild stromal desmoplasia (H and E, staining, × 100). (c, d) Cancer cells were positive for vimentin (× 200) and pax-2 staining (× 400)|
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The patient rejected any kind of chemotherapy when she was informed of the possible side effects. Five months post-surgery, the patient was asymptomatic and received re-examination of CT scan, which showed no evidence of recurrence or metastasis.
| > Discussion|| |
CDC is a rare malignancy of renal cell carcinomas, showing a highly aggressive pattern with early distant metastasis. CDC often presents hematuria, flank pain, a palpable abdominal mass or distant metastasis, but none of which is specific. The diagnosis of CDC still mainly depends on the microscopic and immunohistochemical features to date.
Pickhardt et al. reviewed the pre-operative imaging studies from 17 CDC cases and reported that medullary involvement and an infiltrative appearance were common findings suggesting the diagnosis of CDC, but these features were often overshadowed by an exophytic or expansile component in large tumors. They also mentioned that tumor hypovascularity was seen in all the cases, which is consistent with our findings. Since 90% clear cell RCC are hypervascular, the CTA finding might be a clue to remind surgeons of the possibility of CDC before surgery. SPECT was performed routinely to evaluate the function of the healthy kidney before nephrectomy in our department, but surprisingly it revealed a reduction of GFR of the affected kidney. Clinically deteriorated renal function is frequent in hydronephrosis, nephrotuberculosis, etc., rather than RCC, which depicts that this case might be different before surgery. We speculated that the infiltrative growth pattern of CDC impaired the nephrons, and the mass was bigger, the involved area was larger. We have reviewed previous literature and none discussed this issue before, so we think it's worthy of further exploration.
Radical nephrectomy is the standard treatment for CDC, but due to the highly aggressive behavior and early distant metastasis of CDC, the median survival after surgery is only 22 months. There is still no standard chemotherapy for CDC. Combination of gemcitabine with doxorubicin, platinum salts, INF-α and IL-2 have been proven to have poor response. Novel oral inhibitors of multiple receptor tyrosine kinases, sunitinib and sorefenib, are all reported to have a favorable effect on the metastatic CDC,, but the wild application needs a prospective evaluation. Therefore early detection and combined treatment may be the best treatment strategy to prolong a CDC patient's survival right now. In this case, the tumor was large and protruded the renal capsule, but it was lucky that para-aortic lymph nodes, perirenal and distant metastasis were not observed. Now she has lived disease-free for 5 months without any adjuvant treatment. We would continue the follow up to conclude the case.
It is difficult to differentiate CDC from other types of RCC on the basis of normal imaging findings, but CDC's unique growth pattern and hypovascularity facilitate the early detection of CDC. As to the deteriorated renal function on SPECT, there is no paper analyses of CDC in point of this view to our knowledge. We think this unusual phenomenon is worthy of a much more detailed evaluation to figure out its role in CDC diagnosis.
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[Figure 1], [Figure 2]