British Journal of Radiology (2005) 78, 269-271
© 2005 British Institute of Radiology
doi: 10.1259/bjr/28214940
Erythropoietin-producing renal cell carcinoma arising from autosomal dominant polycystic kidney disease
Y Hama, MD
1
T Kaji, MD
1
K Ito, MD
2
M Hayakawa, MD
2
M Tobe, MD
1 and
S Kosuda, MD
1
Departments of 1 Radiology and 2 Urology, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama, Japan
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Abstract
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Erythropoietin (EPO)-producing renal cell carcinomas (RCC) in patients with chronic renal failure secondary to autosomal dominant polycystic kidney disease (ADPKD) has not previously been reported. We report a case of EPO-producing RCC associated with ADPKD in a 66-year-old woman, and discuss the clinical and radiological findings.
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Introduction
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Erythropoietin (EPO)-producing renal cell carcinomas (RCC) in patients with chronic renal failure is uncommon [1]. The occurrence of EPO-producing RCC in a patient with autosomal dominant polycystic kidney disease (ADPKD) has not previously been reported [2–4]. Here, we report a case of EPO-producing RCC in a patient with ADPKD and discuss the clinical and radiological findings.
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Case report
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A 66-year-old woman, receiving maintenance haemodialysis for 8 years owing to chronic renal failure secondary to ADPKD, was referred to our institution for evaluation of polycythaemia. She had manifested no anaemia for more than 4 months without using recombinant human EPO. Her red blood cell count was 606 x 106 cells mm–3, the haemoglobin concentration was 16.4 g dl–1, and the haematocrit was 53.3%. The serum EPO level was 893 mU ml–1 (normal: 9.1–32.8 mU ml–1). Ultrasound (US) scan showed a homogeneous, round, echogenic area medial to the middle of the left kidney (Figure 1a
). Contrast-enhanced CT scan obtained during the nephrographic phase showed a homogeneously enhancing mass arising from the medial aspect of the left kidney (Figure 1b). On digital subtraction angiography, the lesion was shown to be highly vascularized and supplied mainly by direct branches of the left renal artery (Figure 1c). Pre-operative selective embolisation of the left renal artery was performed using gelatin sponge particles. She underwent radical nephrectomy with curative intent. Pathological examination revealed clear cell RCC. Immunohistochemical staining of the resected specimens showed production of erythropoietin in the tumour cells. Although an elevated serum EPO level decreased to 61 mU ml–1 2 weeks after the nephrectomy. Based on the immunohistochemical and clinical findings, EPO was considered to be produced by RCC arising from ADPKD.
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Figure 1. (a) Longitudinal ultrasound scan showed a homogeneous, round, echogenic mass (arrow) at the medial aspect of the left kidney. (b) Contrast-enhanced CT scan obtained during the nephrographic phase showed a homogeneously enhancing mass (arrow) arising from the medial aspect of the left kidney. (c) Digital subtraction angiography of the left renal artery demonstrated a hypervascular tumour (arrow) which was supplied by direct branches of the left renal artery.
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Discussion
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Serum erythropoietin may be increased in patients on chronic haemodialysis secondary to acquired polycystic disease of the kidneys. The development of RCC in ADPKD is rare and the association of RCC and ADPKD is controversial [2, 3, 5]. The majority of reported cases have been shown to be an incidental finding either on surgical exploration or at autopsy [6]. The serum EPO level is usually low in long-term haemodialysis patients with anaemia [7]. However, increased levels of serum EPO and haemoglobin have been observed in haemodialysis patients because the renal proximal tubule cells produce EPO into the fluid of cysts [4, 8, 9]. Thus, the diagnosis of EPO-producing RCC in such cases is difficult due to overlapping clinical features and marked distortion of architecture [10].
US often fails to reliably differentiate benign haemorrhagic cysts from other complications in patients with ADPKD [11]. CT remains the most widely used modality for assessment and has helped refine the diagnostic work-up of renal masses by allowing image acquisition in various phases of renal enhancement after intravenous administration of a single bolus of contrast material. The nephrographic phase is the most sensitive for tumour detection [12], because the renal parenchyma enhances homogeneously in this phase, allowing a better opportunity for discrimination between the normal renal medulla and masses [13]. In end-stage ADPKD, most of the renal parenchyma is replaced by cysts and fibrotic tissues, and detection of RCC becomes much easier because of the intense enhancement of RCC.
MRI has certain advantages in evaluating patients with ADPKD. The high soft tissue contrast resolution to evaluate the kidneys, cysts, and the possible development of cancer, have shown MRI to be of particular importance in assessing ADPKD patients and defining the possible development of RCC [14]. However, in the present case, clinical signs, US and CT findings were sufficient to reach a pre-operative diagnosis of RCC, and further investigation by MRI was not necessary. Selective angiography, performed for pre-operative embolisation of the tumour vessels, strongly suggested the diagnosis of RCC, although the availability of reliable non-invasive investigations, routine diagnostic angiography is no longer recommended [15].
In conclusion, although a single case cannot be generalized to other ADPKD cases, a continuous increase in serum EPO levels without the use of recombinant human EPO, and a newly developed solid mass with contrast enhancement on CT in a patient with ADPKD may suggest the diagnoses of an EPO-producing RCC.
Received for publication September 1, 2004.
Revision received October 8, 2004.
Accepted for publication December 6, 2004.
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References
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Abstract
Introduction
