Journal of Surgical Oncology 26:77-78 (1984)

Renal Cell Carcinoma in a Horseshoe Kidney

MARSHALL WIENER, MD, DEBA SARMA, MD, AND MOPARTY RAO, MD From the Department of Urology, Tulane Medical Center, New Orleans, and VA Medical Center, Alexandria, and the Department of Pathology, Louisiana State University Medical Center, New Orleans

A case of renal cell carcinoma arising in a horseshoe kidney and presenting with a pathologic fracture of humerus is reported. Relevant literature is discussed.
KEY WORDS: horseshoe

kidney, tumor of horseshoe kidney, cancer of horseshoe kidney, renal cell cancer of horseshoe kidney, clear cell cancer of horseshoe kidney

INTRODUCTION

Renal cell carcinoma arising in a horseshoe kidney is a rare occurrence. Up to 1975, about 55 such cases have been described in the world literature [1]. Skeletal metastasis as the presenting clinical feature for a carcinoma of the horseshoe kidney is most unusual. We have been unable to find such a case in the English literature. CASE REPORT A 58-year-old white man came to the hospital with a painful swelling of the right arm, which the patient had developed soon after getting down from a ladder. Roentgenogram revealed an osteolytic lesion and a pathologic fracture of the right humerus (Fig. 1). An exploration and a biopsy of the fracture site revealed a metastatic renal cell carcinoma. A large amount of tumor was curetted, and an internal fixation of the humerus was done. Additional clinical history failed to uncover any history of weight loss, hematuria, urinary symptoms, or abdominal pain. Two years prior to the present admission, the patient underwent an abdominal aortic aneurysmectomy for an infrarenal aneurysm. Roentgenologic studies done at that time revealed a horseshoe kidney without any lesion. An intravenous urogram revealed a mass in the right upper pole of the horseshoe kidney with distortion of the upper collecting system (Fig. 2). Metastatic work-up was negative except for the lesion in the right humerus. A transperitoneal exploration of the right kidney was done that disclosed a 10-cm2 tumor of the upper pole. A right radical nephrectomy was performed. Pathologic examination confirmed the tumor to be a renal cell carcinoma with no invasion of renal vein or capsule.
1984 Alan R. Liss, Inc.

Fig. 1. Right humerus showing metastatic renal cell carcinoma with pathologic fracture.

Accepted for publication May 23, 1983. Address reprint requests to D. Sarma, MD, 1601 Perdido Street, New Orleans, LA 70146.

Wiener, Sarma, and Rao

Fig. 2. Intravenous urogram showing the renal cell carcinoma in the right upper pole.

Postoperatively, the patient received radiation therapy to the right humerus and systemic chemotherapy (vinblastine, 0.2 mg/kg). Two years later the patient expired with metastatic tumors in the lungs and bones. DISCUSSION Whereas approximately 86% of clinically manifest primary malignant renal tumors are renal cell carcinoma [2], among the cancers of horseshoe kidneys, they comprise about 50% [1]. When compared with the incidence of cancer in a normal kidney, the horseshoe kidney does not appear to have an increased risk of developing malignant tumor [3]. In one study of 105 consecutive cases of horseshoe kidney, only 2 cases of malignant tumors (1 renal cell carcinoma and 1 Wilm's tumor) were found [4]. About one-third of the patients with renal cell carcinoma may have distant metastases at initial presentation [5]. Most frequent sites of metastasis are lung and bones.

Bone being a common metastatic site, first presentation with a pathologic fracture in a renal cell carcinoma arising in a horseshoe kidney is not unexpected. However, survey of the literature shows such an occurrence to be very rare. ACKNOWLEDGMENTS
The authors thank Ms. Karen Dunn for excellent secretarial help.

REFERENCES
1. Buntley D: Malignancy associated with horseshoe kidney. Urology 8:146-148, 1976. 2. Bennington JL. Beckwith JB: Tumors of the kidney, renal pelvis, and ureter. Atlas of tumor pathology. Washington, DC, Armed Forces Institute of Pathology. Fascicle 12:25-29, 1975. 3. Blackard CE, Mellinger GT: Cancer in a horseshoe kidney. Arch Surg 97:616-627, 1968. 4. Kolln CP, Boatman DL, Schmidt JD, Flocks RH: Horseshoe kidney: A review of 105 patients. J Urol 107:203-204, 1972. 5. Holland JM: Cancer of the kidneyNatural history and staging. Cancer 32:1030-1042, 1973.

Weiner M, Sarma DP, Rao M(1984): Renal cell carcinoma in a horseshoe kidney. J Surg Oncol 26:77-78. PMID: 6727390 [PubMed - indexed for MEDLINE]