Clear cell carcinoma in horseshoe kidney: a case report and literature review

Transcription

1 CLINICAL CASE Clear cell carcinoma in horseshoe kidney: a case report and literature review Yaber-Gómez EK, 1 Cortes-Arcos Y, 1 González-Ruiz FG, 1 González-Gomez A, 1 Zuviri-González A, 1 De León-Ángeles P, 1 Cortez-Betancourt R, 1 Barroso-Espinosa M. 2 ABSTRACT RESUMEN Horseshoe kidney is the most frequent kidney fusion alteration. The majority of patients presenting with this pathology are asymptomatic. When symptoms are present they are associated with hydronephrosis, lithiasis, infections and a small percentage are associated with neoplasia. The case of a patient with macroscopic hematuria, palpable mass and pain is presented. Tumor in a horseshoe kidney was detected and patient underwent preoperative embolization and nephrectomy with isthmectomy. Follow-up progression has been satisfactory. El riñón en herradura es la alteración de la fusión renal más frecuente. La mayor parte de los pacientes portadores de ésta patología son asintomáticos. Aquellos pacientes que presentan síntomas, están relacionados con hidronefrosis, litiasis, infecciones y en un menor porcentaje por neoplasias. Se presenta el caso de una paciente quien debutó con hematuria macroscópica, masa palpable y dolor. Se demostró la presencia de una tumoración presente en un riñón en herradura, por lo cual fue sometida a embolización preoperatoria y a nefrectomía con istmectomía. Evolucionó de forma satisfactoria en su seguimiento. Key words: horseshoe kidney, clear cell carcinoma, Mexico. Palabras clave: Riñón en herradura, carcinoma de células claras, México. 1 Urology Service. 2 Pathology Service Centro Médico Nacional 20 de Noviembre ISSSTE, Mexico City Corresponding author: Dr. Efrén K. Yaber Gómez. Félix Cuevas Col. del Valle#540. México, D.F México. Telephone: and Rev Mex Urol 2010;70(2):

2 INTRODUCTION Described for the first time in 1521 by Jacopo Berengario da Carpi, horseshoe kidney is perhaps the most frequent alteration of kidney fusion. It consists of two kidneys joined at their lower poles by parenchymatous or fibrous tissue called isthmus. It occurs in 0.25% of the population. It is more frequent in men with a 2:1 male/female ratio. 1,2 It occurs in the embryo between the 4th and 6th weeks of gestation after the ureteral yolk has entered into the renal blastema. This usually occurs before rotation and the renal pelvises are facing forward. The cause has not been completely identified but it has been suggested that alterations in the position of the umbilical or common iliac artery is the cause, altering the ascent and rotation of the kidneys which end up situated in the lower part of the abdomen. The position of the superior mesenteric artery has also been implicated as a cause of ascent obstruction once the kidneys are fused. The calyces are normal in number but atypical in orientation and blood supply varies widely. 1,3 Almost a third of patients presenting with this congenital malformation remain asymptomatic. Clinical manifestations become apparent as a consequence of hydronephrosis, lithiasis, infection and less frequently, tumor. 1,4 Different abnormalities are associated with horseshoe kidney but carcinoma has been reported in only 123 patients. 5 Forty-seven percent of these cases correspond to clear cell carcinoma, 28% to urothelial carcinoma, 20% to Wilms tumor and 5% to sarcomas. 6 Carcinoma incidence in horseshoe kidney has not been described in the literature but the observation has been made that it is higher than that of the rest of the population. 7 Survival in patients with this type of tumor is related to stage and histopathological grade. 8 Knowledge of preoperative neoplastic localization, extension and vasculature is indispensible as part of the diagnostic approach to horseshoe kidney tumors so that complete resection of the tumor can be carried out without unnecessarily removing functional tissue. Angiography or helical computed tomography (CT) angiography is essential for planning surgical approach due to the great variability of blood vessels. 10 CLINICAL CASE The patient is a 69-year-old woman with no important pathologies in her medical history or drug addictions. Her disease began 6 months before diagnosis and was characterized by pain, a palpable mass at the left flank, macroscopic hematuria and an approximate 5 kg weight loss. Based on these data diagnostic approach was made at a regional general hospital, documenting left kidney tumor, and she was referred to the Centro Médico Nacional 20 de Noviembre for continued study. Upon admittance the patient had hemoglobin 13.3 g/dl, platelets 200,000, prothrombin time 11.3, glucose 74 mg/dl, BUN 12 mg/dl and creatinine 0.7 mg/dl. Liver function tests were normal and chest X-ray revealed no metastasis. Simple contrasted abdominopelvic computed axial tomography (CAT) was done that showed a heterogeneous lesion with calcifications that was dependent on the left kidney with fusion of both kidneys at the lower pole (Images 1 and 2). Preoperative planning included angiogram that showed the left renal artery with multiple ramifications towards the tumor site (Images 3 and 3a). Embolization was carried out on the left renal artery using platinum peripheral coils. Afterwards the patient underwent left radical nephrectomy and isthmectomy by way of midline incision finding a 17 x 10 x 6.5 cm tumor that was dependent on the left kidney and was joined to the right kidney by the isthmus (Image 4). The patient s postoperative progression was satisfactory and she was released 72 hours after surgery. Histopathological study reported a light yellow multinodular tumor with fibrous septae and scant necrotic areas in 80% of the renal parenchyma. Microscopic diagnosis was clear cell carcinoma with 10% necrosis, Fuhrman II, no invasion of the capsule, no lesions at the surgical margins and renal hilum vessels were free from malignant neoplasia. There was no tumor activity in 9 lymph nodes. DISCUSSION Renal cell carcinoma is the most frequent malignant neoplasia associated with horseshoe kidney. The tumor can be localized at any part of the kidney, however, its most common site is the isthmus. 13 In any type of kidney tumor the use of abdominopelvic CAT is essential to determine the presence of contralateral neoplasia, the degree of tumor infiltration into the perirenal fat, adjacent organs and vena cava and/or renal vein involvement. Recently helical tomography with 3D reconstruction has provided important information as to anatomical detail and it is recommended as the only diagnostic imaging study Rev Mex Urol 2010;70(2):

3 Yaber-Gómez EK, et al. Clear cell carcinoma in horseshoe kidney: a case report and literature review Image 1. Abdominopelvic CT showing horseshoe kidney with tumor mass. Image 2. 3D-reconstruction showing fusion of both kidneys and tumor in the left kidney. Image 3. Selective angiogram of left renal artery. Image 3a. Selective embolization of left renal artery. In patients with tumors in ectopic kidneys, routine use of angiography for embolization is not recommended except in patients with important hematuria who are not candidates for radical surgery or in patients who will undergo resection of paravertebral metastatic activity.15 Nevertheless, in patients with horseshoe kidney tumor this study is recommended because vascularity is widely variable. Angiograph facilitates the preservation of the non-affected renal unit and follows the principle of preserving as many nephrons as possible. For these reasons angiograph and selective embolization using platinum peripheral coils were carried out on this patient.10,2,13 Surgical approach was through the midline in order to have better vascular control and to have better exposure of the isthmus for isthmectomy.16,13,6,17 The objective of kidney tumor postoperative surveillance is the early identification of metastatic activity. This activity is related to positive surgical Rev Mex Urol 2010;70(2):

4 Image 4. Macroscopic view of kidney showing tumor in the inferior pole. margins, multifocality and histological grade The possibility of surgical resection is considered the best therapeutic option in cases of resectable disease and is the preferred option in solitary metastatic lesions. In clinical studies early tumor recurrence diagnosis can improve systemic therapy efficacy if tumor load is small. Local recurrence is rare (1.8%) when surgical margins are negative. Contralateral recurrence is also rare (1.2%). When recurrence possibility is low, chest X-ray and ultrasound are appropriate but when risk is moderate or high, chest and abdominal CAT is the study of choice. 21 Recurrence possibility and prognosis are dependent on the same factors established for kidney cancer in non-fused kidneys and can be classified as anatomical, histological, clinical and molecular. In addition prognostic recurrence scales have been developed as well as molecular markers for follow-up. 22 Follow-up of the present case was carried out through chest and abdominal CAT since histological grade was Fuhrman II and tumor size was important. No tumor activity was detected at 7-month follow-up. CONCLUSIONS Horseshoe kidney is the most frequent fusion abnormality and is predominant in men. It is important to underline the fact that a third of patients remain asymptomatic and clinical manifestation is a result of hydronephrosis, lithiasis, infection and less frequently, tumor, as in the present case. There are fewer than 150 cases of malignant tumor in horseshoe kidney reported in the literature making this an important report for the Mexican literature. The use of current imaging studies such as helical tomography with 3D reconstruction is an important diagnostic tool because it greatly facilitates diagnostic approach and the making of surgical decisions. It is important to stress the use of angiographic studies and to evaluate the use of embolization especially in patients with low cardiovascular reserve who do not tolerate hemodynamic changes secondary to hemorrhage. BIBLIOGRAPHY 1. Glenn JF. Analysis of 51 patients with horseshoe kidney. N Eng J Med 1959;261: Jones L, Reeves M, Wingo S. Malignant tumor in a horseshoe kidney. Urol J 2007;4(1), Hohenfellner M. Tumor in the horseshoe kidney: clinical implications and review of embryogenesis. J Urol 1992;147(4): Boatman DL, Cornell SH, Kölln CP. The arterial supply of horseshoe kidney. Am J Roentgenol Radium Ther Nucl Med 1971;113(3): Rev Mex Urol 2010;70(2):

5 5. Blackard CE, Mellinger GT. Cancer in a horseshoe kidney. A report of two cases. Arch Surg 1968;97(4): Otero García JM, Maldonado Alcaráz E, López Samano VA. Carcinoma de células claras en riñón en herradura. Descripción de un caso y revisión de la literatura. Gac Med Mex 2005;141(4); Ben Slama R. Renal adenocarcinoma in horseshoe kidney. Tunis Med 2006;84(12): Vázquez S, Calahorra-Rodríguez A. Patología tumoral en el riñón en herradura. Actas Urol Esp 1994;18: Kim TH. Renal Cell Carcinoma in a Horseshoe Kidney and Preoperative Superselective Renal Artery Embolization: A Case Report. Korean J Radiol 2005;6(3) Arce Y, Trias I, Santaularia JM, Antonio Rosales. Aplicación clínica de las actuales clasificaciones del cáncer renal. Actas Urol Esp 2006;30: Lee CT, Katz J, Fearn PA. Mode of presentation of renal cell carcinoma provides prognostic information. Urol Oncol 2002;7(4): Patard JJ, Leray E, Rodriguez A. Correlation between symptom graduation, tumor characteristics and survival in renal cell carcinoma. Eur Urol 2003;44(2): Rubio-Briones J, Regalado-Pareja R, Sanchez-Martin F. Incidence of tumoural pathology in horseshoe kydneys. Eur Urol 1998;33(2): Lee CT, Hilton S, Russo P. Renal mass within a horseshoe kidney: preoperative evaluation with three-dimensional helical computed tomography. Urology 2001; 57(1): Bakal CW, Cynamon J, Lakritz PS, et al. Value of preoperative renal artery embolization in reducing blood transfusion requirements during nephrectomy for renal cell carcinoma. J Vasc Interv Radiol 1993;4(6): T Oida, T Souma, H Doi, and S Hida Hinyokika Kiyo. Acta urologica Japonica 2002;48(7): Elías R, Rico D, Rodríguez R, et al. Adenocarcinoma renal en el istmo de un riñón en herradura. Rev Arg Urol 2004;69(4): Itano NB, Blute ML, Spotts B, Zincke H. Outcome of isolated renal fossa recurrence after nephrectomy. J Urol 2000;164(2): Sandhu SS, Symes A, A Hern R. Surgical excision of isolated renalbed recurrence after radical nephrectomy for renal cell carcinoma. BJU Int 2005;95(4): Bani-Hani AH, Leibovich BC, Lohse CM. Associations with contralateral recurrence following nephrectomy for renal cell carcinoma using a cohort of 2,352 patients. J Urol 2005;173(2): Patard JJ, Leray E, Rioux-Leclercq N. Prognostic value of histological subtypes in renal cell carcinoma: a multicenter experience. J Clin Oncol ;23(12): Lam JS, Shvarts O, Leppert JT. Renal cell carcinoma 2005: new frontiers in staging, prognostication and targeted molecular therapy. J Urol 2005;173(6): Rev Mex Urol 2010;70(2):