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Mixed Granular-Clear Cell Carcinoma of The Kidney
B. Raofi, M.D. †, M.J. Naderi, M.D.
†, S. Saki, M.D. †, S.A. Rooholamini, M.D.*,
From the Departments of Radiology † Kern Medical Center, Bakersfield, CA * Olive View-UCLA Medical Center, Sylmar, CA
CASE SUMMARY A 56-year-old woman with blunt abdominal trauma was brought in to the emergency room after an accident. The patient was complaining of back pain and distended abdomen. There was a drop in hemoglobin and hematocrit. Computed tomography of the abdomen and an abdominal aortography were done
FIGURE 1. (A and B) Computed tomographic images of the midabdomen with intravenous contrast material injection reveal a large heterogeneously enhanced mass arising from the left kidney, displacing the stomach anteriorly.
The patient is a 56-year-old woman who was brought in to the hospital emergency room after being struck by a motorcycle. The patient complained of back pain and distended abdomen. The routine plain radiographic studies were unremarkable, except for fractures of the right knee, tibia and fibula. Upon admission, hemoglobin was 10 gm/dl and hematocrit 31%. Therefore, computed tomography of the abdomen and pelvis was obtained. The hemoglobin and hematocrit continued to drop with the hematocrit less than 20%. The pulse rate was greater than 145 per minute. Abdominal aortography and selective left renal arteriography were done. The patient continued to complain of back pain and distended abdomen. Hence, an exploratory laparotomy was performed.
FIGURE 2. (A, B, C, D, E, F, G and H) Computed tomographic image of the abdomen with contrast enhancement (A) reveals a large mass arising from the left kidney, displacing the rest of the kidney anteromedially and the stomach to the right. The mass is heterogeneously enhanced. There is a break (arrowhead) through the posteroinferior aspect of the tumor capsule. Perinephric hemorrhage and stranding are seen. Note the large hemorrhage in the left infrarenal space (B). The follow-up CT scan images done two days later (C and D) show progressive intra and retroperitoneal bleeding. Note the perisplenic hemorrhage and the left pleural effusion (C) and subcutaneous soft tissue swelling in the left flank (D). A midstream abdominal aortogram (E) shows a large smooth indentation upon the left lateral aspect of the distal abdominal aorta with some displacement to the right. Selective left renal arteriogram in the early phase (F) demonstrates a large hypervascular tumor with neovascularity, supplied by the left renal artery. Note the tumor stain in the intermediate (G) and late phases (H) of the angiogram.
RADIOLOGIC FINDINGS Computed tomography of the abdomen and pelvis reveals a large retroperitoneal mass arising from the posterior aspect of the left kidney. The mass is demarcated by a capsule from the normal renal parenchyma. The mass has displaced the uninvolved upper pole and a portion of the mid left kidney anteriorly and to the right. The normal portion of the left kidney abuts the posterior aspect of the anterior abdominal wall. There is displacement of the aorta and the stomach to the right by the mass. The spleen is intrinsically intact, but is displaced anteriorly and anterosuperiorly by the mass. The splenic artery and vein, the celiac axis, and the superior mesenteric artery and vein are displaced anteriorly and to the right by the mass. There is a break through the capsule of the tumor at its posteroinferior aspect causing perinephric hematoma. The mass is heterogeneous in density. There are areas of high density within the mass on the unenhanced images, suggesting hemorrhages. Hematomas are also seen in the left perirenal and pararenal spaces. Following intravenous administration of contrast material, scattered areas of enhancement become visible within the mass. These are consistent with the tumor parenchyma. The left adrenal gland is not identified. Two days later, a follow-up CT scan showed progressive perinephric and retroperitoneal hemorrhage on the left side. Left pleural effusion and hemoperitoneum surrounding the spleen are detected at this time. Subcutaneous soft tissue swelling is seen in the left flank. A midstream abdominal aortogram shows a large smooth indentation upon the left lateral aspect of the distal abdominal aorta with some displacement to the right. Selective left renal arteriogram demonstrates a large hypervascular mass with neovascularity supplied by the left renal artery. There is abnormal tumor staining of the mass on the late phase of the angiogram.
DIAGNOSIS
Left renal cell carcinoma, mixed granular-clear cell
type. At surgery, a large retroperitoneal mass was found arising from the left kidney, displacing the adjacent organs to the right side. The mass extended medially beyond the midline, superiorly to the spleen and pancreas, inferiorly to the pelvic inlet, and laterally to the left flank. A multi-layered smooth capsule surrounded the mass. The mass was highly vascular. The mass measured 35x20 cm in diameter and represented a renal tumor with intratumoral hematoma. Hemoperitoneum was also present. The left adrenal gland was not clearly identified. After left radical nephrectomy, open reduction of the right knee, tibial and fibular fractures and internal fixation were done by orthopedic surgeons.
PATHOLOGIC FINDINGS A left radical nephrectomy was done with resection of a portion of the ureter. The specimen measured 24.5x16.5x10 cm and weighed 2530 gm. There were large areas of hemorrhage, necrosis, acute inflammation and thick-walled blood vessels within the neoplasm. The neoplasm was confined by a capsule. The tumor occupied the major portion of the kidney with a small residual normal renal parenchyma left. The neoplasm measured 22.5x16.5x9.5 cm. Multiple well-circumscribed nodules were identified within the mass. No ureteral involvement, lymphadenopathy, or renal vein involvement were found at surgery. The left adrenal gland was not identified. The perinephric adipose tissue revealed multiple areas of hemorrhage. The histological diagnosis was renal cell carcinoma, mixed granular-clear cell type. The neoplasm was Fuhrman grade 2 (2/4). The postoperative course was uneventful and the patient was discharged to return for follow-up care in the orthopedic and oncology clinics.
DISCUSSION Renal cell carcinoma accounts for 3% of all malignant tumors in the adult population. Ninety to 95% of the malignant neoplasms of the kidneys are renal cell carcinoma. Renal cell carcinomas have diverse clinical manifestations and are often asymptomatic at the early stage of the disease. The tumor arises from the proximal renal tubular epithelium. There are sporadic or nonhereditary and hereditary forms of renal cell carcinoma. The hereditary forms of renal cell carcinoma are encountered in von Hippel-Lindau disease, hereditary papillary renal carcinoma (HPRC), familial renal oncocytoma (FRO) associated with Birt-Hogg-Dube syndrome (BHDS), and hereditary renal carcinoma (HRC). The incidence of renal cell carcinoma has been increasing, especially among African-Americans. Renal cell carcinoma is the sixth leading cause of cancer death. The 5-year survival rate is 66% for stage I, 64% for stage II, 42% for stage III, and 11% for stage IV. Renal cell carcinoma is two times more common in men than in women, and it is more common among white Americans of Scandinavian descent than those of Asian ancestry. With the exception of hereditary forms, which are seen in younger individuals, the disease is more commonly seen in the 4th to 6th decades of life. In most of the cases the clinical manifestation is occult. The classic triad of flank pain, hematuria, and flank mass is seen only in 10% of patients and reflects advanced disease. Twenty to thirty percent of patients are asymptomatic and the disease is discovered radiologically. The most common presentations are hematuria (40%), flank pain (40%), flank mass (25%), weight loss (33%), fever (20%), high blood pressure (20%), hypercalcemia (5%), malaise, and night sweats. Left sided varicocele is seen in 2% of males, as a result of obstruction of the gonadal vein. Erythrocytosis, and hypercalcemia may be present. Paraneoplastic syndromes may be seen. Polyneuromyopathy, anemia, fever, cachexia, amyloidosis, weight loss, elevated erythrocyte sedimentation rate, and hypertension may be seen. Thirty percent of patients with renal cell carcinoma present with metastases. The tumor metastasizes to the lungs (75%), soft tissues (36%), bones (20%), liver (18%), skin (8%), and brain (8%). Smoking and obesity are considered among the risk factors. Hypertension, estrogen therapy, analgesic abuse, acquired cystic kidney disease in patients with chronic renal insufficiency, renal dialysis, tuberous sclerosis, renal transplantation with immunosuppressive therapy, and von Hippel-Lindau disease are considered other risk factors for the development of renal cell carcinoma. There are 5 histologic subtypes of renal cell carcinoma. These are clear cell (75%), chromophilic (15%), chromophobic (5%), oncocytoma (3%), and collecting duct (2%). There are four stages of renal cell carcinoma (table I).
Table I – Staging of Renal Cell Carcinoma
CONCLUSION An incidentally discovered large renal cell tumor was found in a patient who had sustained severe blunt abdominal trauma and multiple lower extremity fractures, after being struck by a motorcycle. Intratumoral and perinephric hemorrhages were found. The radiologic features of the concomitant conditions of the renal cell carcinoma and its possible traumatic blast with intratumoral and perinephric hemorrhages are discussed.
REFERENCE · Sachdeva K, Makhoul I, Javeed M et al. Renal Cell Carcinoma. www.emedicine.com/ med/topic2002.htm, January 20, 2005
ACKNOWLEDGMENT The authors express their thanks to Paul Beers and Shervin Ghamghamy for their valuable technical assistance and to René Retana for his secretarial and computer work in the preparation of this manuscript.
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