A case of angiomyolipoma originating from polycystic kidney with horseshoe kidney

A case of renal angiomyolipoma originating from polycystic kidney with horseshoe kidney is reported. A 32-year-old woman visited our hospital with the complaint of proteinuria. with computerized tomographic scan and further examinations the patient was diagnosed as having renal angiomyolipoma with tuberous sclerosis. The tumor originated from a polycystic horseshoe kidney. Three weeks later, she complained of right flank pain and was diagnosed with spontaneous rupture of the angiomyolipoma. Right heminephrectomy was performed and histological examination confirmed the preoperative diagnosis. Some discussion is made on the characteristics and treatment of renal angiomyolipoma, and the statistics on renal diseases with tuberous sclerosis in Japan are presented.     

The limit in differential diagnosis of renal angiomyolipoma from renal cell carcinoma on computerized tomography: a case report

Recent reports have described that the accurate preoperative diagnosis of renal angiomyolipoma is possible with computerized tomography (CT). There are, however, some cases of angiomyolipoma which are indistinguishable from renal cell carcinoma on CT. Herein we report a case of renal angiomyolipoma which CT failed to reveal preoperatively. In a 71-year-old Japanese male, a left renal tumor was incidentally suspected on excretory urogram. Then CT demonstrated a homogeneous mass in the upper part of the left kidney, 6 cm in diameter, which showed a positive attenuation coefficient, slightly denser than renal parenchyma. Selective left renal arteriogram showed hypervascularity without aneurysmal formation or arteriovenous fistula. These radiologic findings strongly suggested renal cell carcinoma and radical left nephrectomy was performed. Histological examination confirmed angiomyolipoma composed mainly of smooth muscle with thick-walled vessels and immature fat cells. We reviewed the literature and discussed the computerized tomographic-pathologic correlation of angiomyolipoma.     

Renal angiomyolipoma: report of 2 cases of tuberous sclerosis in female siblings

Two cases of renal angiomyolipomas in female siblings associated with tuberous sclerosis were reported. The first case was a 47-year-old housewife who visited the hospital because of severe right flank pain. Bilateral renal angiomyolipomas and hemorrhage in the right angiomyolipoma were recognized by computerized tomography, ultrasonography and renal angiography. After 2 months she had another episode of left flank pain and was diagnosed to have hemorrhage in the left renal tumor. The pain remissed by conservative treatment. Bilateral nephrectomy and hemodialysis would be necessary if she were to have a third attack. The second case was her 36-year-old sister with the chief complaint of left flank pain and genital bleeding. CT and renal angiography suggested a large angiomyolipoma in her left kidney and a small one in her right kidney. Left nephrectomy, right partial nephrectomy and hysterectomy were performed to prevent intratumoral hemorrhage. The histopathological diagnosis was angiomyolipoma of kidneys, uterus and lymphnodes of right renal hilus. Although pre-operative differentiation of angiomyolipoma from renal cell carcinoma has been difficult, recently diagnosis has become possible by CT, ultra-sonography and angiography.     

转移性恶性肾血管平滑肌脂肪瘤三例报告

目的 探讨转移性恶性肾血管平滑肌脂肪瘤(AML)的诊断和治疗. 方法转移性恶性AML患者3例.男1例,女2例.年龄分别为36、55和37岁.主诉为持续性患侧腰痛1～6个月.2例B超、CT检查发现患肾实性占位.1例B超见肾肿块内不均匀高回声光团,CT测得脂肪负值信号.2例行根治性肾切除术,1例行肾切除术.3例均未放化疗. 结果 肿块切面灰红、灰黄,质软.病理镜下观察:2例瘤细胞呈上皮样,核有异型,伴坏死;1例大多区域为梭形平滑肌,脂肪细胞和血管混杂生长,部分区域细胞呈上皮样.免疫组化结果 均为VM(+),HMIM5(+),Melan-A(+),S-100(-),CK(-).3例病理诊断均为恶性肾AML,2例为上皮样型,1例为经典型、部分上皮样型.1例术后7年出现肺转移,行肺叶切除加清扫,随访12年无瘤生存.1例术后6年复发,行肿块切除术;术后lO年发现肝、后腹膜转移,再行肿块部分切除,随访12年带瘤生存.1例术后4个月出现肝、后腹膜转移,行肿块部分切除,3个月后失访. 结论 恶性肾AML罕见,诊断依靠病理和免疫组化检查.治疗方法无特殊.     

Spontaneous rupture of renal angiomyolipoma

We report a case of renal angiomyolipoma with retroperitoneal hemorrhage treated by enucleation in a 47 year-old male. The mass in the anterior side of the left kidney, revealed by sonography and CT, was diagnosed as angiomyolipoma with a retroperitoneal hematoma caused by its spontaneous rupture. Removal of hematoma and enucleation of the tumor were performed after the diagnosis. Diagnosis and treatment of ruptured renal angiomyolipoma are discussed.     

原发性肾窦肿瘤三例报告

目的 总结原发性肾窦肿瘤的临床特点及治疗方法.方法 原发性肾窦肿瘤患者3例.女2例,男1例.平均年龄40(33～55)岁.临床表现为腰痛伴血尿1例,单纯腰部疼痛2例.肿瘤位于右侧2例,左侧1例.直径平均5.5(3.5～8.5)cm.3例均经行手术治疗. 结果行右肾窦肿瘤探查术1例,术中病理提示良性肿瘤,单纯切除肿瘤,病理报告为血管平滑肌瘤,随访3年未见复发.1例术中因肿瘤出血及肾盂破坏严重,行右肾切除,病理报告为血管平滑肌脂肪瘤,随访10个月未见复发.1例因肿瘤包绕肾动静脉,局部严重黏连,行左肾切除术,病理报告为脂肪瘤,随访4年未见肿瘤复发.结论 原发性肾窦肿瘤临床罕见,多为良性,易误诊为肾盂肿瘤,CT、MRI、IVU检查有助于鉴别;良性肿瘤直径<4 cm且无症状者可密切随访,有症状或直径>4 cm者可行肿瘤切除;肿瘤累及肾蒂并严重破坏肾盂者可行单纯肾切除;恶性肿瘤应行根治性肾切除.     

A Case of Angiomyolipoma Diagnosed by Thin Slice Non-Enhanced CT and Needle Biopsy

We report a case of a 52-year-old female with two intrarenal tumors in the left kidney and a contralateral non-functioning kidney. Two renal cell carcinomas were suspected on 10 mm and 5 mm thick slices of computed tomography (CT), while angiography could not exclude a diagnosis of angiomyolipoma. Thin section (2 mm thick) non-enhanced CT detected negative attenuation values (indicative of fat) within both tumors, but these values were higher than the value for normal fat tissue. Negative attenuation values within the tumor using non-enhanced thin sections are thought to be essential for a CT diagnosis of angiomyoplipoma, especially when angiomyolipoma is difficult to distinguish from renal cell carcinoma. We performed an ultrasonography-guided needle biopsy of the tumor and pathological examination confirmed the diagnosis of angiomyolipoma, consisting of a rich angiomyomatous element and small amounts of mature adipose tissue.     

A case of renal angiomyolipoma with marked cystic degeneration and pseudoaneurysm

A 59-year-old woman consulted our hospital for a left renal mass which she had been aware of for 4 years. The tumor was in the lower portion of the left kidney. It was a cystic tumor whose wall was enhanced on computed tomography and magnetic resonance imaging. The lesion 3.0 cm in diameter, which was enhanced equally to the aorta, was found in a part of the wall. It was thought to be a pseudoaneurysm by renal angiography. We suspected a cystic renal cell carcinoma because of the plural feeding arteries and tumor staining, and performed left total nephrectomy. Pathological diagnosis was angiomyolipoma with few fat components. To our knowledge only 3 previous cases of renal angiomyolipoma with marked cystic degeneration have been reported in Japan. In all 3 cases, it was difficult in preoperative diagnosis to distinguish angiomyolipoma with cystic degeneration from cystic renal cell carcinoma complicated cyst. Moreover, this is the first reported case of renal angiomyolipoma with marked cystic degeneration and pseudoaneurysm.     

Renal cell carcinoma in a horseshoe kidney with abdominal aortic aneurysm: a case report

We herein report a case of renal cell carcinoma in a horseshoe kidney with an abdominal aortic aneurysm in a 69-year-old man. Radiological examinations showed a left renal tumor, horseshoe kidney and abdominal aortic aneurysm. We performed a left radical nephrectomy with the division of the isthmus and artificial graft through an abdominal transperitoneal approach. Histological findings revealed clear cell type renal cell carcinoma without invasion of the capsule or renal pelvis. Only 31 cases of renal cell carcinoma in a horseshoe kidney have been reported in Japan, and our case is the 32nd. No case with abdominal aortic aneurysm has been reported previously. We assume that abdominal aortic aneurysm was associated with renal cell carcinoma by chance in the horseshoe kidney in this case. The arterial and venous supplies vary from case to case. We emphasize that arteriography and venography are very important preoperative procedures.     

A case of renal cell carcinoma in a horseshoe kidney]

We report a 68-year-old man with renal cell carcinoma in a horseshoe kidney. Abdominal computed tomography revealed a left renal tumor in a horseshoe kidney, and we carried out left radical nephrectomy. Histopathological diagnosis was renal cell carcinoma. Only 19 cases of renal cell carcinoma in a horseshoe kidney have been reported in Japan, and our case was the 20th.     

Solitary fibrous tumor of the adrenal gland with renal cell carcinoma and angiomyolipoma at the same time; a case report

Solitary fibrous tumor (SFT) is a neoplasm of pleura and its occurrence in the retroperitoneal space is rare. We report a case of SFT of the adrenal gland associated with ipsilateral renal cell carcinoma (RCC) and angiomyolipoma (AML). A 48-year-old woman was referred to our hospital for a left renal AML. Computed tomography (CT) in our hospital showed a left adrenal mass (25 x 20 mm). Because the adrenal tumor was nonfunctioning, she was followed at outpatient clinic. Four years later, CT showed an increase in the left adrenal tumor size (42 x 30 mm) and a left RCC. Left adrenectomy and partial nephrectomy for RCC and AML were simultaneously performed. Histological examination revealed adrenal SFT and clear cell carcinoma and AML of the kidney. We present a brief review on histological characteristics of retroperitoneal SFT and its occurrence in the adrenal grand region.     

Two cases of renal angiomyolipoma

Two cases of renal angiomyolipoma without tuberous sclerosis are reported. The first case was of a 35-year-old man with complaints of right upper abdominal and right flank pain. Preoperative diagnosis was right renal angiomyolipoma. Thoracoabdominal radical nephrectomy and lymphadenectomy were performed. The pathological diagnosis was renal angiomyolipoma with lymph node involvement. The second case was of a 46-year-old woman whose left renal mass had been accidentally found by ultrasound study. Preoperative diagnosis was left renal angiomyolipoma. This tumor was enucleated from the left kidney through flank incision.     

Therapeutic embolization of renal angiomyolipoma: a case report

A case of bilateral renal angiomyolipoma without tuberous sclerosis is reported. A 49-year-old woman was admitted to the general practitioner with a sudden onset of severe left flank pain. An excretory urogram and ultrasonogram revealed an enlargement of the left kidney. She was subsequently referred to our clinic for further investigation and treatment. Computed tomographic scan and magnetic resonance imaging using Tl-weighted image showed several tumors with a fatty, dense area in the bilateral kidney. An arteriogram demonstrated a hypervascular renal mass with aneurysms in her left kidney. Diagnosis of bilateral renal angiomyolipoma was confirmed by percutaneous needle biopsy. Superselective embolization of the tumor was successfully performed, preserving normal renal tissue. Gelatin sponges containing Carboquone (CQ sponge) were used as embolic material. Angiomyolipoma has become relatively easy to diagnose by CT, ultrasound, MRI and so on. However, there are some cases of angiomyolipoma which are indistinguishable from renal cell carcinoma using these modes of testing. Therefore, in selecting a conservative management, we indicated that percutaneous biopsy or open biopsy should be done to confirm the results of the above procedures. Moreover, therapeutic embolization for angiomyolipoma was concluded to be very useful.     

Rapidly growing renal angiomyolipoma during pregnancy with tumor thrombus into the inferior vena cava: a case report

A case of renal angiomyolipoma rapidly growing during pregnancy with tumor thrombus into the inferior vena cava is reported. Angiomyolipoma in a 31-year-old woman was incidentally found by ultrasonography. CT scan revealed a fat-containing tumor in the right kidney with 4 cm in diameter. The patient was followed at outpatient clinic without any treatment. Fifteen months later, the post-delivery follow-up CT scan showed that tumor size had grown up to 11 cm in diameter. Although laparoscopic right nephrectomy was tried, open transperitoneal right nephrectomy was performed because the tumor thrombus extending into the inferior vena cava was found during the laparoscopic procedure. Pathological diagnosis was angiomyolipoma of the kidney. There are several reports that indicate angiomyolipoma grows rapidly during pregnancy. Our case demonstrates that careful follow-up is required for angiomyolipoma in women with possible conception and delivery.     

Transarterial renal embolization for enlarged horseshoe kidney

A 56-year-old Japanese man was admitted to our hospital with abdominal fullness in June 2006. He had been diagnosed as having a horseshoe kidney by computed tomography in January 2002. At that time, renal dysfunction (serum creatine: 2.0 mg/dl) was detected. Even after hemodialysis was started for end-stage renal failure in March 2006, his abdominal fullness became progressively worse. CT scanning showed a markedly enlarged horseshoe kidney. Transarterial embolization (TAE) was performed via the right renal arteries with 14 platinum microcoils; the left renal arteries were not embolized in order to preserve sufficient parenchyma and a urine volume of more than 1,000 ml daily. Two years after TAE, a decrease in the size of the left kidney was noted along with the right kidney. However, urine output was still more than 1,000 ml daily. It is possible that one kidney compressed the contralateral kidney, resulting in enlargement of both components of the horseshoe kidney and renal dysfunction. TAE may be a useful option for obstructive uropathy in patients with horseshoe kidney, which has conventionally been treated surgically.     

Tuberous sclerosis, associated with renal cell carcinoma and angiomyolipoma, in a patient who developed endstage renal failure after nephrectomy

We report a case of tuberous sclerosis (TSC) associated with renal cell carcinoma and angiomyolipoma in a patient, who developed endstage renal failure that required hemodialysis after nephrectomy. A 37-year-old woman with TSC was admitted for further investigation of bilateral renal masses detected by computed tomography (CT). Angiography revealed a tumor stain (4 cm in diameter) in the medial portion of the right kidney. Because renal cell carcinoma (RCC) was strongly suspected, right nephrectomy was performed. Her serum creatinine level was already increased, moderately, at 2.4 mg/dl, before the right nephrectomy. Her renal function deteriorated quickly (in 1&frac; years) after the right nephrectomy, and hemodialysis was introduced the next year. The histological findings of the resected right kidney revealed marked intimal thickening of the intralobular arteries. These findings suggested that the renal function loss was not only caused by the nephron mass reduction due to the nephrectomy but was also caused by nephrosclerosis. Though most patients with TSC die before developing endstage renal failure, this patient is currently receiving maintenance hemodialysis and has been followed for 3 years with no recurrence of RCC in the left kidney.     

Horseshoe kidney in an infant associated with ureteropelvic junction and ureterovesical junction obstructions

A 4-month old baby was seen in August, 1982 because of abdominal distension. The findings of the physical examination were normal except for a man's fist sized mass in the left upper abdomen. IVP revealed left nonvisualizing kidney. CT scans revealed a large mass in the left abdomen with low density contents and renal scintigraphy revealed a horseshoe kidney. The tentative diagnosis was horseshoe kidney associated with hydronephrosis due to ureteropelvic junction obstruction. Left pyeloplasty was performed in September 1982 and postoperative X-ray examinations revealed left ureterovesical junction obstruction. Left ureteroneocystostomy was performed in October, 1982 and postoperative course was uneventful. Horseshoe kidney in infants is rare in the Japanese literature and our case is quite unique in that horseshoe kidney is associated with ureteropelvic junction and ureterovesical junction obstruction.     

Mucinous adenocarcinoma of the renal pelvis in the horseshoe kidney: a case report

A 41-year-old man with macroscopic hematuria and abdominal fullness was referred to our hospital. Computed tomography (CT) revealed a left renal pelvic tumor in the horseshoe kidney. We performed left heminephrectomy and ureterectomy. The pathological diagnosis was the mucinous adenocarcinoma in the renal pelvis. He received postoperative adjuvant chemotherapy (CAP therapy). He died of retroperitoneal recurrence 8 months postoperatively. In the literature we found 95 cases of primary adenocarcinoma in the renal pelvis including our case.     

Giant renal calculus in a horseshoe kidney presenting as an abdominal lump

We report a case of giant renal calculus of 14 × 10.5 × 9 cm in size and weighing 790 g, in a horseshoe kidney, along with two large calculi on the opposite side. Patient presented with unusual complaint of an abdominal lump only. Physical examination revealed a stony hard mass on the left side of the abdomen, extending from the subcostal region to just above the iliac crest. A giant renal calculus on the left and two large calculi on the right side of a horseshoe kidney were diagnosed on computed tomography (CT) scan. Percutaneous nephrolithotomy, followed after 5 days by open pyelolithotomy was done for the stones on the right and left side, respectively. Analysis revealed a calcium phosphate stone. This case is worth reporting as it was not only the 8th heaviest renal stone reported in the English literature, but also the first giant stone to be reported in a horseshoe kidney.     

Diagnosis and treatment of renal angiomyolipoma

We have hitherto reported 6 cases of renal angiomyolipoma. Recently, we encountered two more such cases. Case 1 is a 34-year-old woman with fever as the chief complaint. DIP revealed a tumor mass in the right upper pelvic pole. This mass showed a strong echo level on ECHO and adipose tissue of low density on CT scan. Therefore, the patient was diagnosed as having renal angiomyolipoma. Since liposarcoma was not ruled out by the examination of frozen sections during operation, nephrectomy was performed. Case 2 is a 40-year-old woman. Diagnosed as having bilateral renal angiomyolipoma, she underwent right nephrectomy 14 years ago. Two years ago, she had heavy hematuria, and had embolization of the left renal artery. She has had no bleeding since the embolization. We are of the view that ECHO and CT can are very useful for diagnosis of renal angiomyolipoma, and embolization for heavy hematuria, a complication, should be performed first of all.