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.     

Angiomyolipoma: a case report and a statistical study of 147 cases in Japanese literature

A 32-year-old housewife with gross hematuria and right flank pain had excretory urography and angiography performed. A large right renal mass with neovascularity was demonstrated. Computerized tomography also revealed a large well-demarcated renal mass with low value of HU. Right nephrectomy was done and histopathological diagnosis was angiomyolipoma weighing 1100 g. A statistical study was made on 147 cases of renal angiomyolipoma in the Japanese literature including this case. The male to female ratio was 1 to 2.9, and the average age of male and female patients was 37.8 and 39.0 years, respectively. Thirty-eight per cent of the cases were associated with tuberous sclerosis. The main clinical signs were flank pain, flank mass and hematuria. In 80% of the cases, nephrectomy was done because of the difficulty of preoperative differential diagnosis from renal cell carcinoma. Recently, CT and sonography have become a great help in diagnosing angiomyolipoma, because they can demonstrate the fat in the renal mass. Conservative or surgical treatment to save the kidney can be used more often when it becomes possible to make a clear differential diagnosis between angiomyolipoma and other malignant diseases.     

Angiomyolipoma of the kidney: report of three cases and a statistical study of 194 cases in Japan

Three cases of renal angiomyolipoma are reported. The first case was a 53-year-old female with the complaint of left flank pain. In both cases selective angiography revealed a renal tumor, and computerized tomography disclosed a renal mass with low density corresponding to the fat tissue. From several examinations these two cases were preoperatively diagnosed as renal angiomyolipoma and nephrectomy was performed. Histopathological diagnosis was renal angiomyolipoma. The third case was a 64-year-old female with the complaints of left flank pain and macrohematuria. In this case computerized tomography revealed a renal mass which did not have a low density area. The possibility of renal cell carcinoma was considered and nephrectomy was performed. Histological diagnosis was renal angiomyolipoma which was primarily composed of smooth muscle cells. The first case had a calcified lesion in the brain. But there was no evidence of tuberous sclerosis in the following two cases. Some problems in diagnosis and treatment of renal angiomyolipoma are presented, and a statistical study is made on 194 reported cases of renal angiomyolipoma in Japan.     

A case of postpartum spontaneous rupture of an angiomyolipoma

A case of postpartum spontaneous rupture of angiomyolipoma in a 27-year-old woman with the chief complaints of right flank pain and fever was reported. Physical examinations revealed an infant-head sized, hard elastic mass with a smooth surface in the right flank. Laboratory studies showed a decrease of Hb to 9.0 g/dl and Ht to 26.5%, and elevated LDH (2914 IU/l). Angiomyolipoma was suspected with ultrasonography, CT scan and angiography. Transperitoneal right nephrectomy was performed. The right kidney and tumor weighed 1,870 g. Pathological diagnosis also confirmed angiomyolipoma of the kidney. Discussion of the case and a brief review of the literature on spontaneous rupture of angiomyolipoma in the intra- and postpartum are made.     

Wunderlich's Syndrome in a Tuberous Sclerosis Patient

Spontaneous non-traumatic renal hemorrhage known as Wunderlich's syndrome is known to occur in renal angiomyolipoma and may be the first manifestation of the disease. Angiomyolipomas in tuberous sclerosis are usually bilateral and multicentric. A 25yr old female, a case of Tuberous sclerosis with bilateral renal angiomyolipomas presented with right loin pain of increasing severity. On evaluation she had acute abdomen with increasing abdominal distension. Investigations revealed large right perinephric hematoma with bilateral renal angiomyolipomas. Exploratory laparotomy and right nephrectomy was done. Spontaneous hemorrhage in renal angiomyolipoma in a case of tuberous sclerosis and management are discussed.     

Diagnosis and treatment of renal angiomyolipoma

A series of nine patients surgically treated for histologically verified renal angiomyolipoma is presented. Only one patient had extrarenal stigmata of tuberous sclerosis. No evidence of malignancy was found. In three cases a preoperative diagnosis was achieved with computed tomography (CT), use of which is recommended in evaluation of renal tumours. Six angiomyolipomas were solitary and three bilateral. Abdominal or flank pain was present in eight cases and perirenal haemorrhage in four. Solitary tumours were treated with nephrectomy. For bilateral tumour, nephrectomy and conservative renal surgery were used in two cases and bilateral nephrectomy and kidney transplantation were performed in the third case. When adipose tissue containing renal tumour is demonstrated at CT, conservative surgery should be considered.     

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.     

Angiomyolipoma of the kidney: a case report and a statistical study of 429 cases in Japan

A 40-year-old housewife was found to have a renal mass on her left side through an ultrasonogram at the hospital during a medical check up. The patient had a dull pain in her left flank intermittently for three years. She had no personal or family history of stigmas of tuberous sclerosis. The mass showed a high echogenicity on the ultrasonogram and a low density (-84HU) on the CT scan. Preoperative diagnosis was reported as renal angiomyolipoma and an enucleation of the tumor was performed with CUSA. A statistical study was done on data taken from 429 cases of renal angiomyolipoma in the Japanese literature, including our case. The male to female ratio was 1 to 2.9. Thirty three percent of the cases were associated with tuberous sclerosis. The ratio of bilateral cases to unilateral ones was 1:4. The main clinical signs were abdominal pain, abdominal mass and gross hematuria. Nephrectomy was done in 71.0% of the cases, partial nephrectomy and enucleation in 13.7% and embolization in 2.2%. Through the composition of the CT scan and the ultrasonogram, preoperative diagnosis has become possible in many cases, so recently the ratio of nephrectomy has been decreasing. We think partial nephrectomy and enucleation compose the most effective therapy for renal angiomyolipoma.     

Renal cell carcinoma associated with tuberous sclerosis: a case report

Renal angiomyolipoma associated with tuberous sclerosis is well known. On the other hand, few cases of renal cell carcinoma in connection with tuberous sclerosis have been reported. We report a patient with tuberous sclerosis whose kidney was involved with renal cell carcinoma. A 18-year-old woman was first admitted in August 1987 for evaluation of left renal tumor. Diagnosis of tuberous sclerosis was made when she was 11 years old on the basis of mental retardation, papules on her face, seizures, white leaf-shaped macules and periventricular calcifications. Computerized tomographic scan demonstrated a large mass arising from the left kidney and small masses in the right kidney. Angiography confirmed bilateral hypervascular renal tumors. On these bases, a clinical diagnosis of bilateral renal angiomyolipomas was made and surgical treatment of the left kidney was recommended because of its large size. However, her parents did not permit treatment until March, 1988. Finally, left nephrectomy of 4,750 g was performed and histological examination revealed renal cell carcinoma with clusters of spindle cells. In the literature available to us, we found twelve reports of malignant renal tumors associated with tuberous sclerosis including five renal cell carcinomas in Japan.     

Two cases of congenital hydronephrosis discovered by renal trauma

Two cases of congenital hydronephrosis discovered by renal trauma are reported. The first case was a 15-year-old girl. When she was practicing KENDO, her Hakama clung to her legs, and she tumbled. She had emergency operation for abdominal injury. At surgery, no evidence of abdominal injury was detected. Renal trauma was suspected. Right renal angiography revealed hydronephrosis. The second case was a 23-year-old girl, complaining of abdominal pain after stumbling over a block. Intravenous pyelography showed no visualization of the right kidney and CT scan showed an abnormal shadow. Retrograde pyelography and angiography revealed right hydronephrosis. Including our two cases, 47 cases of renal traumata occurring in hydronephrotic kidney in the Japanese literature were reviewed. Thirty five of these cases were males. Many were youths under the age of twenty. Various sports caused many of the renal traumata. 37 of these cases were treated by nephrectomy.     

Spontaneous rupture of a renal angiomyolipoma in pregnancy: a case report]

A 32-year-old woman in the 36th week of pregnancy was admitted to obstetrics with a complaint of left flank pain. Magnetic resonance imaging demonstrated a left perinephric hematoma before the cesarean section. Spontaneous rupture of renal angiomyolipoma was suspected by computed tomographic scan and renal angiography after the cesarean section. We attempted partial nephrectomy, but performed nephrectomy because of perinephric adhesion. This is the 11th case of spontaneous rupture of renal angiomyolipoma during pregnancy in the Japanese literature.     

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.     

Rupture of renal artery aneurysm during pregnancy

We report a case of renal artery aneurysm ruptured during pregnancy. A 32-year-old woman presented at 38th week of gestation with left flank pain. We performed a cesarean delivery for fetal distress, and found retroperitoneal hemorrhage. After delivery, she entered a pre-shock state, and abdominal computed tomography and angiography showed a left renal artery aneurysm and hemorrhage from the aneurysm. Left nephrectomy was performed, and both mother and baby were rescued. Renal artery rupture during pregnancy is a fatal event, but with quick diagnosis and adequate treatment, if possible nephron sparing surgery, most patients, including babies, can be rescued.     

A case of renal pelvic tumor due to phenacetin abuse

An 80-year-old female had been taking phenacetin-containing analgesics due to severe pain in her legs and headache caused by SMON since 1957. The total accumulated dose of phenacetin that she had taken was about 2.3 kg. She visited the department of urology in our hospital complaining of gross hematuria in May, 1987. DIP showed that bilateral kidney were atrophic and her left pyelogram was non-visualized. A solid mass was located in her renal pelvis on the CT scan. Under the diagnosis of a left renal pelvic tumor, nephrectomy was performed on her left kidney in January, 1988. Histological diagnosis was interpreted as a transitional cell carcinoma, at a grade 1 greater than 2 and interstitial nephritis was detected. She died 8 months after the operation because of lung and bone metastasis. In autopsy, interstitial nephritis of the right kidney was also recognized. Our case is the fourth report of renal pelvic tumor due to phenacetin abuse in Japan.     

Nephron-sparing tumorectomy for a large benign renal mass: A case of massive bilateral renal angiomyolipomas associated with tuberous sclerosis

A case of massive bilateral angiomyolipomas (AML) associated with tuberous sclerosis in a 33-year-old woman is reported. She was hospitalized because she had been experiencing abdominal fullness and epigastralgia. Several imaging studies revealed massive bilateral renal tumors and she was diagnosed as having renal AML associated with tuberous sclerosis. Left nephrectomy was carried out after renal arterial embolization for intratumor hemorrhage. Two years after left nephrectomy, nephron-sparing surgery (tumorectomy) for right AML was done because of an increase in the size of the right renal AML and she hoped for a future pregnancy. The left kidney with AML weighed 5700 g and the right AML weighed 1700 g. Postoperative serous creatinine did not differ from that before operation and an increase in the size of the residual tumor was not observed 8 months after operation. We consider that tumorectomy is an effective therapy in patients with a very large tumor involving a solitary kidney.     

Tuberous sclerosis with bilateral renal angiomyolipoma and wunderlich's syndrome

This case describes a 47-year-old female with tuberous sclerosis associated with bilateral giant renal angiomyolipomas. She underwent an initial renal angioembolization in August 2006. After four months, she required emergency nephrectomy due to spontaneous hemorrhage from the angiomyolipoma (Wunderlich's syndrome). Life threatening Wunderlich's syndrome requires emergency nephrectomy, however, renal preservation can be achieved by elective nephron sparing surgery.     

A case of double cancer: renal pelvic transitional cell carcinoma and lung squamous cell carcinoma

A 69-year-old woman was admitted with the chief complaint of gross hematuria and left flank pain ten years after curative right pneumonectomy. Retrograde pyelography showed a filling defect of inferior calyx. Computerized tomography revealed a solid tumor with a low density area arising from the left kidney. The tumor was demonstrated hypovascular by angiography. Left radical nephrectomy by a transabdominal approach was performed. Histological diagnosis was primary transitional cell carcinoma of the left renal pelvis largely replacing the renal parenchyma. Twenty six days after the operation she was discharged. Our case was of double cancer consistent with Warren and Gates criteria and was classified into the nonsimultaneous case according to Moertels criteria. Double cancer of the lung and renal pelvis is very rare and our case seems to be the 7th in the Japanese clinical literature.     

自发性肾包膜下血肿的影像特征与治疗方法选择

目的：探讨自发性肾包膜下血肿的诊治方法。方法：对20例自发性肾包膜下血肿临床资料进行回顾性分析。结果：20例中,行肾切除术6例、根治性肾切除2例、保留肾脏手术1例,血肿穿刺引流6例,肾动脉栓塞术1例,保守治疗4例。痊愈13例,未见血肿复发;死亡4例;失访3例。结论：自发性肾包膜下血肿多见于肾肿瘤,治疗取决于出血的严重程度和原发病。     

A case of focal xanthogranulomatous pyelonephritis difficult to differentiate from renal cell carcinoma

A 34-year-old female with left flank pain persisting for 3 months consulted us on 19 Feb, 2001. Ultrasonography (US), computerized tomography (CT), magnetic resonance imaging (MRI) and renal angiography revealed a cystic renal tumor in the upper pole of the left kidney invading the spleen, and paraaortic lymph node swelling. Left radical nephrectomy combined with splenectomy and partial diaphragmectomy was performed under a tentative diagnosis of renal cell carcinoma. However, histopathological findings revealed xanthogranulomatous pyelonephritis (XGP). XGP is a rare, severe, chronic inflammatory disease characterized by accumulation of lipid laden macrophages. XGP is classified as diffuse or focal type. Preoperative diagnosis of focal XGP is difficult because of radiological similarities to renal cell carcinoma. Our case was more difficult to diagnose because she showed few signs of inflammation and had no history of urinary tract infection or stones.     

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.