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.     

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: 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.     

A case of xanthogranulomatous pyelonephritis presenting with the flank subcutaneous mass

A 51-year-old female exhibited fever, left flank pain and left flank mass in March, 1993. Drip infusion pyelography (DIP) revealed a non-functioning left kidney with shadows of calculi, and abdominal computerized tomography (CT) showed renal calculi and multilocular cystic lesions in the left kidney extending through the perinephric space into the mass on the left flank. Percutaneous nephrostomy and percutaneous drainage were performed, followed by left nephrectomy. Histopathological findings revealed xanthogranulomatous pyelonephritis. There have been a few case reports of xanthogranulomatous pyelonephritis forming nephrocutaneous fistula in the back.     

Angiomyolipoma with a caval thrombus

We report the case of a female patient presenting with flank pain. Abdominal ultrasound revealed a tumor of 8 cm in diameter. After abdominal computerized tomography, the tumor was classified as angiomyolipoma with a tumor thrombus in the inferior vena cava. After nephrectomy, the diagnosis was confirmed histologically. To our knowledge, this is the 11th case of a renal angiomyolipoma extending into the vena cava.     

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.     

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.     

A case report of spontaneous rupture of renal cell carcinoma difficult to be distinguished from angiomyolipoma

A 56-year-old woman was admitted with right flank pain as the chief complaint without any trauma. Abdominal and chest computed tomography (CT scan) demonstrated with low-density area in a retroperitoneal hematoma suspected of spontaneous rupture of angiomyolipoma and hematothorax. Superselective transarterial embolization and drainage of the thoracic cavity were performed. Three months later a right renal tumor in perirenal hematoma was revealed by abdominal CT scan and suspected renal cell carcinoma. Right nephrectomy was performed. Histopathology revealed renal cell carcinoma. Including this case, 31 cases of spontaneous rupture of renal cell carcinoma have been reported in the Japanese literature.     

Two cases of leiomyoma of the kidney]

Renal leiomyomas large enough to be clinically diagnosed are extremely rare. We review 30 cases of clinically diagnosed renal leiomyoma from the literature in Japan, including our 2 new cases. Case 1: In a 52-year-old man with no symptoms a renal mass was found accidentally on an ultrasonogram. CT scan showed a mass with a cystic area at the upper pole of the right kidney. Angiogram showed a hypovascular mass. Case 2: CT scan revealed a cystic mass and angiogram showed an avascular mass at the upper pole of the left kidney in a 19-year-old man having gross hematuria and left flank pain. Transperitoneal nephrectomy was done in both cases. Histologically each tumor was composed of monotonous proliferation of spindle shaped cells without atypia, which showed intense immunoreactivity for alpha-smooth muscle actin. The diagnosis of benign leiomyoma was made in each case. In a review of 30 cases, we found that renal leiomyomas occur most often in female (77%), between decades 2 and 5 of life (median: 46 years). On the angiogram it appears most often as an avascular or hypovascular mass. CT scan shows cystic or mixed solid/cystic or solid lesion, occasionally with calcification. Preoperative diagnosis is extremely difficult to be made. Histologically, fibroma, angiomyolipoma, congenital mesoblastic nephroma and leiomyosarcoma should be differentiated.     

A case of renal angiomyolipoma treated with partial nephrectomy. Diagnostic value of ultrasonography and computed tomography

A 62-year-old woman with renal angiomyolipoma is reported. Ultrasonogram revealed a hyperechoic renal mass and computed tomography showed a fatty density area in the tumor. Preoperative diagnosis of renal angiomyolipoma was made and was confirmed by frozen section during the operation. Partial nephrectomy was performed subsequently. Renal angiomyolipoma is known by its fat content causing high echogenicity in the ultrasonogram and fatty density area in computed tomography. Renal angiomyolipoma, therefore, could be suspected by this combination of findings and conservative surgery should be planned preoperatively.     

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.     

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.     

Retroperitoneal hemorrhage due to spontaneous rupture of adrenal myelolipoma

BACKGROUND: A very rare case of retroperitoneal bleeding due to spontaneous rupture of a large adrenal myelolipoma in a 62-year-old woman is reported. METHODS/RESULTS: She consulted the emergency room of the Nagano Red Cross Hospital with a complaint of sudden left flank pain. A computerized tomography (CT) scan revealed a tumor with areas of fat density and hematoma in the left retroperitoneal space. After her general condition improved, an operation was performed. The tumor strongly adhered to the left kidney and a left nephrectomy with the tumor was curative. Histologic diagnosis was adrenal myelolipoma. No blood transfusion was required. CONCLUSIONS: A CT scan is very useful in the pre-operative diagnosis of adrenal myelolipoma with retroperitoneal hemorrhage. Watch and wait treatments before operation and nephrectomy with adhered tumor are safe and curative.     

Two cases of metastatic renal tumor

Two cases of metastatic renal tumor are reported, one in a 78-year-old male who had undergone total gastrectomy for gastric carcinoma, and the other in a 45-year-old female who had undergone hysterectomy for cervical carcinoma of the uterus. The chief complaint was flank pain and nephrectomy was performed in both cases. Histopathological examination of the removed specimen revealed metastatic renal tumor the primary site of which was probably the stomach in the first case and cervix uteri in the second case. The literature on metastatic renal tumors in Japan is reviewed.     

Laparoscopic nephrectomy for patients with infected nonfunctioning kidney due to ureteropelvic junction stenosis : report of two cases

We report two cases of infected nonfunctioning kidney due to ureteropelvic junction (UPJ) stenosis treated by laparoscopic surgery. Patient 1 : A 78-year-old woman was referred to our clinic complaining of left flank pain and high fever up. Asymptomatic UPJ stenosis with mild hydronephrosis was diagnosed 9 years ago at our clinic. Therefore we followed her up with no treatment. Computed tomography (CT) scan revealed left severe hydronephrosis. Antibiotic chemotherapy was performed without percutaneous nephrostomy. Laparoscopic nephrectomy was performed with the resected renal size of 10 cm in greatest diameter and the operative time of 262 min. The pathological diagnosis was atrophic kidney with pyelonephritis. Patient 2 : A 28-year-old man was referred to our clinic complaining of left flank pain and high fever up. CT scan revealed a left severe hydronephrosis due to UPJ stenosis. Following percutaneous nephrostomy,antibiotic chemotherapy was performed. Laparoscopic nephrectomy was performed with the resected renal size of 6 cm in greatest diameter and the operative time of 140 min. The pathological diagnosis was atrophic kidney with pyelonephritis. Laparoscopic nephrectomy for infected nonfunctioning kidney due to UPJ stenosis is feasible and safe. With dense perinephric adhesions and failure to progress,we should consider early open conversion to reduce morbidity.     

A case of spontaneous disappearance of pulmonary metastasis of renal cell carcinoma following nephrectomy

A 76-year-old man was admitted to our hospital on October 3, 1988 complaining of general fatigue and left flank pain. A large movable mass was palpable in his left flank. Intravenous pyelography and computerized tomography confirmed left renal tumor. Chest X-ray showed a coin lesion in the left lung. Left nephrectomy was performed on October 14, 1988. Histopathological diagnosis was adenocarcinoma of clear cell type. Chest X-rays, on the postoperative eleventh day, showed disappearance of the pulmonary coin lesion which was seen preoperatively. Recurrence of pulmonary metastasis has not been seen for 14 months after operation.     

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.     

A case of renal hemangiopericytoma]

A 27-year-old female was referred with an abdominal mass. Examination revealed a non tender firm mass in the right flank and hypertension (200/100 mmHg). An angiomyolipoma was suspected on computed tomography and arteriography and a radical nephrectomy was performed. On cut section, the kidney was occupied by a well-capsulated, grayish tumor measuring 10 x 9 cm. Pathological diagnosis was a renal hemangiopericytoma without involvement of the capsule. Her blood pressure has normalized after the operation. She has no evidence of recurrence after 18 months' follow-up.     

Spontaneous rupture of a left renal artery aneurysm: a case report

Rupture of a renal artery aneurysm is an acute surgical event associated with high mortality. We report a case of retroperitoneal hemorrhage from a spontaneously ruptured renal artery aneurysm. A 73-year-old woman complained of left flank and abdominal pain. She consulted our department and left retroperitoneal hemorrhage was recognized by abdominal computerized tomography. Selective left renal arteriography revealed a saccular aneurysm arising from the ventral branch of the renal artery, and did not show extravasation of contrast material from the aneurysm. Since it was difficult to remove the aneurysm with preservation of the involved renal unit, we performed left nephrectomy.