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.     

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.     

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.     

Inferior vena cava on left side with left renal cell carcinoma

A case of unilateral inferior vena cava (IVC) on the left side with left renal cell carcinoma and two polar veins is presented. Preoperative computerized tomogram (CT) and angiogram showed IVC on the left side and tumor thrombus in the left renal vein; that information was helpful during radical nephrectomy.     

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.     

Fine needle aspiration biopsy in the diagnosis of renal angiomyolipoma

Exclusive reliance on radiographic techniques for the diagnosis of renal angiomyolipoma can lead to misdiagnosis when the histological status is atypical, computerized tomographic findings are equivocal or renal cell carcinoma coexists. We report our experience and those of others in combining fine needle aspiration biopsy and radiological imaging to identify renal angiomyolipoma. Fine needle aspiration biopsy is safe and provides accurate histological diagnosis of renal angiomyolipoma.     

Computed tomography of perirenal and pararenal inflammatory disease complicating renal calculi

The clinicopathologic features of an angiomyolipoma and a renal cell carcinoma occurring in the same patient are reported. A seventy-four-year-old woman was thought clinically and radiographically to have renal cell carcinoma of the left kidney with a metastasis to the right kidney. At surgery, however, she was found to have an angiomyolipoma in the right kidney and a renal cell carcinoma confined to the left kidney, both of which were resected with a good prognosis for survival. Light microscopy of the angiomyolipoma showed the characteristic admixture of adipose tissue, spindle cells, and hyalinized blood vessels. By electron microscopy, the spindle cells had cytoplasmic myofilaments and pinocytotic vesicles, and were surrounded by a basal lamina, consistent with a smooth muscle cell origin. This case is of interest in light of genetic influences in both of these neoplasms, and from the point of view of patient management.     

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.     

A case of angiomyolipoma in a horseshoe kidney

The patient was 52 years old. She had undergone a breast cancer operation 4 years before this visit. On computed tomography (CT), a left renal tumor in a horseshoe kidney was incidentally pointed out. CT scan showed a 1.8-cm enhanced tumor in the upper pole of the left kidney. It was hyperechoic on ultrasonography. Since renal cell carcinoma could not be excluded preoperatively, left partial nephrectomy was performed. Pathological diagnosis was a renal angiomyolipoma. The incidence of horseshoe kidney is 1 in 400. The occurrence of hydronephrosis, infection and calculous disease is not uncommon. However, a case of angiomyolipoma simultaneously with a horseshoe kidney is very rare, this being the 7th case in the literature.     

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.     

52例肾血管平滑肌脂肪瘤并自发破裂出血的诊治

目的探讨肾血管平滑肌脂肪瘤并自发破裂出血的诊治方法。方法回顾性分析52例手术治疗的肾血管平滑肌脂肪瘤并发自发破裂出血的诊治资料。术前均行B超和CT检查,46例诊断为肾血管平滑肌脂肪瘤并发自发破裂出血,6例未能排除肾细胞癌出血。6例保守治疗,11例行肾动脉栓塞术;35例急诊开放手术。结果6例经保守治疗成功无需手术,11例行肾动脉栓塞术中6例需开放手术;9例行保留肾单位肾切除手术,32例行全肾切除。全肾切除的原因:26例肿瘤广泛侵入肾脏,4例出血,2例未能排除肾细胞癌。术后病理诊断为血管平滑肌脂肪瘤并出血。结论B超和CT是诊断肾血管平滑肌脂肪瘤并出血的重要手段,治疗可以选择保守治疗、肾动脉栓塞或者急诊开放手术。     

Angiomyolipoma in the renal pelvis

A 54-year-old male presented with massive hematuria with bladder tamponade. Computed tomography and magnetic resonance imaging showed a 7.5 x 6.5 x 4.5 cm. mass centered in the left renal pelvis, which is composed of macroscopic fat and soft-tissue component. Differential diagnosis included liposarcoma, lipoma and angiomyolipoma. Urothelial carcinoma was also undeniable because urine cytology was equivocal. Subsequently, a left nephroureterectomy was performed. Pathological examinations revealed a renal angiomyolipoma protruding into the renal pelvis. We present a rare case of angiomyolipoma in the renal pelvis and review the literature concerning these unusual findings of this common neoplasm.     

A case of non-functioning adrenocortical adenoma associated with renal cell carcinoma

Left renal and left adrenal masses were incidentally found by computerized tomography (CT) in a 56-year-old man who was admitted to our hospital for treatment of upper digestive tract hemorrhage. Apparently no clinical signs suggestive of Cushing's syndrome existed. The renal tumor was diagnosed as renal cell carcinoma based on the findings on enhanced CT. 131I-adosterol uptake in the examination of adrenal scintigraphy under dexamethasone suppression was definitely increased in the left adrenal gland, although hormonal examinations of serum and urine for adrenal functions were within the normal range. Plasma adrenocorticotropic hormone (ACTH) and serum cortisol were suppressed by administration of 2 mg dexamethasone for 2 days. The left kidney was radically removed by surgery together with the left adrenal gland. Histological diagnoses were left renal cell carcinoma and adrenocortical adenoma.     

乏脂肪肾血管平滑肌脂肪瘤的CT特征分析（附40例报告）

目的通过分析40例术前诊断为肾癌的乏脂肪肾血管平滑肌脂肪瘤的CT影像学特征,以期提高肾脏肿瘤的诊断准确率,减少过度治疗。方法回顾性分析40例术前影像学诊断为肾癌的乏脂肪血管平滑肌脂肪瘤,评价CT平扫及增强后三期扫描中肿瘤的大小、位置、强化模式、强化程度、是否伴有钙化等。结果肿瘤最大径1.6～7.9（3.48±1.68）cm;38例表现为实性占位,2例表现为囊实性占位（BosniakⅣ型）;1例肿瘤伴有钙化;就强化程度而言,13例表现为轻度强化,18例表现为中度强化,9例表现为明显强化;就强化模式而言,28例表现为延迟强化,12例表现为非延迟强化;10例表现为不均匀强化,30例表现为均匀强化。结论 CT强化程度,强化模式及特殊征象在乏脂肪肾血管平滑肌脂肪瘤的诊断中具有一定价值。     

Diagnosis and management of renal angiomyolipoma

Renal angiomyolipoma (hamartoma) is an uncommon benign tumor of the kidney. Although about 400 cases of renal angiomyolipoma have been reported, it still causes difficulties in diagnosis and treatment. Until a few years ago it was almost impossible to arrive at a correct diagnosis preoperatively, and most of the cases underwent nephrectomy with the wrong diagnosis of renal carcinoma. More recently, abdominal computerized tomography and renal sonography have made it possible to reach the correct preoperative diagnosis in many cases, and thus to avoid unnecessary nephrectomies. We report on our experience with 13 cases of renal angiomyolipoma, and our conservative approach in a number of cases, which has enabled us to preserve renal tissue and function.     

肾血管平滑肌脂肪瘤12例临床分析

报告１２例肾血管平滑肌脂肪瘤，１２例均行Ｂ超检查，８例行ＩＶＵ检查，３例行肾动脉造影，９例行ＣＴ检查，１２例中手术治疗１０例（１１侧肾脏）术后均经病理检查诊断为肾血管平滑肌脂肪瘤，其中１例恶变，２例肿瘤小未予手术，１２例健康复出院，随访６个月～８年，２例分别于术后３年和７年发现对侧肾肿瘤，恶变的１例未见复发和转移，未行手术的２例瘤体未见明显变化，认为肾动脉造影，Ｂ超，ＣＴ对诊断本病具有特异性，但肿     

Extragonadal germ cell tumor coexisted with renal cell carcinoma--a case report

A patient was 31-year-old man with the chief complaint of 38 degrees C fever. He was pointed out left renal tumor by abdominal ultrasonography and computerized tomography (CT). CT revealed left infraclavicular, mediastinal and retroperitoneal lymph nodes swelling and left renal tumor. Serum alpha-fetoprotein (AFP) and beta-human chorionic gonadotropin (HCG-beta) level were elevated. The diagnosis of extragonadal germ cell tumor and left renal cell carcinoma was confirmed pathologically by infraclavicular lymph node and renal biopsy. He was treated with 4 courses of BEP regimen and interferon-alpha, cimetidine therapy for 2 weeks preoperatively. After serum tumor markers were normal level, he underwent left radical nephrectomy and left infraclavicular, mediastinal and retroperitoneal lymph node dissection. The histology of all lymph nodes was necrotic tissue, but operation was incomplete. Therefore VIP therapy was performed postoperatively. This is the first case of extragonadal germ cell tumor coexisted with renal cell carcinoma in the world.     

Fat poor renal angiomyolipoma: patient, computerized tomography and histological findings

PURPOSE: We reviewed our experience with fat poor cases of angiomyolipoma. MATERIALS AND METHODS: The records of patients with angiomyolipoma, as determined by pathological study, from 1998 to 2004 were reviewed by recording patient demographics and outcomes. Fat poor cases were defined as the failure of imaging to demonstrate fat in a lesion. Computerized tomography and histological characteristics were assessed. RESULTS: Histologically confirmed angiomyolipoma was found in 15 patients. Multiple lesions were found in 3 of 15 cases (20%). Of these 15 patients who underwent surgery 11 (73%) had unsuspected angiomyolipoma due to absent fat on computerized tomography and they underwent intervention for presumed renal cell carcinoma. Mean age +/- SD in this group was 54 +/- 15 years and 8 of 11 patients (73%) were female, of whom 4 (50%) had uterine fibroids. These lesions were found incidentally in 7 of 11 cases (64%). Operative complications developed in 2 of 11 patients (18%). Average maximal diameter on pathological evaluation was 3.2 +/- 1.3 cm (range 1.5 to 6). Nonenhanced computerized tomography was available in 7 of 11 cases, of which 3 of 7 (42%) showed hyperdense lesions and 4 of 7 (57%) showed isodense lesions. The percent of fat identified per high power field was less than 25% in 12 of 13 fat poor angiomyolipoma lesions (92%) compared to 2 of 4 classic lesions (50%) known to be angiomyolipoma before surgery (p = 0.04). CONCLUSIONS: We suggest that a general definition of fat poor angiomyolipoma should be the failure of imaging to reveal fat within a lesion, thus, making it unsuspected at surgery. A pathological definition should be less than 25% fat per high power field, which to our knowledge is a formerly undefined quantity. Not all cases are hyperdense on nonenhanced computerized tomography. These lesions cannot be reliably identified by imaging and they should be managed like all enhancing renal masses.     

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.     

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.