A case of extrarenal angiomyolipoma with intestinal localization]

The Authors present a case of extrarenal angiomyolipoma. The tumor arose from the pericolonic fat of the sigmoid colon and, increasing in size, caused intestinal obstruction by extrinsic lesion. Extrarenal angiomyolipoma is extremely rare and to our knowledge this is the first reported case arising from the colonic wall.     

肾血管平滑肌脂肪瘤2例

[例1]女,41岁,因左腰背痛6年,左上腹包块2个月入院。查体：左上腹可触及一肿块,表面光滑,质韧,上界未触及。B超提示腹膜后肿瘤。腹部X线平片：左肾轮廓消失。     

肾血管平滑肌脂肪瘤1例

患者女,31岁,常规体检超声发现左肾轻度积水,为进一步明确原因前来我院就诊。患者无腰痛、尿急、尿频等症状,无肉眼血尿,尿常规检查未见异常。超声：左肾切面形态失常,轮廓尚规则,肾门下方可见大小约59 mm×34 mm的异常强回声,形状呈类圆形,边缘尚整齐,形态规则,向肾外突出,后方回声稍增强;肾盂分离,     

Renal angiomyolipoma with liposarcomatous transformation: a case report and review of the literature

Renal angiomyolipoma is generally benign, although an uncommon subtype (epithelioid angiomyolipoma) may behave more aggressively. Sarcomatous transformation of the disease is exceedingly rare. We report the first case of a high grade round cell liposarcoma arising in a renal angiomyolipoma.     

肾上皮样血管平滑肌脂肪瘤1例报告及文献复习

目的：探讨肾上皮样血管平滑肌脂肪瘤（EAML）的临床病理特征、诊断、鉴别诊断、治疗方法及预后。方法：通过观察1例肾EAML患者的临床病理学表现与免疫组化标记并复习有关文献,讨论其组织学特征及临床生物学行为。结果：CT平扫呈略高密度实性肿块,边界清楚,病灶内无明显脂肪密度;增强扫描密度相对均匀,实质期强化程度最高,排泄期密度下降较少。镜检示肿瘤细胞弥漫分布,瘤体主要成分为上皮样细胞,细胞异型性明显,大小形状不等,核大且染色明显加深,偶有多核巨细胞,核分裂像明显。免疫组化染色显示瘤细胞HMB45＋,SMA＋,CK-。结论：病史、体检结合影像学检查能够给我们提供重要的诊断线索,组织病理学特点结合免疫组化标记有助于该疾病的确诊,治疗方法以手术切除为主,对于预后较差的患者应按照肾细胞癌标准长期随访。     

Epithelioid angiomyolipoma : a case report

A 63-year-old woman with a right renal tumor diagnosed by ultrasound, consulted our hospital in October 2008. The findings of her physical examination were unremarkable. The results of urinalysis and other routine blood tests were normal. The urinary cytology was negative for malignant cells. Dynamic computed tomography showed a right renal mass (diameter, 7.5 cm), which was enhanced in the early phase and washed out in the late phase. We initially thought that the patient had renal cell carcinoma. Therefore, laparoscopic right nephrectomy was performed in October 2008. The tumor section was found to be encapsulated, and focal hemorrhage and necrosis were observed. Histological examination of the tumor by hematoxylin-eosin staining revealed that it contained polygonal cells, eosinophilic cytoplasm and large nuclei. Immunohistochemical staining of anticytokeratin antibodies AE1/AE3 and CAM5.2 (markers for renal cell carcinoma) was negative. However, immunohistochemical staining of HMB-45, a marker for melanoma, was positive. The patient was finally diagnosed with epithelioid angiomyolipoma. She did not show any evidence of tumor recurrence for 25 months after the surgery.     

Extrarenal angiomyolipoma with spontaneous rupture: a case report

We present a very rare case of retroperitoneal extrarenal angiomyolipoma (AML) with spontaneous rupture. A 67-year-old woman without tuberous sclerosis was admitted to our hospital complaining of sudden right flank pain. We suspected right renal AML with rupture by imaging analysis, but the diagnosis was extrarenal AML by surgery and pathological examination.     

Renal cell carcinoma and angiomyolipoma in tuberous sclerosis: case report

We report a case of renal failure in a solitary kidney with manifestation of tuberous sclerosis, synchronous renal cell carcinoma and angiolipomatosis. A review of the literature disclosed the rare coexistence of these 2 tumors in tuberous sclerosis and the diagnostic difficulties in distinguishing these neoplasms.     

Renal angiomyolipoma with a tumor thrombus extending into the right atrium--a case report]

We present a case of spontaneous rupture of renal angiomyolipoma with a tumor thrombus extending from the right renal vein and inferior vena cava to the right atrium. A 41-year-old woman, previously in good health, was referred to our hospital with right flank pain. Computed tomography showed fat densities in both tumor and thrombus. Other imaging examinations also demonstrated a large right renal mass (18 cm in diameter), a long tumor thrombus (13 cm in length) and a small left renal tumor (1.5 cm in diameter). Right nephrectomy and en-bloc removal of the intra caval and intracardiac tumor thrombus were performed on cardiopulmonary bypass. It was pathologically diagnosed as an angiomyolipoma without tuberous sclerosis. At present, three years after surgery the patient is doing well, showing neither metastasis nor increase of the left renal angiomyolipoma. To our knowledge, our case seems to be the 3rd case report of renal angiomyolipoma with a tumor thrombus extending to the right atrium. We conclude that renal angiomyolipoma even with an intra cardiac tumor thrombus can be resected safely and successfully.     

Renal angiomyolipoma: report of 24 cases

A series of 24 patients with renal angiomyolipoma was reviewed. Their ages ranged from 10 to 70 years (average 41). Four cases were associated with tuberous sclerosis, 1 with spinal neurilemmoma, 1 with transitional cell carcinoma and another with renal tuberculosis. The presenting symptoms in decreasing frequencies were flank pain, mass, haematuria, fever, syncope and respiratory distress. Although it has often been said that angiomyolipomas associated with tuberous sclerosis are small and asymptomatic, all 4 such patients in this study had large symptomatic tumours. Before the advent of CT scan and ultrasonography, the pre-operative diagnostic rate for cases unassociated with tuberous sclerosis was 10% (1/10). With the combined use of these 2 modalities, the diagnostic rate increased to 60% (6/10). Most patients in this series were treated with nephrectomy. However, equally good outcomes occurred in 2 patients who received partial nephrectomies. Pre-operative diagnosis now makes it possible to consider more conservative management.     

Cerebellar hemangioblastoma with marked pleomorphism: a case report

We reported an extremely rare case of cerebellar hemangioblastoma with marked pleomorphism and reviewed the literature. A 68-year-old male presented with a one-month history of headache and vomiting. Neurological examination revealed right-sided dysmetria and truncal ataxia. Contrast-enhanced T1-weighted MR imaging revealed a heterogeneously enhancing tumor with solid and cystic components in the right cerebellum. The solid portion of the tumor was low intensity on diffusion-weighted imaging and low intensity on susceptibility-weighted imaging. ¹⁸F-fluorodeoxyglucose PET showed low uptake in the cerebellar tumor and the whole body examination was negative for malignancy. Vertebral angiogram demonstrated moderate tumor staining and no early filling veins. The patient underwent total removal of the tumor through suboccipital craniotomy. Microscopically, the solid tumor contained a cellular rich component consisting of stromal cells and a markedly pleomorphic component including atypical and multinucleated giant cells. The MIB-1 positive rate was 8.2%, which was slightly higher compared to that of hemangioblastomas. We observed strong staining for inhibin-α, aquaporin 1 and neuron specific enolase (NSE) in the tumor cells. PAX-2, cytokeratin and epithelial membrane antigen (EMA) were completely negative in the tumor cells, whereas the tumor cells demonstrated focal staining for CD10. The histological diagnosis was hemangioblastoma. Follow-up MR images showed no evidence of recurrent tumor 14 months after the resection. The study using a combination of immunohistochemical markers (e.g. inhibin-α, aquaporin 1 and PAX-2) is useful for differential diagnosis of hemangioblastoma from metastatic renal cell carcinoma.     

Renal epithelioid angiomyolipoma: 2 Cases report

Introduction

The 2004 World Health Organization Classification of Renal Neoplasms defined epithelioid angiomyolipoma as a potentially malignant mesenchymal neoplasm, characterized by a proliferation of predominantly epithelioid cells with approximately one third of patients experiencing metastases and one half of them having a history of tuberous sclerosis complex.

Extrarenal retroperitoneal epithelioid angiomyolipoma: a case report

We present a very rare case of retroperitoneal extrarenal epithelioid angio-myolipoma (AML). A 64-year-old man without tuberous sclerosis was admitted to our hospital for further examination of an abdominal mass lesion. Imaging analyses revealed a heterodensity and heterointensity retroperitoneal mass covering the left renal surface by ultrasonography, computed tomography and magnetic resonance imaging. Surgical exploration was done, and the tumor was resected with the left kidney radically. Histopathologically high cellular atypia was remarkable, and diagnosis was extrarenal epithelioid AML because of consistent immunostaining positive for HMB-45.     

Renal giant angiomyolipoma in tuberous sclerosis complex: case report and literature review

The aim of this paper is to describe a typical clinical case of tuberous sclerosis complex (Bourneville disease) and discuss controversial issues about the management of this rare condition, with a short revision of the literature. Particularly, we define which is the role of the surgeon in the treatment of this very rare condition, that should be primary approached conservatively.