Malignant epithelioid angiomyolipoma ('sarcoma ex angiomyolipoma') of the kidney: a case report and review of the literature

Malignant epithelioid angiomyolipoma is a recently described rare tumor of the kidney. Its existence has been questioned, however, on the basis of incomplete evidence of malignant behavior, the absence of an associated classic angiomyolipoma component, or the absence of immunoreactivity for HMB-45 in some cases. We describe a case that was HMB-45-positive and arose in association with a classic angiomyolipoma. The patient was treated with a partial nephrectomy. Three years later, she developed rapidly enlarging liver nodules. A fine-needle aspiration of the liver confirmed the presence of pleomorphic epithelioid cells morphologically and immunohistochemically identical to those comprising the primary renal tumor. After two cycles of treatment with doxorubicin, there was a 50% reduction in the size of the tumors with marked improvement in performance status. We believe this case confirms the existence of a malignant epithelioid angiomyolipoma.     

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.     

Massive epithelioid angiomyolipoma of the kidney in a young girl

We report the case of a huge right renal tumor in a 17-year-old girl. Absence of fat on preoperative magnetic resonance imaging suggested renal cell carcinoma, and surgery was performed. Pathologic evaluation with HMB-45 immunohistochemical staining confirmed the diagnosis of epithelioid angiomyolipoma. The tumor consisted predominantly of epithelioid cells, and it could easily be misidentified as a renal cell carcinoma due to the paucity of the fat component. Previous reports have suggested that epithelioid angiomyolipomas have the potential to be malignant, and thus regular postoperative surveillance is recommended. Our patient had no signs of recurrence at her most recent follow up, 12 months after surgery.     

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.     

乏脂性肾血管平滑肌脂肪瘤患者9例临床分析

目的提高对乏脂性肾血管平滑肌脂肪瘤的认识,探讨其诊治方法。方法报告9例经本院收治的乏脂性肾血管平滑肌脂肪瘤的临床资料,回顾性分析其临床特征及影像学表现。9例患者均无明显临床症状。术前超声检查均提示低回声占位,占位可见血流信号。CT检查8例平扫期未见脂肪样低密度。肿瘤动脉期呈现明显强化,CT值最高可达100Hu,实质期肿瘤持续均匀强化。术前诊断为肾脏恶性肿瘤。另1例患者平扫可见局部点状低密度灶,增强扫描点状低密度灶无强化。分析所有患者临床资料并检索Pubmed和CBM数据库,对该疾病相关文献进行复习。结果所有患者均接受手术治疗。1例因患者原因施行根治性肾切除术,余8例患者接受保留肾单位的肾部分切除术。其中6例行腹腔镜肾部分切除术,2例行开放肾部分切除术。术后病理提示肾血管平滑肌脂肪瘤。所有患者均获随访9~36个月,未见肿瘤复发。结论乏脂性肾血管平滑肌脂肪瘤临床少见,对病灶CT的细微分析能够给诊断带来帮助,对于乏脂性肾血管平滑肌脂肪瘤特别是体积较小的肿瘤应该力争行保留肾单位的手术,以减少盲目肾切除造成的过度治疗。     

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.     

Renal epithelioid angiomyolipoma with malignant behavior

The patient was a 22-year-old woman with a history of tuberous sclerosis who complained of abdominal distention. Computed tomography showed bilateral renal masses with minimal fat tissue that were unlike classical angiomyolipoma (AML), as well as enlargement of the paraaortic lymph nodes, pleural effusion, and ascites, similar to other advanced malignant neoplasms such as renal cell carcinoma and renal sarcoma. We performed a left nephrectomy to relieve the symptoms. A pathological examination revealed that the tumor was composed of polygonal epithelioid cells positive for both αSMA and HMB-45, as well as spindle-shaped cells, while immunoreactivity proved the tumor to be an eAML, a subtype of AML with malignant potential. Although the patient was scheduled for postoperative adjuvant chemotherapy, her condition worsened and she died on postoperative day 24 before receiving chemotherapy. We discuss this case with reference to previous reports.     

腹腔镜治疗肾错构瘤并肾静脉及下腔静脉瘤栓1例并文献复习

目的：探讨全腹腔镜治疗肾错构瘤并肾静脉及下腔静脉瘤栓的可行性分析。方法：回顾性分析1例腹腔镜治疗肾错构瘤并肾静脉及下腔静脉瘤栓患者的临床资料。患者,女,26岁,体检时发现右肾占位,B超示右肾窦内可见5．1cm×2．7cm高回声占位,边界欠规则,内见血流。CT示右肾盂旁可见一不规则团块状混杂密度影,大小为4．5cm×2．9cm×1．9cm,可见脂肪成分,最低密度-40HU;病变软组织部分明显强化,增强前后CT值分别为31HU和97HU,病变主要位于肾窦,部分延伸至肾静脉及腔静脉内。检索Pubmed和CBM数据库相关文献进行复习。结果：患者在全麻下行腹腔镜右肾切除及肾静脉、下腔静脉取栓术,瘤栓进入下腔静脉0．6cm。病理诊断右肾错构瘤。术后随访6个月无肿瘤复发和转移。结论：肾错构瘤并。肾静脉及下腔静脉瘤栓临床罕见,对选择性病例行腹腔镜肾切除并行肾静脉及下腔静脉取栓术安全可行。     

Non-ischemic tumor enucleation for renal angiomyolipoma: a case report

A case of renal angiomyolipoma, successfully treated with non-ischemic tumor enucleation, is reported. A 16-year-old Japanese female visited another hospital with a chief complaint of general fatigue. She was diagnosed with angiomyolipoma of the right kidney, 7 cm in the long axis, which developed exteriorly at the lower pole. A nephrectomy was recommended. The patient visited us for a second opinion. We judged that nephron-sparing surgery was applicable to this case. The patient underwent non-ischemic tumor enucleation using a microwave tissue coagulator via retroperitoneal approach. The patient was discharged from our hospital 9 days after the surgery. Since a preoperative diagnosis with renal angiomyolipoma can be obtained relatively easily, maximum efforts for nephron-sparing surgery should be made.     

Coincidental angiomyolipoma and renal cell carcinoma--report of 1 case and review of literature

A patient underwent simple enucleation for a suspected angiomyolipoma in the right kidney and histopathology revealed a mixture of renal cell carcinoma with angiomyolipoma. To our knowledge only 1 other case of a combination of a renomedullary tumor has been reported previously.     

后腹腔镜肾根治性切除、开放腔静脉取栓术治疗巨大肾错构瘤合并下腔静脉瘤栓（附1例报告并文献复习）

目的：报告1例巨大肾错构瘤合并腔静脉瘤栓患者的诊治经过,探讨其治疗选择．为罕见病例的治疗提供借鉴和指导。方法：汇总分析1例巨大肾错构瘤合并腔静脉瘤栓患者的临床资料及诊治经过,查阅文献进行分析讨论。结果：结合病史、体检及辅助检查结果,考虑该患者肾错构瘤合并腔静脉瘤栓诊断明确,行后腹腔镜左肾根治性切除、开放腔静脉瘤栓取出术,术后患者恢复良好出院,病理证实为左肾错构瘤,瘤栓为脂肪成分。结论：肾错构瘤合并肾静脉、腔静脉瘤栓为泌尿外科罕见病例,对瘤体巨大的特殊病例,后腹腔镜肾根治性切除、开放瘤栓取出术是可选术式。在确保手术安全的前提下,能最大程度减少创伤,改善疾病预后及患者生活质量。     

Renal angiomyolipoma: two varieties

Two cases of symptomatic renal angiomyolipoma are presented, and their distinctive clinical, radiologic, and histologic features are discussed. In the first case the scout film demonstrated radiolucency of the tumor which helped to distinguish it from carcinoma. In the second case, which was associated with tuberous sclerosis, the renal lesion was large and unifocal, and the local lymph nodal involvement with tumor raised the possibility of malignant transformation.     

Renal angiomyolipoma with malignant transformation, simultaneous occurrence with malignity and other complex clinical situations

Renal angiomyolipoma is a benign kidney tumor, which is characterized by the presence of mature or immature fat tissue, thick-walled blood vessels, and smooth muscles. However, there is a rare possibility of transformation to a malignancy. This transformation could be toward sarcoma. And also angiomyolipoma could be associated with renal adenocarcinoma in the same kidney. The aim of this study is to show the association of angiomyolipoma with complex clinical situations such as malign transformation, simultaneous occurrence with adenocarcinoma, bilateral tumors with tuberous sclerosis or Wunderlich Syndrome. Findings of clinical presentation, pathological evaluation, urography, ultrasonography, and computerized tomography of 19 patients who were diagnosed angiomyolipoma in our clinic during 1994–2003 were examined retrospectively. Our records indicated that radical nephrectomy was performed in three cases of angiomyolipoma in which the differential diagnosis from adenocarcinoma could not be made and in another case of angiomyolipoma, which was associated with adenocarcinoma. Simple nephrectomy was performed in four symptomatic angiomyolipoma cases with tumor size larger than 4 cm and partial nephrectomy in another case. In one case, renal angiomyolipoma was associated with adenocarcinoma in the same kidney. In one case, post-operative recurrence of angiomyolipoma developed 7 months after nephrectomy. This patient was diagnosed low-grade leiomyosarcoma. Angiomyolipoma is regarded as a benign tumor of the kidney; however, it may also show aggressive behaviors and rarely transform to sarcoma. It may also exist in the same kidney along with adenocarcinoma or sarcoma.     

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.     

Angiomyolipoma with cavoatrial extension

In this paper, we report a case of angiomyolipoma with cavoatrial extension in a 55-yr-old woman. Multislice computed tomography revealed a hypodense mass extending into the right atrium through the right renal vein and inferior vena cava without a renal mass. The patient underwent surgery, and right atrial mass was resected. Pathologic evaluation revealed a tumor consisting of mature adipose tissue, smooth muscle and vessels consistent with angiomyolipoma. Our case reminds us of the rare possibility that angiomyolipoma, which is classified as a benign tumor, may invade the inferior vena cava or right atrium.     

肾错构瘤的诊断与治疗(附13例报告)

目的探讨肾错构瘤的诊断和治疗。方法本组13例患者手术治疗,4例行肿瘤剜除术,6例行患肾部分切除术,1例患者因急腹症就诊探查确诊切除患肾,1例恶变者行肾癌根治术,1例保守治疗。结果所有手术患者术后恢复顺利,术后随访6个月~2年,未见肿瘤复发。结论通过B超、CT等检查,大部分肾错构瘤的患者都可明确诊断,少数瘤体破裂大出血而诊断未明者,宜剖腹探查。手术是治疗肾错构瘤的主要方法,特别是对于肾错构瘤体积大于4cm者应尽早手术治疗。对少数非手术治疗者,应严密观察。手术宜尽量保留有功能的肾组织,恶变者行肾癌根治术。     

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.     

恶性倾向肾巨大血管平滑肌脂肪瘤伴下腔静脉瘤栓1例报告并文献复习

目的：报告1例巨大肾血管平滑肌脂肪瘤合并腔静脉瘤栓病例。通过文献复习,探讨恶性倾向肾血管平滑肌脂肪瘤（renalangiomyolipoma,AML）的诊断与治疗。方法：报道和分析1例巨大AML伴下腔静脉瘤栓的病例资料,并通过查阅文献,分析和探讨具有恶性倾向的AML的临床表现、临床与病理学特性、诊断与鉴别诊断及外科治疗。结果：患者以双下肢肿胀就诊并行CT及MRI检查,提示AML伴肝后下腔静脉瘤栓（MayoIII）形成。行根治性肾切除和腔静脉瘤栓取出术,术后恢复良好。组织病理学证实为AML及静脉内肿瘤栓子。结论：散发的或结节性硬化症（tuberous sclerosis complex,TSC）相关的AML通常为间叶组织来源的良性肿瘤。罕见的,当瘤体侵犯肾静脉或下腔静脉并形成瘤栓时具有侵袭性。这种恶性潜能被认为是上皮样血管平滑肌脂肪瘤（EAMLs）的生物学特性,手术切除肿瘤并取出瘤栓是可能治疗这类AML的惟一有效方法。 我们的研究强调了严密的术前评估、精细的术中操作、审慎的组织病理学检查及密切的术后随访,以求获得更好的预后。     

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.     

Infiltrating recurrent renal angiomyolipoma with fatal outcome

Renal angiomyolipomas are considered to be benign mesenchymal tumors despite histological findings often remarkable for cellular pleomorphism and mitoses. We report a renal angiomyolipoma in a 49-year-old white woman. Surgical treatment was followed by massive recurrence with perforation of the colon and invasion of the diaphragm. The patient died despite surgical debulking and radiation therapy. A review of the literature revealed 1 case of massive recurrence following partial resection of the tumor and 2 in which the tumor showed marked local invasion. We believe that patients with renal angiomyolipoma should be monitored carefully for aggressive tissue infiltration and recurrent tumor.