Hepatic angiomyolipoma: report of 8 cases and review of literature

Hepatic angiomyolipoma is an extremely rare mesenchymal tumor. To study the clinical feature, diagnosis, treatment and prognosis of the rare presentation, the medical records of 8 patients presenting to Cancer Institute and Hospital, CAMS with the diagnosis of hepatic AML from January 1989 to December 2000 were reviewed retrospectively. There were five female and three male, with age ranged from 25 to 59 years (mean 39.6 years). Five tumors located in the right lobe of the liver, and three in the left lobe. Ultrasonography and computed tomography were performed in all cases. Magnetic resonance imaging and angiography were done in some cases. The myoid cells in all tumors showed over-expression of HMB-45. All patients had hepatic resection. Except for one patient died of heart failure after surgery, others survived well to date without tumor. In conclusion, comprehensive imaging features can reveal the characteristic components of hepatic angiomyolipoma. Reaction for HMB-45 can be used to confirm the diagnosis. Surgical resection is an effective treatment of hepatic angiomyolipoma.     

Hepatic angiomyolipoma developing during the follow-up of ulcerative colitis: Report of a case and review of the literature

Hepatic angiomyolipoma is a rare tumor composed of spindle-shaped and epithelioid smooth muscle cells, adipose tissue, and proliferating blood vessels. We report the first documented case of this tumor developing in a patient with ulcerative colitis. A solitary tumor (7.5×7.5×7cm) was detected in the left lateral segment of the liver and a left hepatic lobectomy was performed. The diagnosis of angiomyolipoma was confirmed by a pathological examination. We also review the literature on previously reported cases of hepatic angiomyolipoma.     

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.     

肝血管平滑肌脂肪瘤八例报告

目的：探讨肝血管平滑肌脂肪瘤（AML）的临床表现、诊断、治疗和预后。方法：对1989-2000年间我院手术和病理学检查证实的8例肝AML临床资料进行回顾性分析研究。结果：男3例，女5例。发病年龄25-59岁（平均39.6岁）。肝左叶3例，右叶5例。8例均行超声和CT检查。2例行MRI检查。2例行肝动脉造影。免疫组织化学染色：肿瘤平滑肌样细胞表达HMB-45阳性。8例均手术切除。除1例死于术后心功能衰竭，其余7例随访9个月至12年，未见肿瘤复发。结论：综合影像学检查有助于该肿瘤特征的显示和术前正确诊断。肿瘤平滑肌样细胞表达HMB45阳性，是诊断肝AML的可靠依据。手术切除是肝血管平滑肌脂肪瘤的有效治疗手段。     

Angiomyolipoma of the liver: Its clinical and pathological diversity

Eighty cases (82 tumors) of hepatic angiomyolipoma (AML) from the world literature were reviewed, including our 8 cases. The tumors occurred predominantly in females (female: male ratio, 56∶24). Only six patients (7.5%) had associated tuberous sclerosis. The majority of the tumors (95%) occurred in non-cirrhotic, almost normal liver. The tumors usually presented as a hyperechoic mass on ultrasound (US) examination, a hypodense mass on computed tomography (CT) scan, and hyperintense mass on both T1- and T2-weighted magnetic resonance (MR) imagings. However, the imaging features of the tumor were occasionally variable according to the content of fat tissue component, and the tumors could not be clearly differentiated from other hepatic tumors, particularly hepatocellular carcinoma (HCC), based on the imaging features alone. Tumor location was recorded in 81 tumors; 44 were located in the right lobe, 30 in the left, 5 in the caudate, and 2 in both lobes of the liver. The tumor diameter ranged from 0.3 to 36cm (mean, 8.0±7.0cm). Macroscopically, the tumors usually had no fibrous capsule. The cut surface was yellow to dark brown depending on the amount of fat tissue or blood vessels. The histologic features were quite variable and occasionally included atypical and/or pleomorphic cells, which could lead to the erroneous diagnosis of a variety of benign and malignant tumors, including lipoma, leiomyoma, HCC, hepatoblastoma, leiomyosarcoma, and malignant fibrous histiocytoma. Extramedullary hematopoiesis was found in 33% of the tumors. Reactivity of the tumor cells of the liver with HMB-45, a melanoma-specific antibody, can accurately establish the diagnosis of AML.     

Myolipoma arising from the renal capsule: a case report

A 53-year-old female visited us with an asymptomatic renal mass, which was incidentally found on abdominal ultrasonography (US). US showed a heterogeneous renal mass measuring 5 cm in diameter. Computed tomorraphy and magnetic resonance imaging showed that the tumor was mixed with muscle and fat tissue, faintly enhanced, and located at the lower portion of the left kidney. Although renal angiomyolipoma (AML) was suspected from these findings, we could not rule out a malignant tumor. Therefore, she underwent a laparoscopic partial nephrectomy. Histopathological examination revealed the tumor composed of smooth muscle and mature adipose tissue without cytological atypia arising from the renal capsule. The tumor contained no AML-like blood vessels, and was negative for HMB-45 staining. The final diagnosis was myolipoma arising from the renal capsule. Myolipoma is a rare benign neoplasm in the soft tissue of retroperitoneum and abdominal cavity. Since it is difficult to distinguish myolipoma from other benign and malignant tumors with fat tissue only by imaging studies, a surgical approach should be considered.     

肾上皮样血管平滑肌脂肪瘤诊治探讨

目的 提高肾上皮样血管平滑肌脂肪瘤(EAML)的诊治水平.方法 肾EAML患者5例.男3例,女2例.平均年龄46(26～64)岁.患侧腰部胀痛3例,体检偶然发现2例.肿瘤直径2.9～10.1 cm.B超和CT检查诊断为血管平滑肌脂肪瘤2例,肾癌、肾上腺肿瘤、肝癌各1例.行肾肿瘤剜除术3例,肾楔形切除术1例,肾切除术1例.结果 病理报告:肿瘤由不同比例的异常厚壁血管、平滑肌和脂肪组织构成.上皮样细胞胞质浓密嗜酸性或透亮,有嗜酸性颗粒,并有丰富的血管,上皮样细胞簇常围绕血管.组织学形态表现为圆形、多边形和梭形上皮样细胞灶性分布于脂肪瘤中,上皮样瘤细胞免疫组化标记SMA、Vimentin、HMB45表达阳性.5例病理诊断均为肾EAML.随访2～12个月,未见局部复发及远处转移.结果 肾EAML由厚壁血管、脂肪和平滑肌细胞混合组成,上皮样细胞簇常围绕血管.确诊依赖于临床、影像学及病理学检查相结合,免疫组织化学染色有助于诊断及鉴别诊断.早期诊断及完整切除肿瘤是治疗关键.     

肝脏血管平滑肌脂肪瘤的诊断与治疗

目的 总结肝脏血管平滑肌脂肪瘤的临床、影像及病理特征,探讨提高术前确诊率的途径.方法 回顾性分析2000年至2007年第二军医大学东方肝胆外科医院收治并经病理检查证实的73例肝脏血管平滑肌脂肪瘤患者的影像学特点及诊治经验.根据影像学特点对患者进行分型并进行相应治疗.结果 术前确诊7例.B超检查诊断率为0,CT检查诊断率为13%(7/56),MRI检查诊断率为6%(2/33).根据影像学检查结果分型:血管瘤型6例,脂肪瘤型17例,平滑肌瘤型4例,混合型46例.手术切除72例,RFA治疗1例.24例患者出现肺部感染、胸腔和腹腔积液、轻度肝功能损害.术后免疫组织化学检测阳性率依次为:HMB45＞平滑肌肌动蛋白＞波形蛋白＞增殖细胞核抗原＞CD34＞癌胚抗原蛋白＞CD18＞CD19＞p53.1例术后因复发死亡.结论 肝且庄血管平滑肌脂肪瘤易误诊,影像学分型有助于术前正确诊断,治疗上应积极手术切除.     

5例肝血管平滑肌脂肪瘤的诊断与治疗分析

目的 探讨肝血管平滑肌脂肪瘤（HAML）的临床、影像学、病理学特点及治疗方法。方法 回顾性分析我院2016年1月至2020年10月间收治的5例肝血管平滑肌脂肪瘤病例,并结合相关文献对本病进行探讨。结果 5例肝血管平滑肌脂肪瘤病人均经外科手术治疗,预后良好,所有病人术后随访期间,未见肿瘤复发及转移,无死亡病例。结论 肝血管平滑肌脂肪瘤临床罕见,常缺乏特征性临床表现,早期诊断困难。明确诊断有赖于术后病理及免疫组化结果。治疗方案首选手术治疗,其疗效确切,预后良好。     

A case of renal angiomyolipoma with tumor thrombus

A 42-year-old woman presented with left renal tumor. Computed tomography showed a left renal tumor (6 cm in diameter) and a tumor thrombus at the left renal vein, which had equal density to fat tissue. She was diagnosed with malignant tumor, and underwent radical left nephrectomy and resection of thrombus. Pathological diagnosis was angiomyolipoma with no findings of malignancy. No signs of recurrence or metastasis have been observed for 8 months after the operation.     

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.     

肝巨大血管平滑肌脂肪瘤误诊肝癌1例及文献复习

【摘要】〓目的〓探讨肝血管平滑肌脂肪瘤(HAML)的临床特点、病理及免疫组化特征、诊断及其治疗方法,旨在提高临床诊治水平。方法〓回顾性分析1例于外院误诊为肝癌而于我院经病理证实为肝巨大血管平滑肌脂肪瘤的临床表现、影像学、病理和免疫组化资料并结合文献知识复习。结果〓HAML,多好发于女性,多数无明显不适症状。组织学上,肿瘤主要由平滑肌细胞、血管及脂肪3种成分按不同比例混合而成。免疫组化染色显示:瘤细胞对HMB45、Melan-A、SMA表达阳性。结论〓HAML临床症状缺乏一定特殊性,影像学检查表现多样,术前容易误诊。诊断不明时建议可行肝穿刺活检以进一步明确性质,确诊有赖于术后病理及免疫组化。     

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.     

原发性肾窦肿瘤三例报告

目的 总结原发性肾窦肿瘤的临床特点及治疗方法.方法 原发性肾窦肿瘤患者3例.女2例,男1例.平均年龄40(33～55)岁.临床表现为腰痛伴血尿1例,单纯腰部疼痛2例.肿瘤位于右侧2例,左侧1例.直径平均5.5(3.5～8.5)cm.3例均经行手术治疗. 结果行右肾窦肿瘤探查术1例,术中病理提示良性肿瘤,单纯切除肿瘤,病理报告为血管平滑肌瘤,随访3年未见复发.1例术中因肿瘤出血及肾盂破坏严重,行右肾切除,病理报告为血管平滑肌脂肪瘤,随访10个月未见复发.1例因肿瘤包绕肾动静脉,局部严重黏连,行左肾切除术,病理报告为脂肪瘤,随访4年未见肿瘤复发.结论 原发性肾窦肿瘤临床罕见,多为良性,易误诊为肾盂肿瘤,CT、MRI、IVU检查有助于鉴别;良性肿瘤直径<4 cm且无症状者可密切随访,有症状或直径>4 cm者可行肿瘤切除;肿瘤累及肾蒂并严重破坏肾盂者可行单纯肾切除;恶性肿瘤应行根治性肾切除.     

肝脏罕见肿瘤的诊断和治疗

目的 探讨肝脏罕见肿瘤的诊断和治疗方法.方法 回顾性分析我院2005年5月至2010年1月收治的25例肝脏罕见肿瘤患者的临床病理资料.结果 25例患者中肝局灶性结节性增生6例,肝血管平滑肌瘤、肝门部神经鞘瘤、肝左叶动脉瘤、肝胆管囊腺瘤、肝错构瘤、肝胆管绒毛状腺瘤、肝弥漫性大B细胞淋巴瘤各1例,肝血管平滑肌脂肪瘤2例,肝原发间质瘤2例,肝母细胞瘤5例,肝胚胎性肉瘤3例;术前行B超检查24例,CT检查22例,MRI检查6例,仅有3例(16.7%)检查和术后病理结果一致.术前诊断和术后病理符合5例(20%).25例均行手术切除治疗,包括半肝切除术7例,肝叶切除术7例,肝段切除术9例,肿瘤局部挖除2例.肝脏良性、低度恶性肿瘤及1例弥漫性大B细胞淋巴瘤术后无复发,5例恶性肿瘤随访中3例术后复发行再次手术切除,术后随访无复发;另2例死亡,平均术后生存期4个月.结论肝脏罕见肿瘤影像学诊断率低,手术切除是主要的治疗手段,对能切除的复发性肿瘤性病变应争取再次手术切除.     

Management of Hepatic Angiomyolipoma

Preoperative diagnosis of hepatic angiomyolipoma is difficult, and the treatment for it remains controversial. The aim of this study is to review our experience in the treatment of hepatic angiomyolipoma and to propose a treatment strategy for this disease. We retrospectively collected the clinical, imaging, and pathological features of patients with hepatic angiomyolipoma. Immunohistochemical studies with antibodies for HMB-45, actin, S-100, cytokeratin, vimentin, and c-kit were performed. Treatment experience and long-term follow-up results are summarized. During a period of 9 years, 10 patients with hepatic angiomyolipoma were treated at our hospital. There was marked female predominance (nine patients). Nine patients received surgical resection without complications. One patient received nonoperative management with biopsy and follow-up. One patient died 11 months after surgery because of recurrent disease. We propose all symptomatic patients should receive surgical resection for hepatic angiomyolipoma. Conservative management with close follow-up is suggested in patients with asymptomatic tumors and meet the following criteria: (1) tumor size smaller than 5 cm, (2) angiomyolipoma proved through fine needle aspiration biopsy, (3) patients with good compliance, and (4) not a hepatitis virus carrier.     

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.     

Role of mammalian target of rapamycin inhibitor in the treatment of metastatic epithelioid angiomyolipoma: A case report

Epithelioid angiomyolipoma has malignant potential; however, no effective therapy has been established for advanced cases. A 50‐year‐old woman with a history of right nephrectomy for epithelioid angiomyolipoma was referred to our institution. Computed tomography and magnetic resonance imaging showed multiple tumors in her lung, liver and pelvic cavity. The liver and pelvic tumor specimens obtained by needle biopsy confirmed the diagnosis of epithelioid angiomyolipoma recurrence. The patient was treated with everolimus (10 mg/day). Three months later, pulmonary lesions disappeared; liver and pelvic tumors significantly shrank in size, but the pelvic tumor gradually enlarged again. We carried out surgical resection of the residual liver and pelvic cavity tumors. Although the mammalian target of rapamycin inhibitor seems to be effective for treating epithelioid angiomyolipoma, its long‐term effects remain unknown. Thus, aggressive administration of a multidisciplinary treatment including molecular target therapy and surgical resection is required to improve the prognosis of epithelioid angiomyolipoma.     

Clinico‐pathological correlation of hepatic angiomyolipoma: a series of 23 resection cases

Background

Angiomyolipomas are rare neoplasms of mesenchymal origin and are derived from perivascular epithelioid cells. They usually develop in the kidney and rarely in the liver. Due to their rarity, most hepatic angiomyolipomas have been misinterpreted as hepatocellular carcinoma (HCC) or other hypervascular liver tumours on imaging studies. We aimed to assess the clinico‐pathological correlation of hepatic angiomyolipoma.

Methods

We identified 23 patients with hepatic angiomyolipoma through an institutional database search.

Results

Of 5680 cases of primary liver tumours, 23 (0.4%) had angiomyolipomas (mean age, 43.6 ± 12.4 years; 16 female patients). Hepatitis B virus infection was noted in four patients, whereas a liver mass was incidentally detected on routine health screening in 13 patients. The preoperative diagnoses, before liver biopsy, included HCC in 14, angiomyolipoma in six, focal nodular hyperplasia in two and hepatic adenoma in one patient. Eventually, the preoperative diagnoses were changed to HCC in 12 and hepatic angiomyolipoma in 11 patients. The tumour size was 5.3 ± 4.6 cm, and 22 patients had a single tumour. All tumours exhibited positive findings for human melanoma black?45 and smooth muscle actin staining. During a follow‐up period of 52.2 ± 23.7 months, none of the patients exhibited tumour recurrence or mortality.

Conclusions

Hepatic angiomyolipoma is a rare form of primary liver tumour and is often misdiagnosed as other hypervascular tumours. Although angiomyolipoma is benign in nature, it also has malignant potential; hence, resection is indicated if the tumour grows or malignancy cannot be excluded. Surgical resection is a definitive curative treatment of hepatic angiomyolipoma.     

Development of hepatic angiomyolipoma accompanied with focal nodular hyperplasia long after treatment of pelvic rhabdomyosarcoma

Survivors of childhood cancer have a higher risk of developing a secondary neoplasm in their lifetime. The increased risk of a second malignant neoplasm is related to treatment of the primary tumor and genetic predisposition. We describe a 19-year-old man with 2 hepatic masses, one of which was diagnosed as a hepatic angiomyolipoma and the other as focal nodular hyperplasia 14 years after the treatment of stage IV pelvic rhabdomyosarcoma. The combination of these tumors has not previously been reported in the literature.