Malignant-like angiomyolipoma of the liver: report of one case and review of the literature

Frequently found in kidney, angiomyolipoma is a rare mesenchymal tumor when diagnosed in the liver and usually benign composed of proliferative blood vessels, fatty tissue and smooth muscle. We report the case of a 67-year-old woman who underwent a left hepatectomy for a 4th segment tumor unidentified after imaging and fine needle biopsy. Final anatomopathologic examination revealed an epithelio?d hepatic angiomyolipoma with signs of malignant behaviour as vascular and lymphatics embolus and invaded left portal vein thrombosis. During the subsequent 24-month follow-up, no recurrence was observed. A review of the literature found only two cases of malignant hepatic angiomyolipoma with fatal issue, however, their incidence must be underrated because of their scarcity and the difficulty of their diagnosis, which needs immunohistochemical confirmation with HMB 45 in particularly. Advances in imaging and anatomopathology in particular with the concept of PEComa (Perivascular-Epithelio?d Cell) as the unifying feature should lead to the recognition of the various variant patterns and cell types. The latter which are important for a correct diagnosis, in order to obtain reliable data about frequency, possible malignant behaviour and therefore consensus management for hepatic 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.     

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.     

Laparoscopy-assisted resection of an undiagnosed liver tumor and ascending colon cancer via mini median laparotomy: Report of a case

We describe how we resected a hepatic angiomyolipoma and ascending colon cancer synchronously via laparoscopic left lateral segmentectomy and laparoscopic right colectomy, respectively. The patient was a 72-year-old man, admitted to our hospital after a liver tumor and ascending colon cancer were detected during a general health check. Computed tomography (CT) showed a hypervascular liver tumor mimicking hepatocellular carcinoma, 2 cm in diameter, in segment 3 of the liver. The ascending colon cancer was diagnosed as T2N0M0, Stage I. The left lateral liver and right colon were mobilized laparoscopically and hepatic transaction, followed by resection and anastomosis of the colon, were performed extracorporeally through the same 7-cm upper median incision. This type of laparoscopy-assisted combined resection is useful to obtain a pathological diagnosis of the liver tumor and to remove the entire tumor in a minimally invasive and cosmetic manner.     

Giant hepatic angiomyolipoma simulating focal nodular hyperplasia

A case of hepatic angiomyolipoma occurring in a 41-year-old woman, the eighteenth case in literature, is reported herein. The radiographic imagings of the tumor simulated those of focal nodular hyperplasia; namely, the tumor was hyperechoic on ultrasound, hypodense on computed tomography, hypervascular on angiography, and exhibited an uptake of technetium (Tc)-99m phytate. The diagnosis was established after successful removal of the huge tumor from the right hepatic lobe. Histologically, the tumor was angiomyolipoma which was made up of three components, blood vessels, smooth muscle and fatty tissue. Furthermore, extramedullary hematopoiesis, which is usually seen in this tumor was confirmed. The differentiation of hepatic angiomyolipoma from FNH is also discussed.     

Giant hepatic angiomyolipoma simulating focal nodular hyperplasia

A case of hepatic angiomyolipoma occurring in a 41-year-old woman, the eighteenth case in literature, is reported herein. The radiographic imagings of the tumor simulated those of focal nodular hyperplasia; namely, the tumor was hyperechoic on ultrasound, hypodense on computed tomography, hypervascular on angiography, and exhibited an uptake of technetium (Tc)-99m phytate. The diagnosis was established after successful removal of the huge tumor from the right hepatic lobe. Histologically, the tumor was angiomyolipoma which was made up of three components, blood vessels, smooth muscle and fatty tissue. Furthermore, extramedullary hematopoiesis, which is usually seen in this tumor was confirmed. The differentiation of hepatic angiomyolipoma from FNH is also discussed.     

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.     

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.     

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.     

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.     

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.     

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 two-stage operation successfully performed for giant leiomyosarcoma of the esophagus with hepatic metastasis

Leiomyosarcoma of the esophagus is a rare neoplasm, with only 95 cases having been reported in the literature. Dysphagia is the most commonly noted symptom, however, because of its location in the submucosal layer, the tumor has usually grown to a considerable size by the time this presents. We report herein a case of a 39-year-old man who had no symptoms other than a 7 month history of a cough. After several investigations, the patient underwent resection of the thoracic and abdominal esophagus with lower lobectomy of the right lung through a right and left thoracotomy. The tumor measured 18×15×8cm in length and weighed 1,500g, being the biggest such tumor ever reported. Forty days after the first operation, an extended right hepatic lobectomy of the liver was performed for hepatic metastasis. He was discharged from the hospital 20 days after the second operation and is now doing well. The clinical features and surgical treatment of leiomyosarcoma of the esophagus are discussed herein.     

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。     

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.     

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。     

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.     

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.     

Ruptured hepatic angiolipoma in tuberous sclerosis complex

Tuberous sclerosis complex (TSC) may be associated with hepatic angiomyolipoma. In contradistinction to renal angiomyolipoma this lesion is rare, and nontraumatic hemorrhage has not so far been described. This is the first report of a spontaneously ruptured, surgically treated and histologically verified, hepatic angiomyolipoma in TSC. Anomalies of the vessel walls and consequently the hazard of bleeding are known to increase with the diameter of the tumor in other localizations. It is concluded that in cases with rapid tumor growth and marked vessel anomalies elective surgery should be considered.

---

Ruptur eines Leberangiomyolipoms bei tuberöser Sklerose

Zusammenfassung Die tuberöse Sklersoe (TSC, tuberous sclerosis complex) kann mit Angiomyolipomen der Leber einhergehen. Im Gegensatz zu Angiomyolipomen der Niere sind diese Tumoren selten, und spontane Blutungen wurden bisher nicht beschrieben. Dies ist der erste Fallbericht eines spontan rupturierten, operativ behandelten und histoloisch bestätigten Angiomyolipoms der Leber bei TSC. Es ist bekannt, daß bei anderen Lokalisationen die Anomalien der Gefäßwand und damit die Blutungsgefahr mit dem Durchmesser des Tumors zunehmen. Deshalb sollte bei einer raschen Größenzunahme und ausgeprägten Gefäßanomalien eine elektive, chirurgische Therapie erwogen werden.

     

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.