Clinical review and literature analysis of hepatic epithelioid angiomyolipoma in alcoholic cirrhosis: A case report

BACKGROUND Hepatic epithelioid angiomyolipoma(HEA)has a low incidence and both clinical manifestations and imaging lack specificity.Thus,it is easy to misdiagnose HEA as other tumors of the liver,especially in the presence of liver diseases such as hepatitis cirrhosis.This article reviewed the diagnosis and treatment of a patient with HEA and alcoholic cirrhosis,and analyzed the literature,in order to improve the understanding of this disease.CASE SUMMARY A 67-year-old male patient with a history of alcoholic cirrhosis was admitted due to the discovery of a space-occupying lesion in the liver.Based on the patient’s history,laboratory examinations,and imaging examinations,a malignant liver tumor was considered and laparoscopic partial hepatectomy was performed.Postoperative pathology showed HEA.During outpatient follow-up,the patient showed no sign of recurrence.CONCLUSION HEA is difficult to make a definite diagnosis before surgery.HEA has the poten-tial for malignant degeneration.If conditions permit,surgical treatment is recom-mended.

Treatment of fat-poor renal angiomyolipoma with ectopic blood supply by fluorescent laparoscopy:A case report and review of literature

BACKGROUND Renal angiomyolipoma and renal cell carcinoma are the most common benign and malignant tumors of the kidney respectively,and the preoperative differential diagnosis is crucial due to the wide difference in treatment methods.Fat-poor renal angiomyolipoma is a relatively rare type of in renal angiomyolipoma.Its fat imaging features are not obvious,and it is easily misdiagnosed as renal cell carcinoma.CASE SUMMARY We report the case of a 41-year-old man who complained of osphyalgia.Subsequent abdominal computed tomography scans revealed that a heterogeneous mass was seen in the lower pole of the right kidney,with the size of about 53 mm×47 mm.And showed two right renal arteries,with the mass supplied by an ectopic vessel from the abdominal aorta.Fluorescent laparoscopic blockade of the right renal heterotopic artery and partial nephrectomy was performed.Based on histological and immunohistochemical findings,the tumor was diagnosed as fatpoor renal angiomyolipoma.CONCLUSION The use of fluorescent laparoscopy can effectively help intraoperative management,and the fluorescence pattern provided by intravenous indocyanine green can help suggest the final diagnosis,effectively guide the surgical decisionmaking,and avoid preoperative imaging diagnosis leading to nephrectomy for benign renal tumors,through fluorescent navigation of tumor supply vessel precise block,minimize the loss of renal function.

Fat-poor renal angiomyolipoma with prominent cystic degeneration:A case report and review of the literature

BACKGROUND Angiomyolipoma(AML),the most common benign tumor of the kidney,is usually composed of dysmorphic blood vessels,smooth muscle,and mature adipose tissue.To our knowledge,AML with cystic degeneration has rarely been documented.Cystic degeneration,hemorrhage,and a lack of fat bring great challenges to the diagnosis.CASE SUMMARY A 60-year-old man with hypertension presented with a 5-year history of cystic mass in his left kidney.He fell 2 mo ago.A preoperative computed tomography(CT)scan showed a mixed-density cystic lesion without macroscopic fat density,the size of which had increased compared with before,probably due to hemorrhage caused by a trauma.Radical nephrectomy was performed.Histopathological studies revealed that the lesion mainly consisted of tortuous,ectatic,and thick-walled blood vessels,mature adipose tissue,and smooth muscle-like spindle cells arranged around the abnormal blood vessels.The tumor cells exhibited positivity for human melanoma black-45,Melan-A,smooth muscle actin,calponin,S-100,and neuron-specific enolase,rather than estrogen receptor,progesterone receptor,CD68,and cytokeratin.The Ki-67 labeling index was less than 5%.The final diagnosis was a fat-poor renal AML(RAML)with prominent cystic degeneration.CONCLUSION When confronting a large renal cystic mass,RAML should be included in the differential diagnosis.

Giant Angiomyolipoma Masquerading as Perinephric Abscess: A Diagnostic Conundrum

Renal angiomyolipomas are the most common benign tumours of the kidney accounting for up to 1% of all renal masses. Giant angiomyolipomas which have a size greater than 10 centimetres are rare entities with few cases reported in literature. Small angiomyolipomas are usually a symptomatic and increasing size correlates with symptomatology. These are usually incidentally detected or when symptomatic may present with an abdominal lump, flank pain or hemorrhage. Herein, we report a rare case of 45-year-old lady with giant angiomyolipoma with clinical presentation indistinguishable from perinephric abscess. The case is rare with regards to the large size of tumour and the discordant presentation unusual for an angiomyolipoma.

Primary hepatic epithelioid angiomyolipoma: A malignant potential tumor which should be recognized

AIM: To improve the clinical diagnosis and recognition of hepatic epithelioid angiomyolipoma(HEAML).METHODS: Four cases of primary HEAML were confirmed based on the pathology archive system in our hospital from January 2009 to November 2015. The general state, clinical symptoms, imaging manifestations, histological results and immunohistochemistry of these patients were retrospectively reviewed and analyzed. Studies of HEAML published in the last 15 years were collected from Pub Med and MEDLINE to summarize the clinical symptoms, imaging characteristics, pathological features and management of HEAML.RESULTS: Four cases of primary HEAML were retrieved from our archives. These included three female patients and one male patient, with a mean age of 41.8 ± 11.5 years(ranging from 31 to 56 years). The meantumor size was 7.3 ± 5.5 cm(ranging from 3.0 to 15 cm). In the contrast-enhanced imaging, the tumor was obviously enhanced in the arterial phase, but enhanced continuously or exhibited a slow-density masse during the venous and delayed phases. Histologically, the tumors mainly consisted of epithelioid cells that comprised approximately 95% of the total neoplastic mass. Although no metastases occurred in our patients, pathological studies revealed necrosis, mitotic figures and liver invasion in two patients, which indicates aggressive behavior. Immunohistochemical staining revealed that human melanoma black 45(HMB-45) and Melan-A were positive in 4 cases. We only identified 81 cases with primary HEAML, including our present patients, from 26 articles available from Pub Med and MEDLINE. The majority of the papers were published as case reports. Only 5(5/75, 6%) cases were associated with tuberous sclerosis complex(TSC). More than half(35/66) were discovered incidentally upon physical examination. Approximately 65%(22/34) of the patients were misdiagnosed with HCC or other tumors before surgery. Approximately 10%(8/81) of the patients with HEAML had recurrence or metastasis after surgery, which was a very high and alarming rate.CONCLUSION: HEAML is a very rare primary hepatic tumor that is often misdiagnosed before surgery. Patients should be followed closely after surgery because of its malignant potential.

Hepatic angiomyolipoma-misdiagnosis as hepatocellular carcinoma:A report of 14 cases

Angiomyolipoma (AML) is a rare benign mesenchymal tumor of the liver, composed of a varying heterogeneous mixture of three tissue components: blood vessels, smooth muscle, and adipose cells. It has recently been proposed that the perivascular epithelial cell (PEC) is the common progenitor[1，2] Since its first description by Ishak in 1976[3], there have been more than 100 cases reported in the English literature[4-6]. With the advance of radiological techniques, many more tumors are being diagnosed by the means. But radiological findings of AML may only be suggestive of the lesion; its definitive diagnosis requires histological confirmation[9-19]. Some authors regard renal and hepatic AMLs, pulmonary and soft tissue lymphangiomyomatosis[2], pulmonary and pancreatic clear cell “sugar” tumor, and cardiac rhabdomyoma as closely related groups of tumors, based on their morphologic overlap and common immunoreactivity for HMB-45[l]. They show different microscopic appearances, however, according to their organ of origin. The goals of this study were to highlight more subtle morphology and to gain possible insights into the differential diagnosis that could provide important information about this disease.

Multi-slice computed tomography manifestations of hepatic epithelioid angiomyolipoma

AIM: To explore the characteristics of multi-slice computed tomography (CT) manifestations of hepatic epithelioid angiomyolipoma (HEA), improve the rate of accurate diagnosis, and reduce the misdiagnostic rate.

Liver transplantation for recurrent posthepatectomy malignant hepatic angiomyolipoma:A case report

Hepatic angiomyolipomas(AMLs) are typically benign tumors containing varying amounts of smooth musclecells, adipose tissue, and vessels, and are commonly found in the kidney and occasionally in the liver. The preoperative diagnosis of hepatic AML is primarily made from imaging and fine-needle aspiration biopsy results, though limited experience for such diagnoses can result in misdiagnosis. Some uncommon features of hepatic AML have been reported in the literature without an objective or qualitative consensus. As the majority of cases are benign, conservative treatment of AMLs is recommended. However, in rare cases, liver transplantation has been implemented. Only five cases of malignant hepatic AML have been reported. We report a rare case of recurrent posthepatectomy malignant hepatic AML that was misdiagnosed as liver cancer in a 37-year-old woman, which was treated by liver transplantation. The imaging and pathologic findings are presented in order to provide a more concise description to aid in future diagnoses.

Epithelioid angiomyolipoma of the liver:Cross-sectional imaging findings of 10 immunohistochemically-verified cases

AIM： To retrospectively evaluate the computed tomography （CT）/magnetic resonance imaging （MRI） imaging features of epithelioid angiomyolipoma of the liver （Epi-HAML）, with pathology as a reference. METHODS： The CT/MRI findings （number, diameter, lobar location, and appearance of lesions） in a series of 10 patients with 12 pathologically proven epithelioid angiomyolipomas of the liver were retrospectively analyzed. The imaging features, including attenuation/ signal intensity characteristics, presence of fat, hypervascular, outer rim, and vessels within lesion, were evaluated and compared with that of non-Epi- HAML in 11 patients （13 lesions）. The Fisher exact test was used to compare difference in probability of imaging features between the two types. RESULTS： For 21 patients, CT images of 15 patients and MR images of six patients were available. No patient underwent two examinations. For the 15 patients with a CT scan, all HAML lesions in the two groups （10 Epi-HAML and seven non-Epi-HAML） manifested as hypoattenuation. For the six patients with MRI, all lesions （two Epi-HAML and six non-Epi- HAML） were hypointense on TlWI （fat suppression） and hyperintense on T2WI. There were 10 non-Epi-HAML, but only two Epi-HAML lesions showed the presence of fat, which significantly different between the two types （P = 0.005）. On the dynamic contrast enhancement （DCE） imaging, eight Epi-HAML, and 13 non-Epi lesions manifested as hypervascular. Punctate or curved vessels were displayed in 10 Epi-HAML as well as in nine non- Epi lesions and outer rim enhancement could be found with eight Epi-HAML as well as six non-Epi lesions. CONCLUSION： Little or no presence of adipose tissue was found to be an imaging feature of Epi- HAML, compared with the non-Epi type. In addition, hypervascularity with opacification of central punctiform or filiform vessels on DCE would be a characteristic enhancement pattern for Epi-HAML.

Clinical features of hepatic angiomyolipoma

BACKGROUND: Hepatic angiomyolipoma (HAML) is a rare hepatic mesenchymal tumor. This study was designed to explore its clinical features. METHODS: Clinical data from 26 patients who had been pathologically confirmed with HAML and had received surgical resection at our hospital were analyzed retrospectively. RESULTS: HAML was seen more frequently in females (18/26) in this series, and most of the patients presented no significant symptoms except for one who had a spontaneous rupture hemorrhage. Serum alpha-fetoprotein (AFP), carbohydrate antigen 19-9 (CA19-9) and carcinoembryonic antigen (CEA) were negative in all patients. Imaging presentations were diverse. Pre-operative diagnosis was made in only 3 patients. Pathological study showed that the tumor was composed of adipose tissue, smooth muscle and blood vessels in different proportions. One patient showed hepatic vessel invasion. HMB-45 immunohistochemical staining was positive in all tumors. All patients underwent surgical resection without significant complications. Except for one patient who died 14 months after operation because of recurrent disease, no tumor recurrence was observed in the remaining 25 patients during a 2-3 years follow-up. CONCLUSIONS: Pre-operative diagnosis of HAML is difficult. There are potential risks of spontaneous rupture and malignant transformation. Surgical resection is the treatment of choice for HAML.

Two case reports and literature review for hepatic epithelioid angiomyolipoma: Pitfall of misdiagnosis

BACKGROUND Hepatic epithelioid angiomyolipoma(HEAML) is a rare liver disease and is easily misdiagnosed. Enhanced recognition of HEAML is beneficial to the differential diagnosis of rare liver diseases.CASE SUMMARY We presented two cases of HEAML in Changzheng Hospital, Naval Medical University, and then collected and analyzed all reports about HEAML recorded in PubMed, MEDLINE, China Science Periodical Database, and VIP database from January 2000 to March 2018. A total of 409 cases of HEAML in 97 reports were collected, with a ratio of men to women of 1:4.84 and an age range from 12 years to 80 years(median 44 years). Among the patients with clinical symptoms mentioned, 61.93%(205/331) were asymptomatic, 34.74%(115/331) showed upper or right upper quadrant abdomen discomfort, while a few of them showed abdominal mass, gastrointestinal symptoms, low fever, or weight loss. The misdiagnosis rate of HEAML was as high as 40.34%(165/409) due to its nonspecific imaging findings. Most of the tumors were solitary and round in morphology, with clear boundaries. Ultrasound scan indicated low echo with internal nonuniformity and rich blood supply in most cases. Computer tomography/magnetic resonance imaging enhanced scan showed varied characteristics. The ratio of fast wash-in and fast wash-out, fast wash-in and slow wash-out, and delayed enhancement was roughly 4:5:1. A definite diagnosis of HEAML depended on the pathological findings of the epithelioid cells in lesions and the expression of human melanoma black 45, smooth muscle actin,melanoma antigen, and actin by immunohistochemical staining. HEAML had a relatively low malignant rate of 3.91%. However, surgical resection was the main treatment for HEAML, due to the difficulty diagnosing before operation.CONCLUSION HEAML is a rare and easily misdiagnosed disease, and it should be diagnosed carefully, taking into account clinical course, imaging, pathological,and immunohistochemical findings.

Hepatic angiomyolipoma: Dynamic computed tomography features and clinical correlation

AIM: To study the dynamic computed tomography (CT) features of hepatic angiomyolipoma (AML) in patients with or without tuberous sclerosis complex (TSC).METHODS: The clinical information, CT fi ndings and histopathological results of hepatic AML were analyzed retrospectively in 10 patients.RESULTS: Hepatic AML was prone to occur in female patients (7/10), and most of the patients (8/10) had no specific symptoms. All tumors presented as well-def ined, unenveloped nodules in the liver. Six patients with sporadic hepatic AML had a solitary hepatic nodule with a definite fat component. Non-fat components of the hepatic lesions were enhanced earlier and persistently. Prominent central vessels were noted in the portal venous phase in three patients. In four patients with hepatic AML and TSC, most of the nodules were within the peripheral liver. Seven fat-deficient nodules were found with earlier contrast enhancement and rapid contrast material washout in two patients. Lymphangioleiomyomatosis was found in one patient.CONCLUSION: Imaging features of hepatic AML are characteristic. Correct diagnosis preoperatively can be made in combination with clinical features.

Clinical characteristics and outcomes of patients with hepatic angiomyolipoma:A literature review

First reported in 1976,hepatic angiomyolipoma(HAML)is a rare mesenchymal liver tumor occurring mostly in middle-aged women.Diagnosis of the liver mass is often incidental on abdominal imaging due to the frequent absence of specific symptoms.Nearly 10%of HAMLs are associated with tuberous sclerosis complex.HAML contains variable proportions of blood vessels,smooth muscle cells and adipose tissue,which renders radiological diagnosis hazardous.Cells express positivity for HMB-45 and actin,thus these tumors are integrated into the group of perivascular epithelioid cell tumors.Typically,a HAML appears on magnetic resonance imaging(or computed tomography scan)as a hypervascular solid tumor with fatty areas and with washout,and can easily be misdiagnosed as other liver tumors,particularly hepatocellular carcinoma.The therapeutic strategy is not clearly defined,but surgical resection is indicated for symptomatic patients,for tumors showing an aggressive pattern(i.e.,changes in size on imaging or high proliferation activity and atypical epithelioid pattern on liver biopsy),for large(>5 cm)biopsy-proven HAML,and if doubts remain on imaging or histology.Conservative management may be justified in other conditions,since most cases follow a benign clinical course.In summary,the correct diagnosis of HAML is challenging on imaging and relies mainly on pathological findings.

Granulomatous reaction in hepatic inflammatory angiomyolipoma after chemoembolization and spontaneous rupture

A 77-year-old Japanese woman was transported to a nearby hospital due to sudden abdominal pain and transient loss of consciousness. Abdominal computed tomography(CT) suggested hemoperitoneum and hepatic nodule. She was conservatively treated. Contrast-enhanced CT two months later revealed an increased mass size, and the enhancement pattern suggested the possibility of hepatocellular carcinoma(HCC). Under a clinical diagnosis of HCC, transcatheter arterial chemoembolization(TACE) was performed. A subsequent imaging study revealed that most of the lipiodol used for the embolization was washed out. Therefore, surgical resection was performed. Histologically, the nodule contained numerous inflammatory cells including small lymphocytes, plasma cells and macrophages. Notably, epithelioid granulomatous features with multinucleated giant cells were observed in both the nodule and background liver. Some of the multinucleated giant cells contained oil lipid. Among the infiltrating inflammatory cells,spindle-shaped, histiocytoid or myoid tumor cells with eosinophilic cytoplasm were found. The tumor cells were positive for Melan A and HMB45. The nodule contained many Ig G4-positive plasma cells; these were counted and found to number 72.6 cells/HPF(range: 61-80). The calculated Ig G4:Ig G ratio was 33.2%. The nodule was finally diagnosed as previously ruptured inflammatory angiomyolipoma modified by granulomatous reaction after TACE.

A case of hepatic angiomyolipoma difficult to distinguish from hepatocellular carcinoma

We report a case of hepatic angiomyolipoma with uncommon clinical features. A 56-year-old man presented with a hepatic tumor in the caudate lobe. The tumor was hypoechoic on ultrasonography, showed early-phase hyperattenuation on enhanced computed tomography and did not absorb iron on superparamagnetic iron oxide-enhanced magnetic resonance imaging. Hepatocellular carcinoma was highly suspected, and the patient underwent hepatic resection. Histologically, the tumor was mainly composed of smooth muscle cells and contained small amounts of adipose cells and blood vessels. On immunohistochemical staining, the smooth muscle cells were positive for a melanocytic cell-specific monoclonal antibody. In cases with uncommon features of angiomyolipoma, it is quite difficult to distinguish angiomyolipoma from hepatocellular carcinoma.

Diagnostic analysis of hepatic angiomyolipoma

BACKGROUND: Hepatic angiomyolipoma (HAML) is a rare tumor containing a variable amount of fat, vessels and smooth muscle. We report the image findings on ultra- sonography and computed tomography ( CT) of huge HAML. METHOD; The clinical, imaging and pathological data of a case of HAML were retrospectively collected and ana- lyzed. RESULTS; A huge heterogeneous hyperecho mass with anecho and hypoecho areas inside was found in the left he- patic lobe on ultrasonography. Color Doppler showed blood flow and arterial spectrum in it. CT scan showed a huge heterogeneous solid mass in the left lobe of the liver, with a low density and hypervascular area in arterial phase. The serum tumor marks were all negative. Ultrasound- guided biopsy was taken twice before resection and both showed necrosis tissue and reaction of inflammatory cells. Postoperative pathological results showed that the tumor was composed of epithelioid smooth muscle cells, thick- walled blood vessels and a few adipose cells with necrosis. The immunohistochemistry results showed appea-rance of typical HAML, with HMB-45 positive and alpha fetopro- tein (AFP) negative. CONCLUSIONS: Preoperative diagnosis of HAML relies on combination of CT, MRI and ultrasonography. Our case of HAML showed heterogeneous hyperecho image on ultra- sonography. Ultrasound-guided biopsy combined with morphological manifestation and specimen examination for HMB-45 may be helpful in the diagnosis of HAML.

Coincidence of hepatocelluar carcinoma and hepatic angiomyolipomas in tuberous sclerosis complex: A case report

Tuberous sclerosis complex (TSC) is a dominantly inherited disorder which characterized by the growth of harmatomatous in multiple organs. Unlike the common development of renal angiomyolipoma, hepatic angiomyolipoma rarely occur in patients with TSC. We report here a patient with hepatic angiomyolipomas and concurrent hepatocellular carcinoma in TSC. This represents the first reported case in English literature. In this patient, multiple hepatic angiomyolipomas were diagnosed with recognition of their fat components and typical clinical settings. Hepatocellular carcinoma in the left liver lobe was definitely diagnosed by US guided biopsy. In such clinical settings, fat containing lesions in liver can be reasonably treated as angiomyolipomas, but non fat containing lesions must be differentiated from hepatocellular carcinoma, imaging guided biopsy can be adopted to confirm the diagnosis.

Hepatocellular carcinoma with concomitant hepatic angiomyolipoma and cavernous hemangioma in one patient

The risk of developing hepatocellular carcinoma(HCC) is strongly associated with hepatitis B virus infection.Hepatic angiomyolipoma(AML),a rare benign tumor,is composed of a heterogeneous mixture of adipose cells,smooth muscle cells and blood vessels.Here,we report the case of a 44-year-old man who developed HCC with a concomitant hepatic AML and a cavernous hemangioma,in the absence of cirrhosis.To our knowledge,based on an extensive literature search using the www.pubmed.gov website,this is the first report of an HCC case with both concomitant AML and cavernous hemangioma at the same position in the liver.The presence of the hepatitis B surface antigen was detected,but the liver function was normal.Clinical and pathological data were collected before and during the treatment.Hepatic AML was diagnosed based on the typical histological characteristics and immunohistochemical staining,which revealed,a positive staining with a melanocytic cell-specific monoclonal antibody.There was no evidence of tuberous sclerosis complex in this patient.Although the HCC was poor- to moderately-differentiated,the characteristics of the AML and the cavernous hemangioma in this patient did not match any criteria for malignancy.Hepatectomy followed by transarterial chemoembolization treatment were effective therapeutic methods for the adjacent lesions in this patient.This case is an interesting coincidence.

Embolization of symptomatic renal angiomyolipoma with a mixture of lipiodol and PVA, a mid-term result

Objective： To evaluate the clinical effect of renal artery embolization with a mixture of lipiodol and polyvinyl alcohol （PVA） particles on symptomatic renal angiomyolipoma and to present the mid-term results from our single-center site. Methods： We performed a retrospective review of the seven patients who underwent embolization with a mixture of lipiodol and PVA particles to treat symptomatic renal angiomyolipoma in our center between February 2011 and December 2013. Medical records and follow-up results were reviewed and analyzed. Results： Seven patients underwent eight episodes of embolization using a mixture of lipiodol and PVA particles to treat symptomatic renal angiomyolipoma. One patient required a subsequent embolization of the right kidney 9 months after the initial embolization of the left kidney. No nephrectomy was required in any of these cases during follow-up. None had recurrence of tumor bleeding or rupture during follow-up. Decreased tumor size was achieved in six patients （85.7%） during the mid-term follow-up. Conclusions： Embolization with a mixture of lipiodol and PVA particles is an effective and safe treatment for symptomatic renal angiomyolipoma. Based on the durable mid-term results of the present study, a mixture of lipiodol and PVA particles is recommended for embolization.

Giant exophytic renal angiomyolipoma masquerading as a retroperitoneal liposarcoma: A case report and review of literature

A 42-years-old lady, presented with a large retroperitoneal mass which was preoperatively diagnosed as a retroperitoneal liposarcoma following an image guided core biopsy. She underwent a margin-negative resection of the retroperitoneal mass(multi visceral resection-enbloc excision of the retroperitoneal mass with a left nephrectomy and a segmental descending colectomy). The final histopathological examination of the resected specimen confirmed an exophytic renal angiomyolipoma(AML) which was extending into the retroperitoneum. AML is a rare benign tumor arising most commonly from the kidney. It can sometimes present as a diagnostic challenge as it mimics a retroperitoneal liposarcoma or a fat-containing renal cell carcinomas closely. We present this case to share our experience of managing a case of giant exophytic AML which resembled retroperitoneal liposarcoma closely and resulted into an aggressive surgery.