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Journal of Gastrointestinal Surgery :... Apr 2007Preoperative 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...
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.
Topics: Adult; Angiomyolipoma; Female; Hepatectomy; Humans; Liver Neoplasms; Male; Middle Aged
PubMed: 17436129
DOI: 10.1007/s11605-006-0037-3 -
Seminars in Ultrasound, CT, and MR Feb 2017With the increasing discovery of small renal masses with cross-sectional imaging, there has been the concomitant rise in their treatment. With the intent of early... (Review)
Review
With the increasing discovery of small renal masses with cross-sectional imaging, there has been the concomitant rise in their treatment. With the intent of early curative surgery for a presumed renal cell carcinoma, many renal masses are being resected at surgery without a confirmed diagnosis. Many of them are benign, and some are angiomyolipomas. The diagnosis of renal angiomyolipoma using imaging is, therefore, is as important as ever. Although most, if not all angiomyolipomas with abundant fat are diagnosed readily, some have too little fat to be detected with imaging. This article reviews the current classification, imaging pitfalls, and diagnosis of angiomyolipoma with an emphasis on the fat-poor types. Proper imaging technique, a thorough search for fat, and the appropriate use of percutanoeus biopsy are all needed to eliminate the unnecessary treatment of these benign neoplasms.
Topics: Angiomyolipoma; Diagnosis, Differential; Humans; Kidney; Kidney Neoplasms; Magnetic Resonance Imaging; Tomography, X-Ray Computed
PubMed: 28237279
DOI: 10.1053/j.sult.2016.11.001 -
The American Journal of Case Reports Sep 2017BACKGROUND Angiomyolipoma is a benign tumor composed of blood vessels, smooth muscle cells, and adipose tissue and has been described as belonging to the group of tumors... (Review)
Review
BACKGROUND Angiomyolipoma is a benign tumor composed of blood vessels, smooth muscle cells, and adipose tissue and has been described as belonging to the group of tumors of perivascular epithelioid cell origin (PEComa), commonly found in the kidneys and strongly associated with tuberous sclerosis. Only a few cases of extra-renal angiomyolipoma have previously been reported in the literature, most commonly in the liver. Adrenal angiomyolipoma is very rare, is usually asymptomatic, and is often found incidentally, with only 14 previously reported cases identified in the literature. CASE REPORT We report two cases of adrenal angiomyolipoma that were identified by abdominal computed tomography (CT). The first case presented in a 36-year-old man and was an oval-shaped adrenal mass, measuring 5.2×4.2×3.1 cm. The second case presented in a 61-year-old woman and was a round-shaped mass measuring 8.6×9.5×8.1 cm. Both patients underwent adrenalectomy. Histopathology and immunohistochemistry confirmed the diagnosis of benign angiomyolipoma composed of adipose tissues, blood vessels, and smooth muscle cells. CONCLUSIONS We present two rare cases of adrenal angiomyolipoma. We have reviewed the literature and identified 14 other cases of adrenal angiomyolipoma, and discuss the clinical, radiological, and pathological features of this rare tumor.
Topics: Adrenal Gland Neoplasms; Adrenalectomy; Adult; Angiomyolipoma; Female; Humans; Male; Middle Aged
PubMed: 28904331
DOI: 10.12659/ajcr.903908 -
Nigerian Journal of Clinical Practice Oct 2022Renal angiomyolipoma is the commonest benign solid kidney neoplasm though rare in clinical practice. The advent of radiological imaging techniques, refinement in...
BACKGROUND
Renal angiomyolipoma is the commonest benign solid kidney neoplasm though rare in clinical practice. The advent of radiological imaging techniques, refinement in surgical approach and techniques and availability of mammalian target of rapamycin have improved the outcome in these cohort of patients.
AIM
To report our experience with the surgical management of renal angiomyolipoma in the sub-Saharan Africa.
PATIENTS AND METHODS
This was a retrospective review of the records in the operating theatre book of urology division of patients who underwent radical nephrectomy over a 7-year-period (January 2013 to December 2019). The histologically confirmed renal angiomyolipoma information were retrieved from the records in the Department of Pathology. The clinical data were obtained from the patients' case files by identifying the patient with their registration number and not their names. The clinical features, investigations done, treatment offered, and the outcome of management were recorded in an SPSS version 20. The data was analyzed using statistics of central tendency and percentage.
RESULTS
Only 3 females with symptomatic renal angiomyolipoma were managed. This represented 4.9% of 61 patients with solid renal masses managed in the study period. The mean age was 51.2 (ranged 40-70) years. The mean tumor size was 18.9 cm. All the patients underwent radical nephrectomy. The pre- and postoperative urea and creatinine remained normal. The median follow-up period was 21 (16.5-30) months and were asymptomatic.
CONCLUSION
The incidence of renal angiomyolipoma among solid renal masses is 4.9% in our environment. Open radical nephrectomy is still the preferred method of treatment with satisfactory outcome.
Topics: Female; Humans; Adult; Middle Aged; Aged; Angiomyolipoma; Kidney Neoplasms; Nigeria; Nephrectomy; Retrospective Studies; Hamartoma
PubMed: 36308230
DOI: 10.4103/njcp.njcp_63_21 -
Zhong Nan Da Xue Xue Bao. Yi Xue Ban =... Dec 2022Renal angiomyolipoma (AML) with renal vein, inferior vena cava (IVC), and right atrial embolism is a rare solid tumor, whose etiology and pathogenesis are still unclear.... (Review)
Review
Renal angiomyolipoma (AML) with renal vein, inferior vena cava (IVC), and right atrial embolism is a rare solid tumor, whose etiology and pathogenesis are still unclear. Moreover, it is often misdiagnosed. One patient with renal AML complicated with renal vein, IVC, and right atrial embolism was admitted to the Second Xiangya Hospital of Central South University, who was a 35-year-old female, without any previous medical history, presented with right low back pain for more than 3 years. Computed tomography (CT) scan showed irregular lobulated fatty density mass in the right kidney, renal vein, IVC, and right atrium. The contrast-enhanced scan showed no enhancement of fat components at each phase and mild enhancement of solid components. Radical resection of the right kidney and removal of tumor thrombus were performed, and there was no recurrence 1 year after the operation. It is rare for renal AML to grow along the renal vein, IVC, and extend to the right atrium. Imaging examination is extremely important, and the CT findings of this case are characteristic, but the diagnosis eventually depends on pathological and immunohistochemical examinations.
Topics: Female; Humans; Adult; Vena Cava, Inferior; Angiomyolipoma; Atrial Fibrillation; Kidney Neoplasms; Embolism; Heart Atria; Leukemia, Myeloid, Acute
PubMed: 36748389
DOI: 10.11817/j.issn.1672-7347.2022.220044 -
World Journal of Gastroenterology May 2021First 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... (Review)
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 (, 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.
Topics: Angiomyolipoma; Carcinoma, Hepatocellular; Female; Humans; Liver Neoplasms; Middle Aged; Tomography, X-Ray Computed
PubMed: 34040323
DOI: 10.3748/wjg.v27.i19.2299 -
The Pan African Medical Journal 2013
Topics: Abdominal Pain; Angiomyolipoma; Female; Humans; Liver Neoplasms; Young Adult
PubMed: 24876896
DOI: 10.11604/pamj.2013.16.107.3427 -
PloS One 2018Improving the knowledge of angiomyolipoma physiopathology might help in refining its pharmacological treatment. We investigated if angiomyolipoma cells have migratory...
BACKGROUND
Improving the knowledge of angiomyolipoma physiopathology might help in refining its pharmacological treatment. We investigated if angiomyolipoma cells have migratory properties, how their growth and motility can be influenced by the hormonal milieu, and if this can be related to a specific gender.
METHODS
Primary cells were isolated from angiomyolipomas surgically resected for therapeutical reasons in a female and in a male patient. The genetic control demonstrated no TSC2 deletion. Bi- (wound healing) and three-dimensional (transwell assay) migration were analyzed in vitro in basal conditions and under the influence of 17- β-estradiol and SDF-1α.
RESULTS
Treatment up to 72 hours with 17-β-estradiol (0.1-100 nM), tamoxifen (0.2-20 μM) or with both, does not modify angiomyolipoma cells proliferation. On the other hand, SDF-1α and 17-β-estradiol treatment induce a significant motility increase (both bi- and three-dimensional) which becomes evident already after 2 hours of incubation. Angiomyolipoma cells express mRNA coding for SDF-1α and 17-β-estradiol receptors and secrete both the metalloproteases principally involved in malignant phenotype acquisition, i.e. MMP-2 and MMP-9.
CONCLUSION
Angiomyolipoma cells behave similarly, despite their different source. Primary angiomyolipoma cells migrate in response to hormonal milieu and soluble factors, and produce active metalloproteases, both aspects being consistent with the theory claiming they can migrate to the lungs (and/or other organs) and colonizing them. No main feature, among the aspects we analyzed, seems to be referable to the gender of origin.
Topics: Angiomyolipoma; Cell Movement; Cell Proliferation; Chemokine CXCL12; Estrogens; Female; Gene Expression Regulation, Neoplastic; Humans; Kidney; Male; Matrix Metalloproteinase 2; Matrix Metalloproteinase 9; Primary Cell Culture; Sequence Analysis, DNA; Wound Healing
PubMed: 29920561
DOI: 10.1371/journal.pone.0199371 -
Cancer Control : Journal of the Moffitt... 2022Our study aimed to evaluate the effect of daily oral dose of everolimus in the treatment of patients with tuberous sclerosis complex (TSC) associated with renal...
PURPOSE
Our study aimed to evaluate the effect of daily oral dose of everolimus in the treatment of patients with tuberous sclerosis complex (TSC) associated with renal angiomyolipoma (RAML), and the feasibility and safety of surgical treatment approach.
METHODS
We retrospectively investigated a total of 13 patients diagnosed of TSC-associated renal angiomyolipoma (TSC-RAML) who were scheduled for everolimus therapy. At 3-9 months after starting everolimus therapy, 4 of the study patients were symptomatic and underwent partial renal resection surgery. Two of these surgeries were performed open nephron sparing surgery (NSS) after TAE (Trans-arterial embolization), while the remaining 2 underwent robot-assisted partial nephrectomy (RAPN). A multi-slice helical CT scan performed among all the patients every 3 months, which was used to measure the volume and the density of the lesion.
RESULTS
Follow-up CT images revealed a significant reduction (P < .05) in the RAML volume, at a rate ranging from 11.6 to 42.5%, in response to everolimus therapy (10 mg/day) in TSC-RAML patients. Further, a significant decrease in the mean tumor density (P < .05), as compared to its baseline value, was also observed. Super-selective renal arterial embolization done prior to NSS was effective in reducing the intraoperative bleeding and stabilizing the patient during the NSS procedure: mean warm ischemia time was 29.5 minutes (range 18-40 minutes) and mean intraoperative bleeding volume was 275 mL (range 200-350 mL). Post-surgical (both NSS and RAPN) follow-up showed a favorable perioperative morbidity profile with good renal functional preservation. At the end of 2 years, all patients were well, with no signs of progression or recurrence of the condition, and demonstrated normal renal function.
CONCLUSIONS
The results suggested oral everolimus as an effective non-invasive therapy to treat TSC-RAML patients. Post mTOR inhibitor therapy, NSS and RAPN are preferred mode of surgical intervention in symptomatic patients. TAE prior to NSS is beneficial.
Topics: Humans; Angiomyolipoma; Everolimus; Kidney Neoplasms; Retrospective Studies; Tuberous Sclerosis
PubMed: 36471546
DOI: 10.1177/10732748221140266 -
PloS One 2023Differentiation of fat-poor angiomyolipoma (fp-AMLs) from renal cell carcinoma (RCC) is often not possible from just visual interpretation of conventional... (Meta-Analysis)
Meta-Analysis
PURPOSE
Differentiation of fat-poor angiomyolipoma (fp-AMLs) from renal cell carcinoma (RCC) is often not possible from just visual interpretation of conventional cross-sectional imaging, typically requiring biopsy or surgery for diagnostic confirmation. However, radiomics has the potential to characterize renal masses without the need for invasive procedures. Here, we conducted a systematic review on the accuracy of CT radiomics in distinguishing fp-AMLs from RCCs.
METHODS
We conducted a search using PubMed/MEDLINE, Google Scholar, Cochrane Library, Embase, and Web of Science for studies published from January 2011-2022 that utilized CT radiomics to discriminate between fp-AMLs and RCCs. A random-effects model was applied for the meta-analysis according to the heterogeneity level. Furthermore, subgroup analyses (group 1: RCCs vs. fp-AML, and group 2: ccRCC vs. fp-AML), and quality assessment were also conducted to explore the possible effect of interstudy differences. To evaluate CT radiomics performance, the pooled sensitivity, specificity, and diagnostic odds ratio (DOR) were assessed. This study is registered with PROSPERO (CRD42022311034).
RESULTS
Our literature search identified 10 studies with 1456 lesions in 1437 patients. Pooled sensitivity was 0.779 [95% CI: 0.562-0.907] and 0.817 [95% CI: 0.663-0.910] for groups 1 and 2, respectively. Pooled specificity was 0.933 [95% CI: 0.814-0.978]and 0.926 [95% CI: 0.854-0.964] for groups 1 and 2, respectively. Also, our findings showed higher sensitivity and specificity of 0.858 [95% CI: 0.742-0.927] and 0.886 [95% CI: 0.819-0.930] for detecting ccRCC from fp-AML in the unenhanced phase of CT scan as compared to the corticomedullary and nephrogenic phases of CT scan.
CONCLUSION
This study suggested that radiomic features derived from CT has high sensitivity and specificity in differentiating RCCs vs. fp-AML, particularly in detecting ccRCCs vs. fp-AML. Also, an unenhanced CT scan showed the highest specificity and sensitivity as compared to contrast CT scan phases. Differentiating between fp-AML and RCC often is not possible without biopsy or surgery; radiomics has the potential to obviate these invasive procedures due to its high diagnostic accuracy.
Topics: Humans; Carcinoma, Renal Cell; Angiomyolipoma; Retrospective Studies; Diagnosis, Differential; Kidney Neoplasms; Tomography, X-Ray Computed; Sensitivity and Specificity; Leukemia, Myeloid, Acute
PubMed: 37498830
DOI: 10.1371/journal.pone.0287299