Diagnosis Of Angiomyolipoma Research Articles

Metastasis and Recurrence Retroperitoneal Angiomyolipoma: a Case Report

Introduction: Angiomyolipoma (AML) is often a benign tumor found in the kidney. Case presentation: We report a very rare case of retroperitoneal AML with two relapses and metastasis that presented with complaints of abdominal pain. Discussion: Extrarenal angiomyolipoma (ERAML) is a very rare tumor, often benign, and mostly found in the liver and then in the retroperitoneal area. Diagnosis of retroperitoneal AML is very difficult, and the best imaging is CTA and CT. We found only a few reports of metastasis and recurrence in the publication. Conclusion: retroperitoneal angiomyolipoma (RAML) often behaves benignly; according to several studies of metastasis and recurrence, it shows the need for frequent follow-ups after surgery.

Angiomyolipoma in the Temporal Region: A Case Report

Abstract: Angiomyolipomas are benign tumors (consisting of fat tissues, smooth muscles, and blood vessels) that are often found in the kidney or liver. However, they are rarely found in the head and neck region and require proper management. We reported a 13-year-old girl presented with a mass in the right temple since last year. Musculoskeletal ultrasound (US) and contrast-enhanced computed tomography (CT) scan showed a well-defined hypoechoic lesion in the right temporal subfascial region, with a suspicion of a hemangioma. The patient underwent resection of the lesion. The diagnosis of angiomyolipoma was confirmed through a histopathological examination. The patient was subsequently discharged on the third postoperative day and had no further treatment. In conclusion, extraperitoneal angiomyolipomas are rarely found, and the diagnosis is confirmed by histopathological examination. The management of temporal angiomyolipomas is tumor resection. Keywords: temporal angiomyolipomas; histopathological diagnosis; surgical treatment

Minimal Fat Content in Papillary Renal Cell Carcinoma Diagnosed with Dual-Layer Dual-Energy CT.

A 56-year-old man with a previous right nephrectomy for multiple papillary renal cell carcinomas (pRCC) underwent a follow-up CT scan. Using a dual-layer dual-energy CT (dlDECT), we demonstrated the presence of a small amount of fat in a 2.5 cm pRCC that mimicked the diagnosis of angiomyolipoma (AML). Histological examination demonstrated the absence of macroscopic intratumoral adipose tissue, showing a fair amount of enlarged foam macrophages loaded with intracytoplasmic lipids. The presence of fat density in an RCC is an extremely rare occurrence in the literature. To our knowledge, this is the first description using dlDECT of a minimal amount of fat tissue in a small RCC due to the presence of tumor-associated foam macrophages. Radiologists should be aware of this possibility when characterizing a renal mass with DECT. The option of RCCs must be considered, especially in the case of masses with an aggressive character or a positive history of RCC.

MP64-14 CRITICAL EXAMINATION OF SURVEILLANCE AS a MANAGEMENT STRATEGY IN RENAL ANGIOMYOLIPOMA

You have accessJournal of UrologyCME1 Apr 2023MP64-14 CRITICAL EXAMINATION OF SURVEILLANCE AS a MANAGEMENT STRATEGY IN RENAL ANGIOMYOLIPOMA Katiana Vazquez-Rivera, Brooklyn, Ny, Stephen W. Reese, Lennert Eismann, Arnold Oparanozie, Sari Khaleel, Jonathan Coleman, Paul Russo, and A. Ari Hakimi Katiana Vazquez-RiveraKatiana Vazquez-Rivera More articles by this author , Brooklyn Brooklyn More articles by this author , Ny Ny More articles by this author , Stephen W. ReeseStephen W. Reese More articles by this author , Lennert EismannLennert Eismann More articles by this author , Arnold OparanozieArnold Oparanozie More articles by this author , Sari KhaleelSari Khaleel More articles by this author , Jonathan ColemanJonathan Coleman More articles by this author , Paul RussoPaul Russo More articles by this author , and A. Ari HakimiA. Ari Hakimi More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000003322.14AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: There currently remains debate about the need for intervention and surveillance in patients diagnosed with a renal angiomyolipoma (AML). Surveillance has typically been considered in masses ≤4 cm and many still advocate for select intervention in large tumors, women of child-bearing age and patients with limited access to emergency care, however little empirical data supports these practices. The objective of this study is to report our institutional experience of patients with AMLs on active surveillance (AS). METHODS: In this retrospective single-institution study at a large academic institution, we identified a cohort of patients with a radiographic diagnosis of AML. Many of these patients were placed on a surveillance protocol at the discretion of their physician. Patient demographics, tumor characteristics and intervention rates were captured. Additionally, we modeled the likelihood of requiring intervention while on surveillance using a Cox proportional hazard model. RESULTS: The study included 163 patients with a radiographic diagnosis of AML from 1997 to 2021. In the cohort, 133 (81.6%) patients were placed on surveillance and the rest underwent upfront intervention. Most patients were women (n=141, 86.0%) and median age was 57. Patients who received upfront intervention (n=30) vs. surveillance (n=133) were more likely to be younger (51 vs. 59 years, p=0.02), have a smaller mass (1.6 vs. 2.1 cm, p=0.02) and be symptomatic on presentation (26.7% vs. 5.3%, p<0.01). Of the patients who received upfront intervention, 6 (20%) had pain and 2 (6.7%) had hematuria as their primary symptom. Median time on surveillance was 6.7 years (95% CI: 5.6-7.7) and patients who received an intervention on surveillance did so at median of 3.2 years (95% CI: 0.9-4.2). Of those on surveillance, 23 (17.3%) ultimately received an intervention, including 11 (47.8%) who had a mass >4 cm and 1 (4.4%) patient had an urgent intervention. Median yearly growth rate of AML was significantly different in those who received an intervention vs. those who did not (7.25% vs. 2.16%, p=0.01). Modeling likelihood of intervention while on surveillance, patients who had hematuria (HR: 9.15, p=0.04), mass size >4 cm (HR: 5.40, p<0.01) and higher growth rate (HR: 1.02, p<0.01) were more likely to receive an intervention. CONCLUSIONS: Many patients with an AML can be safely watched on active surveillance, however some patients may ultimately require intervention. Factors associated with increased likelihood of intervention on surveillance include hematuria, large mass size and rapid growth rate. Source of Funding: None © 2023 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 209Issue Supplement 4April 2023Page: e886 Advertisement Copyright & Permissions© 2023 by American Urological Association Education and Research, Inc.MetricsAuthor Information Katiana Vazquez-Rivera More articles by this author Brooklyn More articles by this author Ny More articles by this author Stephen W. Reese More articles by this author Lennert Eismann More articles by this author Arnold Oparanozie More articles by this author Sari Khaleel More articles by this author Jonathan Coleman More articles by this author Paul Russo More articles by this author A. Ari Hakimi More articles by this author Expand All Advertisement PDF downloadLoading ...

Diagnostic performance of the “drooping” sign in CT diagnosis of exophytic renal angiomyolipoma

To evaluate the prevalence of angular interface and the "drooping" sign in exophytic renal angiomyolipomas (AMLs) and the diagnostic performance in differentiating exophytic lipid-poor AMLs from other solid renal masses. This IRB-approved, two-center study included 185 patients with 188 exophytic solid renal masses < 4cm with histopathology and pre-operative CT within 30days of surgical resection or biopsy. Images were reviewed for the presence of angular interface and the "drooping" sign qualitatively by three readers blinded to the final diagnosis, with majority rules applied. Both features were assessed quantitatively by cohort creators (who are not readers) independently. Free-marginal kappa was used to assess inter-reader agreement and agreement between two methods assessing each feature. Fisher's exact test, Mann-Whitney test, and multivariable logistic regression with two-tailed p < 0.05 were used to determine statistical significance. Diagnostic performance was assessed. Ninety-four patients had 96 AMLs, and 91 patients had 92 non-AMLs. Seventy-four (77%) of AMLs were lipid-poor based on quantitative assessment on CT. The presence of angular interface and the "drooping" sign by both qualitative and quantitative assessment were statistically significantly associated with AMLs (39% (qualitative) and 45% (quantitative) vs 15% (qualitative) and 13% (quantitative), and 48% (qualitative) and 43% (quantitative) vs 4% (qualitative) and 1% (quantitative), respectively, all p < 0.001) in univariable analysis. In multivariable analysis, only the "drooping" sign in either qualitative or quantitative assessment was a statistically significant predictor of AMLs (both p < 0.001). Inter-reader agreement for the "drooping" sign was moderate (k = 0.55) and for angular interface was fair (k = 0.33). Agreement between the two methods of assessing the "drooping" sign was substantial (k = 0.84) and of assessing the angular interface was moderate (k = 0.59). The "drooping" sign both qualitatively and quantitatively, alone or in combination of angular interface, had very high specificity (96-100%) and positive predictive value (PPV) (89-100%), moderate negative predictive value (62-68%), but limited sensitivity (23-49%) for lipid-poor AMLs. The "drooping" sign by both qualitative and quantitative assessment is highly specific for lipid-rich and lipid-poor AMLs. This feature alone or in combination with angular interface can aid in CT diagnosis of lipid-poor AMLs with very high specificity and PPV.

Angiomyolipoma with epithelial cysts, an unexpected discovery in a gunshot abdomen: a single case report

BackgroundAngiomyolipoma with epithelial cysts is a rare variant of an angiomyolipoma that typically occurs sporadically. Patients with pre-existing kidney lesion or disease have significantly increased risk of morbidity and mortality associated with trauma. Abnormal and diseased kidneys are frequently injured by low or insignificant velocity impacts. The vulnerability of these kidneys is related to the type of pathology. However, cystic or hydronephrotic kidneys are more susceptible to trauma. Recent studies have shown an association of this rare entity with tuberous sclerosis complex. Despite the rarity of genetic association of Angiomyolipoma with epithelial cysts and tuberous sclerosis, the emerging findings raise the necessity of genetic testing of these lesions to confirm a remote possibility of tuberous sclerosis complex.Case presentationWe report a case of a 38-year-old male patient who sustained a right renal injury as a result of a gunshot to the abdomen and underwent an emergency nephrectomy due to hemodynamic instability. A grossly looking shattered kidney was surgically removed. The morphology in conjunction with immunohistochemistry of the specimen favoured a diagnosis of Angiomyolipoma with epithelial cysts.ConclusionThis is the first reported case of angiomyolipoma with epithelial cysts diagnosed on a nephrectomy specimen from renal trauma. We aim to highlight an approach to renal trauma on a kidney with pre-existing AMLEC as well as an approach to adult cystic renal neoplasms and to propose the importance of genetic testing for Tuberous Sclerosis.

Renal Angiomyolipoma: A Diagnostic and Therapeutic Challenge.

Angiomyolipomas (AML) represent less than 10% of renal tumours. They are most often detected incidentally during imaging tests, but there are several histological variants that pose difficulties in the radiological differential diagnosis. Their identification should allow the loss of renal parenchyma due to embolization or radical surgery to be prevented. Retrospective study of patients undergoing kidney surgery with post-surgical pathological diagnosis of AML at the Álvaro Cunqueiro Hospital (2016-2021). Patients with a radiological diagnosis of AML who underwent surgery based on clinical criteria were excluded. 18 patients were registered, allowing for the assessment of 18 renal tumours. All of the cases were diagnosed incidentally. Preoperative radiological diagnosis was: 9 lesions suggestive of renal cell carcinoma (RCC) (50%), 7 cases suggestive of RCC vs. AML (38.9%) and 2 lesions suggestive of AML vs. retroperitoneal liposarcoma (11.1%). Histological variants of AML were found in 61.1% of cases (n = 11). The most widely used surgical technique was partial nephrectomy, in 66.67% of cases. The radiological differential diagnosis of AML, and particularly its variants, with malignant lesions have important limitations either due to the predominance or scarcity of any of the AML components. Some cases can also pose difficulties at the histological level. This fact highlights the importance of the specialization of uroradiologists and uropathologists and the performance of kidney-sparing therapeutic techniques.

Single-port robot-assisted retroperitoneal surgery: A feasible approach

IntroductionThe concept of surgery through a single incision has been pursued in the field of minimal invasion for the treatment of different pathologies. This, added to a retroperitoneal approach, implies less aggression for the patient at different levels. We describe the first cases of single-port robot-assisted retroperitoneal surgery (SP-RARS) in our country using the da Vinci Xi® system. Material and methodsWe present two cases of patients with right adrenal masses larger than 4 cm. The first case was a 55-year-old woman with an incidental diagnosis of angiomyolipoma, and the second case was a 62-year-old man with a non-functioning adrenal adenoma. Both cases were operated through a single 4-cm subcostal incision using the da Vinci Xi® system, annulling arm 4 without reallocating laterality on the surgeon’s console. ResultsBoth surgeries were performed with console times of 75 and 150 min, respectively, with access and docking times below 30 min. Bleeding was less than 100 cc in both surgeries without the need to make accessory incisions or leave a drainage tube. The surgical specimens were removed through the same initial incision without the need for enlargement. Postoperative intravenous analgesia was not necessary in any case (VAS 0) and hospital stay was less than 24 h in both patients without reporting any complications. ConclusionsSP-RARS is a feasible approach using the da Vinci Xi® system.

Cirugía retroperitoneal asistida por robot por puerto único: un abordaje factible

IntroductionThe concept of surgery through a single incision has been pursued in the field of minimal invasion for the treatment of different pathologies. This, added to a retroperitoneal approach, implies less aggression for the patient at different levels. We describe the first cases of single-port robot-assisted retroperitoneal surgery (SP-RARS) in our country using the Da Vinci Xi® system. Material and methodsWe present 2 cases of patients with right adrenal masses larger than 4cm. The first case was a 55-year-old woman with an incidental diagnosis of angiomyolipoma, and the second case was a 62-year-old man with a non-functioning adrenal adenoma. Both cases were operated through a single 4cm subcostal incision using the Da Vinci Xi® system, annulling arm 4 without reallocating laterality on the surgeon's console. ResultsConsole times for surgeries were 75 and 150min, with access and docking times below 30min. Bleeding was less than 100cc in both surgeries with no need of accesory trocars, make new incisions or leave a drainage tube. The surgical specimens were removed through the same initial incision without the need for enlargement. Postoperative intravenous analgesia was not necessary in any case (VAS 0) and hospital stay was less than 24h in both patients, without reporting any complications. ConclusionsSP-RARS is a feasible approach using the Da Vinci Xi® system.

DIAGNOSTIC VALUE OF MULTI-SLICE CT SCAN FOR RENAL ANGIO- MYOLIPOMA DETECTION: A CASE REPORT

Background: Angiomyolipoma (AML) is a rare solid benign tumor that occurs at many sites, more commonly in the kidney, which arises from either the renal pelvis or the sinus, also referred to as renal hamartoma. AML is composed of blood vessels, smooth muscle cells, and fat hence the name. They are seen 80-90% as isolated lesions that occur sporadically and seen 25-50% of patients with tuberous sclerosis. AML may hard to be distinguished from renal cell carcinoma because of its presentation as enlarged lump. Computed tomography may help to diagnose AML. To get a definite diagnosis of AML, excision and histopathological examination is recommended in symptomatic patients, especially to prevent rupture hemorrhage. Objective: The purpose of this study was to evaluate multi-slice computed tomography with contrast in a male patient aged 49 years with Angiomyolipoma Case description: We herein present a case of renal AML in a 49 years old male patient, who presented with left flank pain for ~1 year. He has no history of hematuria and dysuria and tuberous sclerosis. Multi-slice computed tomography with contrast demonstrates a hypodense inhomogenous mass measuring 11x9,3 cm in the upper pole of the left kidney, hypertrophy of the left kidney, and hump sign of left kidney. At the later date, the patient underwent total left nephrectomy at another hospital and the postoperative histopathological examination confirmed the lesion as an AML. Conclusion: Angiomyolipoma is a rare benign tumor which usually occurs in the kidney that can be confused with malignant tumor. Ultrasonography and computed tomography can help to diagnose AML. The symptomatic patient requires surgical intervention.

Association of CT Scan Parameters with the Risk of Renal Angiomyolipoma Rupture; a Brief Report.

Introduction:Rupture of renal angiomyolipoma (AML) is an emergency and life-threatening complication. This study aimed to evaluate the association of computed tomography (CT) scan parameters with the risk of rupture in renal AMLs.Methods:In this retrospective cross-sectional study, patients who were referred to a referral university hospital with diagnosis of AML, between 2007 and 2019, were included. Patients were divided into ruptured and non-ruptured cases based on surgery and CT scan findings and the baseline characteristics as well as CT scan parameters were compared between the two groups.Results:20 AML patients with the mean age of 39.6 ± 12.5 years were included (75% female). The lesion was ruptured in 8 (40%) patients. The mean size of the lesion was ‎97.0 ± 15.9 mm‎‏ in the ruptured and ‏‎72.0 ± 29.4‎‏ in the non‎-‎ruptured AML ‏‎(‎p ‎= ‎‏0.045). The ‎mean fat density based on non-contrast enhanced CT (NCCT) scan (-‎56.1 ± 16.3 ‎vs ‎-‎‎74.9±24.1; ‏p = ‏‎0.018) and contrast enhanced CT (CECT) scan (-‎20.8 ± 16.9 ‎vs ‎-‎‎50.5 ± 31.7; ‏p ‏= ‏‎0.‎016) was significantly higher in the ruptured cases. Total tumor density based on NCCT scan was significantly greater in the ruptured ‎AMLs ‏(‏‎19.6 ± 25.9 ‎‏ vs‎ ‎-22.7±41.6, p=0.033). Conclusion:It seems that some CT scan parameters such as mean fat density and ‎total tumor density ‏could be used for differentiation between ruptured and non-ruptured AMLs.

Long-standing Renal Hydatid Cyst Mimicking an Angiomyolipoma: A Rare Case Report

Primary hydatid cyst of the kidney is very rare and accounts for 1-4% of all hydatid disease. Authors hereby reports an unusual case of an isolated, long-standing renal hydatid cyst, mimicking an angiomyolipoma on imaging. A 72-year-old female, from a rural area, presented with pain in the right flank since two years. She also complained of mild fever off and on, since one year. A 10×10 cm, non tender lump was palpated in the right flank. Ultrasonography and computerised tomography showed a large rightsided renal mass measuring 10 cms and a diagnosis of angiomyolipoma was rendered. A right sided open/simple nephrectomy was done. The kidney was large, cystic, firm and was sent for histopathological examination. On gross examination, a large, unilocular, thick- walled, cystic lesion was seen. Cut-surface showed heterogeneous, creamy coloured necrotic contents, with few nodular gelatinous masses. On microscopy, characteristic acellular, laminated structure of the ectocyst of Echinococcus granulosus was seen. Extensive calcification and ossification of the cyst wall with bone marrow production was also seen. Contents of the cyst were necrotic and no daughter cysts were seen. Adipose tissue of the bone marrow was mistaken on imaging for the lipomatous component of an angiomyolipoma. Isolated renal hydatid cyst is very uncommon and though active hydatid cysts do have characteristic radiological findings, they can be misdiagnosed as a benign renal tumours. Renal cysts or abscesses. Old cases show degenerative changes as well as calcification and are difficult to diagnose preoperatively. Surgical treatment of renal hydatid cyst depends on the size, number of cysts, exact intrarenal location and residual renal function.

A giant renal angiomyolipoma: a rare case report and review of literature

Renal angiomyolipomas (AMLs) are the most common benign tumours of the kidney that occur sporadically in 80% of the patients and are seen associated with genetic conditions such as tuberous sclerosis in rest of the patients. The diagnosis of renal AML pre-operatively have been made easier with the advent of good imaging modalities. The management of these tumours depends on factors such as size, clinical presentation, extent of parenchymal involvement and ranges from a wait and watch strategy to a radical nephrectomy. Herewith, we reported of a giant renal AML who presented with complaints of lump in abdomen with headache and palpitations. She was diagnosed to have a giant renal AML on imaging. The patient was managed with radical nephrectomy after renal AML. A brief case report with review literature was presented here.

Giant Renal Angiomyolipoma by the Example of Clinical Cases

The paper presents two clinical cases of patients with giant renal angiomyolipomas (AML), in one of whom its course was complicated by intratumoral hemorrhage. It describes key diagnostic criteria for computed tomography (CT), as well as the distinctive features of other neoplasms that should undergo a differential diagnosis. The similar clinical presentations and morphological characteristics of different renal neoplasms can cause certain diagnostic difficulties; however, the carefully collected historical data and distinctive criteria allow AML to be identified. Due to its high sensitivity and specificity, abdominal contrast-enhanced CT is an effective imaging technique in the detection and differential diagnosis of giant renal AML.

Renal epithelioid angiomyolipoma: computed tomography manifestation and radiologic-pathologic correlation depending on different epithelioid component percentages.

Renal epithelioid angiomyolipoma (EAML) is a rare and potentially malignant mesenchymal lesion mainly composed of epithelioid cells. Although some case reports or small case series have been published, the computed tomography (CT) manifestations and radiologic-pathologic correlation depending on different epithelioid component percentages have not been studied before. To investigate the CT manifestation and radiologic-pathologic correlation between renal EAML and angiomyolipoma (AML) with epithelioid component. The clinicopathologic and imaging data of 53 patients with an original diagnosis of EAML or AML with epithelioid component were retrospectively collected from three hospitals. All tissue specimens were re-sectioned and re-observed under the microscope. Samples were divided into an EAML group (≥ 80% epithelioid component, n = 25) and AML with epithelioid component group (5% ≤ epithelioid component < 80%, n = 28). Two radiologists reviewed the images in consensus, describing and comparing the CT manifestation, including the long diameter of the tumor, morphology, presence of necrosis or cystic change, hemorrhage, fat, calcification, enlarged blood vessels, and dynamic enhancement pattern according to the Hounsfield unit value of each CT phase between the two groups. The radiologic-pathologic correlation depending on the different percentages of epithelioid component were studied. The long diameter of the tumor, presence of necrosis or cystic change, fat, enhancement pattern, and tumor-to-cortex enhancement ratio of the cortical phase between the two groups were significantly different (z = - 2.932, P = 0.003; χ2 = 18.020, P < 0.001; χ2 = 16.377, P < 0.001; P = 0.020; and T = - 3.944, P < 0.001, respectively). In multivariate logistic regression analysis, the significant predictive factors of EAML included the presence of necrosis or cystic change [odds ratio (OR) 11.864, P = 0.001] and absence of fat (OR 0.095, P = 0.003). Correlation analysis found that the presence of necrosis or cystic change (r = 0.679, P < 0.001) and fat (r = - 0.603, P < 0.001) were both moderately related to the epithelioid component percentage. The combined model based on the presence of necrosis or cystic change and absence of fat yielded the best diagnostic performance in discriminating EAML and AML with epithelioid component with the highest area under the curve (0.887). EAML has characteristic CT signs; these characteristic CT signs are closely related to the epithelioid component percentage. The presence of necrosis or cystic change and the absence of fat were independent predictors of EAML.

Epithelioid angiomyolipoma of the pancreas: A case report and review of the literature

BACKGROUNDAngiomyolipomas (AMLs), belonging to the family of mesenchymal tumors, are considered benign lesions that occur mostly in the kidney or as a part of tuberous sclerosis. Epithelioid AML (EAML) is a rare type of AML that appears to have malignant potential. Extrarenal AMLs usually occur in the liver according to the retrieved literature reports. There have been only two previous reports of monofocal primary AML of the pancreas; however, no cases of primary monotypic EAML of the pancreas have been reported.CASE SUMMARYAn asymptomatic 59-year-old woman incidentally revealed a tumor during abdominal ultrasound examination. Routine blood tests and physical examination were within normal limits. Abdominal ultrasound revealed a 1.9-cm hypoechogenic mass in the tail of the pancreas, clearly visualized by endoscopic ultrasound. However, contrast-enhanced abdominal computed tomography scans did not demonstrate the lesion. A subsequent gadolinium-enhanced magnetic resonance imaging scan showed that the lesion had some characteristic manifestations. The lesion was initially thought to be a neuroendocrine tumor (asymptomatic PanNET). After surgical resection, histopathology and immunohistochemistry confirmed the diagnosis of EAML. At the 6-mo follow-up, no recurrence, spread, or metastasis was identified on computed tomography or magnetic resonance imaging.CONCLUSIONThe preoperative diagnosis of pancreatic AML is extremely difficult. Imaging techniques are essential for providing valuable morphological features for differential diagnosis.

Primary soft tissue angiomyolipoma in a dog with unusual clinical features

Angiomyolipoma is an extremely rare neoplasm in animals. It belongs to a group of perivascular epithelioid cell tumours (so-called PEComas). This study reports a case of primary soft tissue angiomyolipoma in a dog with some unusual clinical features. A 4-year old female Labrador dog with a rapidly growing pelvic tumour measuring 30 × 20 cm with a short history of 12 weeks was presented. The tumour was well-circumscribed and pushed into the right vaginal wall and into the perineum. The tumour was completely surgically excised. An extensive histological examination was performed, including immunohistochemical analysis. Histology revealed a mesenchymal neoplasm consisting of three tissue components – mature adipose tissue (which dominated), vessels, and smooth muscle (spindle) cells. The lesion showed positive immunohistochemical staining with smooth muscle actin and desmin in mature-appearing smooth muscle cells and S-100 protein positivity in adipocytes. The diagnosis of angiomyolipoma must be considered in different types of benign and malignant tumours of various lineages. The diagnostic approach to soft tissue tumours, including angiomyolipoma, requires optimal processing and sectioning of resected specimens. This paper is believed to be the first case of primary soft tissue angiomyolipoma reported in the veterinary literature.

RENAL ANGIOMYOLIPOMAS: ANALYSIS OF 15 CASES

Giriş: Anjiomyolipom (AML) matür yağ dokusu, kalın duvarlı damar yapıları, düz kas dokusu ve melanin sentezleyen özel miyoid hücrelerin değişen oranlarda gözlendiği benign mezankimal bir tümördür. Bu makalede 2008-2018 yılları arasında bölümümüzde renal AML tanısı almış farklı klinik ve mikroskopik özellikleri olan olgular ile patolojide tanı koymada yararı olacak güncel bilgileri sunmayı amaçladık. Gereç ve Yöntemler: Tıbbı Patoloji Anabilim Dalımızda 2008-2018 yılları arasında tanı almış 15 Renal AML olgusu retrospektif olarak değerlendirilmiştir. Bulgular: Onbeş olgunun 2’si erkek, 13’ü kadın olup, yaşları 37-74 (ortalama yaş 59±10) arasında değişmekteydi. Boyutları 0,7- 15 cm arasında, ortalama 5.1±3,2 cm idi. Olgulardan birinde tüberoskleroz olduğu tespit edildi. Yedi olguda kapsül bütünlüğünde kayıp ve bir olguda perforasyon ile uyumlu alanlar izlendi. Mikroskobik incelemede 13 olguda matür yağ dokusu, kalın duvarlı damar yapıları ve iğsi epiteloid hücreler izlendi. Dört olgu yağ dokudan zengin, 1 olgu iğsi epiteloid hücrelerden zengin ve 3 olgu damar yapılarından zengindi. Sonuç: Renal AML farklı klinik özellikler, mikroskopik ve makroskopik bulgular ile karakterize bir tümördür. İnsidental olarak saptanabileceği gibi çok büyük boyutlara ulaşıp kanama ve perforasyon gibi komplikasyonlarla tespit edilebilir. Tüm komponentler her zaman birlikte bulunmayıp belirli komponentlerin baskın olabileceğini ayırıcı tanıda akılda tutmak önemlidir.Anahtar kelimeler: Anjiomyolipom, tüberoskleroz, böbrek

Angiomyolipoma managed by partial nephrectomy: Outcomes and follow-up

Angiomyolipoma (AML) is a benign neoplasm and consists of thick-walled poorly organized blood vessels, smooth muscle, and varying levels of mature adipose tissue. We evaluated the impact of clinical characteristics, particularly tumor diameter, and surgical treatment in the form of partial nephrectomy on the outcome of sporadic renal AML. Patients undergoing partial nephrectomy during the period Jan 2000 to Dec 2016 with a final diagnosis of angiomyolipoma formed the study group. Patient demographic data was collected, and data from during and after surgery was also reviewed. The median age of the patients was 51 (38-55) years. At presentation, most patients were asymptomatic (6/8, 75%) with the AML presenting as an incidental finding on imaging done for other reasons. There was classical triphasic AML, consisting of elements of adipose tissue, smooth muscle and abnormal blood vessels, in 5/10 tumours (50%). The more aggressive epitheliod variant AML was present in 2/10 (20%). The median follow-up of the patients was 38 (29-64) months. During the follow-up period one (12.5%) patient who had two lesions progressively showed evidence of rising creatinine and decreasing creatinine clearance. AML is a benign renal neoplasm and should be treated initially conservatively. Surgical intervention when required should be nephron sparing so as to reduce the incidence of perioperative complications, loss of renal units, and development of CKD.

Diagnostic Performance of MRI in the Detection of Renal Lipid-Poor Angiomyolipomas: A Systematic Review and Meta-Analysis.

Background Lipid-poor angiomyolipomas (AMLs) are challenging to differentiate from other renal lesions at imaging and often necessitate biopsy or surgery. If sufficiently accurate, MRI may play a role as a replacement test for biopsy. Purpose To perform a systematic review to evaluate the diagnostic performance of MRI for lipid-poor AMLs in patients with renal masses. Materials and Methods A systematic review of MEDLINE, EMBASE, Scopus, the Cochrane Library, and the "gray literature" (conference proceedings) was performed without language restriction through July 18, 2019, with the assistance of a health sciences librarian. Original articles with more than 10 patients evaluating the diagnostic performance of MRI, with histopathologic findings used as the reference standard, for the diagnosis of lipid-poor AMLs in patients with renal masses were included. Studies including AMLs with macroscopic fat and studies with insufficient data were excluded. Patient, clinical, MRI, and diagnostic performance parameters were independently acquired by two authors. Meta-analysis was performed by using a random-effects or bivariate mixed-effects regression model depending on the number of studies. Risk of bias of individual studies was evaluated by using Quality Assessment of Diagnostic Accuracy Studies-2. Results Twenty-three studies with 2196 patients and 25 contingency tables were included. The pooled sensitivity, specificity, and area under the receiver operating characteristic curve were 83% (95% confidence interval [CI]: 72%, 90%), 90% (95% CI: 84%, 94%), and 0.93 (95% CI: 0.91, 0.95), respectively. Considerable variability was present for several variables, including MRI parameters; however, subgroup analysis did not identify MRI sequence or field strength as sources for variability. All studies were at high risk of bias for index test domain because no reported thresholds were prespecified. Conclusion MRI shows promising accuracy for detecting lipid-poor angiomyolipomas (area under the receiver operating characteristic curve, >0.9), indicating a potential role as a replacement test for biopsy in selected patients. Studies evaluating MRI accuracy with a pragmatic algorithm and prespecified threshold may be helpful to confirm this potential role in the management pathway. © RSNA, 2020 Online supplemental material is available for this article.