Giant Angiomyolipoma Research Articles

Giant renal angiomyolipomas in tuberous sclerosis complex.

Angiomyolipomas are the benign tumours of the kidney which occur either sporadically or in association with tuberous sclerosis complex (TSC). Symptoms typically develop with an increase in the size of the lesion. We present a clinical image of a patient with giant renal angiomyolipomas who had other clinical features of TSC (facial angiofibromas, periungual fibroma, subependymal nodules in the brain, and lung cysts).

A Giant Renal Angiomyolipoma: Case Report and Review of Literature

A Giant Renal Angiomyolipoma: Case Report and Review of Literature

Transarterial embolization in Wunderlich syndrome due to recanalization of giant renal angiomyolipoma pseudoaneurysm: a case report and literature review

BackgroundAcute spontaneous bleeding from renal angiomyolipoma (AML) is one of the causes of Wunderlich syndrome, a rare and potentially fatal clinical condition. Clinical deterioration will occur if there is a delay in urgent management. There are several management options for renal angiomyolipoma rupture. However, until now little is known about the case of recanalization from post-coil embolization of renal angiomyolipoma. There is no guideline about embolization technique for the management of recurrent bleeding after embolization or coil recanalization of renal angiomyolipoma.Case presentationA 55-year-old male has Wunderlich syndrome caused by recurrent bleeding of giant AML of the left renal due to coil recanalization compounded by a pseudoaneurysm and other bleeding site in bilateral giant renal angiomyolipoma which is showed by contrast-enhanced abdominal computed tomography scan. The patient underwent urgent transarterial embolization and some blood transfusion. Clinical improvement occurred and the patient discharged several days later.ConclusionsEmbolization for spontaneous bleeding or rebleeding of renal pseudoaneurysms may become the first choice of treatment in bilateral multiple renal angiomyolipoma rather than other managements which are available to preserve renal function.

A Life-threatening Case of Giant Bilateral Renal Angiomyolipoma: A Case Report

Renal angiomyolipoma (AML) is a rare tumor with an incidence of 0.3% - 3%. We reported a case of a 41-year-old male patient who presented with gross hematuria and hemorrhagic shock, due to a right giant angiomyolipoma he underwent urgent right nephrectomy by subcostal laparotomy, total weight of the mass was 6 Kg, histological examination concluded in a renal angiomyolipoma. Treatment of renal AML depends on the clinical presentation, tumor size, and single or multiple lesions: single small (< 4 cm) asymptomatic lesions require only clinical and radiological follow-up, however giant symptomatic (hematuria), life-threatening masses require urgent multidisciplinary treatment and especially surgery. Giant renal bilateral AML is very rare, conservative treatment in the absence of hemorrhage should always be first proposed to preserve renal function as possible.

The Difficulties of Diagnosis Giant Angiomyolipoma of the Liver: Clinical Case and Literature Review

Purpose: To report a clinical case demonstrating difficulties of diagnosis and successful surgical treatment of myomatous type of angiomyolipoma of the liver without simultaneous damage to the kidneys.Material and methods: A female patient, 49 years old, was admitted to the hospital in August 2023, had no specific complaints, an abdominal ultrasound revealed extraperitoneal extraorgan mass in the epigastrium and mesogastrium and masses in the right lobe of liver, most likely hemangioma. The contrast-enhanced MSCT was performed on admission. Patient was rehospitalized for a surgery in September. During the operation the tumor was removed and sent for histological examination, a further immunohistochemical study was also carried out.Results: According to the results of MSCT in S3, S4, S5 of the liver oval-shaped mass (198×104×177 mm) with heterogeneous density and solid, cystic and fat features was found. Three hypovascular hypodense inclusions sizes up to 11 mm was also identified in S7/8. There was no pathologic formation in the kidneys. The liver tumor was surgically removed, the resected material was directed to a scheduled histological examination. To confirm the diagnosis an IHC analysis was also done and it revealed the expression of melanocytic (HBM-45) and smooth muscle (SMA) markers.Discussion: In this case we encountered very sparsely described in the world literature localization of angiomyolipoma — isolated hepatic one (without kidney damage). Like other authors we encountered the similarity of muomatous tumor type to the HCC and adenoma of the liver due to the characteristic features of visualization: hypervascularity, early contrast enhancement into an arterial phase and reduced density relative to the preserved parenchyma of the liver into a delayed phase of contrast.Conclusion: This case demonstrates the difficulty of diagnosing the myomatous type of AML simulating the HCC. As one of the most informative methods of diagnosis, we single out MSCT, which allowed us to think about the possibility of a benign nature of the tumor. In our case the main features excluding HCC were the absence of rapid “washout” of contrast drug, the presence of lipomatous areas and no accumulation of contrast agent by the capsule.

Huge renal angiomyolipoma

Renal angiomyolipoma (AML) is a rare solid benign tumor composed of varying amounts of mature adipose tissue, smooth muscle, and thick-walled vessels. This article presents a clinical observation and immediate results of a two-stage treatment of giant renal AML (preliminary superselective embolization with further partial nephrectomy) in a 58-year-old woman.

Endovascular Treatment and Follow-up of Retroperitoneal Hemorrhage Caused by Bilateral Giant Renal Angiomyolipoma

Endovascular Treatment and Follow-up of Retroperitoneal Hemorrhage Caused by Bilateral Giant Renal Angiomyolipoma

Giant renal angiomyolipomas – Can they be managed safely through minimally invasive selective angioembolisation? Case series and literature review

Objective: The objective was to determine whether giant renal angiomyolipomas (AMLs) can be managed through selective angioembolisation (SA) and compare outcomes to nephron-sparing surgery (NSS) and nephrectomy. Methods: A retrospective case series was compiled from a prospectively maintained database, commenced in 2011 of renal AMLs that underwent SA. We extracted patient demographics, size of AMLs, intervention and outcomes. A literature review of case reports and case series was performed on the management of giant renal AMLs managed through SA, NSS or nephrectomy. Results: Of 30 AMLs that underwent SA, 6 patients met the inclusion criteria. The mean diameter of AMLs prior to embolisation was 14.3 cm. All embolised AMLs decreased in size post-embolisation by an average of 18% (mean: 39-month follow-up). There were no complications in our cohort’s follow-up period, including no rise in any patients’ creatinine associated with SA. Our literature review of 284 articles found 82 articles pertaining to 102 giant renal AMLs. Our review identified SA, NSS and nephrectomy to all be effective management pathways for giant AMLs with minimal complications. Total nephrectomy did result in five patients requiring dialysis post-procedure. Conclusions: SA is an effective intervention for giant AMLs with comparable outcomes to NSS and nephrectomy and should be considered a suitable management option for giant renal AMLs. Level of evidence: Not applicable

Endovascular Management of Ruptured Renal Angiomyolipoma: A Case Report

Renal angiomyolipoma is classified as a benign tumour comprising fat, smooth muscle cells, and vascular tissue. Renal giant angiomyolipoma with rupture is a relatively rare clinical emergency condition. Here, the authors present a case of a 37-year-old female patient who presented with complaints of right flank pain for six hours. The patient was anaemic and did not have haematuria. On non contrast Computed Tomography (CT) imaging, there was a right perirenal haematoma with a mixed-density, fat-containing tumour in the upper interpolar region of the right kidney. On Contrast-Enhanced Computed Tomography (CECT) imaging, the right renal tumour showed multiple abnormal tortuous vessels with moderate to intense enhancement, consistent with a large angiomyolipoma. She was managed with primary therapeutic endovascular embolisation for a ruptured right renal angiomyolipoma under local anaesthesia via a right transfemoral artery approach. The procedure was uneventful, and postembolisation syndrome was managed with medications. The patient was discharged in a stable condition. During the one-month follow-up, the patient’s symptoms improved, and the need for major surgery like nephrectomy was avoided. Super selective embolisation to devascularise the tumour and preserve renal parenchyma was done under local anaesthesia via a right transfemoral approach without any complications. Postembolisation syndrome was managed medically

Case report: A hybrid technique for a safe nephrectomy in a giant kidney angiomyolipoma

BackgroundGiant angiomyolipoma is usually associated with genetic syndromes and complications (spontaneous rupture and bleeding, hematuria, hypertension) and mass-related symptoms (flank and abdominal pain).Case presentationWe present a case of a 20-year-old woman suffering from tuberous sclerosis who was referred to our hospital with a giant angiomyolipoma causing abdominal pain. A contrast-enhanced computed tomography showed a left angiomyolipoma, measuring 28 cm × 17 cm × 27 cm. After a multidisciplinary team discussion, the patient was submitted for a nephrectomy. Percutaneous temporary occlusion of the main renal artery was achieved through an endovascular balloon catheter. Through the balloon catheter guidewire, 2,500 IU of heparin was infused to reduce the risk of tumor vein thrombosis and venous embolism. This allowed a safe kidney manipulation through a left thoracoabdominal approach. The postoperative course was uneventful. Pathology showed a 40 cm × 30 cm × 9 cm and 10 kg AML. One year after surgery, the patient is on follow-up, and her estimated glomerular filtration is 120.5 ml/min/1.73 m2.ConclusionThe present case showed that the endovascular control of the main renal artery could be considered a useful approach to safely managing huge renal masses when renal hilar control is expected to be very difficult.

Spontaneous rupture of an intratumoral pseudoaneurysm in a giant renal angiomyolipoma

We reported a rare case of a spontaneous rupture of an intratumoral pseudoaneurysm in a giant renal angiomyolipoma of a 52-year-old lady. The initial presentation was a sudden onset of right hypochondriac pain, nausea, and vomiting. CT scan revealed large heterogenous exophytic enhancing mass with mixed solid and fat density within, arising from the right kidney likely represent a giant right renal angiomyolipoma. There is associated right perinephric hematoma and active bleeding within the mass. No features suggestive of tuberous sclerosis. Subsequent right renal angiogram revealed a pseudoaneurysm of an inferior segmental right renal artery and emergency embolization was done with successful obliteration of the aneurysmal sac and devascularization of the mass.

A Clinical Challenge Case of Bilateral Giant Renal Angiomyolipoma Associated With Tuberous Sclerosis Complex

A 27-year-old female was referred to the urology clinic because of an incidentally found bilateral giant renal angiomyolipoma associated with tuberous sclerosis complex (TSC-RAML) discovered during workup for menorrhagia. Six years ago, she began to develop a bulging belly, which she thought was due to obesity. She had multiple angiofibromas on her face, multiple toenail fibromas on her toes (Fig. 1). Whole-genome sequencing analysis of plasma revealed pathogenic mutation of TSC2 gene (base change: C.1513C >T;amino acid change: p.R505X; Mutation abundance: heterozygous; GloriousMed Technology, China).

A case of giant nasal septal angiomyolipoma.

Angiomyolipoma is an extremely rare, benign mesenchymal tumor of the nasal cavity, primarily common in the kidney and secondarily common in the liver. According to the author's knowledge, no cases of angiomyolipoma of the nasal septum have been identified to date. We report a case of a patient with a giant angiomyolipoma at the posterior end of the nasal septum who recovered after surgery without any complication.

Giant hepatic angiomyolipoma presenting with severe anemia: A surgical case report and review of literature.

BackgroundAngiomyolipoma (AML) is a solid benign neoplasm with mesenchymal features. The clinical signs and symptoms of hepatic angiomyolipoma are nonspecific, and treatment strategy is variable.PresentationA 35-years-old male patient has admitted to the hospital with symptoms of severe anemia. Abdominal multi-slice computed tomography (MSCT) and Gadoxetic acid (GA)-enhanced magnetic resonance imaging (MRI) revealed a heterogeneous 23 × 17 cm-in-sized tumor with heterogeneous enhancement and increased angiogenesis. Percutaneous hepatic biopsy was proceeded and in immunohistochemistry, tumor cells responded positively to the HMB-45, SMA, and Glutamine stains, the CD-34 stain was positive for blood vessels as well as Ki-67 sporadically positive, but the Heppar1, S-100, CK stains reacted negative. The final pathologic result was consistent with the primary hepatic angiomyolipoma. The final surgical management was extended right hepatectomy with ligation of the right hepatic artery (RHA) and vein (RHV), as well as the middle hepatic vein (MHV), one month after portal embolization as well as to enlarge the remnant liver volume.DiscussionA hepatic angiomyolipoma (HAML) primary origin is relatively rare, the clinical manifestations are variable and non-specific. Histological examination and immunohistochemistry staining are considered as the gold standard for HAML diagnosis. HAML are commonly expressed benign behaviors, but HAML cases with malignant behaviors were reported in a cumulative incidence. Radical surgery must be still the most effective and major treatment approach.ConclusionThe present case being the first case with initial syndrome of severe anemia accounted in the English literature. Giant angiomyolipoma is composed of blood vessels and could lead to extensive internal tumoral hemorrhage. We here present a report of this case with had primary hepatic angiomyolipoma with clinical picture of severe anemia.

ОСОБЕННОСТИ ДИАГНОСТИКИ И ЛЕЧЕНИЯ ГИГАНТСКОЙ АНГИОМИОЛИПОМЫ ПОЧКИ (КЛИНИЧЕСКИЙ СЛУЧАЙ)

Introduction. One of the rare complex diseases in clinical practice is Bourneville-Pringle disease (tuberous sclerosis). It is a genetically determined polysystemic disease with an autosomal dominant type of inheritance, one of the manifestations of which are renal angiomyolipomas. Angiomyolipoma is a benign kidney tumor. It consists of epithelioid, smooth muscle cells and adipose tissue and has abundant abnormal vascularization. Aim. We analyze a complex clinical case in a patient with a giant angiomyolipoma of a single kidney. Material and methods. Patient S., 58 years old, was urgently admitted to the urology department. The patient complained of pain in the left lumbar region, in the left half of the abdomen, blood impurities in the urine. General clinical, laboratory and instrumental research tools were conducted. Results and discussion. According to the spiral CT of the kidneys with contrasting, in the left kidney a giant mass with a violation of its architectonics, with signs of hemorrhage and a formed hematoma in the upper third of the mass and hemorrhagic impregnation of its lower half was found. Surgical treatment was prescribed, during which embolization of the segmental arteries of the left kidney, selective angiography of the left renal artery, total nephrectomy on the left with a successful outcome were performed. Conclusion. On the basis of the given clinical case, it should be concluded that despite the benign nature of the renal angiomyolipoma, the tumor has reached gigantic sizes due to the late treatment of the patient caused by concomitant oligophrenia. In this regard, the late diagnosis has led to life-threatening complications that required immediate decision-making. The given clinical observation shows the success of the chosen surgical tactics in the form of a radical operation that allowed saving the life of the patient with a rare disease. The patient was discharged from the hospital for outpatient follow-up by an urologist and a nephrologist. Regular hemodialysis, monitoring with the help of laboratory and instrumental research tools are prescribed. The patient was put on the waiting list for renal trans- plantation in specialized centers in Russia.

Pulmonary lymphangioleiomyomatosis with an associated giant renal angiomyolipoma.

Pulmonary lymphangioleiomyomatosis (LAM), a rare, progressive disease predominantly affecting the lungs of women of reproductive age, is often associated with renal angiomyolipoma (AML). We report the case of a 29-year-old female patient who presented to our obstetrics department at 37 weeks’ gestation, complaining of abdominal pain, and constipation. Ultrasound noted a viable singleton with a large left-sided abdominal mass. After undergoing a caesarean section, she was referred to our urology department to assess her flank mass further. Computed tomography demonstrated a large, exophytic left renal mass measuring 22 cm x 16 cm x 13 cm, suggestive of an AML and numerous bilateral pulmonary cysts. A diagnosis of LAM and associated unilateral giant renal AML was made. As soon as she had fully recovered from her caesarean section, we removed the huge AML via a standard left-sided open nephrectomy without incident. We report this rare case of giant AML associated with LAM and review the literature about the association of these two conditions.

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 Bilateral Angiomyolipoma Associated with Tuberous Sclerosis of Bourneville: Exceptional Case and Therapeutic Dilemma

Angiomyolipoma (AML) is a mesenchymal tumour composed of variable proportions of adipose tissue and vascular and smooth muscle elements. It can cause potentially life-threatening complications.
 This report aims to describe a bilateral angiomyolipoma associated with tuberous sclerosis of Bourneville; the second aim is to discuss the treatment modalities of this disease.
 A 51-year-old woman with abdominal mass and characteristic triad: Epilepsy, mental retardation and skin lesions (adenoma) (Fig. 1), with no notion of hematuria or abdominal pain, abdominal ultrasonography and computed tomography showing bilateral renal masses of 28.4×17 cm on the left kidney and 22× 11.7 cm on the right, respectively (Fig. 2-3). Serum creatinine was 13.4g/l.
 Body-scan imaging finds cardiac rhabdomyoma (Fig. 4) and brain calcifications (Fig. 5). the patient underwent surveillance with scan imaging every month, and at the last control, she was asymptomatic, serum creatinine was still normal. The management of giant bilateral AML is a complex and multifactorial decision. Patients can knowingly choose an active surveillance program, even with giant AML, but the economic situation and mental status can limit the therapeutic choice.

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.

Deep Learning Plus Three-Dimensional Printing in the Management of Giant (>15 cm) Sporadic Renal Angiomyolipoma: An Initial Report

ObjectiveTo evaluate the feasibility and effectivity of deep learning (DL) plus three-dimensional (3D) printing in the management of giant sporadic renal angiomyolipoma (RAML).MethodsThe medical records of patients with giant (>15 cm) RAML were retrospectively reviewed from January 2011 to December 2020. 3D visualized and printed kidney models were performed by DL algorithms and 3D printing technology, respectively. Patient demographics and intra- and postoperative outcomes were compared between those with 3D-assisted surgery (3D group) or routine ones (control group).ResultsAmong 372 sporadic RAML patients, 31 with giant ones were eligible for analysis. The median age was 40.6 (18–70) years old, and the median tumor size was 18.2 (15–28) cm. Seventeen of 31 (54.8%) had a surgical kidney removal. Overall, 11 underwent 3D-assisted surgeries and 20 underwent routine ones. A significant higher success rate of partial nephrectomy (PN) was noted in the 3D group (72.7% vs. 30.0%). Patients in the 3D group presented a lower reduction in renal function but experienced a longer operation time, a greater estimated blood loss, and a higher postoperative morbidity. Subgroup analysis was conducted between patients undergoing PN with or without 3D assistance. Despite no significant difference, patients with 3D-assisted PN had a slightly larger tumor size and higher nephrectomy score, possibly contributing to a relatively higher rate of complications. However, 3D-assisted PN lead to a shorter warm ischemia time and a lower renal function loss without significant difference. Another subgroup analysis between patients under 3D-assisted PN or 3D-assisted RN showed no statistically significant difference. However, the nearness of tumor to the second branch of renal artery was relatively shorter in 3D-assisted PN subgroup than that in 3D-assisted RN subgroup, and the difference between them was close to significant.Conclusions3D visualized and printed kidney models appear to be additional tools to assist operational management and avoid a high rate of kidney removal for giant sporadic RAMLs.