Giant Angiomyolipoma Research Articles

Therapeutic Potential of Everolimus on Core Autism Symptoms and Increasing Serum Ceruloplasmin and Transferrin Levels in a Pubescent Boy with Tuberous Sclerosis

Background: The neuropsychiatric clinical manifestations of tuberous sclerosis complex (TSC) include social and behavioral impairment, similar to the core symptoms of autism spectrum disorder (ASD), as well as subependymal giant cell astrocytomas (SEGAs) and renal angiomyolipomas (AMLs). Case report: The present study examined the clinical effects of 24 weeks of therapy with the mTOR inhibitor everolimus on the social and behavioral symptoms that are similar to the core ASD symptoms and neurobiological mechanisms underlying the effects of everolimus in an 11-year-old boy with TSC. After the initial physical and psychological screening and Magnetic resonance imaging (MRI) examinations, the patient received 4.4 mg/day everolimus for 24 weeks. To study neurobiological mechanism, we examined the relationship between the efficacy of everolimus and serum levels of superoxide dismutase (SOD) and the copper- and iron-binding antioxidants ceruloplasmin (Cp) and transferrin (Tf). Results MRI results revealed a Subependymal giant cell astrocytoma (SEGA) located at the foramen of Monro and a renal angiomyolipoma (AML) on the patient’s kidney. Everolimus remarkably improved the patient’s social and behavioral impairments over the course of the 24-week treatment without apparent reduction in the size of the SEGA and AML. Serum Cp and Tf levels were gradually increased in response to the improvement in symptoms. Conclusion: As everolimus increased antioxidant capacity and elevated serum VEGF levels, this study firstly revealed that increased antioxidant activity related to copper and iron may contribute to the remarkable improvement in the core social and behavioral ASD symptoms caused by 24-week everolimus treatment.

Спонтанный разрыв гигантской ангиомиолипомы почки

Спонтанный разрыв гигантской ангиомиолипомы почки

Effect of everolimus on skin lesions in patients being treated for subependymal giant cell astrocytoma and renal angiomyolipoma: Final 4-year results from EXIST-1 and EXIST-2

Effect of everolimus on skin lesions in patients being treated for subependymal giant cell astrocytoma and renal angiomyolipoma: Final 4-year results from EXIST-1 and EXIST-2

A clinical observation of cool-tip radiofrequency ablation assisted enucleation for giant renal angiomyolipoma

To initially explore the clinical effect of cool-tip radiofrequency ablation combined with enucleation for the giant hamartoma of kidney with narrow base and export-oriented way of growth. The clinical date of 15 patients including 6 male and 9 female with special hamartoma of kidney underwent cool-tip radiofrequency ablation assisted enucleation from July 2011 to October 2014 were reviewed.The median age was 49 years (ranging from 35 to 71 years). There were 6 cases with left renal tumor, 8 cases with right renal tumor and 1 case with solitary kidney tumor.All patients were confirmed by B ultrasound or CT scan, the mean diameter of hamartoma of kidney was 9.7 cm(8.5-12.7)cm, all tumors were located distant from the collecting system and presents with a special way of growth.The preoperative hemoglobin was (129±18)g/L, SCr was (92±41)μmol/L, glomerular filtration rate (GFR) was (32±12)ml·min(-1)·1.73 m(-2). Cool-tip radiofrequency ablation assisted enucleation was technically successful in all patients.The mean operative duration was (115±31)minutes, and the average intraoperative bleeding was (72±21)ml with no blooding transfusion.The postoperative hospital stay was(7±2)days, and the postoperative hemoglobin was(129±18)g/L, SCr (92±41)μmol/L, GFR(30±15)ml·min(-1)·1.73 m(-2). No statistic change of hemoglobin and SCr or glomerular filtration rate after operations(all P>0.05). Postoperative pathology showed that all cases were hamartoma of kidney.During a mean follow-up period of 19.5 months, none of them had local tumor recurrence or chronic renal insufficiency. Cool-tip radiofrequency ablation assisted enucleation is both safe and effective in the treatment of huge hamartoma of kidney with a narrow base and export-oriented way of growth. The short-term follow-up shows a satisfactory therapeutic effect.

Combination of Superselective Arterial Embolization and Radiofrequency Ablation for the Treatment of a Giant Renal Angiomyolipoma Complicated with Caval Thrombus.

This is a case of a 78-year-old male patient with multiple angiomyolipomas of a solitary right kidney. The largest of these tumors (maximum diameter: 13.4 cm) caused significant extrinsic compression of the inferior vena cava complicated by thrombosis of this vessel. Treatment of thrombosis with anticoagulants had been ineffective and the patient had experienced a bleeding episode from the largest right renal angiomyolipoma, which had been treated by transarterial embolization in another institution, 4 months prior to our intervention. Our approach included superselective transarterial embolization of the dominant, right kidney angiomyolipoma with hydrogel microspheres, which was combined, 20 days later, with ultrasonographically guided radiofrequency ablation. Both interventions were uneventful. Computed tomography 2 months after ablation showed a 53% reduction in tumor volume, reduced space-occupying effect on inferior vena cava, and resolution of caval thrombus. Nine months after intervention the patient has had no recurrence of thrombosis or hemorrhage and no tumor regrowth has been observed. The combination of superselective transarterial embolization and radiofrequency ablation seems to be a feasible, safe, and efficient treatment of large renal angiomyolipomas.

A Giant Angiomyolipoma of the Adrenal Gland: A Rare Case Report with Review of Literature

A Giant Angiomyolipoma of the Adrenal Gland: A Rare Case Report with Review of Literature

Successful management of a giant angiomyolipoma with partial nephrectomy

Renal angiomyolipoma (AML) is largely defined as a benign tumor with 3 components: adipose tissue, abnormally thick-walled blood vessels and smooth muscle. Varying sizes of AML has been reported in the literature, with the general consensus that a size greater than 10 cm can be termed a giant AML and are associated with increased complications. This report discusses a case of giant AML with a diameter of up to 24 cm that underwent a successful partial nephrectomy without prior embolization.

A Rare Case Report: A Giant Angiomyolipoma Located in the Small Intestine

A Rare Case Report: A Giant Angiomyolipoma Located in the Small Intestine

Giant renal angiomyolipoma – case report

renal angiomyolipoma is rare benign renal neoplasm composed of variable amounts of adipose tissue, smooth muscle tissue originating from perivascular epithelial cells and blood vessels. the incidence of renal angiomyolipoma is from 0,3% to 3,0%. tumors over 10 cm are diagnosed rare. clinically renal angiomyolipoma is usually associated with tuberous sclerosis complex and is more frequent in women. about 20% of these tumor are seen in patients with the tuberous sclerosis complex and 80% patients with tuberous sclerosis complex develop renal angiomyolipomas. the tumor is diagnosed by ultrasound, computer tomography scan (ct), magnetic resonance imaging (mri), histology and immunohistochemistry. the authors of this article presented a case of a 47-year-old caucasian woman who was admitted to the hospital because of giant renal angiomyolipoma. the patient underwent radical left nephrectomy without complications. the authors performed a literature review on giant renal angiomyolipoma treatment.

Giant renal angiomyolipomas in a patient with tuberous sclerosis.

Giant renal angiomyolipomas in a patient with tuberous sclerosis.

Angiomiolipoma renal gigante y linfangioleiomiomatosis pulmonar esporádica no filiada. A propósito de un caso

Angiomyolipoma (AML) is a benign mesenchymal tumor composed of mature adipose tissue, aberrant blood vessels and smooth muscle, with a prevalence of approximately 1-3% of all renal masses. It has been reported as an isolated presentation, as sporadic pulmonary lymphangioleiomyomatosis and / or in the context of tuberous sclerosis syndrome, with the last 2 contexts being more difficult in terms of diagnosis and management of curative intent. The case is presented of a patient diagnosed with giant renal angiomyolipoma and pulmonary lymphangioleiomyomatosis, diagnosed and managed with left nephrectomy prior to arterial embolization in the Fundación Cardiovascular de Colombia. ConclusionRenal angiomyolipoma is a disease of low prevalence, of incidental diagnosis in most cases, and does not require intervention but rather follow-up by the urologist. However, when faced with patients with large tumors and / or multiple masses, coupled with clinical manifestations, an association with pulmonary lymphangioleiomyomatosis and / or tuberous sclerosis should be kept in mind, as they are entities that are a challenge from diagnosis to treatment by a multidisciplinary team, in order to improve the quality of life of affected patients.© 2013 Sociedad Colombiana de Urología. Published by Elsevier España, S.L. All rights reserved.

A Rare Case Report: A Giant Angiomyolipoma Located in The Small Intestine

A Rare Case Report: A Giant Angiomyolipoma Located in The Small Intestine

Giant angiomyolipoma with Wunderlich's syndrome in a patient with tuberous sclerosis

Giant angiomyolipoma with Wunderlich's syndrome in a patient with tuberous sclerosis

Management of expanding giant renal angiomyolipoma in pregnancy

Management of expanding giant renal angiomyolipoma in pregnancy

Anaesthesia for autotransplantation after extracorporeal nephron sparing surgery for bilateral giant renal angiomyolipoma

Extracorporeal ‘work bench surgery’ with subsequent autotransplantation is a challenge from both anaesthetic and surgical point of view when performed bilaterally or in a solitary kidney. A 28-year-old female with bilateral giant angiomyolipoma of kidneys was taken up for renal autotransplantation. Patient had a huge tumour, which was the largest reported exophytic tumour to be excised by this technique. Both kidneys were operated at an interval of 1 month, under combined general and epidural anaesthesia. Anaesthetic challenges faced during the procedure were maintenance of adequate perfusion of the grafted kidneys, containment of massive blood loss and coagulopathy during the perioperative period. Patient recovered in due course with functioning autotransplanted kidney. A careful pre-operative preparation with intraoperative maintenance of adequate blood volume and blood pressure is the key for graft survival.

Angiomyolipoma of a kidney: A case report

Angiomyolipoma is a rare benign tumour of the kidney. They are composed of abnormal vasculature, smooth muscle and adipose elements. They may be associated with Tuberous sclerosis. The unilateral presentation is uncommon. Angiomyolipoma is presented as an enlarge lump in the abdomen may mimic malignant lesion such as renal cell carcinoma, yet malignancy has to be ruled out. The diagnosis of this condition may not be straight forward with imaging alone. Excision is recommended for definite histopathological diagnosis (in symptomatic patients) and to prevent the potential risk of haemorrhage and malignancy. A case of benign giant Angiomyolipoma is presented here because of its uncommon occurrence.

Everolimus for the Treatment of Subependymal Giant Cell Astrocytoma Probably Causing Seizure Aggravation in a Child with Tuberous Sclerosis Complex: A Case Report

We are reporting on a 13.5-year-old girl with tuberous sclerosis complex (TSC) who was treated with everolimus because of giant cell astrocytoma and bilateral angiomyolipoma. She suffered from pharmacoresistant partial epilepsy with clusters of tonic and tonic-clonic seizures. Treatment with carbamazepine and sulthiame had led to a stable situation for more than 2.5 years. The dosage of everolimus had to be increased and refractory status epilepticus followed after 12 days. In the absence of any other possible cause, we believe that the status epilepticus was provoked by everolimus. So far, only a few cases of possible seizure aggravation by everolimus have been reported. The clinical relevance of possible negative effects in epileptic patients remains unclear. Similar observations should be documented and reported.

Giant Renal Angiomyolipoma: Unusual Cause of Huge Abdominal Mass

We present the imaging and histopathological characteristics of a giant renal angiomyolipoma (AML) in a 49-year-old female patient, who presented with bloating sensation in the abdomen and a steadily increasing abdominal girth for about 3 years. Contrast enhanced computed tomography (CECT) scan films of abdomen revealed that a large fat containing tumor had replaced the left kidney while displacing the rest of the abdominal contents toward the other side of the midline. Intraoperatively the left kidney was completely replaced by a fat containing tumor. The recovered surgical specimen measured 39 cm × 25 cm × 9 cm and weighed 7500 g. Histopathological investigation with immuno-histochemical staining of the specimen with hydroxy beta-methylbutyric acid-45 confirmed this lesion as AML. CECT scan of the head did not show any lesion suggestive of tuberous sclerosis. The giant tumor of the present case is the heaviest AML in both syndromic and sporadic categories and largest by dimensions as sporadic AML ever reported in the literature.

Pulmonary Embolization as the Primary Clinical Manifestation of Giant Renal Angiomyolipoma

Pulmonary Embolization as the Primary Clinical Manifestation of Giant Renal Angiomyolipoma

Renal transplant in a tuberous sclerosis patient with bilateral giant renal angiomyolipomas and concurrent renal carcinoma

Co-existence of angiomyolipoma (AML) and renal cell carcinoma (RCC) in the same tumor mass is very rare and only eight cases have been reported. We present a case of a young female with tuberous sclerosis complex (TSC) with bilateral huge renal AMLs. Both tumors were removed, one of which revealed co-incidental RCC. She was subsequently successfully transplanted a kidney from her brother and is maintaining normal graft function eight months post-transplant. No recurrence or metastases of RCC has been detected till the last follow-up.