Renal Angiomyolipoma Presenting Research Articles

Management Outcomes of Large Renal Angiomyolipoma Presenting with Wunderlich Syndrome-Experience from a Tertiary Center.

Renal angiomyolipoma is an uncommon, benign-mixed mesenchymal tumor consisting of thick-walled blood vessels, smooth muscles, and mature adipose tissues. Twenty percent of these tumors are associated with tuberous sclerosis. Wunderlich syndrome (WS), an acute nontraumatic spontaneous perirenal hemorrhage, can be a presentation of large angiomyolipoma. This study evaluated the presentation, management, and complications of renal angiomyolipoma with WS in eight patients who presented to the emergency department between January 2019 and December 2021. The presenting symptoms included flank pain, palpable mass, hematuria, and bleeding in the perinephric space on computerized tomography. Demographic data, symptoms at presentation, comorbidities, hemodynamic parameters, the association with tuberous sclerosis, transfusion requirements, need for angioembolization, surgical management, Clavien-Dindo complication, duration of hospital stay, and 30-day readmission rates were evaluated. The mean age of presentation was 38 years. Of the eight patients, five (62.5%) were females and 3(37.5%) were males. Two (25%) patients had tuberous sclerosis with angiomyolipoma, and three (37.5%) patients presented with hypotension. The mean packed cell transfusion was three units, and the mean tumor size was 7.85 cm (3.5-25 cm). Three of them (37.5%) required emergency angioembolization to prevent exsanguination. Embolization was unsuccessful in one patient (33%) who underwent emergency open partial nephrectomy, and one (33%) patient developed post-embolization syndrome. A total of six patients underwent elective surgery-four underwent partial nephrectomy (laparoscopic - 1, robotic - 1, open - 2) and two underwent open nephrectomy. Three patients encountered Clavien-Dindo complications (Grade 1, n = 2 and IIIA, n = 2). WS is a rare, life-threatening complication in patients with large angiomyolipoma. Judicious optimization, angioembolization, and prompt surgical intervention will help deliver better outcomes.

Ruptured Renal Angiomyolipoma Presenting with Isolated Flank Pain: A Case Report

Ruptured Renal Angiomyolipoma Presenting with Isolated Flank Pain: A Case Report

Bleeding renal angiomyolipoma presenting as duodenal obstruction

We report a case of a 60-year-old woman who had a delayed presentation of duodenal obstruction as a result of a bleeding right renal angiomyolipoma (AML) with retroperitoneal hematoma. Her duodenal obstruction did not improve upon conservative management, and a computed tomography (CT)-guided drainage of the retroperitoneal hematoma was subsequently performed. Post-intervention, CT scan confirmed hematoma resolution, and she was able to resume normal diet afterwards. We present this first reported case of a bleeding renal AML with retroperitoneal hematoma causing duodenal obstruction and discuss on the management of such condition.

Angiomyolipoma presenting as renal abscesses: a consideration for further evaluation of renal abscess

This is to describe an usual presentation of renal angiomyolipoma as renal abscess This is a case report of a 51 year old man with clinical and radiological features suggestive of renal abscess. The patient had exploratory laparatomy and a simple nephrectomy for a non functioning kidney which was more like a bag of pus. The histological report however revealed renal abscess coexisting with angiomyolipoma supporting previous reports that renal abscess may be a harbinger of other renal pathologies. Abscesses are usually managed by drainage and the dictum of 'wherever there is pus let it out' holds generally but this does not always suffice in renal abscesses because renal abscesses are often found in the background of other diseases which may be of more clinical importance.

Renal Angiomyolipoma Presenting as Fever of Unknown Origin

A 50 yr man presented with fatigue and fever. He was found to have a 7.2 cm left renal mass. Radical nephrectomy was done. The mass was HMB-45 negative renal angiomyolipoma. This patient did not have tuberous sclerosis. His fever resolved after the tumor excision. This case is unique because of the unusual morphology and histology of the tumor. It reiterates that getting rid of inflammatory cytokines being produced by the tumor will cause resolution of fever.

Vaginal Mass With Urinary Incontinence: A Rare Presentation

To our knowledge, there are only few published cases of benign renal AML presenting with tumor thrombus in females. We present a new case of this uncommon complication of a benign renal tumor.Epithelioid angiomyolipoma is a recently described rare variant of renal angiomyolipoma.It can occur in patients with or without tuberous sclerosis, and may potentially bemalignant. Benign renal angiomyolipoma (AML) rarely presents with evidence of extension into the renal vein, inferior vena cava (IVC) or atrium. We report a case of a benign renal AML with a tumor thrombus extending into the IVC in a 46-year-old female who presented with right-sided flank pain associated with a right sided abdominal mass. Right Radical nephrectomy with IVC tumor thrombectomy was done. Patient is totally asymptomatic. At 1 month after surgery, an abdominal ultrasound showed no evidence of thrombus within the IVC. CT scan of the abdomen at 3 months post-operatively showed no evidence of recurrence. Surgical treatment of angiomyolipoma with IVC thrombus is warranted in view of risk of malignancy and to prevent tumor embolus to the heart or lungs.

Life threatening intraperitoneal hemorrhage with abdominal compartment syndrome: Unusual presentation of renal angiomyolipoma

Life threatening intraperitoneal hemorrhage with abdominal compartment syndrome: Unusual presentation of renal angiomyolipoma

Endovascular Therapy for Patients with Renal Angiomyolipoma Presenting with Retroperitoneal Haemorrhage

We report our experience treating four patients with acutely bleeding angiomyolipoma (AML) of sizes between 4 and 12 cm who were managed with endovascular embolisation with a mean follow-up of 10 months. In our case series, we demonstrate that endovascular embolisation in the acute setting for bleeding AMLs is a viable treatment option. AML should be in the differential diagnosis of acutely bleeding renal masses, even when there is no fat assessed by computed tomography (CT) imaging in the renal mass.

Multifocal renal angiomyolipoma presenting as massive intraabdominal hemorrhage

Angiomyolipomata of the kidney are unusual lesions composed of abnormal thick walled blood vessels, smooth muscle and adipose elements. These are asymptomatic and occasionally present with flank pain, a palpable mass or gross hematuria. They may be associated with tuberous sclerosis. The risk of bleeding is increased with size, and lesions greater than 4 cm have more than 50% chance of significant bleeding. An unusual case of multifocal renal angiomyolipoma associated with tuberous sclerosis and presenting as massive intra abdominal hemorrhage is reported.

Spontaneous intraperitoneal haemorrhage of a renal angiomyolipoma presenting with an acute abdomen

A 36 year old male patient presented to the emergency department with pain over the right iliac fossa and 2 episodes of fainting over the past 2 days. He was found to have tenderness and rigidity over the right side of the abdomen, tachycardia and severe pallor. A CT scan suggested features of lacerated right kidney. An exploratory laparotomy was performed.1.5 litres of intra peritoneal blood was found followed by a huge haemorrhagic mass involving the kidney. A right sided nephrectomy was performed and the specimen was sent for histopathological examination. The biopsy report stated that it was an angiomyolipoma. Angiomyolipomas are benign lesions found in the kidney and are usually asymptomatic. Retroperitoneal haemorrage can occur but intraperitoneal haemorrhage though extremely rare is a dangerous complication.

Polycythemia vera as a presentation of renal angiomyolipoma: a case report

IntroductionAngiomyolipoma is a common benign renal tumor composed of thick-walled blood vessels, smooth muscle, and adipose tissue. It may be found incidentally during workup for suspected renal disease. Although angiomyolipoma may present as a palpable, tender renal mass with flank pain and gross or microscopic hematuria, many patients are asymptomatic. Erythrocytosis is an unusual presentation, and malignant transformation may be suspected. This report describes a rare case of a woman diagnosed with renal angiomyolipoma and polycythemia vera. The report discusses the differential diagnosis using erythropoietin, erythropoietin-receptor and Janus kinase 2.Case presentationA 79-year-old Chinese woman was diagnosed with erythrocytosis according to World Health Organization criteria. An upper left renal pole angiomyolipoma was successfully ablated after multiple phlebotomy treatments. Red cell count immediately returned to normal, but gradually increased after 4 months. Polycythemia vera was finally diagnosed by positive mutation of Janus kinase 2 and negative erythropoietin protein expression. Her clinical symptoms improved with regular phlebotomy and hydroxyurea treatment.ConclusionConcurrent occurence of angiomyolipoma and polycythemia vera is rare. Polycythemia vera can be easily missed. Polycythemia vera can be confirmed with high specificity and sensitivity by the acquired somatic mutation. Surgical intervention for this renal tumor should be avoided unless malignancy or renal cell carcinoma is suspected or to prevent spontaneous rupture of larger tumors.

UP-1.014: Spontaneous Bleeding from Renal Angiomyolipoma Presenting with Haemoperitoneum

UP-1.014: Spontaneous Bleeding from Renal Angiomyolipoma Presenting with Haemoperitoneum

Renal angiomyolipoma with inferior vena caval thrombus in a 32-year-old male

Renal angiomyolipoma (AML) rarely presents with evidence of extension into the renal vein, inferior vena cava (IVC) or atrium. We report a case of a renal AML with a tumor thrombus to the IVC in a 32-year-old male. The patient subsequently underwent a right radical nephrectomy with IVC tumor thrombectomy. To our knowledge, there are four published cases of renal AML presenting with tumor thrombus in males. This case report describes the management of the youngest male ever to develop a renal AML with IVC tumor thrombus.

Renal angiomyolipoma presenting with massive retroperitoneal haemorrhage due to deranged clotting factors: a case report

BackgroundAngiomyolipomata of the kidney are unusual lesions composed of abnormal vasculature, smooth muscle, and adipose elements. They may be associated with tuberous sclerosis and occasionally present with flank pain, a palpable mass, and gross haematuria. As angiomyolipomata grow their risk of bleeding increases, with a greater than 50% chance of significant bleeding in lesions > 4 cm; anticoagulant therapy accentuates this risk.Case presentationA case of massive retroperitoneal haemorrhage in a patient on warfarin is presented. The underlying diagnosis of renal angiomyolipoma was diagnosed based on CT findings. Emergency resuscitation and selective interpolar arterial embolization was performed which saved the patient's life as well as his kidney.ConclusionThis case illustrates the clinical scenario of massive retroperitoneal haemorrhage in an anticoagulated patient with renal angiomyolipomata. In the emergent situation, adequate resuscitation along ABC principles, as well as control of haemorrhage with either nephrectomy (partial or radical), non-selective renal arterial embolization, or selective embolization of the feeding vessel(s), is necessary. For this to occur, it is imperative to consider the diagnosis early in warfarinized patients (and others at risk of bleeding) who present with abdominal pain. The authors hope this case report highlights to readers the clinical scenario of massive retroperitoneal haemorrhage in anticoagulated patients with renal angiomyolipomata so that they can deal appropriately with such presentations.

Angimiolipoma renal como causa de shock hemorrágico agudo

Kidney Angiomyolipoma as Aetiology of Haemorragic ShockThis is a case of retroperitoneal hematoma and hypovolemic shock as a presentation of renal angiomyolipoma, a rarely benign and asymptomatic tumor.The massive and spontaneous bleeding of the tumor was diagnosed by an abdominal ecography and confirmed by an abdominal computer tomography in the emergency room.Although that kind of tumor needs non-aggressive treatment, as a general rule, because of the size and severity of the following symptoms, the patient has been submitted to a selective embolization of the renal mass when he was hemodinamically stabilized.The evolution was favorable; it was not necessary to do more aggressive surgical handling neither to urgently remove the angiomyolipoma.

Spontaneous Hemorrhage from Renal Angiomyolipoma Presenting with Fever of Unknown Origin

Spontaneous Hemorrhage from Renal Angiomyolipoma Presenting with Fever of Unknown Origin

Renal Angiomyolipoma Presenting as Acute Abdominal Emergency: A Case Report

A 47-year-old woman presented as an acute abdominal emergency with rapid progression to shock following spontaneous rupture of a left renal angiomyolipoma. The diagnosis was missed clinically and on computerized tomographic scan. At different stages of management based on her pattern of symptoms, signs and radiologic appearances of her lesion, she was thought to have ruptured ectopic pregnancy, torsion of a left ovarian cyst, acute salpingitis, spontaneous rupture of the spleen, leaking abdominal aortic aneurysm and eventually left renal cell carcinoma until the histology of her left nephroureterectomy specimen became available. Even though a rare entity, with a high index of suspicion and a good knowledge of the pathognomonic features on ultrasound and computerized tomographic scanning, the diagnosis should be more easily made to facilitate quicker decisions on management. (Nig J Surg Res 2001; 3: 181 – 184) KEY WORDS: Renal Angiomyolipoma, Acute Abdomen

Ruptured renal angiomyolipoma presenting as renal colic

The case of a patient with acute onset of flank pain and hematuria is presented. Initial therapy was directed toward relief of pain believed to be caused by renal colic. It was not until the patient developed atypical features that the true diagnosis, ruptured renal angiomyolipoma, was discovered. The case and discussion emphasize the need to carefully consider a complete differential diagnosis when evaluating patients with flank pain and hematuria who have atypical clinical features or an atypical course.

Huge Renal Angiomyolipoma Presenting with Bleeding in a 76-Year-Old Woman

Renal angiomyolipoma is a rare, benign and hypervascular tumour. Its symptoms may be acute, with severe bleeding, or protracted, few and recurrent, due to bleeding and pressure. A symptomatic case is presented. The patient seems to be the oldest on record, and with the longest-lasting symptoms and the biggest tumour.

Angiomyolipoma presenting as fever of unknown origin

A case of renal angiomyolipoma presenting as fever of unknown origin unassociated with tuberous sclerosis is described. Important features include (1) infrequent occurrence in absence of tuberous sclerosis, (2) angiographic appearance suggesting neoplasm, (3) absence of documented malignant tissue transformation, and (4) presenting symptoms most frequently included abdominal pains, palpable flank mass and hematuria. Surgical removal of the lesion in this instance was associated with cessation of fever.