[A Case of Renal Epithelioid Angiomyolipoma with Recurrence in the Abdominal Cavity].
Maki FujiwaraAkihiro HoshiyamaTakehiro TakahashiYasushi TakashimaYuka KohnoMasakatsu UedaHiroaki KawanishiKazuhiro Okumura
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Abstract:
A 37-year-old man with no symptoms or family history of tuberous sclerosis complex presented to our hospital with abdominal pain in 2013. Abdominal computed tomography (CT) revealed a rupture in the right renal angiomyolipoma (AML) having a maximum diameter of 7 cm. He had undergone a transarterial embolization. Follow-up CT showed an increase in the size of the right tumor to 11 cm, and therefore, right nephrectomy was performed in 2016. The diagnosis of epithelioid AML (EAML) was confirmed. In 2019, he was diagnosed with a solitary tumor near right-sided transverse colon, which was resected and showed recurrence of EAML. He was disease-free 6 months after surgery. EAML has malignant potential, with 30-50% of reported EAML cases resulting in local recurrence or distant metastasis. Previous recurrence or metastasis may occur 0.25-12 years postoperatively. Furthermore, multiple and unresectable recurrences or metastases, arising early in the postoperative period may lead to a poor outcome. Therefore, close and long-term follow-up is required.Keywords:
Angiomyolipoma
Transverse colon
Abdominal cavity
The association between renal carcinoma and angiomyolipoma is rare. Only 14 cases have been reported in the literature. The purpose of this paper is to present an additional case and review the literature on this association.A healthy 42 year old woman was found to have a left flank mass incidentally when she presented for a Papanicolaou smear. The computerised tomography scan revealed a left lower pole renal mass consistent with a renal cell carcinoma. A nephrectomy was performed and the patient recovered uneventfully. The nephrectomy specimen was processed routinely. In addition to haematoxylin and eosin staining, immunohistochemistry for CAM 5.2, vimentin, CD34, antismooth muscle actin, and HMB45 was carried out. Transmission electron microscopy was also performed.Macroscopically, the lower pole of the kidney contained a well circumscribed, non-encapsulated, tan coloured tumour with a large area of central haemorrhage measuring 10.5 cm. In addition, there was a 0.4 cm poorly circumscribed unencapsulated yellow nodule adjacent to the tumour. Microscopically, the larger tumour showed characteristic features of an oncocytoma. Numerous mitochondria were seen on electron microscopy. The smaller yellow nodule was an angiomyolipoma.This paper presents an additional case of oncocytoma associated with angiomyolipoma. Of the 15 cases described in the literature, three were associated with the tuberous sclerosis complex, all from a single study. In tuberous sclerosis, angiomyolipomas are more commonly associated with renal cell carcinoma. If angiomyolipomas are found incidentally in nephrectomy specimens together with other tumours, it is important to exclude tuberous sclerosis retrospectively.
Angiomyolipoma
Nodule (geology)
Clear cell carcinoma
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Wünderlich syndrome (WS) is a spontaneous retroperitoneal hemorrhage confined to the subcapsular or perinephric space without a history of trauma. Since it is a rare condition with a significant mortality rate if not treated timely, it is essential to identify its risk factors and early clinical manifestations for a favorable outcome. Various conditions are associated, but the most common causes are benign and malignant renal neoplasms. We present a 26-year-old female with a history of tonic-clonic seizures who presented to the ED with intense abdominal pain located on the right flank with a palpable mass. Management included IV fluids and blood transfusion. She underwent a right total nephrectomy. She was later diagnosed with tuberous sclerosis. A 44-year-old female with a three-year history of right costovertebral pain and recurrent urinary tract infections that presented to the ED with acute right flank pain was diagnosed with WS secondary to an angiomyolipoma and underwent right total nephrectomy. WS is a very rare pathology that represents a diagnostic challenge for the physician. The treatment will depend on the hemodynamic condition of the patient. Active follow-up should be reserved for those who have small tumors, are asymptomatic, and have hemodynamic stability. Surgical or radiology intervention is reserved for those who are hemodynamically unstable or who have a suspicion of renal cell carcinoma.
Angiomyolipoma
Flank pain
Retroperitoneal space
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Introduction. Angiomyolipomas are the most common benign tumor of the kidney, associated with Tuberous Sclerosis in 20% of cases and arising sporadically in 80% of cases. Renal angiomyolipomas are neoplasms of mesenchymal origin with varying proportions of vasculature, smooth muscle spindle cells, and adipocytes, making management of such neoplasms a challenging endeavor. Possible management options include partial or radical nephrectomy and segmental renal artery embolization. Case Presentation. A 61-year-old woman admitted for a large retroperitoneal hemorrhage was discovered to have a giant, sporadic, 3818.3 g, 30.0 × 26.5 × 18.0 cm left perinephric angiomyolipoma. Given her hemodynamic instability upon presentation, she underwent segmental arterial embolization, followed by an open left partial nephrectomy. Ten-month follow-up revealed no noticeable loss of renal function. Discussion. Literature review revealed occasional renal angiomyolipomas of comparable size, with all angiomyolipomas larger than this requiring treatment with radical nephrectomy. Conclusion. We show that nephron-sparing surgery may be considered in the treatment of even the largest of renal angiomyolipomas.
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Angiomyolipoma
Arterial Embolization
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To explore the diagnosis and treatment of tuberous sclerosis complex complicated with renal angiomyolipoma.The clinical data of 22 patients with tuberous sclerosis complex complicated with renal angiomyolipoma were analyzed retrospectively.There were 12 males and 10 females with a mean age of 23 (1-46) years. All of them had bilateral multiple renal angiomyolipomas. The mean tumor size was 8.5 (0.7-18.0) cm in diameter. The presence or absence of clinical symptoms showed a significant correlation with tumor size.Eight patients with angiomyolipoma under 4 cm in diameter were continuously monitored at an outpatient clinic. There were 8 patients with lesions of 4-10 cm.Five of them underwent nephron-sparing surgeries and another 3 monitored at an outpatient clinic. There were 6 patients with tumor over 10 cm. Three of them underwent transcatheter arterial embolization and one case took rapamycin after embolization.One patient with concurrent renal cell carcinoma underwent partial nephrectomy. Chronic renal failure occurred in one case dying of pulmonary lymphangiomyomatosis with serious hemoptysis. During a mean follow-up of 25 months, neither severe renal hemorrhage nor symptomatic aggravation was found.In the case of rapamycin, there was 10% reduction in the size of angiomyolipoma.Most cases of tuberous sclerosis complex are complicated with bilateral multiple renal angiomyolipoma. The small lesions under 4 cm in diameter may be monitored at an outpatient clinic.For those larger (>4 cm) or symptomatic ones, medication, embolization or surgery is necessary.
Angiomyolipoma
Outpatient clinic
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Tuberous sclerosis complex (TSC) is an autosomal-dominant neurocutaneous disorder involving the skin, brain, kidney, heart and other organs. Renal manifestations are mainly angiomyolipomas, renal cysts and cancer. We report three female patients, mean aged of 31 years (range: 22-37), with a family history of TSC in one case. Cutaneous and nervous symptoms were found in all patients. Clinical complaints were mainly abdominal pain (3 cases), hematuria (2 cases), right flank palpable mass (1 case). Angiomyolipomas (AML) were diagnosed by ultrasonography in all patients, associated to renal cysts in one case. A patient underwent total right nephrectomy without any event during the follow-up. In a second patient, a selective arterial embolization of AML was indicated. Total left nephrectomy was performed due to the persistence of hematuria and the increased flank mass. Pathological examination of the kidney revealed a clear cell carcinoma. The third patient with small AML, associated with renal cysts, required careful monitoring. Renal manifestations in TSC are frequent and serious, they are the second leading cause of death after nervous lesions. Clinical and morphological aspects are variable and different therapeutic indications must be discussed.
Abdominal mass
Angiomyolipoma
Flank pain
Abdominal ultrasonography
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Angiomyolipoma
Flank pain
Retroperitoneal Hemorrhage
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A 49-year-old woman with tuberous sclerosis visited our hospital complaining of left abdominal pain. She had been diagnosed with bilateral renal angiomyolipoma (AML) when she was 32 years old and had been on maintenance hemodialysis since the age of 44. Magnetic resonance imaging showed bilateral giant renal AML and hemorrhage in the AML of the left kidney. Transthoracolumbar left nephrectomy was performed in March 2008. The resected specimen weighed 3.2 kg. The histological diagnosis was consistent with AML. In addition, the Japanese literature on giant renal AML was reviewed and discussed.
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Abdominal mass
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Angiomyolipoma
Macroscopic hematuria
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A 37-year-old man with no symptoms or family history of tuberous sclerosis complex presented to our hospital with abdominal pain in 2013. Abdominal computed tomography (CT) revealed a rupture in the right renal angiomyolipoma (AML) having a maximum diameter of 7 cm. He had undergone a transarterial embolization. Follow-up CT showed an increase in the size of the right tumor to 11 cm, and therefore, right nephrectomy was performed in 2016. The diagnosis of epithelioid AML (EAML) was confirmed. In 2019, he was diagnosed with a solitary tumor near right-sided transverse colon, which was resected and showed recurrence of EAML. He was disease-free 6 months after surgery. EAML has malignant potential, with 30-50% of reported EAML cases resulting in local recurrence or distant metastasis. Previous recurrence or metastasis may occur 0.25-12 years postoperatively. Furthermore, multiple and unresectable recurrences or metastases, arising early in the postoperative period may lead to a poor outcome. Therefore, close and long-term follow-up is required.
Angiomyolipoma
Transverse colon
Abdominal cavity
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