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Renal Angiomyolipoma In A Solitary Functioning Kidney Discovered Post Partum.

机译:产后发现肾功能性肾功能不全。

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Background:Though rare, angiomyolipoma (AML), is the most common mesenchymal tumour of the kidney. AML is a benign tumour composed of varying amounts of mature adipose tissue, smooth muscle and thick walled blood vessels. The incidence in the general population is between 0.07% and 0.3%. . The association between renal AML and pregnancy is a rare one. The first case of renal AML associated with bleeding in pregnancy was reported in 1964. Since then, only 15 cases have been published in the literature. This is the first report in the literature of a large asymptomatic angiomyolipoma in a solitary functioning kidney found immediately post partum in a 28 year old female.Case Presentation:A 28 year old female was referred to the general surgeons from the labour ward 24hrs post partum with a non tender mass palpable in the right flank detected on routine examination. An ultrasound scan of the abdomen revealed a mass of mixed echogenicity involving and expanding the upper pole of the right kidney. In addition the left kidney was atrophic and non functioning (subsequent radionuclide scan). Consequently a decision to biopsy under ultrasound guidance was made. Histology confirmed a mixed mesenchymal tumour of mature fat, smooth muscle and vasculature, confirming the diagnosis of AML. The patient remained asymptomatic with no deterioration in renal function. She was then referred to a tertiary referral centre for selective embolization of the feeding vessels following which the patient made a complete recovery.Conclusions:The specific risk of renal AML and pregnancy is that of spontaneous haemorrhage and rupture. Although most AML are benign and asymptomatic, the literature suggests that symptoms develop in up to 80% of patients when tumour size reaches 4cm or greater. The most severe and life threatening symptom is that due to haemorrhage. Treatment options for AML include conservative and interventional (total/partial nephrectomy, cryoptherapy, embolization).The current belief is that tumour size should determine whether prophylactic treatment is offered to women of child bearing age due to the possible dangerous (predominantly haemorrhagic) events that may occur in pregnancy. This case provides evidence that it may be possible to continue pregnancy and achieve a normal vaginal delivery when treated conservatively. Patients with angiomyolipoma who intend on becoming pregnant should be counselled about the risks and the option of treating these tumours prophylactically also discussed. Background Though rare, angiomyolipoma (AML), is the most common mesenchymal tumour of the kidney. Initially described by Grawitz in 1900, AML is a benign tumour composed of varying amounts of mature adipose tissue, smooth muscle and thick walled blood vessels1. The incidence in the general population is between 0.07% and 0.3%. Clinically, two distinct presentations of renal AML are usually seen. Between 40 and 80% of patients with tuberous sclerosis have renal AML, which tend to be multiple, bilateral, asymptomatic and often less than 2cm in diameter2. However, approximately 50% of symptomatic AMLs occur in patients who do not have tuberous sclerosis. These tumours usually are single and unilateral. The association between renal AML and pregnancy is a rare one. The specific risk of spontaneous haemorrhage and rupture poses a difficult diagnostic and therapeutic challenge. The first case of renal AML associated with bleeding in pregnancy was reported in 1964. Since then, only 15 cases have been published in the literature3,4,5. There are however no cases in the literature described in the immediate post partum period. This is the first report in the literature of a large asymptomatic angiomyolipoma in a solitary functioning kidney found immediately post partum in a 28 year old female. We review and discuss renal AML in pregnancy, along with its diagnostic and therapeutic challenges. Case History A 28 year old female was referred to the general surgeons fr
机译:背景:血管平滑肌脂肪瘤(AML)虽然很少见,但却是最常见的肾脏间质瘤。 AML是一种良性肿瘤,由不同数量的成熟脂肪组织,平滑肌和厚壁血管组成。普通人群的发病率在0.07%至0.3%之间。 。肾AML与妊娠之间的关联是罕见的。 1964年报告了首例妊娠出血相关的肾性AML。此后,文献中仅发表了15例。这是文献中关于一名28岁女性在产后立即发现的孤立无功能肾大无症状性血管平滑肌脂肪瘤的文献报道。病例报告:一名28岁女性在产后24小时被转诊给劳动病房的普通外科医师常规检查发现右胁可触及非触痛性肿块。腹部超声检查显示大量混合回声,累及右肾上极。另外,左肾萎缩且无功能(随后的放射性核素扫描)。因此,决定在超声引导下进行活检。组织学证实是成熟脂肪,平滑肌和脉管系统的间质混合瘤,确诊为AML。该患者无症状,肾功能未见恶化。然后,她被转诊到三级转诊中心进行选择性的栓塞供血血管,之后患者完全康复。结论:肾AML和妊娠的特殊风险是自发性出血和破裂。尽管大多数AML是良性和无症状的,但文献表明,当肿瘤大小达到4cm或更大时,多达80%的患者会出现症状。最严重和威胁生命的症状是出血引起的。 AML的治疗选择包括保守治疗和介入治疗(全肾/部分肾切除术,冷冻疗法,栓塞术)。目前的看法是,肿瘤的大小应确定是否由于可能发生的危险(主要是出血性)事件而对育龄妇女提供预防性治疗可能发生在怀孕期间。该病例提供的证据表明,如果保守治疗,则有可能继续怀孕并实现正常的阴道分娩。打算怀孕的血管平滑肌脂肪瘤患者应被告知其风险,并应讨论预防性治疗这些肿瘤的选择。背景技术血管平滑肌脂肪瘤(AML)尽管很少见,但却是最常见的肾脏间质瘤。 AML由Grawitz于1900年首次描述,是一种良性肿瘤,由不同数量的成熟脂肪组织,平滑肌和厚壁血管组成1。普通人群的发病率在0.07%至0.3%之间。在临床上,通常会看到两种不同的肾AML表现。结节性硬化症患者中有40%至80%患有肾性AML,多发性,双侧,无症状且直径通常小于2cm2。但是,约有50%的症状性AML发生在没有结节性硬化症的患者中。这些肿瘤通常是单发的和单侧的。肾AML与妊娠之间的关联是罕见的。自发性出血和破裂的特定风险带来了困难的诊断和治疗挑战。 1964年报告了首例妊娠出血相关的肾性AML。从那时起,文献15、3仅发表了文献3、4、5。但是,在产后立即没有文献记载。这是文献中首次报道一名28岁女性在产后立即发现孤立的功能正常的肾中无症状的大血管平滑肌脂肪瘤。我们审查和讨论妊娠肾AML,以及其诊断和治疗挑战。病史一名28岁的女性被转诊至全科医生

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