{"title":"Giant renal angiomyolipoma (RAML): A case report and literature review.","authors":"Ahsan Nasim, Adeena Azam, Amna Faheem Mufti, Hafiz Usman Azeem","doi":"10.47391/JPMA.21576","DOIUrl":null,"url":null,"abstract":"<p><p>Renal angiomyolipoma is a benign, rare type of tumour which consists of mature adipose tissue, thick-walled blood vessels, and smooth muscle cells. This tumour can grow to a large size, and is then defined as giant. We are describing the case of a 34-year-old female with a giant renal angiomyolipoma, measuring 32cm x 28cm x 6cm, which caused her pain and constipation. The mass was removed successfully, and partial nephrectomy was done. The patient had an uncomplicated postsurgical clinical course, and renal function was preserved. Renal angiomyolipoma is a hamartomatous, benign mass with variable components. It can occur sporadically or in association with genetic syndromes such as tuberous sclerosis. Symptoms include abdominal pain and constipation. Potentially dangerous sequelae include retroperitoneal haemorrhage and shock. The treatment for these tumours is surgery with open nephrectomy. Other treatment modalities have also been developed. Diagnostic workup should be done for genetic syndromes whenever necessary.</p>","PeriodicalId":54369,"journal":{"name":"Journal of the Pakistan Medical Association","volume":"75 7","pages":"1131-1134"},"PeriodicalIF":0.8000,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of the Pakistan Medical Association","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.47391/JPMA.21576","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"MEDICINE, GENERAL & INTERNAL","Score":null,"Total":0}
引用次数: 0
Abstract
Renal angiomyolipoma is a benign, rare type of tumour which consists of mature adipose tissue, thick-walled blood vessels, and smooth muscle cells. This tumour can grow to a large size, and is then defined as giant. We are describing the case of a 34-year-old female with a giant renal angiomyolipoma, measuring 32cm x 28cm x 6cm, which caused her pain and constipation. The mass was removed successfully, and partial nephrectomy was done. The patient had an uncomplicated postsurgical clinical course, and renal function was preserved. Renal angiomyolipoma is a hamartomatous, benign mass with variable components. It can occur sporadically or in association with genetic syndromes such as tuberous sclerosis. Symptoms include abdominal pain and constipation. Potentially dangerous sequelae include retroperitoneal haemorrhage and shock. The treatment for these tumours is surgery with open nephrectomy. Other treatment modalities have also been developed. Diagnostic workup should be done for genetic syndromes whenever necessary.
肾血管平滑肌脂肪瘤是一种罕见的良性肿瘤,由成熟的脂肪组织、厚壁血管和平滑肌细胞组成。这种肿瘤可以长到很大,然后被定义为巨型肿瘤。我们正在描述一个34岁的女性病例,她患有巨大的肾血管平滑肌脂肪瘤,尺寸为32cm x 28cm x 6cm,导致她疼痛和便秘。肿块成功切除,行部分肾切除术。患者术后临床过程简单,肾功能完好。肾血管平滑肌脂肪瘤是一种错构瘤状的良性肿块,具有多种成分。它可以偶尔发生或与遗传综合征如结节性硬化症有关。症状包括腹痛和便秘。潜在的危险后遗症包括腹膜后出血和休克。治疗这些肿瘤的方法是手术和开放性肾切除术。其他治疗方式也已开发出来。必要时应对遗传综合征进行诊断检查。
期刊介绍:
Primarily being a medical journal, JPMA publishes scholarly research focusing on the various fields in the areas of health and medical education. It publishes original research describing recent advances in health particularly clinical studies, clinical trials, assessments of pathogens of diagnostic importance, medical genetics and epidemiological studies. Review articles highlighting importance of various issues in the domain of public health, drug research and medical education are also accepted. As a leading journal of South Asia, JPMA remains cognizant of the recent advances in the rapidly growing fields of biomedical sciences, it invites and encourages scholars to write short reviews and invited editorials on the emerging issues. We particularly aim to promote health standards of developing countries by encouraging manuscript submissions on issues affecting the public health and health delivery services.