Shankar Sushma, Hulikanthimatt Kiran Shankar, Awatti Shreeharsha Mallappa, Narayanrao Suresh Turuvekere
Assistant Professor, Department of Pathology, Sri Devaraj Urs Medical College , SDUAHER, Tamaka, Kolar, India .
Post Graduate Student, Department of Surgery, Sri Devaraj Urs Medical College , SDUAHER, Tamaka, Kolar, India .
J Clin Diagn Res. 2015 Aug;9(8):ED01-3. doi: 10.7860/JCDR/2015/13349.6289. Epub 2015 Aug 1.
Renal Angiomyolipoma (renal AML) is a benign clonal neoplasm with a incidence of 0.3-3%, occurring as isolated sporadic entity or in association with Tuberous sclerosis (TS) in 80% cases. Multiple, bilateral renal AML are considered diagnostic of Tuberous sclerosis. Wunderlich's syndrome, a urological emergency is a spontaneous nontraumatic bleeding into subcapsular and or peri-renal space and is a life threatening complication of renal AML occurring in 10% cases which has to be timely diagnosed and treated. Here, we present an unusual case of TS with bilateral, multifocal renal AML in a 25-year-old female who presented with Wunderlich's syndrome, which is a rare but life threatening complication that has to be suspected, timely diagnosed and treated. We have discussed the importance of early diagnosis, timely treatment, follow up and education of patient and relatives of the possible complications associated.
肾血管平滑肌脂肪瘤(肾AML)是一种良性克隆性肿瘤,发病率为0.3%-3%,可作为孤立的散发性病变出现,或在80%的病例中与结节性硬化症(TS)相关。多发、双侧肾AML被认为是结节性硬化症的诊断依据。Wunderlich综合征是一种泌尿外科急症,是指自发性非创伤性出血进入肾包膜下和/或肾周间隙,是肾AML的一种危及生命的并发症,发生率为10%,必须及时诊断和治疗。在此,我们报告一例罕见病例,一名25岁女性患有双侧、多灶性肾AML并伴有Wunderlich综合征,这是一种罕见但危及生命的并发症,必须予以怀疑、及时诊断和治疗。我们讨论了早期诊断、及时治疗、随访以及对患者及其亲属进行可能相关并发症教育的重要性。