Introduction And Importance: Renal angiomyolipoma (AML) are benign tumors, often incidentally diagnosed with rupture being the commonest complication and cause of mortality. These tumors are rare with a higher prevalence among patients with tuberous sclerosis and female predominance. Management is dependent on tumor size and whether or not the tumor has ruptured.
Case Presentation: 32-year-old female presenting with sudden right flank pain with shock without history of prior trauma or surgeries. Underwent imaging revealing a suspected ruptured AML thus underwent emergent nephrectomy with admission to intensive care.
Clinical Discussion: Wunderlich syndrome manifests as the Lenk triad, which includes acute flank pain, flank mass, and hypovolemic shock with signs of internal bleeding such as hematuria. It is a rare manifestation signifying spontaneous renal hemorrhage. Due to the instability of the patient had to undergo emergency laparotomy and nephrectomy.
Conclusion: Renal AML occur as a rare benign phenomenon which when ruptured are associated with high mortality rate if not treated promptly in a setting with specialized treatment and intensive unit care. We hope that through our experience patients presenting with Lenk's triad are identified early for adequate intervention.
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http://dx.doi.org/10.1016/j.ijscr.2023.109073 | DOI Listing |
Clin J Gastroenterol
January 2025
Department of Gastroenterology and Hepatology, Osaka University Graduate School of Medicine, Suita, Japan.
A 55-year-old man with tuberous sclerosis complex (TSC) was diagnosed with left renal angiomyolipoma (AML), a group of perivascular epithelioid cell tumors called PEComas. He had received the mTOR inhibitor everolimus, which resulted in a complete response. However, a left renal mass relapsed in two years, followed by the occurrence of a hepatic mass five months later.
View Article and Find Full Text PDFFront Oncol
December 2024
Fujian Provincial Hospital, Provincial Clinical Medical College, Fujian Medical University, Fuzhou, China.
Purpose: This study aims to assess the diagnostic efficacy of conventional ultrasound (CUS) and contrast-enhanced ultrasound (CEUS) in detecting fat-poor angiomyolipomas(AML) with dimensions less than 5 cm. Additionally, the study seeks to identify independent indicators for predicting the presence of fat-poor AML.
Methods: We conducted a retrospective analysis of patients diagnosed with renal AML and renal cell carcinoma, who were admitted and underwent surgery at Fujian Provincial Hospital from January 2013 to October 2023.
J Cytol
November 2024
Department of Pathology, Cytogenetics Unit, Kidwai Memorial Institute of Oncology, Bangalore, Karnataka, India.
Background: The role of fine needle aspiration cytology (FNAC) in the diagnosis of renal malignancies is established and has been getting more precise and important over a period of time. Knowledge of the pathology of uncommon renal neoplasms along with radiological and clinical correlations often aids in correct diagnosis.
Aims: The present study aims to describe the cytomorphological and immunohistochemical findings in the varied spectrum of renal tumors, other than renal cell carcinomas (RCC).
Diseases
December 2024
Department of Pediatrics, Dokkyo Medical University, Tochigi 321-0293, Japan.
Background: Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder characterized by mutations in the TSC1 and TSC2 genes, leading to the dysregulation of the mammalian target of rapamycin (mTOR) pathway. This dysregulation results in the development of benign tumors across multiple organ systems and poses significant neurodevelopmental challenges. The clinical manifestations of TSC vary widely and include subependymal giant cell astrocytomas (SEGAs), renal angiomyolipomas (AMLs), facial angiofibromas (FAs), and neuropsychiatric conditions such as autism spectrum disorder (ASD).
View Article and Find Full Text PDFTher Clin Risk Manag
December 2024
Department of Medicine, Chang Gung University, Taoyuan City, Taiwan.
Background: Evaluating risk factors for bleeding events in robot-assisted partial nephrectomy (RAPN) for renal angiomyolipoma (RAML) is essential for improving surgical outcomes.
Methods: We performed a retrospective analysis of patients who underwent RAPN for renal masses between May 2019 and June 2023 at a single medical center, categorizing them into AML and non-AML groups. We assessed demographic data, perioperative complications, and postoperative outcomes.
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