We present a 37-year-old female with a history of tuberous sclerosis. She developed flank pain, hypotension, and a sudden drop in hemoglobin levels which prompted a work-up. A computed tomography scan demonstrated enlarged heterogeneous kidneys and a large complex collection in the right kidney, likely hemorrhagic in nature. The findings were suspicious for bilateral angiomyolipomas with retroperitoneal hematoma. She was treated with bilateral transarterial embolization. She subsequently developed recurrent fever and was suspected of having emphysematous pyelonephritis, for which she underwent a right nephrectomy. The entire kidney was replaced by a tan bulging, lobular mass, with scattered tan-yellow nodules. Microscopic examination revealed an angiomyolipoma with an incidental oncocytoma. The association of renal angiomyolipoma and renal oncocytoma is rare and only 16 cases have been previously reported.
Download full-text PDF |
Source |
---|
Epilepsia
January 2025
Department of Neurosciences, Faculty of Medicine, Université de Montréal, Montreal, Quebec, Canada.
Objective: Tuberous sclerosis complex (TSC) is a monogenetic disorder associated with sustained mechanistic target of rapamycin (mTOR) activation, leading to heterogeneous clinical manifestations. Epilepsy and renal angiomyolipoma are the most important causes of morbidity in adult people with TSC (pwTSC). mTOR is a key player in inflammation, which in turn could influence TSC-related clinical manifestations.
View Article and Find Full Text PDFDiagn Pathol
January 2025
Department of Diagnostic Pathology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
Background: Perivascular epithelioid cell tumors (PEComas) rarely appear in the head and neck region. This case report describes two transcription factor E3 (TFE3)-rearranged PEComa cases, consisting of one in the orbit and one in the nasal cavity.
Case Presentation: Both cases demonstrated sheet-like or focal nested architecture and comprised epithelioid cells with abundant clear to eosinophilic cytoplasm and vascular stroma.
Ann Diagn Pathol
January 2025
Department of Pathology and Laboratory Medicine, Brigham and Women's Hospital, Boston, USA. Electronic address:
Intrarenal hemangiomas lack concise clinicopathologic information, due to the predominance of single case reports and inclusion of other vascular neoplasms and hemangiomas of perirenal, hilar, and renal vein origin. Herein, in this multi-institutional study we evaluate clinicopathologic features of 39 intrarenal hemangiomas. The median age was 62 years (range = 27-94 years; 2:1 male to female ratio), with left-sided predominance (left = 21, right = 13; one case was bilateral).
View Article and Find Full Text PDFClin 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.
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!