{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T16:30:14Z","timestamp":1780590614242,"version":"3.54.1"},"reference-count":0,"publisher":"MJH Life Sciences","issue":"3810","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,10,2]]},"abstract":"<jats:p>A 47-year-old woman with a history of drug-resistant epilepsy during childhood presented to the emergency department with sudden dyspnea and chest pain. Upon admission, her oxygen saturation was 88%. A chest CT scan revealed pulmonary cystic lesions consistent with lymphangioleiomyomatosis and a right spontaneous pneumothorax, which resolved with the placement of a chest tube. Physical examination revealed a hypopigmented macule on the skin of the lumbar region, facial angiofibromas, and periungual fibromas. An abdominal MRI documented multiple bilateral renal tumors that were hypointense on T2-weighted imaging and showed a black boundary artifact, suggestive of fat-poor angiomyolipomas (AMLs). Subsequent percutaneous biopsy of the largest renal tumor confirmed the diagnosis of angiomyolipoma (positive for HMB-45 on immunohistochemistry). The brain MRI revealed subependymal nodules. The pulmonary function tests showed a mild obstructive pattern. Germline genetic testing confirmed the suspected diagnosis, and the patient started oral systemic treatment with everolimus (Afinitor) 10 mg once daily, along with dexamethasone rinses for prophylaxis.<\/jats:p>","DOI":"10.46883\/2024.25921027","type":"journal-article","created":{"date-parts":[[2024,10,30]],"date-time":"2024-10-30T15:39:11Z","timestamp":1730302751000},"page":"376-380","source":"Crossref","is-referenced-by-count":1,"title":["Hereditary Renal Tumor Syndromes and the Use of mTOR Inhibitors"],"prefix":"10.46883","member":"25947","published-online":{"date-parts":[[2024,10,21]]},"container-title":["ONCOLOGY"],"original-title":[],"language":"en","deposited":{"date-parts":[[2024,10,30]],"date-time":"2024-10-30T15:39:12Z","timestamp":1730302752000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.cancernetwork.com\/view\/hereditary-renal-tumor-syndromes-and-the-use-of-mtor-inhibitors"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,10,2]]},"references-count":0,"issue-title":["October 2024"],"journal-issue":{"issue":"3810","published-online":{"date-parts":[[2024,10,21]]},"published-print":{"date-parts":[[2024,10,2]]}},"URL":"https:\/\/doi.org\/10.46883\/2024.25921027","relation":{},"ISSN":["0890-9091"],"issn-type":[{"value":"0890-9091","type":"print"}],"subject":[],"published":{"date-parts":[[2024,10,2]]}}}