{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,14]],"date-time":"2026-03-14T21:22:43Z","timestamp":1773523363785,"version":"3.50.1"},"reference-count":20,"publisher":"The Endocrine Society","issue":"3","license":[{"start":{"date-parts":[[2026,2,24]],"date-time":"2026-02-24T00:00:00Z","timestamp":1771891200000},"content-version":"vor","delay-in-days":1,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,2,23]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>A 19-year-old woman presented with severe hypertension, hypokalemia, and clinical signs of Cushing syndrome (CS). Computed tomography imaging revealed a large left adrenal mass encasing a major vessel, a left renal lesion, a right ovarian teratoma, and multiple pulmonary micronodules. 18F-fluorodeoxyglucose positron emission tomography (18-FDG-PET) showed hypermetabolism in the adrenal mass, lung nodules, and teratoma. Biochemical work-up confirmed adrenocorticotropin-independent CS and elevated adrenal androgens, consistent with metastatic, unresectable adrenocortical carcinoma. The patient was started on mitotane and metyrapone, followed by 8 cycles of etoposide-doxorubicin-cisplatin plus mitotane. Despite treatment, the disease progressed. Adrenal biopsy revealed high tumor-infiltrating lymphocytes, low tumor mutation burden, low programmed death-ligand 1 expression, and microsatellite stability. Germline testing identified a TP53 pathogenic variant, confirming Li-Fraumeni syndrome. A single dose of pembrolizumab was administered as salvage therapy but led to grade 3 immune-related hepatitis and prolonged hospitalization, requiring cessation of both pembrolizumab and mitotane. Remarkably, a follow-up scan 3 months later showed 60% regression of the adrenal mass, stable renal and ovarian lesions, and resolution of lung nodules. The patient underwent complete surgical resection, achieving full remission. More than 1 year later, she remains disease free.<\/jats:p>","DOI":"10.1210\/jcemcr\/luaf279","type":"journal-article","created":{"date-parts":[[2025,10,30]],"date-time":"2025-10-30T13:22:26Z","timestamp":1761830546000},"source":"Crossref","is-referenced-by-count":1,"title":["Exceptional Response to a Single Dose of Pembrolizumab as Salvage Therapy for Metastatic Adrenocortical Carcinoma"],"prefix":"10.1210","volume":"4","author":[{"ORCID":"https:\/\/orcid.org\/0009-0007-8051-4608","authenticated-orcid":false,"given":"Laurie","family":"Branchaud-Croiseti\u00e8re","sequence":"first","affiliation":[{"name":"Division of Endocrinology, Department of Medicine, Research Center, Centre Hospitalier de L\u2019Universit\u00e9 de Sherbrooke (CRCHUS) , Sherbrooke, QC , J1H 5H3","place":["Canada"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emma","family":"Turcotte","sequence":"additional","affiliation":[{"name":"Division of Oncology, Department of Medicine, Research Center, CRCHUS , Sherbrooke, QC , J1H 5H3","place":["Canada"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jean-Fran\u00e7ois","family":"Castilloux","sequence":"additional","affiliation":[{"name":"Division of Oncology, Department of Medicine, Research Center, CRCHUS , Sherbrooke, QC , J1H 5H3","place":["Canada"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8000-4313","authenticated-orcid":false,"given":"Isabelle","family":"Bourdeau","sequence":"additional","affiliation":[{"name":"Division of Endocrinology, Department of Medicine, Research Center, Centre Hospitalier de L\u2019Universit\u00e9 de Montr\u00e9al (CRCHUM) , Montr\u00e9al, QC , H2X 0A9","place":["Canada"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fr\u00e9d\u00e9ric","family":"Mercier","sequence":"additional","affiliation":[{"name":"Division of General Surgery, Department of Surgery, Centre Hospitalier de L\u2019Universit\u00e9 de Montr\u00e9al (CHUM) , Montr\u00e9al, QC , H2X 0C1","place":["Canada"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9791-2118","authenticated-orcid":false,"given":"Matthieu","family":"St-Jean","sequence":"additional","affiliation":[{"name":"Division of Endocrinology, Department of Medicine, Research Center, Centre Hospitalier de L\u2019Universit\u00e9 de Sherbrooke (CRCHUS) , Sherbrooke, QC , J1H 5H3","place":["Canada"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"80","published-online":{"date-parts":[[2026,2,24]]},"reference":[{"issue":"4","key":"2026022405043917600_luaf279-B1","doi-asserted-by":"crossref","first-page":"G1","DOI":"10.1530\/EJE-18-0608","article-title":"European Society of Endocrinology clinical practice guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European network for the study of adrenal 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