title: Rapid and Complete Remission of Metastatic Adrenocortical Carcinoma Persisting 10 Years After Treatment With Mitotane Monotherapy: Case Report and Review of the Literature.
abstract: Mitotane has been used for more than 5 decades as therapy for adrenocortical carcinoma (ACC). However its mechanism of action and the extent of tumor response remain incompletely understood. To date no cases of rapid and complete remission of metastatic ACC with mitotane monotherapy has been reported. A 52-year-old French Canadian man presented with metastatic disease 2 years following a right adrenalectomy for stage III nonsecreting ACC. He was started on mitotane which was well tolerated despite rapid escalation of the dose. The patient course was exceptional as he responded to mitotane monotherapy after only few months of treatment. Initiation of chemotherapy was not needed and he remained disease-free with good quality of life on low maintenance dose of mitotane during the following 10 years. A germline heterozygous TP53 exon 4 polymorphism c.215C>G (p. Pro72Arg) was found. Immunohistochemical stainings for IGF-2 and cytoplasmic β-catenin were positive. Advanced ACC is an aggressive disease with poor prognosis and the current therapeutic options remain limited. These findings suggest that mitotane is a good option for the treatment of metastatic ACC and might result in rapid complete remission in selected patients.
authors.affiliation: From the Division of Endocrinology (NEG, GR, AL, IB), Department of Medicine; Department of Pathology (ML); Division of Nuclear Medicine (CC), Department of Radiology; Division of Oncology (HO), Department of Medicine; Division of Urology (PP), Department of Surgery; Division of Endocrinology (AS), Department of Medecine, Hôpital Honoré Mercier, Saint-Hyacinthe, Québec, Canada; and Division of Endocrinology (TLM), Medical Clinical Department, Health Sciences Centre, Universidade Estadual de Londrina (UEL), Paraná, Brazil.
authors.forename: Nada
authors.initials: N
authors.lastname: El Ghorayeb
authors.forename: Geneviève
authors.initials: G
authors.lastname: Rondeau
authors.forename: Mathieu
authors.initials: M
authors.lastname: Latour
authors.forename: Christian
authors.initials: C
authors.lastname: Cohade
authors.forename: Harold
authors.initials: H
authors.lastname: Olney
authors.forename: André
authors.initials: A
authors.lastname: Lacroix
authors.forename: Paul
authors.initials: P
authors.lastname: Perrotte
authors.forename: Alexis
authors.initials: A
authors.lastname: Sabourin
authors.forename: Tania L
authors.initials: TL
authors.lastname: Mazzuco
authors.forename: Isabelle
authors.initials: I
authors.lastname: Bourdeau
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery; D018268:Adrenocortical Carcinoma / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery; D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use*; D002170:Canada; D016158:Genes, p53 / Q000235:genetics; D006801:Humans; D007335:Insulin-Like Growth Factor II / Q000276:immunology; D008297:Male; D008875:Middle Aged; D008939:Mitotane / Q000627:therapeutic use*; D009362:Neoplasm Metastasis; D020641:Polymorphism, Single Nucleotide; D011788:Quality of Life; D012074:Remission Induction; D051176:beta Catenin
chemical_list: D018931:Antineoplastic Agents, Hormonal; C035170:IGF2 protein, human; D051176:beta Catenin; D007335:Insulin-Like Growth Factor II; D008939:Mitotane