title: Metastatic esthesioneuroblastoma secreting adrenocorticotropic hormone in pediatric patients.
abstract: The purpose of this article was to report a pediatric case of secondary cervical esthesioneuroblastoma involving the parapharyngeal lymph nodes. A 3-year-old boy came to our clinical observation because of a right lymphonodal mass evidenced by nuclear magnetic resonance and a diagnosis of Cushing syndrome associated with ectopic adrenocorticotropic hormone secretion, moon face, central obesity, asthenia, and hirsutism. At the age of 10 months, the patient underwent endoscopic surgery for asportation of the World Health Organization stage IV esthesioneuroblastoma. At 38 months of age, the patient underwent right parapharyngeal lymphadenectomy with surgical access by a double mandibulectomy. After surgery, serum ACTH, cortisolemia, and urinary excretion of cortisol were within the reference range. Blood pressure was recorded at 110/70 mm Hg. Moon face disappeared, as well as central obesity and hirsutism. Clinical report is presented together with brief review of literature.
authors.affiliation: Department of Maxillo-Facial Surgery, IRCCS San Matteo Hospital, University of Pavia, Pavia, Italy.
authors.forename: Silvestre
authors.initials: S
authors.lastname: Galioto
authors.forename: Alessandro
authors.initials: A
authors.lastname: Di Petrillo
authors.forename: Mauro
authors.initials: M
authors.lastname: Pastori
authors.forename: Alberto
authors.initials: A
authors.lastname: Arecchi
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000378:metabolism*; D002675:Child, Preschool; D003480:Cushing Syndrome / Q000209:etiology* / Q000601:surgery; D018304:Esthesioneuroblastoma, Olfactory / Q000378:metabolism* / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008197:Lymph Node Excision; D008207:Lymphatic Metastasis; D008297:Male; D008334:Mandible / Q000601:surgery; D009296:Nasal Cavity / Q000601:surgery*; D009669:Nose Neoplasms / Q000378:metabolism* / Q000473:pathology*
chemical_list: D000324:Adrenocorticotropic Hormone