title: Neuroendocrine carcinoma in an adolescent with hypercortisolemia.
abstract: We present a 16-year-old boy with weakness, hypercortisolemia, and markedly elevated adrenocorticotropic hormone. Computed tomographic imaging revealed hepatic lesions and a calcified pancreatic mass. Biopsy of the hepatic lesions revealed moderately differentiated neuroendocrine carcinoma. The primary tumor could not be determined. The patient received neoadjuvant chemotherapy with carboplatin and etoposide followed by therapeutic bilateral adrenalectomy and tumor debulking. Despite significant clinical improvement, restaging revealed progressive hepatic disease. The patient died 9 months after diagnosis. Autopsy revealed disseminated neuroendocrine carcinoma. The rarity of this tumor compels a cooperative investigational model involving pediatric and adult oncologists.
authors.affiliation: Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, TN, USA.
authors.forename: Emily L
authors.initials: EL
authors.lastname: Fagan
authors.forename: Jeremy S
authors.initials: JS
authors.lastname: Slone
authors.forename: Ashley H
authors.initials: AH
authors.lastname: Shoemaker
authors.forename: Jennifer
authors.initials: J
authors.lastname: Black
authors.forename: Jordan
authors.initials: J
authors.lastname: Berlin
authors.forename: Michael
authors.initials: M
authors.lastname: E Engel
mesh_terms: D000293:Adolescent; D000315:Adrenalectomy; D000308:Adrenocortical Hyperfunction / Q000150:complications*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D016190:Carboplatin / Q000008:administration & dosage; D018278:Carcinoma, Neuroendocrine / Q000209:etiology* / Q000628:therapy*; D003131:Combined Modality Therapy; D005047:Etoposide / Q000008:administration & dosage; D006801:Humans; D008297:Male; D020360:Neoadjuvant Therapy; D014057:Tomography, X-Ray Computed
chemical_list: D005047:Etoposide; D016190:Carboplatin