title: A case of corticotroph carcinoma that caused multiple cranial nerve palsies, destructive petrosal bone invasion, and liver metastasis.
abstract: A 52-year-old woman experienced sudden onset of double vision due to a right abducens nerve palsy and was diagnosed as having a pituitary macroadenoma that invaded into the right cavernous sinus. Otherwise, she was asymptomatic despite marked elevation of ACTH (293 pg/ml) and cortisol (24.6 μg/dl) levels. The patient underwent transsphenoidal surgery followed by γ-knife radiosurgery (GKR), which healed the diplopia and ameliorated the hypercortisolemia. The excised tumor was diffusely stained for ACTH with a high (15%) Ki-67 labeling index. Early tumor recurrence occurred twice thereafter, producing right lower cranial nerve palsies with petrosal bone destruction at 8 months and an ipsilateral oculomotor nerve palsy at 12 months after GKR; all palsies resolved completely with the second and third GKRs. Hypercortisolemia worsened rapidly soon after the third GKR, and the patient developed marked weight gain, hypokalemia, and hypertension. Multiple liver lesions were incidentally detected with computer tomography and identified as metastatic pituitary tumor on immunohistochemistry. An ACTH-producing adenoma should be followed carefully for early recurrence and/or metastatic spread when the tumor is an invasive macroadenoma with a high proliferation marker level. The unique aggressive behavior and high potential for malignant transformation of this case are discussed.
authors.affiliation: Department of Medicine II, Institute of Clinical Endocrinology, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, Japan. miki-masa@abox3.so-net.ne.jp
authors.forename: Masami
authors.initials: M
authors.lastname: Ono
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Miki
authors.forename: Kosaku
authors.initials: K
authors.lastname: Amano
authors.forename: Motohiro
authors.initials: M
authors.lastname: Hayashi
authors.forename: Takakazu
authors.initials: T
authors.lastname: Kawamata
authors.forename: Toshiro
authors.initials: T
authors.lastname: Seki
authors.forename: Kazue
authors.initials: K
authors.lastname: Takano
authors.forename: Satosi
authors.initials: S
authors.lastname: Katagiri
authors.forename: Masakazu
authors.initials: M
authors.lastname: Yamamoto
authors.forename: Toshio
authors.initials: T
authors.lastname: Nishikawa
authors.forename: Osami
authors.initials: O
authors.lastname: Kubo
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Sano
authors.forename: Tomokatsu
authors.initials: T
authors.lastname: Hori
authors.forename: Yoshikazu
authors.initials: Y
authors.lastname: Okada
mesh_terms: D000236:Adenoma / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D000324:Adrenocorticotropic Hormone / Q000378:metabolism; D014408:Biomarkers, Tumor / Q000378:metabolism; D001859:Bone Neoplasms / Q000556:secondary*; D003389:Cranial Nerve Diseases / Q000209:etiology / Q000473:pathology*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000378:metabolism; D008113:Liver Neoplasms / Q000556:secondary*; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D009364:Neoplasm Recurrence, Local / Q000473:pathology*; D010243:Paralysis / Q000209:etiology / Q000473:pathology; D010579:Petrous Bone / Q000473:pathology*; D047748:Pituitary ACTH Hypersecretion / Q000150:complications / Q000473:pathology; D010903:Pituitary Gland, Anterior / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D010911:Pituitary Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D016634:Radiosurgery; D012772:Shock, Septic
chemical_list: D014408:Biomarkers, Tumor; D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone