Case of Corticotroph Carcinoma that Caused Multiple Cranial Nerve Palsies, Destructive Petrosal Bone Invasion, and Liver Metastasis

• Published in: Endocrine pathology 2011-03, Vol.22 (1), p.10-17
• Main Authors: Ono, Masami, Miki, Nobuhiro, Amano, Kosaku, Hayashi, Motohiro, Kawamata, Takakazu, Seki, Toshiro, Takano, Kazue, Katagiri, Satosi, Yamamoto, Masakazu, Nishikawa, Toshio, Kubo, Osami, Sano, Toshiaki, Hori, Tomokatsu, Okada, Yoshikazu
• Format: Article
• Language: English
• Subjects: Abducens nerve; Adenoma; Adenoma - metabolism; Adenoma - pathology; Adenoma - surgery; Adrenocorticotropic hormone; Adrenocorticotropic Hormone - metabolism; Aggressive behavior; Biomarkers, Tumor - metabolism; Bone loss; Bone Neoplasms - secondary; Brain tumors; Carcinoma; Corticotroph carcinoma; Cranial Nerve Diseases - etiology; Cranial Nerve Diseases - pathology; Cranial nerves; Cushing syndrome; Endocrinology; Fatal Outcome; Female; Humans; Hydrocortisone; Hydrocortisone - metabolism; Hypokalemia; Liver; Liver Neoplasms - secondary; Medicine; Medicine & Public Health; Metastases; Middle Aged; Neoplasm Invasiveness; Neoplasm Recurrence, Local - pathology; Oculomotor nerve; Oncology; Paralysis; Paralysis - etiology; Paralysis - pathology; Pathology; Petrous Bone - pathology; Pituitary; Pituitary (anterior); Pituitary ACTH Hypersecretion - complications; Pituitary ACTH Hypersecretion - pathology; Pituitary carcinoma; Pituitary Gland, Anterior - metabolism; Pituitary Gland, Anterior - pathology; Pituitary Gland, Anterior - surgery; Pituitary Neoplasms - metabolism; Pituitary Neoplasms - pathology; Pituitary Neoplasms - surgery; Radiosurgery; Shock, Septic; Sinus; Surgery; Tumors; γ-Knife radiosurgery
• ISSN: 1046-3976; 1559-0097
• DOI: 10.1007/s12022-010-9144-5

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.