Category: Pituitary Disorders/Neuroendocrinology
We reported the case with pituitary apoplexy following CRH administration for inferior petrosal sinus sampling.
Methods : Reviewed clinical, laboratory including IPSS, imaging and pathology data.
A 42-year-old woman who was healthy presented for progressive weakness and sudden onset of headache. Physical exam showed high blood pressure, and ecchymoses on extremities. BMI was 23 kg/m2. Initial labs were unremarkable except low K 2.2 mmol/L (ref: 3.4-5.3 mmol/L). MRI/MRA brain showed bilateral vertebral arteries dissection and incidentally pituitary macroadenoma size 2.3 cm extending to left cavernous sinus. Further investigations showed elevated morning cortisol 51.2 ug/dL (ref: 4-22 ug/dL), 24-hour urine cortisol 17097 ug/d (ref: 3.5-45 ug/d), ACTH 270 pg/mL (ref: <47 pg/mL). One and 8 mg Dexamethasone suppression test were not suppressed, morning cortisol were 37.5 ug/dL, and 67.3 ug/dL, respectively. CT chest abdomen and pelvis was unremarkable except bilateral adrenal hyperplasia. Ketoconazole was started and titrated up to 400 mg TID. IPSS was done to confirm pituitary origin, which showed significantly elevated baseline ACTH on the left 4605 pg/mL and kept increasing to 19290 pg/mL until the end of the study (+20 min) without shot period of peak. Next day, she developed left eye ptosis with ophthalmoplegia, severe headache and legs weakness. MRI/MRV brain showed no cavernous sinus thrombosis, but slightly expanding pituitary adenoma further to left cavernous sinus. Dexamethasone and Enoxaparin were started. She underwent semi-urgent endoscopic transnasal surgery. Intraoperatively found hemorrhagic areas in the tumor. Pathology report revealed necrotic and hemorrhagic pituitary adenoma. Immunohistochemistry showed high ACTH but Ki-67 and p53 were both low. Her cortisol level was significantly reduced. Ptosis and ophthalmoplegia are recovering.
IPSS has high sensitivity and specificity (80-100%, >95 %) to confirm Cushing disease. Although invasive, IPSS has been known to be low risk with major neurologic complications only 0.2%. Pituitary apoplexy following endocrine tests are rare but reported mainly in GnRH and TRH stimulation test. CRH induced pituitary apoplexy in Cushing disease during IPSS was reported once but not well described and unclear mechanism. By reviewing our IPSS data, baseline ACTH was significantly high which responded to CRH immediately but kept rising. Mechanism is unclear, however this unusual pattern of ATCH may associate to CRH induced pituitary apoplexy in Cushing disease.
Pituitary apoplexy is a rare condition in Cushing disease. We present a rare case of CRH induced pituitary apoplexy in Cushing disease during IPSS.
Jutarat Sangtian– Fellow, Division of Endocrinology, Diabetes and Metabolism, University of Minnesota, Minneapolis, Minnesota
Darin Ruanpeng– Fellow, Division of Endocrinology, Diabetes and Metabolism, University of Minnesota, Minnesota
Takako Araki– Assistant Professor, Division of Endocrinology, Diabetes and Metabolism, University of Minnesota, Minnesota
Division of Endocrinology, Diabetes and Metabolism, University of Minnesota
Division of Endocrinology, Diabetes and Metabolism, University of Minnesota, Minnesota