2018MedicineOpen access

Invasive ACTH-secreting pituitary macroadenoma in remission after transsphenoidal resection

Zhe Zhuang, Xiaohai Liu, Xinjie Bao, Boju Pan, Kan Deng, Yong Yao, Wei Lian, Bing Xing, Huijuan Zhu, Lin Lü, Renzhi Wang, Ming Feng

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Abstract

RATIONALE: Knosp grade 4 adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma is a rare cause of Cushing disease. After the 1st surgery, the remission rate among these patients is extremely low. PATIENT CONCERNS: We presented a case of a 33-year-old female with classical Cushingoid symptoms. Further investigations revealed ATCH-dependent hypercortisolemia, as well as a Knosp grade 4 pituitary macroadenoma. DIAGNOSIS: Cushing disease, caused by a Knosp grade 4 pituitary macroadenoma. INTERVENTIONS: The patient underwent endoscopic endonasal transsphenoidal surgery with the assistance of intraoperative transsphenoidal Doppler and image-guidance devices. OUTCOMES: Pathologic examinations confirmed that the lesion was an ACTH-secreting pituitary adenoma. The patient was in biochemical remission after surgery. Her postoperative magnetic resonance imaging showed gross-total tumor resection. There was no evidence of recurrence during the 1-year follow-up. LESSONS: With intraoperative Doppler and image-guidance, gross-total resection and biochemical remission can be achieved in Cushing disease when the internal carotid artery is completely encased by the pituitary adenoma.

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RATIONALE: Knosp grade 4 adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma is a rare cause of Cushing disease. After the 1st surgery, the remission rate among these patients is extremely low. PATIENT CONCERNS: We presented a case of a 33-year-old female with classical Cushingoid symptoms. Further investigations revealed ATCH-dependent hypercortisolemia, as well as a Knosp grade 4 pituitary macroadenoma. DIAGNOSIS: Cushing disease, caused by a Knosp grade 4 pituitary macroadenoma. INTERVENTIONS: The patient underwent endoscopic endonasal transsphenoidal surgery with the assistance of intraoperative transsphenoidal Doppler and image-guidance devices. OUTCOMES: Pathologic examinations confirmed that the lesion was an ACTH-secreting pituitary adenoma. The patient was in biochemical remission after surgery. Her postoperative magnetic resonance imaging showed gross-total tumor resection. There was no evidence of recurrence during the 1-year follow-up. LESSONS: With intraoperative Doppler and image-guidance, gross-total resection and biochemical remission can be achieved in Cushing disease when the internal carotid artery is completely encased by the pituitary adenoma.

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Available abstract

RATIONALE: Knosp grade 4 adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma is a rare cause of Cushing disease. After the 1st surgery, the remission rate among these patients is extremely low. PATIENT CONCERNS: We presented a case of a 33-year-old female with classical Cushingoid symptoms. Further investigations revealed ATCH-dependent hypercortisolemia, as well as a Knosp grade 4 pituitary macroadenoma. DIAGNOSIS: Cushing disease, caused by a Knosp grade 4 pituitary macroadenoma. INTERVENTIONS: The patient underwent endoscopic endonasal transsphenoidal surgery with the assistance of intraoperative transsphenoidal Doppler and image-guidance devices. OUTCOMES: Pathologic examinations confirmed that the lesion was an ACTH-secreting pituitary adenoma. The patient was in biochemical remission after surgery. Her postoperative magnetic resonance imaging showed gross-total tumor resection. There was no evidence of recurrence during the 1-year follow-up. LESSONS: With intraoperative Doppler and image-guidance, gross-total resection and biochemical remission can be achieved in Cushing disease when the internal carotid artery is completely encased by the pituitary adenoma.

Key concepts: Medicine, Cushingoid, Transsphenoidal surgery, Cushing Disease, Pituitary adenoma, Adrenocorticotropic hormone, Adenoma, Hypercortisolemia

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