Tags

Type your tag names separated by a space and hit enter

Cushing Syndrome Caused by Ectopic Adrenocorticotropic Hormone-Secreting Pituitary Adenomas: Case Report and Literature Review.
World Neurosurg. 2020 10; 142:75-86.WN

Abstract

BACKGROUND

Ectopic adrenocorticotropic hormone-secreting pituitary adenomas (EAPAs) are rare adenomas causing Cushing syndrome, which are located in ectopic locations outside the sella turcica. No more than 100 cases of this entity have been reported. Because of its rarity and nonspecific clinical and biochemical features, EAPA is often misdiagnosed, leading to surgical failure and delayed treatment. In the article, the cases of 3 patients with suprasellar EAPAs treated at our institution are reported and a review of the literature is presented.

CASE DESCRIPTION

All 3 patients with Cushing syndrome had ectopic pituitary adenomas located in the suprasellar region and identifiable on preoperative sellar magnetic resonance imaging. Preoperative laboratory evaluation in all patients showed identical results to those observed in Cushing disease. In 2 patients, craniotomies were performed directly to achieve total tumor resection and clinical remission. One patient, who underwent previous negative transsphenoidal exploration, achieved full remission through a second craniotomy despite pulmonary infection as a postoperative complication.

CONCLUSIONS

Although EAPA is an extremely rare entity, it should be considered as a differential diagnosis of Cushing disease. Because of its similar clinical and biochemical behavior to intrasellar pituitary adrenocorticotropic hormone adenoma in Cushing disease, careful examination of the potential occurrence of EAPA on preoperative radiologic imaging is of considerable significance to avoid unnecessary surgery and achieve improved outcomes. Surgical resection EAPA remains the first choice of treatment, and the optimal surgical approach ought to be determined according to the adenoma features, the general condition of the patient, and the surgeon's experience.

Authors+Show Affiliations

Department of Neurosurgery, Chinese Academy of Medical Sciences and Peking Union Medical College, Peking Union Medical College Hospital, Beijing, China; School of Medicine, Tsinghua University, Beijing, China.Department of Endocrinology, Chinese Academy of Medical Sciences and Peking Union Medical College, Peking Union Medical College Hospital, Beijing, China.Department of Neurosurgery, Chinese Academy of Medical Sciences and Peking Union Medical College, Peking Union Medical College Hospital, Beijing, China. Electronic address: jackietz@163.com.Department of Neurosurgery, Chinese Academy of Medical Sciences and Peking Union Medical College, Peking Union Medical College Hospital, Beijing, China.Department of Neurosurgery, Chinese Academy of Medical Sciences and Peking Union Medical College, Peking Union Medical College Hospital, Beijing, China.Department of Neurosurgery, Chinese Academy of Medical Sciences and Peking Union Medical College, Peking Union Medical College Hospital, Beijing, China.Department of Neurosurgery, Chinese Academy of Medical Sciences and Peking Union Medical College, Peking Union Medical College Hospital, Beijing, China.Clinical Biobank, Chinese Academy of Medical Sciences and Peking Union Medical College, Peking Union Medical College Hospital, Beijing, China.Department of Neurosurgery, Chinese Academy of Medical Sciences and Peking Union Medical College, Peking Union Medical College Hospital, Beijing, China.Department of Endocrinology, Chinese Academy of Medical Sciences and Peking Union Medical College, Peking Union Medical College Hospital, Beijing, China.Department of Neurosurgery, Chinese Academy of Medical Sciences and Peking Union Medical College, Peking Union Medical College Hospital, Beijing, China.

Pub Type(s)

Case Reports
Journal Article
Review

Language

eng

PubMed ID

32592962

Citation

Sun, Xiaolin, et al. "Cushing Syndrome Caused By Ectopic Adrenocorticotropic Hormone-Secreting Pituitary Adenomas: Case Report and Literature Review." World Neurosurgery, vol. 142, 2020, pp. 75-86.
Sun X, Lu L, Feng M, et al. Cushing Syndrome Caused by Ectopic Adrenocorticotropic Hormone-Secreting Pituitary Adenomas: Case Report and Literature Review. World Neurosurg. 2020;142:75-86.
Sun, X., Lu, L., Feng, M., Fan, Y., Bao, X., Dai, C., Deng, K., Guo, D., Yao, Y., Zhu, H., & Wang, R. (2020). Cushing Syndrome Caused by Ectopic Adrenocorticotropic Hormone-Secreting Pituitary Adenomas: Case Report and Literature Review. World Neurosurgery, 142, 75-86. https://doi.org/10.1016/j.wneu.2020.06.138
Sun X, et al. Cushing Syndrome Caused By Ectopic Adrenocorticotropic Hormone-Secreting Pituitary Adenomas: Case Report and Literature Review. World Neurosurg. 2020;142:75-86. PubMed PMID: 32592962.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cushing Syndrome Caused by Ectopic Adrenocorticotropic Hormone-Secreting Pituitary Adenomas: Case Report and Literature Review. AU - Sun,Xiaolin, AU - Lu,Lin, AU - Feng,Ming, AU - Fan,Yanghua, AU - Bao,Xinjie, AU - Dai,Congxin, AU - Deng,Kan, AU - Guo,Dan, AU - Yao,Yong, AU - Zhu,Huijuan, AU - Wang,Renzhi, Y1 - 2020/06/24/ PY - 2020/05/18/received PY - 2020/06/15/revised PY - 2020/06/17/accepted PY - 2020/6/28/pubmed PY - 2021/1/7/medline PY - 2020/6/28/entrez KW - Craniotomy KW - Cushing disease KW - Cushing syndrome KW - Ectopic pituitary adenoma KW - Suprasellar SP - 75 EP - 86 JF - World neurosurgery JO - World Neurosurg VL - 142 N2 - BACKGROUND: Ectopic adrenocorticotropic hormone-secreting pituitary adenomas (EAPAs) are rare adenomas causing Cushing syndrome, which are located in ectopic locations outside the sella turcica. No more than 100 cases of this entity have been reported. Because of its rarity and nonspecific clinical and biochemical features, EAPA is often misdiagnosed, leading to surgical failure and delayed treatment. In the article, the cases of 3 patients with suprasellar EAPAs treated at our institution are reported and a review of the literature is presented. CASE DESCRIPTION: All 3 patients with Cushing syndrome had ectopic pituitary adenomas located in the suprasellar region and identifiable on preoperative sellar magnetic resonance imaging. Preoperative laboratory evaluation in all patients showed identical results to those observed in Cushing disease. In 2 patients, craniotomies were performed directly to achieve total tumor resection and clinical remission. One patient, who underwent previous negative transsphenoidal exploration, achieved full remission through a second craniotomy despite pulmonary infection as a postoperative complication. CONCLUSIONS: Although EAPA is an extremely rare entity, it should be considered as a differential diagnosis of Cushing disease. Because of its similar clinical and biochemical behavior to intrasellar pituitary adrenocorticotropic hormone adenoma in Cushing disease, careful examination of the potential occurrence of EAPA on preoperative radiologic imaging is of considerable significance to avoid unnecessary surgery and achieve improved outcomes. Surgical resection EAPA remains the first choice of treatment, and the optimal surgical approach ought to be determined according to the adenoma features, the general condition of the patient, and the surgeon's experience. SN - 1878-8769 UR - https://www.unboundmedicine.com/medline/citation/32592962/Cushing_Syndrome_Caused_by_Ectopic_Adrenocorticotropic_Hormone_Secreting_Pituitary_Adenomas:_Case_Report_and_Literature_Review_ DB - PRIME DP - Unbound Medicine ER -