Cushing's syndrome attributable to ectopic secretion of corticotropin in a patient with two neuroendocrine tumors

Roberto Salvatori, Danilo Fintini, William H. Westra, Steve Y. Cho, Richard D. Schulick

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

Objective: To report a case of ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) in a patient with two distinct neuroendocrine tumors and to highlight the difficulties of establishing the differential diagnosis of EAS. Methods: We describe the clinical presentation of the current case, discuss its management, and report the results of molecular studies undertaken to determine whether the two tumors had a common origin. Results: A 52-year-old woman presented with obvious features of Cushing's syndrome. Findings on hormonal evaluation were consistent with EAS. Pituitary magnetic resonance imaging revealed normal findings. Computed tomographic imaging disclosed two masses, one in the lung and one in the pancreas. Somatostatin receptor scintigraphy showed uptake only in the pancreatic mass, which was surgically removed. It was a well-differentiated neuroendocrine tumor, with negative immunostaining for ACTH. Hypercortisolemia did not resolve after removal of the pancreatic tumor. The lung mass was subsequently excised, and pathology examination showed a carcinoid tumor with immunostaining for ACTH. Thereafter, plasma ACTH became unmeasurable. The two tumors had similar patterns of X-chromosome inactivation; thus, whether they arose independently could be neither confirmed nor excluded. Conclusion: This case demonstrates that, in the presence of more than one neuroendocrine tumor, somatostatin receptor scintigraphy may misguide the decision regarding the appropriate surgical course in patients with EAS, and it highlights the need for accurate studies to determine the source of ACTH in patients with EAS.

Original languageEnglish
Pages (from-to)656-659
Number of pages4
JournalEndocrine Practice
Volume12
Issue number6
Publication statusPublished - Nov 2006

Fingerprint

Neuroendocrine Tumors
Cushing Syndrome
Adrenocorticotropic Hormone
Somatostatin Receptors
Radionuclide Imaging
Paraneoplastic Endocrine Syndromes
X Chromosome Inactivation
Neoplasms
Lung
Carcinoid Tumor
Pancreas
Differential Diagnosis
Magnetic Resonance Imaging
Pathology

ASJC Scopus subject areas

  • Endocrinology
  • Endocrinology, Diabetes and Metabolism

Cite this

Cushing's syndrome attributable to ectopic secretion of corticotropin in a patient with two neuroendocrine tumors. / Salvatori, Roberto; Fintini, Danilo; Westra, William H.; Cho, Steve Y.; Schulick, Richard D.

In: Endocrine Practice, Vol. 12, No. 6, 11.2006, p. 656-659.

Research output: Contribution to journalArticle

Salvatori, Roberto ; Fintini, Danilo ; Westra, William H. ; Cho, Steve Y. ; Schulick, Richard D. / Cushing's syndrome attributable to ectopic secretion of corticotropin in a patient with two neuroendocrine tumors. In: Endocrine Practice. 2006 ; Vol. 12, No. 6. pp. 656-659.
@article{a71a49109e6a4070b5d568b4988e8916,
title = "Cushing's syndrome attributable to ectopic secretion of corticotropin in a patient with two neuroendocrine tumors",
abstract = "Objective: To report a case of ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) in a patient with two distinct neuroendocrine tumors and to highlight the difficulties of establishing the differential diagnosis of EAS. Methods: We describe the clinical presentation of the current case, discuss its management, and report the results of molecular studies undertaken to determine whether the two tumors had a common origin. Results: A 52-year-old woman presented with obvious features of Cushing's syndrome. Findings on hormonal evaluation were consistent with EAS. Pituitary magnetic resonance imaging revealed normal findings. Computed tomographic imaging disclosed two masses, one in the lung and one in the pancreas. Somatostatin receptor scintigraphy showed uptake only in the pancreatic mass, which was surgically removed. It was a well-differentiated neuroendocrine tumor, with negative immunostaining for ACTH. Hypercortisolemia did not resolve after removal of the pancreatic tumor. The lung mass was subsequently excised, and pathology examination showed a carcinoid tumor with immunostaining for ACTH. Thereafter, plasma ACTH became unmeasurable. The two tumors had similar patterns of X-chromosome inactivation; thus, whether they arose independently could be neither confirmed nor excluded. Conclusion: This case demonstrates that, in the presence of more than one neuroendocrine tumor, somatostatin receptor scintigraphy may misguide the decision regarding the appropriate surgical course in patients with EAS, and it highlights the need for accurate studies to determine the source of ACTH in patients with EAS.",
author = "Roberto Salvatori and Danilo Fintini and Westra, {William H.} and Cho, {Steve Y.} and Schulick, {Richard D.}",
year = "2006",
month = "11",
language = "English",
volume = "12",
pages = "656--659",
journal = "Endocrine Practice",
issn = "1530-891X",
publisher = "American Association of Clinical Endocrinology",
number = "6",

}

TY - JOUR

T1 - Cushing's syndrome attributable to ectopic secretion of corticotropin in a patient with two neuroendocrine tumors

AU - Salvatori, Roberto

AU - Fintini, Danilo

AU - Westra, William H.

AU - Cho, Steve Y.

AU - Schulick, Richard D.

PY - 2006/11

Y1 - 2006/11

N2 - Objective: To report a case of ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) in a patient with two distinct neuroendocrine tumors and to highlight the difficulties of establishing the differential diagnosis of EAS. Methods: We describe the clinical presentation of the current case, discuss its management, and report the results of molecular studies undertaken to determine whether the two tumors had a common origin. Results: A 52-year-old woman presented with obvious features of Cushing's syndrome. Findings on hormonal evaluation were consistent with EAS. Pituitary magnetic resonance imaging revealed normal findings. Computed tomographic imaging disclosed two masses, one in the lung and one in the pancreas. Somatostatin receptor scintigraphy showed uptake only in the pancreatic mass, which was surgically removed. It was a well-differentiated neuroendocrine tumor, with negative immunostaining for ACTH. Hypercortisolemia did not resolve after removal of the pancreatic tumor. The lung mass was subsequently excised, and pathology examination showed a carcinoid tumor with immunostaining for ACTH. Thereafter, plasma ACTH became unmeasurable. The two tumors had similar patterns of X-chromosome inactivation; thus, whether they arose independently could be neither confirmed nor excluded. Conclusion: This case demonstrates that, in the presence of more than one neuroendocrine tumor, somatostatin receptor scintigraphy may misguide the decision regarding the appropriate surgical course in patients with EAS, and it highlights the need for accurate studies to determine the source of ACTH in patients with EAS.

AB - Objective: To report a case of ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) in a patient with two distinct neuroendocrine tumors and to highlight the difficulties of establishing the differential diagnosis of EAS. Methods: We describe the clinical presentation of the current case, discuss its management, and report the results of molecular studies undertaken to determine whether the two tumors had a common origin. Results: A 52-year-old woman presented with obvious features of Cushing's syndrome. Findings on hormonal evaluation were consistent with EAS. Pituitary magnetic resonance imaging revealed normal findings. Computed tomographic imaging disclosed two masses, one in the lung and one in the pancreas. Somatostatin receptor scintigraphy showed uptake only in the pancreatic mass, which was surgically removed. It was a well-differentiated neuroendocrine tumor, with negative immunostaining for ACTH. Hypercortisolemia did not resolve after removal of the pancreatic tumor. The lung mass was subsequently excised, and pathology examination showed a carcinoid tumor with immunostaining for ACTH. Thereafter, plasma ACTH became unmeasurable. The two tumors had similar patterns of X-chromosome inactivation; thus, whether they arose independently could be neither confirmed nor excluded. Conclusion: This case demonstrates that, in the presence of more than one neuroendocrine tumor, somatostatin receptor scintigraphy may misguide the decision regarding the appropriate surgical course in patients with EAS, and it highlights the need for accurate studies to determine the source of ACTH in patients with EAS.

UR - http://www.scopus.com/inward/record.url?scp=33847759540&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=33847759540&partnerID=8YFLogxK

M3 - Article

VL - 12

SP - 656

EP - 659

JO - Endocrine Practice

JF - Endocrine Practice

SN - 1530-891X

IS - 6

ER -