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Cushing's Syndrome Due to a Corticotropin-Releasing Hormone- and Adrenocorticotrophic Hormone-Producing Neuroendocrine Pancreatic Tumor

Standard

Cushing's Syndrome Due to a Corticotropin-Releasing Hormone- and Adrenocorticotrophic Hormone-Producing Neuroendocrine Pancreatic Tumor. / Sauer, Nina; Zur Wiesch, Clarissa Schulze; Flitsch, Jörg; Saeger, Wolfgang; Klutmann, Susanne; Zustin, Josef; Lübke, Andreas; Aberle, Jens.

In: ENDOCR PRACT, Vol. 20, No. 4, 2014, p. e53-7.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Sauer, N, Zur Wiesch, CS, Flitsch, J, Saeger, W, Klutmann, S, Zustin, J, Lübke, A & Aberle, J 2014, 'Cushing's Syndrome Due to a Corticotropin-Releasing Hormone- and Adrenocorticotrophic Hormone-Producing Neuroendocrine Pancreatic Tumor', ENDOCR PRACT, vol. 20, no. 4, pp. e53-7. https://doi.org/10.4158/EP13001.CR

APA

Sauer, N., Zur Wiesch, C. S., Flitsch, J., Saeger, W., Klutmann, S., Zustin, J., Lübke, A., & Aberle, J. (2014). Cushing's Syndrome Due to a Corticotropin-Releasing Hormone- and Adrenocorticotrophic Hormone-Producing Neuroendocrine Pancreatic Tumor. ENDOCR PRACT, 20(4), e53-7. https://doi.org/10.4158/EP13001.CR

Vancouver

Sauer N, Zur Wiesch CS, Flitsch J, Saeger W, Klutmann S, Zustin J et al. Cushing's Syndrome Due to a Corticotropin-Releasing Hormone- and Adrenocorticotrophic Hormone-Producing Neuroendocrine Pancreatic Tumor. ENDOCR PRACT. 2014;20(4):e53-7. https://doi.org/10.4158/EP13001.CR

Bibtex

@article{e790823c78654a9aaa5732b9aca822a2,
title = "Cushing's Syndrome Due to a Corticotropin-Releasing Hormone- and Adrenocorticotrophic Hormone-Producing Neuroendocrine Pancreatic Tumor",
abstract = "Objective: To our knowledge, only 2 cases of pancreatic neuroendocrine tumors have been described as the source of corticotropin-releasing hormone (CRH) in Cushing's syndrome. Here, we describe a case of ectopic adrenocorticotrophic hormone (ACTH-) and CRH-production caused by a pancreatic neuroendocrine tumor.Methods: We analyzed and summarized the patient's medical history, physical examination results, laboratory data, imaging studies, and histopathologic results.Results: An endocrinologic workup revealed massive ACTH-dependent hypercortisolism. Pituitary magnetic resonance imaging (MRI) showed no pathologic findings and led to extensive imaging in search of the suspected ectopic lesion. Ketoconazole treatment was initiated. Rapid deterioration of the patient's clinical condition due to escalating cortisol levels and resulting sepsis required an emergency adrenalectomy to control the hypercortisolism. A positron emission tomography-computed tomography (PET-CT) scan revealed a hepatic lesion, which was biopsied. Histology of the lesion showed a well-differentiated endocrine tumor. Subsequent scintigraphy with octreotide (a somatostatin [SMS] analog) detected a pancreatic tumor, which was endosonographically confirmed. The initiated SMS therapy was followed by a distal splenopancreatectomy and a right hemihepatectomy. Immunostaining of the specimen showed positive expression for CRH and ACTH.Conclusion: We conclude that SMS-scintigraphy did have an additional diagnostic benefit compared to PET-CT. In hypercortisolemic patients, rapid endocrinologic evaluation is crucial to prevent rapid deterioration and a possible fatal outcome.",
author = "Nina Sauer and {Zur Wiesch}, {Clarissa Schulze} and J{\"o}rg Flitsch and Wolfgang Saeger and Susanne Klutmann and Josef Zustin and Andreas L{\"u}bke and Jens Aberle",
note = "Wolfgang Saeger INTERNE Person ",
year = "2014",
doi = "10.4158/EP13001.CR",
language = "English",
volume = "20",
pages = "e53--7",
journal = "ENDOCR PRACT",
issn = "1530-891X",
publisher = "American Association of Clinical Endocrinology",
number = "4",

}

RIS

TY - JOUR

T1 - Cushing's Syndrome Due to a Corticotropin-Releasing Hormone- and Adrenocorticotrophic Hormone-Producing Neuroendocrine Pancreatic Tumor

AU - Sauer, Nina

AU - Zur Wiesch, Clarissa Schulze

AU - Flitsch, Jörg

AU - Saeger, Wolfgang

AU - Klutmann, Susanne

AU - Zustin, Josef

AU - Lübke, Andreas

AU - Aberle, Jens

N1 - Wolfgang Saeger INTERNE Person

PY - 2014

Y1 - 2014

N2 - Objective: To our knowledge, only 2 cases of pancreatic neuroendocrine tumors have been described as the source of corticotropin-releasing hormone (CRH) in Cushing's syndrome. Here, we describe a case of ectopic adrenocorticotrophic hormone (ACTH-) and CRH-production caused by a pancreatic neuroendocrine tumor.Methods: We analyzed and summarized the patient's medical history, physical examination results, laboratory data, imaging studies, and histopathologic results.Results: An endocrinologic workup revealed massive ACTH-dependent hypercortisolism. Pituitary magnetic resonance imaging (MRI) showed no pathologic findings and led to extensive imaging in search of the suspected ectopic lesion. Ketoconazole treatment was initiated. Rapid deterioration of the patient's clinical condition due to escalating cortisol levels and resulting sepsis required an emergency adrenalectomy to control the hypercortisolism. A positron emission tomography-computed tomography (PET-CT) scan revealed a hepatic lesion, which was biopsied. Histology of the lesion showed a well-differentiated endocrine tumor. Subsequent scintigraphy with octreotide (a somatostatin [SMS] analog) detected a pancreatic tumor, which was endosonographically confirmed. The initiated SMS therapy was followed by a distal splenopancreatectomy and a right hemihepatectomy. Immunostaining of the specimen showed positive expression for CRH and ACTH.Conclusion: We conclude that SMS-scintigraphy did have an additional diagnostic benefit compared to PET-CT. In hypercortisolemic patients, rapid endocrinologic evaluation is crucial to prevent rapid deterioration and a possible fatal outcome.

AB - Objective: To our knowledge, only 2 cases of pancreatic neuroendocrine tumors have been described as the source of corticotropin-releasing hormone (CRH) in Cushing's syndrome. Here, we describe a case of ectopic adrenocorticotrophic hormone (ACTH-) and CRH-production caused by a pancreatic neuroendocrine tumor.Methods: We analyzed and summarized the patient's medical history, physical examination results, laboratory data, imaging studies, and histopathologic results.Results: An endocrinologic workup revealed massive ACTH-dependent hypercortisolism. Pituitary magnetic resonance imaging (MRI) showed no pathologic findings and led to extensive imaging in search of the suspected ectopic lesion. Ketoconazole treatment was initiated. Rapid deterioration of the patient's clinical condition due to escalating cortisol levels and resulting sepsis required an emergency adrenalectomy to control the hypercortisolism. A positron emission tomography-computed tomography (PET-CT) scan revealed a hepatic lesion, which was biopsied. Histology of the lesion showed a well-differentiated endocrine tumor. Subsequent scintigraphy with octreotide (a somatostatin [SMS] analog) detected a pancreatic tumor, which was endosonographically confirmed. The initiated SMS therapy was followed by a distal splenopancreatectomy and a right hemihepatectomy. Immunostaining of the specimen showed positive expression for CRH and ACTH.Conclusion: We conclude that SMS-scintigraphy did have an additional diagnostic benefit compared to PET-CT. In hypercortisolemic patients, rapid endocrinologic evaluation is crucial to prevent rapid deterioration and a possible fatal outcome.

U2 - 10.4158/EP13001.CR

DO - 10.4158/EP13001.CR

M3 - SCORING: Journal article

C2 - 24325992

VL - 20

SP - e53-7

JO - ENDOCR PRACT

JF - ENDOCR PRACT

SN - 1530-891X

IS - 4

ER -