Abstract #1086883: A Rare Case of Ectopic ACTH Syndrome Associated With a Hepatocellular Carcinoma

Abstract Background Manifestations of hypokalaemia in ectopic adrenocorticotropic hormonesyndrome(EAS) vary from mild muscle weakness to life-threatening arrhythmia. Herein, we present a rare case of EAS with concomitant rhabdomyolysis(RM) as a result of intractable hypokalaemia. Case presentation A 64-year-old man was admitted for limb weakness and facial hyperpigmentation for 2 weeks. Lab tests revealed intractable hypokalaemia (lowest at 1.8 mmol/L) and metabolic alkalosis. The diagnosis of RM was based on a creatine kinase(CK)level of 5 times the upper limit. The elevated CK and myohemoglobin (Mb) levels returned to within the normal range after the alleviation of hypokalaemia. The patient was diagnosed with ACTH-dependent Cushing’s syndrome (CS) based on unsuppressed serum cortisol after a low-dose dexamethasone suppression test(LDDST) and remarkably elevated ACTH levels. The diagnosis of EAS was made based on the results of a high-dose dexamethasone suppression test(HDDST) and bilateral inferior petrosal sinus sampling(BIPSS). Multiple lymph nodes in the left supraclavicular fossa, right root of neck, mediastinum and bilateral hili of the lung were found with abnormal uptake of 68Ga-DOTA-NOC. Mediastinoscopic lymph node biopsy was performed. The pathological diagnosis was small-cell and large-cell neuroendocrine carcinoma with positive ACTH staining. The patient was prescribed mifepristone and received one cycle of chemotherapy. The patient could not tolerate subsequent chemotherapy and died of dyscrasia. Conclusions RM is a rare complication of EAS with insidious onset and atypical clinical manifestations. Serum potassium levels should be vigilantly monitored to avoid RM in EAS.

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A rare case of ectopic ACTH syndrome due to recurrence of olfactory neuroblastoma

Endocrine Abstracts ◽

10.1530/endoabs.63.ep17 ◽

2019 ◽

Author(s):

Hironori Abe ◽

Hirotsugu Suwanai ◽

Takuya Ishikawa ◽

Fumiyoshi Yakou ◽

Natsuko Hara ◽

...

Keyword(s):

Rare Case ◽

Olfactory Neuroblastoma ◽

Ectopic Acth Syndrome ◽

Ectopic Acth

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A rare case of ectopic ACTH syndrome originated from malignant renal paraganglioma

Endocrine Abstracts ◽

10.1530/endoabs.41.ep76 ◽

2016 ◽

Author(s):

Esra Tutal ◽

Demet Yilmazer ◽

Taner Demirci ◽

Erman Cakal ◽

Evrim Cakir ◽

...

Keyword(s):

Rare Case ◽

Ectopic Acth Syndrome ◽

Ectopic Acth

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A rare case of ectopic ACTH syndrome originating from malignant renal paraganglioma

Archives of Endocrinology and Metabolism ◽

10.1590/2359-3997000000240 ◽

2017 ◽

Vol 61 (3) ◽

pp. 291-295 ◽

Cited By ~ 2

Author(s):

Esra Tutal ◽

Demet Yılmazer ◽

Taner Demirci ◽

Evrim Cakır ◽

Salih Sinan Gültekin ◽

...

Keyword(s):

Rare Case ◽

Ectopic Acth Syndrome ◽

Ectopic Acth

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Adrenocortical Ultrastructure in Ectopic Acth Syndrome

Proceedings, annual meeting, Electron Microscopy Society of America ◽

10.1017/s0424820100091172 ◽

1976 ◽

Vol 34 ◽

pp. 238-239

Author(s):

K. Kovacs ◽

E. Horvath ◽

W. Singer

Keyword(s):

Adrenal Cortex ◽

Zona Glomerulosa ◽

Ectopic Acth Syndrome ◽

Pituitary Neoplasms ◽

Electron Microscopic ◽

Adrenocortical Cells ◽

Cell Hyperplasia ◽

Ectopic Acth ◽

Ectopic Acth Production ◽

Microscopic Findings

Secretion of ACTH by non-pituitary neoplasms is recognized with increasing frequency. While the clinical and biochemical changes associated with ectopic ACTH production have been extensively studied recently, relatively little attention was focused on the morphology of the adrenal cortex and, to our knowledge, the fine structure of the adrenocortical cells in cases of ectopic ACTH syndrome has not been described so far. We report here the electron microscopic findings in the adrenal cortex of a 50-year-old man with a pancreatic apudoma. The patient showed the characteristic clinical and biochemical features of ectopic ACTH syndrome and because of extensive hypercorticism, underwent bilateral adrenalectomy.By light microscopy, the adrenal cortices showed extensive compact cell hyperplasia and lipid depletion. The zona glomerulosa was present in small foci and, except for a few places, fasciculata cells were noted under the fibrous capsule.

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Electron Microscopic investigation of crooke’s change in the pituitaries of patients with hypercorticism

Proceedings, annual meeting, Electron Microscopy Society of America ◽

10.1017/s0424820100156225 ◽

1989 ◽

Vol 47 ◽

pp. 848-849

Author(s):

E. Horvath ◽

K. Kovacs ◽

L. Stefaneanu ◽

N. Losinski

Keyword(s):

Nucleolar Organizer ◽

Microscopic Investigation ◽

Electron Microscopic Investigation ◽

Ectopic Acth Syndrome ◽

Nucleolar Organizer Regions ◽

Electron Microscopic ◽

Human Pituitary ◽

Ectopic Acth ◽

Crooke’S Hyalinization

Human pituitary corticotropins have unique morphologic markers: bundles of type-1 filaments, measuring approximately 70 A in width and representing cytokeratin. The extreme ring-like accumulation of type-1 filaments, known as Crooke's hyalinization, signals functional suppression of the corticotropins and occurs in endogenous and exogenous glucocorticoid excess, caused by ACTH-secreting pituitary adenoma, glucocorticoid secreting adrenocortical tumor, ectopic ACTH-syndrome and administration of pharmacologic doses of glucocorticoids. Cells of autonomous corticotroph adenomas usually do not show Crooke's hyalin change. A minority of these tumors, however, retains sensitivity to the negative feed-back effect of elevated blood glucocorticoid levels and display typical Crooke’s change.In the present study pituitary corticotropins in various phases of Crooke's hyalinization were investigated in patients with glucocorticoid excess of various origin, applying histology, immunocytochemistry, count of argyrophilic nucleolar organizer regions (AgNOR), and transmission electron microscopy.

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Abstract #21: Ectopic ACTH Syndrome from Cervical Cancer

Endocrine Practice ◽

10.1016/s1530-891x(20)43869-8 ◽

2003 ◽

Vol 9 ◽

pp. 8

Author(s):

Kashif M. Munir ◽

Richard B. Horenstein

Keyword(s):

Cervical Cancer ◽

Ectopic Acth Syndrome ◽

Ectopic Acth

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Assessment of endocrine disorders of the hypothalamicpituitary axis by nuclear medicine techniques

Nuklearmedizin ◽

10.1055/s-0038-1625645 ◽

2002 ◽

Vol 41 (02) ◽

pp. 80-90 ◽

Cited By ~ 2

Author(s):

F. Jockenhövel ◽

P. Theissen ◽

M. Dietlein ◽

W. Krone ◽

H. Schicha ◽

...

Keyword(s):

Nuclear Medicine ◽

Pituitary Adenomas ◽

Dopamine D2 Receptor ◽

Somatostatin Receptor ◽

D2 Receptor ◽

Ectopic Acth Syndrome ◽

Endocrine Disorders ◽

Ectopic Acth ◽

Number Of Patients ◽

Hypothalamic Pituitary Axis

SummaryThe following article reviews nuclear medicine techniques which can be used for assessment of endocrine disorders of the hypothalamic-pituitary axis. For planar and SPECT imaging somatostatin-receptor- and dopamine- D2-receptor-scintigraphy are the most widely distributed techniques. These nuclear medicine techniques may be indicated in selected cases to answer differential diagnostic problems. They can be helpful to search for presence and localization of receptor positive tissue. Furthermore they can detect metastasis in the rare cases of a pituitary carcinoma. Scintigraphy with Gallium-67 is suitable for further diagnostic evaluation in suspected hypophysitis. Other SPECT radiopharmaca do not have relevant clinical significance. F-18-FDG as PET radiopharmacon is not ideal because obvious pituitary adenomas could not be visualized. Other PET radiopharmaca including C-11-methionine, C-11-tyrosine, F-18-fluoroethylspiperone, C-11-methylspiperone, and C-11-raclopride are available in specialized centers only. Overall indications for nuclear medicine in studies for the assessment of endocrine disorders of the hypothalamic-pituitary-axis are rare. Original studies often report only about a small number of patients. According to the authors’ opinion the relevance of nuclear medicine in studies of clinically important endocrinologic fields, e. g. localization of small ACTH-producing pituitary adenomas, tumor localization in ectopic ACTH syndrome, localization of recurrent pituitary tissue, assessment of small incidentalomas, can not be definitely given yet.

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