CORTICOSTERONE SECRETING ADRENAL CARCINOMA AND EMPTY SELLA TURCICA: A CASE REPORT

ABSTRACT Pituitary adenoma was suspected in a woman of 74 with hypercorticism, hypokalaemic alkalosis and radiographically enlarged sella turcica. However, non-suppressibility of steroid excretion by high-dose dexamethasone and low plasma concentration of ACTH suggested adrenal tumour. Detailed analysis of urinary steroid excretions demonstrated unusually large amounts of corticosterone metabolites, 14.6 mg/24 h compared to a mean normal value of 0.5 mg. The basal levels of the remaining pituitary hormones were unremarkable. The patient died incidentally before a planned adrenalectomy. The autopsy disclosed an adrenal carcinoma and an empty sella turcica. The enlarged pituitary fossa was lined by a narrow rim of histological normal pituitary tissue.

Download Full-text

Empty Sella Prevalence: Step By Step

Russian Open Medical Journal ◽

10.15275/rusomj.2021.0206 ◽

2021 ◽

Vol 10 (2) ◽

Author(s):

Victoria B. Sharavii ◽

Natalya A. Shnayder ◽

Lada K. Sharavii ◽

Marina M. Petrova ◽

Regina F. Nasyrova

Keyword(s):

Pituitary Gland ◽

Subarachnoid Space ◽

Sella Turcica ◽

Empty Sella ◽

Systematic Search ◽

Pituitary Fossa ◽

The World

An empty sella (ES), also known as an empty sella turcica (empty pituitary fossa) is defined as an intrasellar herniation of subarachnoid space within the sella which leads to flattening of the pituitary gland towards the posteroinferior wall. Purpose — to analyze studies of ES prevalence in the world. Methods — We carried out a systematic search in both Russian and English databases (E-library, PubMed, GoogleScholar, OxfordPress, ClinicalKeys) using keywords “empty sella”, “empty sella turcica”, “empty pituitary fossa”, “prevalence” and their combinations. The period of search: 2000-2020. It is also worth noting that earlier publications of historical interest were included in the review. Results — We have analyzed 23 studies. They can be roughly devided into two groups: the first group – screening studies of ES, the second group – targeted studies of ES.

Download Full-text

Cushing's syndrome due to unilateral nodular adrenal hyperplasia: a new pathophysiological entity?

Acta Endocrinologica ◽

10.1530/acta.0.0930495 ◽

1980 ◽

Vol 93 (4) ◽

pp. 495-504 ◽

Cited By ~ 10

Author(s):

Robert G. Josse ◽

Robert Bear ◽

Kalman Kovacs ◽

H. Patrick Higgins

Keyword(s):

Adrenal Gland ◽

Cushing’S Syndrome ◽

Plasma Cortisol ◽

Electron Microscopic Examination ◽

Cushing's Syndrome ◽

High Dose ◽

Adrenal Hyperplasia ◽

Electron Microscopic ◽

High Dose Dexamethasone ◽

Steroid Excretion

Abstract. A case is presented of a 43-year-old Caucasian female with Cushing's syndrome, malignant hypertension and renal insufficiency. Plasma cortisol values were elevated (19.5 μg/100 ml at 0800 h and 18.5 μg/100 ml at 2200 h) with loss of diurnal variation and failure of suppression with low and high dose dexamethasone. 17-ketogenic steroid excretion remained unchanged after metyrapone administration. Adrenal vein plasma cortisol values showed prominent left-sided gradient. Gross, histologic and electron-microscopic examination of the left adrenal gland revealed changes of nodular adrenal hyperplasia. The presence of a suppressed right adrenal gland was confirmed post-operatively by a five day ACTH infusion. This case may represent a previously unreported variant of Cushing's syndrome due to unilateral nodular adrenal hyperplasia.

Download Full-text

Late-Onset Isolated Corticotrope Deficiency in a Woman with Down Syndrome

Case Reports in Endocrinology ◽

10.1155/2021/5562831 ◽

2021 ◽

Vol 2021 ◽

pp. 1-4

Author(s):

Ibtissem Oueslati ◽

Marwa Ben Jemaa ◽

Meriem Yazidi ◽

Fatma Chaker ◽

Melika Chihaoui

Keyword(s):

Down Syndrome ◽

Late Onset ◽

Copy Number Variants ◽

Sella Turcica ◽

Pituitary Hormones ◽

Empty Sella ◽

Magnetic Resonance Imaging Scan ◽

The Past ◽

Low Blood Pressure ◽

Thyroperoxidase Antibodies

Isolated corticotrope deficiency is a rare cause of secondary adrenocortical insufficiency. Its occurrence in patients with Down syndrome is exceptional. Herein, we report a case of an isolated corticotrope deficiency diagnosed at the age of 33 years in a woman with Down syndrome and discuss its possible mechanisms. A 33-year-old woman with Down syndrome was referred to our department for the investigation of low blood pressure. She complained of asthenia, dizziness, and palpitation with arterial hypotension for the past 4 years. The thyroid function was normal and anti-thyroperoxidase antibodies were negative. The peak of cortisol level in response to the insulin-induced hypoglycemia test was 9.4 μg/dl. ACTH level was normal, indicating corticotrope deficiency. Other pituitary hormones were normal. Magnetic resonance imaging scan revealed a partially empty sella turcica. Genetic analysis showed no mutations and no copy number variants of the TBX19 and NFKB2 genes. The mechanism of isolated corticotrope deficiency is unclear, but it may be induced by autoimmune mechanism in similar to other disorders of patients with Down syndrome.

Download Full-text

Primary hypothyroidism associated with empty sella turcica and hypopituitarism

Medicinski pregled ◽

10.2298/mpns0508410m ◽

2005 ◽

Vol 58 (7-8) ◽

pp. 410-413 ◽

Cited By ~ 2

Author(s):

Maja Milosevic ◽

Milos Stojanovic ◽

Milica Nesovic

Keyword(s):

Sella Turcica ◽

Pituitary Hormones ◽

Empty Sella ◽

Primary Hypothyroidism ◽

Pituitary Hormone ◽

Pituitary Insufficiency ◽

Central Hypothyroidism ◽

Empty Sella Syndrome ◽

The Third ◽

High Level

Introduction Empty sella syndrome is a rather frequent neuroradiological finding in the general population and can be associated with hypopituitarism. Examinations reveal low pituitary hormone levels and lack of response to stimuli. Most patients suffer from central hypothyroidism as part of pituitary insufficiency. Primary hypothyreoidism is a rare finding in these patients. Case report We present 3 patients: one female and two male, suffering from complete hypopituitarism, as part of the empty sella syndrome diagnosed due to low concentrations of all pituitary hormones, elevated TSH and low thyroid hormones. TRH, LHRH, ACTH and ITT tests, as well as IGF1 have confirmed hypopituitarism and primary hypothyroidism. CT and NMR in all three patients showed empty sella without a tumor in it. The diagnosis of primary hypothyrodism in the first patient was made before hypopituitarism has taken place, or at the same time in the second patient, whereas in the third patient it was diagnosed twenty years later. In two patients anti-TPO and anti-Tg antibody levels were high, and in the third patient they were not elevated. It can be assumed that the etiology of primary hypothyrodism in all three patients was of autoimmune origin, which caused thyroid hypofunction. High level of TSH in all three patients and especially in the patient whose hypopituitarism was diagnosed twenty years later, showed presence of thyrotrophic cells in the pituitary. Evaluation of the hypothalamic-pituitary-thyroid axis was carried out during the complete substitution therapy of hypopituitarism. Conclusion Diagnosing primary hypothyrodism associated with hypopituitarism helps improving the knowledge on empty sella syndrome and points to different clinical syndromes characterized by lack of mixoedema, although approach to therapy is the same for both primary and central hypothyroidism.

Download Full-text

OESTROGENS IN VIRILIZING ADRENAL CARCINOMA

Acta Endocrinologica ◽

10.1530/acta.0.0520443 ◽

1966 ◽

Vol 52 (3) ◽

pp. 443-454 ◽

Cited By ~ 2

Author(s):

George I. Loutfi ◽

Kendall Emerson

Keyword(s):

Primary Tumour ◽

Total Phenolic ◽

Adrenal Carcinoma ◽

The Third ◽

Original Tumour ◽

Subsequent Decrease ◽

Before And After ◽

One Year ◽

Steroid Excretion ◽

Urinary Steroid

ABSTRACT The urinary excretion of oestrone, 17β-oestradiol and oestriol, together with total phenolic steroids and 17-ketosteroids, were measured in three patients with virilizing adrenal carcinoma before and after removal of the primary tumour and again after the development of functioning metastasis. The patient who excreted chiefly oestrone had a 10-year survival after removal of the original tumour in spite of the presence of metastases, whereas one of the two subjects who excreted predominantly oestriol died within one year after removal of the primary tumour. In the third case the primary tumour was almost completely necrotic at the time of surgery 4 years ago. Corticotrophin stimulation increased the excretion of the predominant metabolite by the primary tumour, whereas dexamethasone restored a more normal pattern of steroid excretion and reduced the total urinary phenolic steroids. The steroid excretion by the metastases, on the other hand, was not affected by either procedure in 2 cases. In the third patient necrosis and disappearance of liver metastases with a subsequent decrease of urinary steroid excretion was observed following the infusion of corticotrophin.

Download Full-text

Enlargement of the sella turcica in pseudotumor cerebri

Journal of Neurosurgery ◽

10.3171/2013.10.jns131265 ◽

2014 ◽

Vol 120 (2) ◽

pp. 538-542 ◽

Cited By ~ 20

Author(s):

Sung-eun E. Kyung ◽

James V. Botelho ◽

Jonathan C. Horton

Keyword(s):

Intracranial Pressure ◽

Pituitary Gland ◽

Sella Turcica ◽

Pseudotumor Cerebri ◽

Empty Sella ◽

Slight Reduction ◽

Mr Images ◽

Pituitary Fossa ◽

Cross Sectional ◽

Bony Enlargement

Object The sella turcica usually appears partially empty in MR images obtained from patients with chronic elevation of intracranial pressure. The authors measured the size of the sella turcica to determine if enlargement of the pituitary fossa explains the partially empty sella associated with pseudotumor cerebri. Methods The medical records from 2005 to 2011 of a single neuro-ophthalmologist were searched to identify consecutive patients with pseudotumor cerebri. Age-matched control patients were selected from the same practice. The sella turcica and pituitary gland were measured on sagittal T1-weighted MR images. Results Measurements were obtained for 48 patients with pseudotumor cerebri and 48 controls. The cross-sectional area of the sella was 38% greater in the patients with pseudotumor cerebri, with only a slight reduction in mean pituitary gland size. Conclusions Chronic elevation of intracranial pressure is associated with bony enlargement of the sella turcica. Enlargement of the sella turcica contributes to its partially empty appearance.

Download Full-text

Reconstruction of the Pituitary Fossa in Transsphenoidal Surgery: An Experience of 140 Cases

Neurosurgery ◽

10.1227/00006123-198509000-00009 ◽

1985 ◽

Vol 17 (3) ◽

pp. 453-458 ◽

Cited By ~ 54

Author(s):

Renato Spaziante ◽

Enrico de Divitiis ◽

Paolo Cappabianca

Keyword(s):

Cerebrospinal Fluid ◽

Transsphenoidal Surgery ◽

Sella Turcica ◽

Empty Sella ◽

Transsphenoidal Approach ◽

Cerebrospinal Fluid Leakage ◽

Pituitary Fossa ◽

Postoperative Course ◽

Precise Method ◽

Surgical Condition

Abstract Even though the main problems of transsphenoidal surgery are encountered during the intra- and suprasellar portions of the operation, proper reconstruction of the sella turcica plays an important role in the overall results, greatly influencing the final outcome. Improper measures may bring about a complicated postoperative course, whereas a wise and precise method allows one to control any surgical condition, preventing the more dangerous consequences of the transsphenoidal approach (i.e., empty sella, cerebrospinal fluid leakage, hemorrhage, infection, etc.). The authors review the different methods of sellar plugging and, on the basis of their own experience, indicate the most suitable one for each of the more usual situations. A good result is achievable if only natural materials are used and one avoids synthetic prostheses. Extradural packing is the technique of choice whenever a hermetic closure and a particularly effective intrasellar plug are required.

Download Full-text

Pituitary volume in patients with Primary Empty Sella and clinical relevance to pituitary hormone secretion: A retrospective single center study

Current Medical Imaging Formerly Current Medical Imaging Reviews ◽

10.2174/1573405617666210525111218 ◽

2021 ◽

Vol 17 ◽

Author(s):

Gamze Akkus ◽

Sinan Sözütok ◽

Fulya Odabaş ◽

Bilen Onan ◽

Mehtap Evran ◽

...

Keyword(s):

Anterior Pituitary ◽

Hormone Secretion ◽

Sella Turcica ◽

Volume Measurement ◽

Pituitary Hormones ◽

Empty Sella ◽

Pituitary Hormone ◽

Pituitary Dysfunction ◽

Volume Measurements ◽

Primary Empty Sella

Background: According to neuroradiological findings, empty sella seems to be deprived of pituitary tissue in sella turcica. Changing size of the pituitary volume is closely related to the occurrence of primary empty sella. The aim of the study is to determine pituitary dysfunction in patients with partial or total primary empty sella and the significance of pituitary volume measurements in these patients. Methods: This study was designed retrospectively. 67 patients (55 females, 12 males) diagnosed with primary empty sella syndrome between the years of 2015-2019 were included in the study. Patients were divided into two groups: partial (PES) and total (TES) empty sella by magnetic resonance imaging (MRI). Basal anterior pituitary and its hormones were assessed. We also included 26 healthy control subjects (19 females, 7 males) to compare the differences in pituitary volumes. Volumes were measured by using Osirix Dicom Viewer ( Pixmeo SARL, Geneve, Swiss) in 3.0 Tesla scanner MRI. Results: 82.1% (n=55) of all patients were PES and the others were (n=12) TES. Hypopituitarism, known as one or more pituitary hormones deficiency, was found in 12 patients (17.9%). While 9 of them had total PES, the others had partial PES. Secondary adrenal insufficiency and gonadotropin deficiency were more prevalent in patients with TES. Mean volume measurements of patients with TES, PES and healthy subjects were 0.23±0.17, 0.35±0.15, 0.54±0.17 cm3, respectively. Except for IGF1 values (p=0.026), there was not any significant correlation found between the anterior pituitary hormones and volume measurements. Conclusion: Although volume measurement has helped in the diagnosis of pituitary empty sella (partial or total), it does not seem to have any significant correlation with pituitary secretory function.

Download Full-text