scholarly journals MECHANISMS IN ENDOCRINOLOGY: Hypophysitis: diagnosis and treatment

2018 ◽  
Vol 179 (3) ◽  
pp. R151-R163 ◽  
Author(s):  
Mamta N Joshi ◽  
Benjamin C Whitelaw ◽  
Paul V Carroll
Keyword(s):  
Imaging Techniques ◽  
Immunosuppressive Agents ◽  
Treatment Strategies ◽  
Rare Condition ◽  
Diagnostic Value ◽  
Serum Markers ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Pituitary Mass ◽  
Pituitary Antibodies

Hypophysitis is a rare condition characterised by inflammation of the pituitary gland, usually resulting in hypopituitarism and pituitary enlargement. Pituitary inflammation can occur as a primary hypophysitis (most commonly lymphocytic, granulomatous or xanthomatous disease) or as secondary hypophysitis (as a result of systemic diseases, immunotherapy or alternative sella-based pathologies). Hypophysitis can be classified using anatomical, histopathological and aetiological criteria. Non-invasive diagnosis of hypophysitis remains elusive, and the use of currently available serum anti-pituitary antibodies are limited by low sensitivity and specificity. Newer serum markers such as anti-rabphilin 3A are yet to show consistent diagnostic value and are not yet commercially available. Traditionally considered a very rare condition, the recent recognition of IgG4-related disease and hypophysitis as a consequence of use of immune modulatory therapy has resulted in increased understanding of the pathophysiology of hypophysitis. Modern imaging techniques, histological classification and immune profiling are improving the accuracy of the diagnosis of the patient with hypophysitis. The objective of this review is to bring readers up-to-date with current understanding of conditions presenting as hypophysitis, focussing on recent advances and areas for future development. We describe the presenting features, investigation and diagnostic approach of the patient with likely hypophysitis, including existing conventional techniques and those in the research/development arena. Hypophysitis usually results in acute and persistent pituitary hormone deficiency requiring long-term replacement. Management of hypophysitis includes control of the inflammatory pituitary mass using a variety of treatment strategies including surgery and medical therapy. Glucocorticoids remain the mainstay of medical treatment but other immunosuppressive agents (e.g. azathioprine, rituximab) show benefit in some cases, but there is a need for controlled studies to inform practice.

Waxing and waning of a pituitary mass in a young woman with combined pituitary hormone deficiency (CPHD) due to a PROP-1 mutation

Pituitary ◽  
2006 ◽  
Vol 9 (1) ◽  
pp. 47-52 ◽  
Author(s):  
Sergio Oliva Nascif ◽  
Teresa Cristina Vieira ◽  
João Carlos Ramos-Dias ◽  
Ana-Maria Judith Lengyel ◽  
Julio Abucham
Keyword(s):  
Young Woman ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Pituitary Mass ◽  
Pituitary Hormone Deficiency ◽  
Combined Pituitary Hormone Deficiency

scholarly journals Rathke’s Cleft Cyst Abscess—An Unusual Guest in The Sella

2021 ◽  
Author(s):  
Piyush Lodha ◽  
Srinivas Rao P ◽  
Vijay Sheker Reddy Danda ◽  
Gollapudi Prakash Rao
Keyword(s):  
Visual Fields ◽  
Empty Sella ◽  
Abscess Formation ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Pituitary Mass ◽  
Empty Sella Syndrome ◽  
Rim Enhancement ◽  
Bitemporal Hemianopia

AbstractAbscess formation within a Rathkes’s cleft cyst (RCC) is extremely rare, particularly at a young age. We report the case of a young girl with abscess formation in RCC. A 21-year-old female presented with headache, vomiting, visual deterioration and features suggestive of hypopituitarism. She had bitemporal hemianopia with impairment of visual acuity. MRI revealed a cystic lesion in the sella with suprasellar extension and peripheral rim enhancement. On the basis of history and imaging, this was indistinguishable from more commonly encountered pituitary pathology. She underwent transsphenoidal decompression, which revealed yellowish purulent material that when cultured grew Staphylococcus epidermidis. Histological examination revealed numerous neutrophils and cyst wall lining with features characteristic of RCC. Postoperatively, she received antibiotics and replacement therapy for hypopituitarism. Three months later, she experienced deterioration in visual fields. Considering persistent disease, she underwent redo surgery which revealed similar findings. Postsurgery, pituitary MRI revealed an empty sella syndrome. Thereafter, follow-up for 1 year was stable with permanent diabetes insipidus and multiple pituitary hormone deficiency on supplementation. Although uncommon, we recommend considering RCC abscess as a differential diagnosis of a pituitary mass lesion, as predicting its presence can be difficult preoperatively. Persistent or recurrent disease is common in these cases, so timely diagnosis and adequate surgical drainage leads to lower morbidity and mortality.

Free-Floating Thrombus in the Distal Internal Carotid Artery Causing a Stroke

2020 ◽  
Author(s):  
Spyros Papadoulas ◽  
Konstantinos Moulakakis ◽  
Natasa Kouri ◽  
Petros Zampakis ◽  
Stavros K. Kakkos
Keyword(s):  
Carotid Artery ◽  
Internal Carotid Artery ◽  
Medical Management ◽  
Internal Carotid ◽  
Treatment Strategies ◽  
Rare Condition ◽  
Residual Stenosis ◽  
Floating Thrombus ◽  
Distal Internal Carotid Artery ◽  
Carotid Bulb

AbstractWe present a patient suffering from a stroke with a free-floating thrombus extending up to the distal internal carotid artery. The thrombus was totally resolved after a 2-week anticoagulation regimen without leaving behind any severe residual stenosis in the carotid bulb. The optimal treatment of this rare condition remains uncertain. We report some important treatment strategies that have been used in the literature, emphasizing the anticoagulation as the mainstay of therapy. Immediate surgical and interventional manipulations carry the risk of thrombus dislodgement and embolization and should be considered if there are recurrent symptoms despite medical management.

Metabolic status of patients with combined pituitary hormone deficiency due to PROP1 mutation

2020 ◽  
Author(s):  
Damian Rogoziński ◽  
Aleksandra Gilis-Januszewska ◽  
Łukasz Kluczyński ◽  
Magdalena Godlewska ◽  
Alicja Hubalewska-Dydejczyk
Keyword(s):  
Metabolic Status ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Pituitary Hormone Deficiency ◽  
Combined Pituitary Hormone Deficiency

scholarly journals Current Trends in Non-Invasive Imaging of Interactions in the Liver Tumor Microenvironment Mediated by Tumor Metabolism

Cancers ◽  
2021 ◽  
Vol 13 (15) ◽  
pp. 3645
Author(s):  
Isabel Theresa Schobert ◽  
Lynn Jeanette Savic
Keyword(s):  
Tumor Microenvironment ◽  
Liver Cancer ◽  
Cancer Cells ◽  
Imaging Techniques ◽  
Treatment Strategies ◽  
Tumor Metabolism ◽  
Resistance Mechanisms ◽  
Metabolic Reprogramming ◽  
Non Invasive

With the increasing understanding of resistance mechanisms mediated by the metabolic reprogramming in cancer cells, there is a growing clinical interest in imaging technologies that allow for the non-invasive characterization of tumor metabolism and the interactions of cancer cells with the tumor microenvironment (TME) mediated through tumor metabolism. Specifically, tumor glycolysis and subsequent tissue acidosis in the realms of the Warburg effect may promote an immunosuppressive TME, causing a substantial barrier to the clinical efficacy of numerous immuno-oncologic treatments. Thus, imaging the varying individual compositions of the TME may provide a more accurate characterization of the individual tumor. This approach can help to identify the most suitable therapy for each individual patient and design new targeted treatment strategies that disable resistance mechanisms in liver cancer. This review article focuses on non-invasive positron-emission tomography (PET)- and MR-based imaging techniques that aim to visualize the crosstalk between tumor cells and their microenvironment in liver cancer mediated by tumor metabolism.

A novel diagnostic tool for the evaluation of hypothalamic-pituitary region and diagnosis of growth hormone deficiency: pons ratio

2020 ◽  
Vol 33 (6) ◽  
pp. 735-742
Author(s):  
Meliha Demiral ◽  
Mehmet Salih Karaca ◽  
Edip Unal ◽  
Birsen Baysal ◽  
Rıza Taner Baran ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Growth Hormone Deficiency ◽  
Cost Benefit ◽  
Practical Method ◽  
Hormone Deficiency ◽  
Small Scale ◽  
Pituitary Hormone ◽  
Clinical Value ◽  
Pubertal Status ◽  
Sensitive Tool

AbstractBackgroundsLimitations in the evaluation of the pituitary size and changes according to pubertal status make its validity questionable. Recently, in a small-scale study, pons ratio (PR) has been suggested as a more sensitive tool for diagnosis and etiological evaluation of growth hormone deficiency (GHD). The aim of the study is to evaluate the diagnostic value of PR in the diagnosis of GHD.MethodsWe retrospectively evaluated the pituitary magnetic resonance imaging (MRI) of 133 patients with a diagnosis of GHD. Primary axis (PA) was assigned as a line crossing the mid-sagittal dorsum sella and fourth ventricle. PR was defined as the pons height above the PA divided by total pons height. The PR of patients with GHD was compared to subjects without GHD.ResultsStudy included 133 patients with GHD and 47 controls. In total, 121 (91%) patients had isolated GHD and 12 (9%) patients had multiple pituitary hormone deficiency. The PR of the patient group (mean: 0.32 ± 0.89; range: 0.14–0.63) was significantly higher than controls (mean: 0.26 ± 0.067; range 0.19–0.44) (p: 0.000). The optimal cut-off value of PR for GHD diagnosis was 0.27 (sensitivity 71% specificity 56%). There was a negative correlation between anterior pituitary height (APH)-SDS and PR (p: 0.002; r: −0.27). APH was increased, but PR remained unchanged in pubertal patients (p: 0.089).ConclusionsPR measurement is a noninvasive, practical method with a cost-benefit clinical value. As it is not affected by pubertal status, PR is potentially a more sensitive tool for evaluation of pituitary gland in GHD patients compared to APH.

scholarly journals Allelic Variants in Established Hypopituitarism Genes Expand Our Knowledge of the Phenotypic Spectrum

Genes ◽  
2021 ◽  
Vol 12 (8) ◽  
pp. 1128
Author(s):  
Marilena Nakaguma ◽  
Nathalia Garcia Bianchi Pereira Ferreira ◽  
Anna Flavia Figueredo Benedetti ◽  
Mariana Cotarelli Madi ◽  
Juliana Moreira Silva ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Male Patient ◽  
Missense Variant ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Incomplete Penetrance ◽  
Growth Hormone Gene ◽  
Posterior Lobe ◽  
Allelic Variants ◽  
Phenotypic Spectrum

We report four allelic variants (three novel) in three genes previously established as causal for hypopituitarism or related disorders. A novel homozygous variant in the growth hormone gene, GH1 c.171delT (p.Phe 57Leufs*43), was found in a male patient with severe isolated growth hormone deficiency (IGHD) born to consanguineous parents. A hemizygous SOX3 allelic variant (p.Met304Ile) was found in a male patient with IGHD and hypoplastic anterior pituitary. YASARA, a tool to evaluate protein stability, suggests that p.Met304Ile destabilizes the SOX3 protein (ΔΔG = 2.49 kcal/mol). A rare, heterozygous missense variant in the TALE homeobox protein gene, TGIF1 (c.268C>T:p.Arg90Cys) was found in a patient with combined pituitary hormone deficiency (CPHD), diabetes insipidus, and syndromic features of holoprosencephaly (HPE). This variant was previously reported in a patient with severe holoprosencephaly and shown to affect TGIF1 function. A novel heterozygous TGIF1 variant (c.82T>C:p.Ser28Pro) was identified in a patient with CPHD, pituitary aplasia and ectopic posterior lobe. Both TGIF1 variants have an autosomal dominant pattern of inheritance with incomplete penetrance. In conclusion, we have found allelic variants in three genes in hypopituitarism patients. We discuss these variants and associated patient phenotypes in relation to previously reported variants in these genes, expanding our knowledge of the phenotypic spectrum in patient populations.

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