scholarly journals GHRH secretion from a pancreatic neuroendocrine tumor causing gigantism in a patient with MEN1

2021 ◽  
Vol 2021 ◽  
Author(s):  
Vinaya Srirangam Nadhamuni ◽  
Donato Iacovazzo ◽  
Jane Evanson ◽  
Anju Sahdev ◽  
Jacqueline Trouillas ◽  
...  
Keyword(s):  
Neuroendocrine Tumour ◽  
Primary Tumour ◽  
Elevated Serum ◽  
Pancreatic Neuroendocrine Tumor ◽  
Pituitary Surgery ◽  
List Type ◽  
Ki 67 ◽  
Subtotal Parathyroidectomy ◽  
Cross Sectional ◽  
Ectopic Acromegaly

Summary A male patient with a germline mutation in MEN1 presented at the age of 18 with classical features of gigantism. Previously, he had undergone resection of an insulin-secreting pancreatic neuroendocrine tumour (pNET) at the age of 10 years and had subtotal parathyroidectomy due to primary hyperparathyroidism at the age of 15 years. He was found to have significantly elevated serum IGF-1, GH, GHRH and calcitonin levels. Pituitary MRI showed an overall bulky gland with a 3 mm hypoechoic area. Abdominal MRI showed a 27 mm mass in the head of the pancreas and a 6 mm lesion in the tail. Lanreotide-Autogel 120 mg/month reduced GHRH by 45% and IGF-1 by 20%. Following pancreaticoduodenectomy, four NETs were identified with positive GHRH and calcitonin staining and Ki-67 index of 2% in the largest lesion. The pancreas tail lesion was not removed. Post-operatively, GHRH and calcitonin levels were undetectable, IGF-1 levels normalised and GH suppressed normally on glucose challenge. Post-operative fasting glucose and HbA1c levels have remained normal at the last check-up. While adolescent-onset cases of GHRH-secreting pNETs have been described, to the best of our knowledge, this is the first reported case of ectopic GHRH in a paediatric setting leading to gigantism in a patient with MEN1. Our case highlights the importance of distinguishing between pituitary and ectopic causes of gigantism, especially in the setting of MEN1, where paediatric somatotroph adenomas causing gigantism are extremely rare. Learning points It is important to diagnose gigantism and its underlying cause (pituitary vs ectopic) early in order to prevent further growth and avoid unnecessary pituitary surgery. The most common primary tumour sites in ectopic acromegaly include the lung (53%) and the pancreas (34%) (1): 76% of patients with a pNET secreting GHRH showed a MEN1 mutation (1). Plasma GHRH testing is readily available in international laboratories and can be a useful diagnostic tool in distinguishing between pituitary acromegaly mediated by GH and ectopic acromegaly mediated by GHRH. Positive GHRH immunostaining in the NET tissue confirms the diagnosis. Distinguishing between pituitary (somatotroph) hyperplasia secondary to ectopic GHRH and pituitary adenoma is difficult and requires specialist neuroradiology input and consideration, especially in the MEN1 setting. It is important to note that the vast majority of GHRH-secreting tumours (lung, pancreas, phaeochromocytoma) are expected to be visible on cross-sectional imaging (median diameter 55 mm) (1). Therefore, we suggest that a chest X-ray and an abdominal ultrasound checking the adrenal glands and the pancreas should be included in the routine work-up of newly diagnosed acromegaly patients.

Uncommon sites for metastasis of neuroendocrine tumor in adults

2009 ◽  
Vol 27 (15_suppl) ◽  
pp. e15683-e15683
Author(s):  
R. Srirajaskanthan ◽  
K. Desai ◽  
A. Jayaratnam ◽  
E. Carras ◽  
C. Toumpanakis ◽  
...  
Keyword(s):  
Breast Cancer ◽  
Pet Imaging ◽  
Neuroendocrine Tumour ◽  
Imaging Modality ◽  
Primary Tumour ◽  
Somatostatin Receptor ◽  
Histological Evaluation ◽  
Ocular Involvement ◽  
Metastatic Focus ◽  
Cross Sectional

e15683 Background: Neuroendocrine tumours are relatively slow growing tumours. They often present with significant metastatic disease affecting liver, lymph nodes, lungs and bone. However tumour masses may be found at unusual/uncommon sites e.g. breast, orbital soft tissue and the heart raising the possibility of this being metastatic focus or presence of another primary tumour. Detection of these sites could have a significant impact on the available treatment options. Aim: to determine the most appropriate imaging modality and type of tumour metastases. Methods: We reviewed 300 consecutive clinic patients. We identified 18 patients with metastasis at uncommon sites i.e. breast, orbital and cardiac. We retrospectively evaluated clinical notes and recent radiological investigations of these patients. To characterise these lesions additional investigations included cross sectional imaging, PET imaging (68Gallium DOTA Octreotate PET and 18F- FDG PET) and histological evaluation of the metastasis where appropriate. Patients with breast metastasis underwent bilateral mammogram, patients with peri-ocular involvement underwent MRI of the brain and the orbit. Results: 18 patients had tumour masses at uncommon sites. Of these 15 masses were in the breast; 4 were in the orbital muscles and 2 patients had pericardial metastasis. Of the 15 patients with breast lesions 12 had confirmed neuroendocrine tumour metastases and 3 had breast cancer. It should be noted that breast cancer lesions were positive on the 68Gallium Octreotate PET imaging. One patient who had a defined metastasis in the pericardium showed avid uptake on the 68Gallium DOTA Octreotate PET and scan and cardiac MRI, the other patient had pericardial metastases confirmed at post mortem. Conclusions: Clear knowledge of these uncommon sites of metastasis is useful in terms of arranging further investigations and excluding other cancers. It is also important to realise that although somatostatin receptor scintigraphy especially 68Gallium DOTA Octreotate PET is very useful in detecting NET metastasis, it may also show avid uptake in patients with breast cancer and hence histological evaluation of these lesions are important. Undoubtedly within our cohort of patients and generally there is an under-diagnosis of lesions in uncommon sites. No significant financial relationships to disclose.

scholarly journals Diagnostic Role and Therapeutic Perspectives of Endoscopic Ultrasound for Pancreatic Neuroendocrine Tumor

2021 ◽  
Vol 26 (1) ◽  
pp. 1-9
Author(s):  
Jun-Ho Choi
Keyword(s):  
Neuroendocrine Tumor ◽  
Endoscopic Ultrasound ◽  
Imaging Techniques ◽  
Preoperative Assessment ◽  
Pancreatic Neuroendocrine Tumor ◽  
Fine Needle Biopsy ◽  
Effective Treatment Option ◽  
Curative Surgery ◽  
Ki 67 ◽  
Cross Sectional

Pancreatic neuroendocrine tumors (PNETs) are rare tumors with malignant potential, but their incidence has appreciably increased over the last few decades. Diagnosis of PNETs is often difficult with conventional imaging methods. Cross-sectional imaging localizes less than 10% of PNETs less than 1 cm in diameter. Endoscopic ultrasound (EUS) has been shown to be superior to other imaging techniques in the preoperative localization and diagnosis of PNETs. The finding of a hyperenhanced lesion on contrastenhanced EUS was highly predictive of PNETs different from adenocarcinoma. Preoperative assessment of tumor differentiation and Ki-67 is an important prognostic factor for grading. Thus, EUS-guided fine needle biopsy may play a key role in the work-up of nonfunctioning PNETs, and attempts to measure Ki-67 on cytologic and histologic samples have been made. According to the European Neuroendocrine Tumor Society guidelines, there is no clear evidence of a survival benefit of surgery for nonfunctioning G1 PNETs <2 cm in diameter. The benefits of curative surgery must be weighed against operative morbidity and mortality. Although surgical resection remains the mainstay for PNETs, EUS-guided ablation offers a relatively safe and effective treatment option in patients deemed unfit for, or who do not want to undergo, surgery. EUS-guided ablation provides an attractive therapeutic nonsurgical option for patients; however, further studies that better elucidate the long-term outcomes with standardization of technique will help define its role in the treatment of PNETs. The role of EUS in the diagnosis and treatment of PNETs is expected to make much progress in the future.

scholarly journals Phaeochromocytoma and Acromegaly: a unifying diagnosis

2014 ◽  
Vol 2014 ◽  
Author(s):  
C Mumby ◽  
J R E Davis ◽  
J Trouillas ◽  
C E Higham
Keyword(s):  
Pituitary Gland ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Rare Condition ◽  
Ct Scanning ◽  
Pituitary Surgery ◽  
Pathogenic Mutation ◽  
Tolerance Test ◽  
Oral Glucose ◽  
List Type

Summary A 52-year-old lady was referred after a 5 cm left adrenal mass was detected on computed tomography (CT) scanning. She was asymptomatic although was noted to have acromegalic facies. Blood pressure (BP) was normal but plasma normetanephrines were raised to 2.81 mmol/l (<1.09) and urinary normetadrenaline excretion 5.3 μmol/24 h (0–4.3). Adrenal biochemistry screen was otherwise normal. Metaiodobenzylguanidine (MIBG) scan demonstrated uptake in the adrenal lesion. Growth hormone (GH) nadir on oral glucose tolerance test (OGTT) was 2.2 ng/ml with an elevated IGF1 level of 435 ng/ml (72–215), confirming acromegaly biochemically. The remainder of the pituitary screen was normal. A magnetic resonance imaging (MRI) scan of the pituitary revealed an enlarged pituitary gland with a microadenoma/cyst of 2–3 mm in diameter. Alpha blockade was achieved with a titrated dose of phenoxybenzamine before a successful laparoscopic hand-assisted left adrenalectomy. Postoperative biochemical testing revealed a normal plasma normetanephrine level of 0.6 nmol/l (<1.09) and a metanephrine level of 0.35 nmol/l (<0.46 nmol/l). Nadir on OGTT was normal at 0.07 ng/ml with an IGF1 level within the reference range at 111 ng/ml (75–215). Histology demonstrated a well-circumscribed and encapsulated oval mass with microscopic features typical for a phaeochromocytoma. The sections stained strongly positive for GHRH in 20% of cells on immunocytochemistry. Genetic analysis showed no pathogenic mutation. This is a report of the rare condition of a phaeochromocytoma co-secreting GHRH resulting in clinical and biochemical acromegaly. Neuroendocrine tumours can stain positive for GHRH without coexisting acromegaly, but the resolution of patient symptoms and normalisation of serum GH and IGF1 levels following surgery imply that this was functional secretion. Pituitary surgery should be avoided in such cases. Learning points Incidental findings on imaging require thorough investigation to determine the presence of serious pathology. Acromegaly and phaeochromocytoma are rarely coincident in the same patient. If this occurs, co-secretion of GHRH from the phaeochromocytoma or the presence of underlying genetic abnormalities must be considered. Acromegaly is due to ectopic GHRH-secreting neuroendocrine tumours in <1% of cases, most commonly pancreatic or bronchial lesions. Co-secretion of GHRH from a phaeochromocytoma is extremely rare. In such cases, the pituitary gland may appear enlarged but pituitary surgery should be avoided and surgical treatment of the neuroendocrine tumour attempted.

scholarly journals Glucagonoma-associated dilated cardiomyopathy refractory to somatostatin analogue therapy

2019 ◽  
Vol 2019 ◽  
Author(s):  
Michal Barabas ◽  
Isabel Huang-Doran ◽  
Debbie Pitfield ◽  
Hazel Philips ◽  
Manoj Goonewardene ◽  
...  
Keyword(s):  
Dilated Cardiomyopathy ◽  
Cardiac Failure ◽  
Neuroendocrine Tumour ◽  
Left Ventricular ◽  
Somatostatin Analogue ◽  
List Type ◽  
Cross Sectional ◽  
Dominant Feature ◽  
Cross Sectional Imaging ◽  
Sectional Imaging

Summary A 67-year-old woman presented with a generalised rash associated with weight loss and resting tachycardia. She had a recent diagnosis of diabetes mellitus. Biochemical evaluation revealed elevated levels of circulating glucagon and chromogranin B. Cross-sectional imaging demonstrated a pancreatic lesion and liver metastases, which were octreotide-avid. Biopsy of the liver lesion confirmed a diagnosis of well-differentiated grade 2 pancreatic neuroendocrine tumour, consistent with metastatic glucagonoma. Serial echocardiography commenced 4 years before this diagnosis demonstrated a progressive left ventricular dilatation and dysfunction in the absence of ischaemia, suggestive of glucagonoma-associated dilated cardiomyopathy. Given the severity of the cardiac impairment, surgical management was considered inappropriate and somatostatin analogue therapy was initiated, affecting clinical and biochemical improvement. Serial cross-sectional imaging demonstrated stable disease 2 years after diagnosis. Left ventricular dysfunction persisted, however, despite somatostatin analogue therapy and optimal medical management of cardiac failure. In contrast to previous reports, the case we describe demonstrates that chronic hyperglucagonaemia may lead to irreversible left ventricular compromise. Management of glucagonoma therefore requires careful and serial evaluation of cardiac status. Learning points: In rare cases, glucagonoma may present with cardiac failure as the dominant feature. Significant cardiac impairment may occur in the absence of other features of glucagonoma syndrome due to subclinical chronic hyperglucagonaemia. A diagnosis of glucagonoma should be considered in patients with non-ischaemic cardiomyopathy, particularly those with other features of glucagonoma syndrome. Cardiac impairment due to glucagonoma may not respond to somatostatin analogue therapy, even in the context of biochemical improvement. All patients with a new diagnosis of glucagonoma should be assessed clinically for evidence of cardiac failure and, if present, a baseline transthoracic echocardiogram should be performed. In the presence of cardiac impairment these patients should be managed by an experienced cardiologist.

scholarly journals Palpation thyroiditis following subtotal parathyroidectomy for hyperparathyroidism

2016 ◽  
Vol 2016 ◽  
Author(s):  
Elizabeth M Madill ◽  
Shamil D Cooray ◽  
Leon A Bach
Keyword(s):  
Elevated Serum ◽  
Mechanical Damage ◽  
Thyroid Stimulating Hormone ◽  
List Type ◽  
Subtotal Parathyroidectomy ◽  
Tertiary Hyperparathyroidism ◽  
Adrenergic Antagonists ◽  
Potential Risks ◽  
Unexplained Symptoms ◽  
Clinically Significant

Summary Thyrotoxicosis is an under-recognised but clinically important complication of parathyroidectomy. We report a case of a 37-year-old man with tertiary hyperparathyroidism who initially developed unexplained anxiety, diaphoresis, tachycardia, tremor and hyperreflexia one day after subtotal parathyroidectomy. Thyroid biochemistry revealed suppressed thyroid stimulating hormone and elevated serum free T4 and free T3 levels. Technetium-99m scintigraphy scan confirmed diffusely decreased radiotracer uptake consistent with thyroiditis. The patient was diagnosed with thyrotoxicosis resulting from palpation thyroiditis. Administration of oral beta-adrenergic antagonists alleviated his symptoms and there was biochemical evidence of resolution fourteen days later. This case illustrates the need to counsel patients about thyroiditis as one of the potential risks of parathyroid surgery. It also emphasises the need for biochemical surveillance in patients with unexplained symptoms in the post-operative period and may help to minimise further invasive investigations for diagnostic clarification. Learning points Thyroiditis as a complication of parathyroidectomy surgery is uncommon but represents an under-recognised phenomenon. It is thought to occur due to mechanical damage of thyroid follicles by vigorous palpation. Palpation of the thyroid gland may impair the physical integrity of the follicular basement membrane, with consequent development of an inflammatory response. The majority of patients are asymptomatic, however clinically significant thyrotoxicosis occurs in a minority. Patients should be advised of thyroiditis/thyrotoxicosis as a potential complication of the procedure. Testing of thyroid function should be performed if clinically indicated, particularly if adrenergic symptoms occur post-operatively with no other cause identified.

CONCENTRATION OF THE SERUM TGF-BETA1 IN HEALTHY ADULT PEOPLE

2014 ◽  
pp. 90-93
Author(s):  
Van Tuan Nguyen ◽  
Tam Vo ◽  
Bui Bao Hoang
Keyword(s):  
Transforming Growth Factor ◽  
Healthy Adult ◽  
Age Groups ◽  
Elevated Serum ◽  
Cross Sectional Study ◽  
Healthy People ◽  
Sectional Study ◽  
Transforming Growth Factor Beta1 ◽  
Cross Sectional ◽  
And Gender

Elevated serum Transforming growth factor-beta1 (TGF-beta1) levels have been linked to tissue fibrosis including chronic kidney disease. Objectives: (1) Investigate serum TGF-beta1 levels in healthy adult people and (2) Examined the relation between serum TGF-beta1 level and gender, age, body mass index (BMI). Method: A cross-sectional study. TGF-beta1 were quantified by ELISA. Results: Levels of serum TGF-beta1 in healthy people were 13,45 ± 7,17 ng/mL mL (0,59 - 33,10 ng/mL). There are no difference of serum TGF-beta1 levels between men and women, between the age groups (<40 years, 40 to < 60 years and ≥ 60 years), between BMI groups < 23 and BMI group ≥ 23. Key words: TGF-beta1, healthy people

scholarly journals The association between serum alanine aminotransferase and hypertension: A national based cross-sectional analysis among over 21 million Chinese adults

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Jiajing Jia ◽  
Ying Yang ◽  
Fangchao Liu ◽  
Minjin Zhang ◽  
Qin Xu ◽  
...  
Keyword(s):  
Alanine Aminotransferase ◽  
Elevated Serum ◽  
Population Based ◽  
Reproductive Age ◽  
Chinese Adults ◽  
Cross Sectional ◽  
Serum Alanine Aminotransferase ◽  
Cross Sectional Analysis ◽  
Registration Data ◽  
Sectional Analysis

Abstract Background Inconsistent results were found in the association between serum alanine aminotransferase (ALT) and hypertension among population-based studies. This study evaluated the association between ALT and hypertension among Chinese reproductive-age population by utilizing registration data from National Free Pre-pregnancy Checkups Project in 2016–2017. Methods The 21,103,790 registered participants were eligible for analysis, including women who were 20–49 years old and men who were 20–59 years old with available data for ALT and blood pressure (BP). Logistic regression was conducted to estimate odds ratio (OR) for the association between ALT and hypertension as a binary outcome. Linear regression was used to examine the association between ALT and BP as a continuous outcome. Results In total, 4.21% of the participants were hypertensive, and 11.67% had elevated ALT (> 40 U/L). Hypertension prevalence was 3.63% and 8.56% among participants with normal and elevated ALT levels. A strong linear relationship was found between serum ALT levels and the odds of hypertension after adjustment for potential confounders. The multivariable-adjusted ORs for hypertension were 1, 1.22 (1.21, 1.22), 1.67 (1.65 1.68), 1.78 (1.76, 1.80), and 1.92 (1.90, 1.94) in participants with ALT levels of ≤ 20, 20.01–40, 40.01–60, 60.01–80, and > 80 U/L, respectively. Systolic and diastolic BPs rose by 1.83 and 1.20 mmHg on average, for each 20 U/L increase in ALT (P for trend < 0.001). The association was consistent among subgroups and tended to be stronger among populations who are overweight (body mass index ≥ 24 kg/m2) (χ2 = 52,228, P < 0.001), alcohol drinking (χ2 = 100,730, P < 0.001) and cigarette smoking (χ2 = 105,347, P < 0.001). Conclusions Our cross-sectional analysis suggested a linear association between serum ALT and hypertension or BP, which indicated that abnormal liver metabolism marked by elevated serum ALT could play a role in hypertension or elevated BP condition.

scholarly journals Environmentally Friendly and Multifunctional Shaddock Peel-Based Carbon Aerogel for Thermal-Insulation and Microwave Absorption

2021 ◽  
Vol 13 (1) ◽  
Author(s):  
Weihua Gu ◽  
Jiaqi Sheng ◽  
Qianqian Huang ◽  
Gehuan Wang ◽  
Jiabin Chen ◽  
...  
Keyword(s):  
Microwave Absorption ◽  
Thermal Insulation ◽  
Freeze Drying ◽  
Thermal Infrared ◽  
Carbon Aerogel ◽  
Carbon Aerogels ◽  
List Type ◽  
Drying Method ◽  
Cross Sectional ◽  
Compression Resistance

Highlights The eco-friendly shaddock peel-derived carbon aerogels were prepared by a freeze-drying method. Multiple functions such as thermal insulation, compression resistance and microwave absorption can be integrated into one material-carbon aerogel. Novel computer simulation technology strategy was selected to simulate significant radar cross-sectional reduction values under real far field condition. . Abstract Eco-friendly electromagnetic wave absorbing materials with excellent thermal infrared stealth property, heat-insulating ability and compression resistance are highly attractive in practical applications. Meeting the aforesaid requirements simultaneously is a formidable challenge. Herein, ultra-light carbon aerogels were fabricated via fresh shaddock peel by facile freeze-drying method and calcination process, forming porous network architecture. With the heating platform temperature of 70 °C, the upper surface temperatures of the as-prepared carbon aerogel present a slow upward trend. The color of the sample surface in thermal infrared images is similar to that of the surroundings. With the maximum compressive stress of 2.435 kPa, the carbon aerogels can provide favorable endurance. The shaddock peel-based carbon aerogels possess the minimum reflection loss value (RLmin) of − 29.50 dB in X band. Meanwhile, the effective absorption bandwidth covers 5.80 GHz at a relatively thin thickness of only 1.7 mm. With the detection theta of 0°, the maximum radar cross-sectional (RCS) reduction values of 16.28 dB m2 can be achieved. Theoretical simulations of RCS have aroused extensive interest owing to their ingenious design and time-saving feature. This work paves the way for preparing multi-functional microwave absorbers derived from biomass raw materials under the guidance of RCS simulations.

scholarly journals Elevated serum IL-10 is associated with severity of neonatal encephalopathy and adverse early childhood outcomes

2021 ◽  
Author(s):  
Raymand Pang ◽  
Brian M. Mujuni ◽  
Kathryn A. Martinello ◽  
Emily L. Webb ◽  
Angela Nalwoga ◽  
...  
Keyword(s):  
Early Childhood ◽  
Neonatal Mortality ◽  
Child Death ◽  
Elevated Serum ◽  
Adverse Outcomes ◽  
List Type ◽  
Perinatal Infection ◽  
Neonatal Encephalopathy ◽  
Early Childhood Outcomes ◽  
Sub Saharan

Abstract Background Neonatal encephalopathy (NE) contributes substantially to child mortality and disability globally. We compared cytokine profiles in term Ugandan neonates with and without NE, with and without perinatal infection or inflammation and identified biomarkers predicting neonatal and early childhood outcomes. Methods In this exploratory biomarker study, serum IL-1α, IL-6, IL-8, IL-10, TNFα, and VEGF (<12 h) were compared between NE and non-NE infants with and without perinatal infection/inflammation. Neonatal (severity of NE, mortality) and early childhood (death or neurodevelopmental impairment to 2.5 years) outcomes were assessed. Predictors of outcomes were explored with multivariable linear and logistic regression and receiver-operating characteristic analyses. Results Cytokine assays on 159 NE and 157 non-NE infants were performed; data on early childhood outcomes were available for 150 and 129, respectively. NE infants had higher IL-10 (p < 0.001), higher IL-6 (p < 0.017), and lower VEGF (p < 0.001) levels. Moderate and severe NE was associated with higher IL-10 levels compared to non-NE infants (p < 0.001). Elevated IL-1α was associated with perinatal infection/inflammation (p = 0.013). Among NE infants, IL-10 predicted neonatal mortality (p = 0.01) and adverse early childhood outcome (adjusted OR 2.28, 95% CI 1.35–3.86, p = 0.002). Conclusions Our findings support a potential role for IL-10 as a biomarker for adverse outcomes after neonatal encephalopathy. Impact Neonatal encephalopathy is a common cause of child death and disability globally. Inflammatory cytokines are potential biomarkers of encephalopathy severity and outcome. In this Ugandan health facility-based cohort, neonatal encephalopathy was associated with elevated serum IL-10 and IL-6, and reduced VEGF at birth. Elevated serum IL-10 within 12 h after birth predicted severity of neonatal encephalopathy, neonatal mortality, and adverse early childhood developmental outcomes, independent of perinatal infection or inflammation, and provides evidence to the contribution of the inflammatory processes. Our findings support a role for IL-10 as a biomarker for adverse outcomes after neonatal encephalopathy in a sub-Saharan African cohort.

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