Abstract:
:Phaeochromocytomas and paragangliomas (PPGLs) are rare tumours that arise from the adrenal medulla or extra-adrenal sympathetic or parasympathetic paraganglia. Recent advances in genetics have greatly enhanced understanding of the pathogenesis and molecular physiology of PPGL. Concomitantly, advances in molecular imaging mean four techniques are now available for use in PPGLs: [123 I]-MIBG coupled with SPECT/CT; [18 F]- FDG, [68 Ga]-DOTATATE and [18 F]-FDOPA coupled with PET/CT. Each modality relies on unique cellular uptake mechanisms that are contingent upon the tumour's molecular behaviour-which, in turn, is determined by the tumour's genetic profile. This genotype-phenotype correlation means the appropriate choice of radiotracer may depend on the known (or suspected) underlying genetic mutation, in addition to the clinical indication for the scan-whether confirming diagnosis, staging disease, surveillance or determining eligibility for radionuclide therapy. Given these rapid recent changes in genetic understanding and molecular imaging options, many clinicians find it challenging to choose the most appropriate scan for an individual with PPGL. To this end, recent guidelines published by the European Association of Nuclear Medicine and the Society of Nuclear Medicine and Molecular Imaging (EANM/SNMMI) have detailed the preferred radiotracer choices for individuals with PPGL based on their genotype and/or clinical presentation, providing timely clarity in this rapidly moving field. The current review summarizes the implications of the genotype-phenotype relationship of PPGL, specifically relating this to the performance of molecular imaging modalities, to inform and enable practising endocrinologists to provide tailored, personalized care for individuals with PPGL.
journal_name
Clin Endocrinol (Oxf)journal_title
Clinical endocrinologyauthors
Ryder SJ,Love AJ,Duncan EL,Pattison DAdoi
10.1111/cen.14375subject
Has Abstractpub_date
2020-12-09 00:00:00eissn
0300-0664issn
1365-2265pub_type
杂志文章abstract::A 24-year-old negress with the triad of acanthosis nigricans, hirsutism associated with polycystic ovaries and insulin resistance is reported. Metabolic studies were done 3 years after a bilateral ovarian wedge resection. Partial remission of the hirsutism and return of menstrual cycles occurred after surgery. Extreme...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1982.tb02632.x
更新日期:1982-07-01 00:00:00
abstract:OBJECTIVE:Abdominal visceral (VAT) and subcutaneous (SAT) adipose tissues contribute to obesity, but may have different cardiometabolic risk profiles. We examined and compared the associations of abdominal VAT and SAT with metabolic risk factors in a large cohort of Chinese adults. METHODS:This study was based on cros...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.13013
更新日期:2016-07-01 00:00:00
abstract:OBJECTIVE:Patients on long-term dialysis may develop secondary hyperparathyroidism (SHPT), which causes varying degrees of bone mass loss. This condition is treated with parathyroidectomy (PTX). We investigated whether serial serum bone turnover markers could predict changes in bone mineral density (BMD) after PTX. DE...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2011.04265.x
更新日期:2012-05-01 00:00:00
abstract::Exposure to maternal hypothyroxinemia during pregnancy, which is characterized by low free T4 but normal thyroid-stimulating hormone (TSH) levels, can negatively affect the foetus. This review provides an overview of present findings concerning the association between maternal hypothyroxinemia during pregnancy and chi...
journal_title:Clinical endocrinology
pub_type: 杂志文章,评审
doi:10.1111/cen.12227
更新日期:2013-08-01 00:00:00
abstract:OBJECTIVE:Familial isolated primary hyperparathyroidism (FIHP) is a rare hereditary disorder. We present four patients from a single family with FIHP, and genetic analysis of their parathyroid adenomas and parathyroid carcinoma. DESIGN:DNA was extracted from tumours resected at surgery. Tumours were examined for loss ...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1046/j.1365-2265.1998.00354.x
更新日期:1998-01-01 00:00:00
abstract:CONTEXT:Steroid 5α-reductase type 2 deficiency (5α-RD2) is a male-limited, autosomal recessive inherited disease. Affected 46, XY individuals usually present with ambiguous genitalia at birth. An early and precise diagnosis is of great value to the long-term prognosis of the disease. OBJECTIVE:To describe the clinical...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.12456
更新日期:2014-11-01 00:00:00
abstract:OBJECTIVES:Adrenocortical tumours in man are characterized mainly on biochemical, anatomical and histological grounds which establish their secretory pattern and, with some uncertainty, their benign or malignant nature. To study further these tumours and eventually to shed some light on their pathogenesis, we determine...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1994.tb02485.x
更新日期:1994-04-01 00:00:00
abstract:OBJECTIVE:Mutations in the melanocortin-4 receptor gene (MC4R) are the most common monogenic form of human obesity. However, the contribution of MC4R mutations to obesity in Chinese has not been investigated. We studied the frequency of MC4R mutations in an obese southern Chinese population and the functional consequen...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2006.02573.x
更新日期:2006-08-01 00:00:00
abstract::We have examined the characteristics of circulating immunoreactive human calcitonin (IR-hCT) by studying its different molecular weight (MW) forms under various secretory conditions in a patient with medullary carcinoma of the thyroid. Plasma IR-hCT (318 mg/l basal) increased 9 and 12 times, respectively, after calciu...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1980.tb01032.x
更新日期:1980-08-01 00:00:00
abstract:OBJECTIVE:Parathyroid carcinoma remains difficult to diagnose. Recently, it has been shown that mutations in the HRPT2 gene (encoding parafibromin) are associated with the development of parathyroid carcinoma. Although MEN1 is not typically thought to be involved in carcinoma formation, parathyroid carcinoma may be an ...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2007.02894.x
更新日期:2007-09-01 00:00:00
abstract::A single fasting level of serum prolactin was measured in each of sixty control subjects and eighty-three psychiatric patients of both sexes who had been on neuroleptic therapy for 2-4 weeks (acute treatment) or at least 5 years (chronic treatment) and who were aged either 17-45 or 48-85 years. All groups of patients ...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1976.tb01953.x
更新日期:1976-05-01 00:00:00
abstract:BACKGROUND AND OBJECTIVE:It has been well documented that treatment of chronic hepatitis B and C infection with interferon alpha (IFN-alpha) can lead to the induction of thyroid autoantibodies and hypothyroidism. Thyrotoxicosis, however, is less frequently observed and less well characterized. PATIENTS AND METHODS:We ...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1046/j.1365-2265.2002.01553.x
更新日期:2002-06-01 00:00:00
abstract:OBJECTIVE:Growth deficiency is commonly seen in polytransfused beta-thalassaemia patients, especially in adolescence. It is not completely dependent on the lack of their pubertal growth spurt. GH impairment at different levels (hypothalamic or pituitary) and/or a reduced IGF-1 synthesis have been suggested the main cau...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1046/j.1365-2265.1997.1951005.x
更新日期:1997-06-01 00:00:00
abstract::A modified short ACTH test for the detection of heterozygote carriers of 21-hydroxylase deficiency (21-OHD) was applied to twenty-one controls and fourteen parents of children with 21-OHD. The following modifications were introduced: (1) Endogenous ACTH was suppressed by dexamethasone administration prior to the test,...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1980.tb01372.x
更新日期:1980-06-01 00:00:00
abstract::Nine acromegalic patients were treated by the somatostatin analogue SMS 201-995 octreotide (Sandostatin (octreotide), Sandoz, Basle, Switzerland] 250 micrograms/day in 4 divided s.c. injections (50 + 50 + 50 + 100 micrograms) for 1 month, 250 micrograms/24 has continuous s.c. infusions for another month and thereafter...
journal_title:Clinical endocrinology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1111/j.1365-2265.1989.tb00266.x
更新日期:1989-06-01 00:00:00
abstract::Uncontrolled hyperthyroidism in pregnancy poses a risk to both mother and foetus, and the optimal treatment strategy in this setting remains elusive. Instigation of pharmacological therapy or an alternative intervention during pregnancy requires careful consideration, and the evidence that has underpinned our choice o...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.12577
更新日期:2015-04-01 00:00:00
abstract:OBJECTIVE:High overnight plasma growth hormone (GH) levels in insulin-dependent diabetes mellitus (IDDM) are reflected in both an increase in the GH pulse amplitude and elevated baseline GH concentrations. To determine whether these are a result of an increase in GH secretory episodes, we undertook deconvolution analys...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1993.tb00978.x
更新日期:1993-01-01 00:00:00
abstract:OBJECTIVE:The distinction between benign and malignant well-differentiated endocrine tumours is hard to achieve. The aim of the present study was to determine whether detection of telomerase or quantification of human telomerase reverse transcriptase protein subunit (hTERT) differ between benign and malignant endocrine...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2005.02417.x
更新日期:2006-01-01 00:00:00
abstract:OBJECTIVE:Women with Turner's syndrome (TS) have recently been shown to be at an increased risk of developing chronic liver disease. There has been some concern that oestrogen replacement therapy may exacerbate hepatic dysfunction. The aim of this study was to assess hepatic function in women with TS and to determine t...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1046/j.1365-2265.2001.01321.x
更新日期:2001-08-01 00:00:00
abstract::The effect of chlorpromazine (CPZ) on prolactin (PRL) secretion was studied in fourteen sexually immature males; nine with idiopathic hypogonadotrophic hypogonadism and five who proved to be normal early pubertal boys. Initially, clinical features and basal levels of testosterone, LH and FSH in serum collected in the ...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1982.tb00723.x
更新日期:1982-04-01 00:00:00
abstract:OBJECTIVE:Poorly differentiated thyroid cancer (PDTC) is a rare, but aggressive thyroid cancer (TC) and a main cause of death from non-anaplastic follicular cell-derived TC. Assessing the risk of PDTC-related death and the risk of recurrence is important for clinicians. The recent American Thyroid Association (ATA) 201...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.13910
更新日期:2019-08-01 00:00:00
abstract::Five patients with Bloom's syndrome aged from 2 8/12 to 27 years, all of whom had hypogonadism, were subjected to an i.v. LHRH test and two of them to an i.m. HCG test. There was increased responsiveness of plasma LH and FSH, indicating that the hypogonadism is primary in nature and of early development. The tubular e...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1977.tb02013.x
更新日期:1977-04-01 00:00:00
abstract:OBJECTIVES:Hyponatraemia is strongly associated with increased inpatient mortality, but it is unknown whether hyponatraemia per se contributes to excess mortality. Our hypothesis was that if hyponatraemic patients had significantly greater mortality compared with controls despite no difference with regard to gender, ag...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.12429
更新日期:2014-09-01 00:00:00
abstract::We describe here two patients with hypothyroidism due to pituitary-hypothalamic disease in whom basal thyrotrophin (TSH) levels measured by radioimmunoassay (RIA) were elevated yet when measured by a cytochemical bioassay (CBA) were found to be normal. This finding and the absence of the normal rise of thyroid hormone...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1978.tb02174.x
更新日期:1978-05-01 00:00:00
abstract:OBJECTIVES:A population-based study was undertaken to determine the mortality and morbidity for people with hypoparathyroidism compared to the general population. METHODS:In this study, patients identified with chronic hypoparathyroidism using data linkage from regional datasets were compared with five age- and gender...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.13895
更新日期:2019-02-01 00:00:00
abstract:OBJECTIVE:The disruption between the brain and the spinal cord leads to a decentralized sympathetic nervous system in people with chronic, cervical spinal cord lesions. These tetraplegic subjects are prone to disorders of energy metabolism and osteoporosis, and they experience alterations in their body composition with...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2005.02204.x
更新日期:2005-02-01 00:00:00
abstract:OBJECTIVE:Infusion of GH secretagogues appears to be a novel endocrine approach to reverse the catabolic state of critical illness, through amplification of the endogenously blunted GH secretion associated with a substantial IGF-I rise. Here we report the dynamic characteristics of spontaneous nightly TSH and PRL secre...
journal_title:Clinical endocrinology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1046/j.1365-2265.1997.3371118.x
更新日期:1997-11-01 00:00:00
abstract:BACKGROUND:Papillary thyroid cancer (PTC) is a common endocrine cancer and commonly presents with lymph node (LN) metastases. The role of surgical removal of the central cervical LN compartment is poorly defined. There are no prospective randomized controlled trials addressing the relevance to the extent of the initial...
journal_title:Clinical endocrinology
pub_type: 杂志文章,meta分析,评审
doi:10.1111/j.1365-2265.2011.04162.x
更新日期:2012-01-01 00:00:00
abstract::The inactivation of synthetic [3H]thyrotrophin-releasing hormone (TRH) by plasma was studied in rats treated with propylthiouracil (PTU) alone or with PTU and thyroxine. 48 h after the onset of treatment with thyroxine, the capacity of rat plasma to inactivate [3H]TRH was significantly increased. The percentage of dea...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1976.tb01961.x
更新日期:1976-07-01 00:00:00
abstract::Raised activity of the LH axis caused by activating mutations of LH receptor gene presents with precocious puberty in boys, analogous to the presentation of LH secreting pituitary adenomas (Faggiano et al., 1983; Ambrosi et al., 1990). LH "hyperactivity' in females appears to have no effect. Hyperactivity of the FSH a...
journal_title:Clinical endocrinology
pub_type: 杂志文章,评审
doi:10.1046/j.1365-2265.1996.8680879.x
更新日期:1996-12-01 00:00:00