Chromogranin A Levels in Diagnosis, Treatment and Follow-Up of 42 Patients with Non-Functioning Pancreatic Endocrine Tumours

Nikou, George C.; Marinou, Kyriakoula; Θωμάκος, Πέτρος; Papageorgiou, Dimitrios; Sanzanidis, V.; Νικολάου, Παναγιώτα; Kosmidis, Chris; Moulakakis, Antonios; Mallas, E.

doi:10.1159/000152000

Cited by 75 publications

(47 citation statements)

References 25 publications

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“…Regarding the role of disease extent, the Nehar study (Nehar et al 2004) also found a significant difference in the rate of CgA elevation between metastatic and non-metastatic patients, namely 73 vs 26%. This dramatic impact on CgA sensitivity was further confirmed by Nikou et al (2008), analyzing a cohort of non-functional pancreatic NEN, who found CgA alterations in the totality of patients with liver metastases, whereas the rate was much lower, 66.6%, in those subjects without liver involvement. According to these data, sensitivity of the CgA test can be considered as acceptable only for functional and advanced NENs.…”

Section: Sensitivitymentioning

confidence: 73%

“…This was due to the well-demonstrated relationship with disease stage/extent, which represents the main predictor of clinical outcome (Ahmed et al 2009). Indeed, the majority of authors demonstrated higher marker levels in patients with extensive metastases, as compared with those having localized disease or even limited hepatic involvement (Janson et al 1997, Tomassetti et al 2001b, Nehar et al 2004, Campana et al 2007, Zatelli et al 2007, Nikou et al 2008. Furthermore, Arnold et al (2008) reported a direct correlation between the CgA increase and the extent of liver involvement.…”

Section: Circulating Cga As a Prognostic Marker In Nenmentioning

confidence: 99%

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Chromogranin A as circulating marker for diagnosis and management of neuroendocrine neoplasms: more flaws than fame

Marotta

Zatelli

Sciammarella³

et al. 2018

Endocrine-Related Cancer

125

104

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Owing to the heterogeneity of neuroendocrine neoplasms (NENs), the availability of reliable circulating markers is critical for improving diagnostics, prognostic stratification, follow-up and definition of treatment strategy. This review is focused on chromogranin A (CgA), a hydrophilic glycoprotein present in large dense core vesicles of neuroendocrine cells. Despite being long identified as the most useful NEN-related circulating marker, clinical application of CgA is controversial. CgA assays still lack standardization, thus hampering not only clinical management but also the comparison between different analyses. In the diagnostic setting, clinical utility of CgA is limited as hampered by (a) the variety of oncological and non-oncological conditions affecting marker levels, which impairs specificity; (b) the fact that 30-50% of NENs show normal CgA, which impairs sensitivity. Regarding the prognostic phase, there is prospective evidence which demonstrates that advanced NENs secreting CgA have poorer outcome, as compared with those showing non-elevated marker levels. Although the identification of cut-offs allowing a proper risk stratification of CgA-secreting patients has not been performed, this represents the most important clinical application of the marker. By contrast, based on prospective studies, the trend of elevated circulating CgA does not represent a valid indicator of morphological evolution and has therefore no utility for the follow-up phase. Ultimately, current knowledge about the role of the marker for the definition of treatment strategy is poor and is limited by the small number of available studies, their prevalent retrospective nature and the absence of control groups of untreated subjects.

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Section: Sensitivitymentioning

confidence: 73%

Section: Circulating Cga As a Prognostic Marker In Nenmentioning

confidence: 99%

Chromogranin A as circulating marker for diagnosis and management of neuroendocrine neoplasms: more flaws than fame

Marotta

Zatelli

Sciammarella³

et al. 2018

Endocrine-Related Cancer

125

104

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“…Cg A is currently the best available biomarker for the diagnosis of NETs. It is useful in establishing the diagnosis, predicting disease recurrence, outcome and efficacy of therapy [19,20]. Urinary 5-hydroxyindoleacetic acid (5-HIAA) is the urinary breakdown of serotonin.…”

Section: Discussionmentioning

confidence: 99%

Multiple endocrine neoplasia type 1: a case report and review of the literature

et al. 2014

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AbstractMultiple endocrine neoplasia syndrome, type 1 (MEN1) is an underdiagnosed autosomal dominant inherited cancer predisposition syndrome with inter- and intrafamilial variability without a known genotype-phenotype correlation. Disease is caused by mutations in the MEN1 gene located on chromosome 11, but other genes (CDKN1B, AIP) and mechanisms might be involved too. We performed retrospective case series study of MEN1 syndrome patient and his family and present our experience of management of genetically confirmed 9 MEN1 syndrome large family members from Lithuania with novel MEN1 gene mutation (MEN1 exon 6 — c. 879delT (p.Pro293Profs*76)) and delineate its clinical phenotype. At present the diagnosis of MEN1 syndrome must be established by direct mutation testing. MEN1 syndrome patients, their relatives and patients suspected of MEN1 are eligible for mutation testing. Patients with MEN1 have a shorter life expectancy than the general population. MEN1 patients and mutation carriers should be subjected to periodic screening in order to detect manifestations in an early stage. Early genetic diagnosis and subsequent periodic screening is associated with less morbidity and mortality at follow-up. Our study confirmed the absence of genotype-phenotype correlation and showed high intrafamilial clinical expression variability of the MEN1 syndrome.

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“…insulin (insulinoma), gastrin (gastrinomas), glucagon (glucagonomas), and serotonin (small bowel NET). Chromogranin A (CgA) is another biomarker that has been studied extensively and found to be expressed by normal and malignant neuroendocrine cells [9,10] . The function of CgA is not completely understood.…”

Section: Pancreatic Neuroendocrine Tumorsmentioning

confidence: 99%

“…[9] Some authors caution that poorly differentiated tumors [9] and insulinomas [11] do not cause CgA levels to rise and may therefore be missed. Despite these limitations, CgA is still considered the best available biomarker for detecting NETs and assessing tumor burden, disease progression, and response to therapy [9][10][11] .…”

Section: Pancreatic Neuroendocrine Tumorsmentioning

confidence: 99%

Thymic neuroendocrine tumor in a patient with multiple endocrine neoplasia type I : The limitations of surveillance

Zibert

Hobbs

2014

CRIM

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Multiple endocrine neoplasia type I (MEN1) is an autosomal dominant disorder associated with pituitary and parathyroid adenomas and neuroendocrine tumors (NETs) of the pancreas. Pancreatic NETs, most of which are non-functional, are now the primary life-threatening manifestation of MEN1. Some patients with this disorder develop NETs of the thymus, which are typically characterized by aggressive behavior and malignant potential. Although rare in the general population, 25% of all reported thymic NETs have occurred in patients with MEN1. With the aid of advancements in imaging and biomarkers, the clinician is now better equipped in detecting NETs in this high-risk group. In this report, we present a patient whose incidentally discovered thymic NET led to the diagnosis of MEN1. We provide an overview of MEN1-associated NETs with a specific focus on the use of chromogranin A (CgA) in disease surveillance.

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Chromogranin A Levels in Diagnosis, Treatment and Follow-Up of 42 Patients with Non-Functioning Pancreatic Endocrine Tumours

Cited by 75 publications

References 25 publications

Chromogranin A as circulating marker for diagnosis and management of neuroendocrine neoplasms: more flaws than fame

Chromogranin A as circulating marker for diagnosis and management of neuroendocrine neoplasms: more flaws than fame

Multiple endocrine neoplasia type 1: a case report and review of the literature

Thymic neuroendocrine tumor in a patient with multiple endocrine neoplasia type I : The limitations of surveillance

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