Calcitonin-Secreting Pancreatic Neuroendocrine Tumors: A Case Report and Review of the Literature

Kováčová, Martina; Filková, Marta; Potočárová, Mária; Kiňová, Soňa; Pajvani, Utpal B.

doi:10.4158/ep13505.cr

Cited by 13 publications

(5 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Hypersecretion of calcitonin by NENs is rare; in fact, in functional NENs, calcitonin secretion is less common than other hormone secretions causing endocrine paraneoplastic syndromes (79). Table 2 summarizes the main features of the calcitonin.-secreting NENs reported in the literature (1,5,7,40,80,81,82,83,84,85,86,87,88,89,90,91,92,93,94). Pancreatic, laryngeal and lung NENs are most frequently associated with hypercalcitoninemia, but calcitonin secretion has also been described in duodenal, esophageal, cutaneous and paranasal NENs.…”

Section: False Positives and False Negativesmentioning

confidence: 99%

ENDOCRINE TUMOURS: Calcitonin in thyroid and extra-thyroid neuroendocrine neoplasms: the two-faced Janus

Giannetta

Guarnotta

Altieri

et al. 2020

European Journal of Endocrinology

View full text Add to dashboard Cite

An increased calcitonin serum level is suggestive of a medullary thyroid cancer (MTC), but is not pathognomonic. The possibility of false positives or other calcitonin-secreting neuroendocrine neoplasms (NENs) should be considered. Serum calcitonin levels are generally assessed by immunoradiometric and chemiluminescent assays with high sensitivity and specificity; however, slightly-moderately elevated levels could be attributable to various confounding factors. Calcitonin values >100 pg/mL are strongly suspicious of malignancy, whereas in patients with moderately elevated values (10-100 pg/mL) a stimulation test may be applied to improve diagnostic accuracy. Although the standard protocol and the best gender-specific cut-offs for calcium stimulated calcitonin are still controversial, the fold of the calcitonin increase after stimulation seems to be more reliable. Patients with MTC show stimulated calcitonin values at least 3-4 times higher than the basal values, whereas calcitonin-secreting NENs can be distinguished from a C-cell disease by the absence of or <2-fold response to stimulation. The measurement of calcitonin in fine-needle aspirate washout (FNA-CT) and calcitonin immunocytochemical staining from thyroid nodules are ancillary methods that may significantly improve MTC diagnosis. The present review examines the gray areas in the interpretation of calcitonin measurement in order to provide a tool to clarify the origin of calcitonin secretion and differentiate the behavior of the two-faced Janus of neuroendocrinology: intra-thyroid (MTC) and extra-thyroid NENs.

show abstract

Section: False Positives and False Negativesmentioning

confidence: 99%

ENDOCRINE TUMOURS: Calcitonin in thyroid and extra-thyroid neuroendocrine neoplasms: the two-faced Janus

Giannetta

Guarnotta

Altieri

et al. 2020

European Journal of Endocrinology

View full text Add to dashboard Cite

show abstract

“…Functional pNETs ectopically secreting calcitonin were rarely reported in the literature (8, 9). However, most recently ten cases of pNET-secreting calcitonin were reported (10). To the best of our knowledge, only one case of pNET secreting both GHRH and calcitonin has been described previously (11).…”

Section: Discussionmentioning

confidence: 99%

pNET co-secreting GHRH and calcitonin: ex vivo hormonal studies in human pituitary cells

Zornitzki

Rubinfeld

Lysyy

et al. 2016

Endocrinology, Diabetes &Amp; Metabolism Case Reports

View full text Add to dashboard Cite

SummaryAcromegaly due to ectopic GHRH secretion from a neuroendocrine tumor (NET) is rare and comprises <1% of all acromegaly cases. Herein we present a 57-year-old woman with clinical and biochemical features of acromegaly and a 6 cm pancreatic NET (pNET), secreting GHRH and calcitonin. Following surgical resection of the pancreatic tumor, IGF1, GH and calcitonin normalized, and the clinical features of acromegaly improved. In vitro studies confirmed that the tumor secreted large amounts of both GHRH and calcitonin, and incubation of pNET culture-derived conditioned media stimulated GH release from a cultured human pituitary adenoma. This is a unique case of pNET secreting both GHRH and calcitonin. The ability of the pNET-derived medium to stimulate in vitro GH release from a human pituitary-cell culture, combined with the clinical and hormonal remission following tumor resection, confirmed the ectopic source of acromegaly in this patient.Learning points Signs, symptoms and initial work-up of acromegaly due to ectopic GHRH secretion are similar to pituitary-dependent acromegaly. However, if no identifiable pituitary lesion is found, somatostatin receptor scan and further imaging (CT, MRI) should be performed.Detection of GHRH in the blood and in the tumor-derived medium supports the diagnosis of ectopic GHRH secretion.Functional bioactivity of pNET-secreted GHRH can be proved in vitro by releasing GH from human pituitary cells.

show abstract

“…In similar cases, a step-wise exclusion of non-MTC hypercalcitonemia could initially include a careful medical history for drugs known to raise Ct levels such as omeprazole, b-blockers, or glucocorticoids (3), along with estimation of renal function and calcium and gastrin levels (19). In the absence of such causes, assay interactions could also be considered as they can give false Ct elevations (17).…”

Section: Discussionmentioning

confidence: 99%

Pancreatic Neuroendocrine Tumors Presenting with Hypercalcitonemia

Chrisoulidou

Kaltsas

Mandanas

et al. 2015

AACE Clinical Case Reports

View full text Add to dashboard Cite

Objective: Pancreatic neuroendocrine tumors (pNETs) can be associated with discrete clinical syndromes due to the secretion of a variety of bioactive substances that are used to confirm the diagnosis and as potential tumor markers. Calcitonin (Ct) is mainly secreted by medullary thyroid carcinoma (MTC) but is also rarely secreted by pNETs, occasionally leading to diagnostic confusion. We report the diagnosis of 4 patients with metastatic pNETs and associated Ct secretion who were initially evaluated for thyroid pathology. Methods: We describe the histories of 4 patients who were referred for evaluation of thyroid diseases and proved to have metastatic pNETs and associated Ct secretion. One of the patients had erroneously undergone thyroidectomy as hypercalcitonemia was attributed to MTC. Results: Patients presented with variable Ct levels (4-fold up to 35-fold elevations), and all had thyroid nodules that were considered the cause of hypercalcitonemia. Ultrasonography and fine-needle aspiration cytology (FNAC) were used to exclude the thyroid origin of high Ct. As a result, investigations were directed towards other causes of hypercalcitonemia and pancreatic tumors were discovered. Conclusions: As in the majority of cases, Ct secretion is not associated with an obvious clinical syndrome, and these cases may be misdiagnosed as MTC, particularly in the absence of locally compressive symptoms from pNETs. Making the wrong diagnosis has potentially deleterious effects and may lead to unnecessary interventions such as a thyroidectomy and late diagnosis of an advanced pNET. (AACE Clinical Case Rep. 2015;1:e269-e272) Abbreviations: CgA = chromogranin A; Ct = calcitonin; FNAC = fine-needle aspiration cytology; IHC = immunohistochemistry; LI = labeling index; MTC = medullary thyroid carcinoma; NET = neuroendocrine tumor; pNET= pancreatic neuroendocrine tumors; SSA = somatostatin analogue.

show abstract

Calcitonin-Secreting Pancreatic Neuroendocrine Tumors: A Case Report and Review of the Literature

Cited by 13 publications

References 15 publications

ENDOCRINE TUMOURS: Calcitonin in thyroid and extra-thyroid neuroendocrine neoplasms: the two-faced Janus

ENDOCRINE TUMOURS: Calcitonin in thyroid and extra-thyroid neuroendocrine neoplasms: the two-faced Janus

pNET co-secreting GHRH and calcitonin: ex vivo hormonal studies in human pituitary cells

Pancreatic Neuroendocrine Tumors Presenting with Hypercalcitonemia

Contact Info

Product

Resources

About