Extrathyroidal Calcitonin Secreting Tumors

Giannetta, Elisa; Gianfrilli, Daniele; Pozza, Carlotta; Lauretta, Rosa; Graziadio, Chiara; Sbardella, Emilia; Baroli, Alberto; Caronna, Roberto; Chirletti, Piero; Lenzi, Andrea; Isidori, Andrea M.

doi:10.1097/md.0000000000002419

Cited by 16 publications

(10 citation statements)

References 13 publications

(13 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…To the best of our knowledge, the few cases of calcitonin-producing insulinoma reported in the literature were not associated to metastases in liver or in other sites. In our case, immunohistochemistry of the liver lesion was negative for calcitonin; however, it should be noted that immunostaining of hepatic lesions secondary to calcitonin-producing PNETs may be negative for calcitonin probably because metastases can dedifferentiate and lose their secretory activity [34].…”

Section: Discussioncontrasting

confidence: 58%

Malignant Insulinoma with Multiple Liver Metastases and Hypercalcitoninemia in a Patient with Type 2 Diabetes Mellitus Presenting as Recurrent Episodes of Diaphoresis due to Severe Hypoglycemia

Ciacciarelli

Caruso

Rengo

et al. 2020

Case Reports in Endocrinology

View full text Add to dashboard Cite

Insulinoma is an insulin-producing pancreatic neuroendocrine tumor that can be malignant in about 10% of cases. Locoregional invasion, lymph node metastases, or remote metastases are the main criteria of malignant insulinoma. Its incidence in patients with pre-existing diabetes mellitus (DM) is exceptionally rare. In this report, we describe a 66-year-old man with long-standing type 2 DM who presented with recurrent episodes of diaphoresis due to severe hypoglycemia despite the withdrawal of insulin therapy, hypercalcitoninemia, and biochemical and radiological findings suggestive of metastatic malignant insulinoma. Unfortunately, after few days of diazoxide treatment, edema, hypotension, oliguria, and water retention were observed, patient's clinical status deteriorated rapidly, and he died in our department from acute renal failure.

show abstract

Section: Discussioncontrasting

confidence: 58%

Malignant Insulinoma with Multiple Liver Metastases and Hypercalcitoninemia in a Patient with Type 2 Diabetes Mellitus Presenting as Recurrent Episodes of Diaphoresis due to Severe Hypoglycemia

Ciacciarelli

Caruso

Rengo

et al. 2020

Case Reports in Endocrinology

View full text Add to dashboard Cite

show abstract

“…Finally, other two cases of metastasizing moderately differentiated laryngeal NENs that shared many similarities with MTC were described in the literature (10,13). Calcitonin may therefore be regarded not only as a marker of MTC but also of well to moderately differentiated laryngeal NENs as well as of NENs of other sites (7).…”

Section: Discussionmentioning

confidence: 91%

“…Calcitonin can be a further neuroendocrine marker of laryngeal NENs (6). In addition, other NENs [pancreatic NENs (PanNENs) and pheochromocytomas] expressed calcitonin, generally in the presence of inappropriately elevated serum calcitonin levels (7,8). Particularly, calcitonin was expressed in 10.9% of the cases in a clinical-pathological study of 229 PanNENs, suggesting that calcitonin immunoreactivity is not an exceptional event in PanNENs (9).…”

Section: Introductionmentioning

confidence: 99%

Laryngeal Neuroendocrine Tumor With Elevated Serum Calcitonin: A Diagnostic and Therapeutic Challenge. Case Report and Review of Literature

et al. 2020

Self Cite

View full text Add to dashboard Cite

Introduction: Laryngeal neuroendocrine neoplasms (NENs) are a rare group of NENs of the neck, which commonly show immunostaining for calcitonin. Laryngeal NENs with calcitonin hypersecretion and lymph node metastases represent a diagnostic and therapeutic challenge, which should be included in the differential diagnosis of medullary thyroid carcinoma (MTC). We report a complex case of laryngeal NEN with calcitonin hypersecretion and a review of the literature. Case Presentation: A 59-year-old man presented with dysphagia, dyspnea, and lateral cervical mass; he was a smoker. At first imaging, a laryngeal lesion with lateral cervical lymphadenopathies was found, and it resulted as a moderately differentiated neuroendocrine tumor (G2), Ki67 = 5%, positive for calcitonin. Increased levels of serum calcitonin (50 pg/ml) were found. The patient started somatostatin analogs for lesions positivity to somatostatin receptor-based imaging. After 5 months, the disease progressed at 18F-fluorodeoxyglucose (18 F-FDG) PET-CT, and also new painful cutaneous lesions occurred. Considering high serum levels of calcitonin, differential diagnosis with MTC was required. Patient performed a thyroid color Doppler ultrasound, nodule fine needle aspiration, calcitonin dosage in fine needle washout fluid, and a calcium gluconate stimulation test. After multidisciplinary evaluation, we decided to perform a total thyroidectomy associated with lateral cervical lymphadenectomy and resection of skin metastases. No MTC was found. Two of the five resected lymph nodes, left upper parathyroid, and skin lesions were metastases of NEN G2, positive for calcitonin. After 2 months, new painful skin lesions occurred, and a target therapy with everolimus 10 mg/day was started. After 6 months of therapy, partial metabolic response with a reduction of 53.7% of radiotracer uptake at primary tumor was detected together with an improvement of patient's quality of life. Conclusions: The present case is the seventh described in the literature of laryngeal NEN associated with elevated serum calcitonin levels and the first case with parathyroid metastasis, suggesting the importance of a correct differential diagnosis between MTC and calcitonin-secreting laryngeal NEN, using an integrated approach of biochemistry and advanced imaging. This is also the first time that somatostatin analogs and then everolimus were used in this setting, resulting in clinical and partial metabolic response.

show abstract

“…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%

“…NENs often express peptide(s) or amine(s), especially insulin, gastrin, and serotonin. Calcitonin-secreting NENs have been described in various organs including the lung, pancreas, larynx, bladder and ovaries (3,4,5,6,7,8). Their frequency may be underestimated, as serum calcitonin measurement or immunohistochemical staining for calcitonin is not routinely performed on NENs of various origins, and even when calcitonin serum levels are measured, an elevated level may be misinterpreted as MTC, especially in patients with thyroid nodules, leading to unnecessary thyroidectomies.…”

Section: Introductionmentioning

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

Self Cite

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

Extrathyroidal Calcitonin Secreting Tumors

Cited by 16 publications

References 13 publications

Malignant Insulinoma with Multiple Liver Metastases and Hypercalcitoninemia in a Patient with Type 2 Diabetes Mellitus Presenting as Recurrent Episodes of Diaphoresis due to Severe Hypoglycemia

Malignant Insulinoma with Multiple Liver Metastases and Hypercalcitoninemia in a Patient with Type 2 Diabetes Mellitus Presenting as Recurrent Episodes of Diaphoresis due to Severe Hypoglycemia

Laryngeal Neuroendocrine Tumor With Elevated Serum Calcitonin: A Diagnostic and Therapeutic Challenge. Case Report and Review of Literature

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

Contact Info

Product

Resources

About