2014
DOI: 10.1210/jc.2014-1315
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Parathyroid Hormone-Related Peptide (PTHrP) Secretion by Gastroenteropancreatic Neuroendocrine Tumors (GEP-NETs): Clinical Features, Diagnosis, Management, and Follow-Up

Abstract: Hypersecretion of PTHrP by metastatic GEP-NETs is very rare and seems to be exclusively associated with metastatic pancreatic NETs. PTHrP production has major clinical impact because poorly controllable hypercalcemia is associated with increased morbidity and mortality. The most successful treatment options for PTHrP-producing GEP-NETs are SSAs and PRRT using radiolabeled SSAs. Isotonic saline and bisphosphonates can be considered as supportive therapies.

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Cited by 56 publications
(47 citation statements)
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“…In refractory cases, intravenous etomidate has successfully been used but requires intensive monitoring [57] . Hypersecretion of parathyroid hormone-related peptide by metastatic NET seems to be exclusively associated with metastatic pancreatic NET and has a major clinical impact because poorly controllable hypercalcemia is associated with increased morbidity and mortality [58] . In such cases and other more rare syndromes, early diagnosis and management are essential [54] .…”
Section: Syndromes Related To Ectopic Hormonal Secretionmentioning
confidence: 99%
“…In refractory cases, intravenous etomidate has successfully been used but requires intensive monitoring [57] . Hypersecretion of parathyroid hormone-related peptide by metastatic NET seems to be exclusively associated with metastatic pancreatic NET and has a major clinical impact because poorly controllable hypercalcemia is associated with increased morbidity and mortality [58] . In such cases and other more rare syndromes, early diagnosis and management are essential [54] .…”
Section: Syndromes Related To Ectopic Hormonal Secretionmentioning
confidence: 99%
“…Another interesting aspect in our case was the control of tumor secretion of PTHrP provided by everolimus, even at the time of clear radiological progression. PTHrP-producing NETs are rare and some reports have shown limited-time control of hypercalcemia with somatostatin analogs, radiolabeled agents and even sunitinib [10]. An anti-secretory activity of everolimus in patients with insulinomas [11] and carcinoid syndrome [12] has been reported, but we are unaware of other reports of everolimus successfully controlling malignant hypercalcemia related to PTHrP-producing NET.…”
Section: Discussionmentioning
confidence: 99%
“…Because of the death of these mice at birth the role of PTHrP in the physiology of islets is not known. Neuroendocrine tumors secreting PTHrP resulting in hypercalcemia has been described [76,78].…”
Section: Pthrp and The Pancreasmentioning
confidence: 99%