OBJECTIVE: Pancreatic endocrine tumors (PETs) are characterized by the presence of hormone syndromes. Reports focusing on calcitonin-secreting PET (CTsPETs) are very rare. This study aimed to define a CTsPET-associated syndrome in regard to chemical, anatomical, and developmental aspects. METHODS: A computerized MEDLINE search was conducted under the search items: "pancreatic endocrine tumor," "calcitonin," "neuroendocrine pancreatic tumor," and "pancreas." Results of clinical, histopathological, immunohistochemical, and biochemical assessments of all patients identified with CTsPET were registered and statistically analyzed. RESULTS: Thirty-seven patients with CTsPET were identified. Mean serum calcitonin was elevated to the 89.2-fold of the upper reference value. Main symptoms were watery diarrhea (51.4%) and abdominal pain (35.1%). Most patients (59.5%) presented with metastatic spread at the time of diagnosis. Of all patients, 66.7% were alive after a mean follow-up of 28.9 months. Survival was higher in patients who underwent more aggressive surgical therapies independent from tumor sizes and in those with no metastases at the time of diagnosis. CONCLUSIONS: High calcitonin levels should always raise suspicion of medullary thyroid carcinomas. However, when thyroid examination remains without pathological findings, a CTsPET should be excluded. An aggressive surgical approach even in cases with large primary tumor sizes may lead to a longer survival.
OBJECTIVE:Pancreatic endocrine tumors (PETs) are characterized by the presence of hormone syndromes. Reports focusing on calcitonin-secreting PET (CTsPETs) are very rare. This study aimed to define a CTsPET-associated syndrome in regard to chemical, anatomical, and developmental aspects. METHODS: A computerized MEDLINE search was conducted under the search items: "pancreatic endocrine tumor," "calcitonin," "neuroendocrine pancreatic tumor," and "pancreas." Results of clinical, histopathological, immunohistochemical, and biochemical assessments of all patients identified with CTsPET were registered and statistically analyzed. RESULTS: Thirty-seven patients with CTsPET were identified. Mean serum calcitonin was elevated to the 89.2-fold of the upper reference value. Main symptoms were watery diarrhea (51.4%) and abdominal pain (35.1%). Most patients (59.5%) presented with metastatic spread at the time of diagnosis. Of all patients, 66.7% were alive after a mean follow-up of 28.9 months. Survival was higher in patients who underwent more aggressive surgical therapies independent from tumor sizes and in those with no metastases at the time of diagnosis. CONCLUSIONS: High calcitonin levels should always raise suspicion of medullary thyroid carcinomas. However, when thyroid examination remains without pathological findings, a CTsPET should be excluded. An aggressive surgical approach even in cases with large primary tumor sizes may lead to a longer survival.
Authors: Henning Dralle; Thomas J Musholt; Jochen Schabram; Thomas Steinmüller; Andreja Frilling; Dietmar Simon; Peter E Goretzki; Bruno Niederle; Christian Scheuba; Thomas Clerici; Michael Hermann; Jochen Kußmann; Kerstin Lorenz; Christoph Nies; Peter Schabram; Arnold Trupka; Andreas Zielke; Wolfram Karges; Markus Luster; Kurt W Schmid; Dirk Vordermark; Hans-Joachim Schmoll; Reinhard Mühlenberg; Otmar Schober; Harald Rimmele; Andreas Machens Journal: Langenbecks Arch Surg Date: 2013-03-03 Impact factor: 3.445
Authors: M Falconi; B Eriksson; G Kaltsas; D K Bartsch; J Capdevila; M Caplin; B Kos-Kudla; D Kwekkeboom; G Rindi; G Klöppel; N Reed; R Kianmanesh; R T Jensen Journal: Neuroendocrinology Date: 2016-01-05 Impact factor: 4.914
Authors: Tiziana Feola; Giulia Puliani; Franz Sesti; Roberta Modica; Marco Biffoni; Cira Di Gioia; Raffaella Carletti; Emanuela Anastasi; Valentina Di Vito; Roberta Centello; Andrea Lenzi; Andrea M Isidori; Antongiulio Faggiano; Elisa Giannetta Journal: Front Endocrinol (Lausanne) Date: 2020-07-16 Impact factor: 5.555
Authors: Andrew T Magis; Noa Rappaport; Matthew P Conomos; Gilbert S Omenn; Jennifer C Lovejoy; Leroy Hood; Nathan D Price Journal: Sci Rep Date: 2020-10-01 Impact factor: 4.379