Exp Clin Endocrinol Diabetes 2006; 114(5): 217-221
DOI: 10.1055/s-2006-924257
Case Report

J. A. Barth Verlag in Georg Thieme Verlag KG Stuttgart · New York

Hypercalcitoninemia in a Patient with a Recurrent Goitre and Insulinoma: a Case Report

P. Pusztai1 , B. Sármán1 , G. Illyés2 , E. Székely2 , I. Péter4 , K. Boer5 , T. Tihanyi3 , K. Rácz1
  • 12nd Department of Medicine, Semmelweis University, Budapest
  • 22nd Department of Pathology, Semmelweis University, Budapest
  • 31st Department of Surgery, Semmelweis University, Budapest
  • 4National Institute of Oncology, Budapest
  • 5Department of Oncology, St. Margit Hospital, Budapest, Hungary
Further Information

Publication History

Received: July 21, 2005 First decision: November 4, 2005

Accepted: February 3, 2006

Publication Date:
28 June 2006 (online)

Abstract

Serum calcitonin has become a very sensitive and specific marker for medullary thyroid carcinoma that should be determined in patients with nodular thyroid disease. However, a few earlier reports indicated that tumors other than medullary thyroid carcinoma including insulinomas arising from pancreatic islet cells may also produce calcitonin. Of the few cases of calcitonin-producing insulinomas previously reported, most had incomplete data or lack of documentation of the association between raised serum calcitonin concentration and immunohistochemical detection of calcitonin in pancreatic islet cell tumors. In this paper we are reporting a 54-year-old woman with a history of partial thyroidectomy for multinodular goitre at the age of 50 yrs, she was evaluated for a 2-months history of fasting hypoglycemia (plasma glucose 1.9 mmol/L during a supervised fast), raised serum insulin (at the time of hypoglycemia 88.8 µU/ml; normal, 5 - 35 µU/ml) and C-peptide levels (at the time of hypoglycemia 6.1 ng/ml; normal, 1.37 - 3.51 ng/ml), markedly increased serum calcitonin concentration (481 pg/ml; normal, < 9.9 pg/ml), and an enlarged residual thyroid gland. Aspiration biopsy of the thyroid was negative for parafollicular C-cell hyperplasia or medullary thyroid carcinoma. Abdominal ultrasound and CT scan revealed a tumor in the head of the pancreas, which was surgically removed. Histopathological evaluation of the pancreatic tumor showed typical features of a neuroendocrine neoplasm with strong immunostaining for both insulin and calcitonin. After removal of the pancreatic tumor, clinical symptoms resolved and biochemical markers normalized (serum insulin, 14.9 µU/ml; C-peptide, 3.0 ng/ml; calcitonin, 2.9 pg/ml) confirming the causal relationship between insulinoma and markedly increased serum calcitonin levels.

References

  • 1 Abe Y, Utsunomiya H, Tsutsumi Y. Atypical carcinoid tumor of the lung with amyloid stroma.  Acta Pathol Jap. 1992;  42 286-292
  • 2 Akerström G, Hellman P, Hessman O, Osmak L. Surgical treatment of endocrine pancreatic tumors.  Neuroendocrinology. 2004;  80 62-66
  • 3 Bugalho M J, Roque L, Sobrinho L G, Hoog A, Nunes J F, Almeida J M, Leitao C N, Santos J R, Pereira M C, Santos M A, Soares J. Calcitonin-producing insulinoma: clinical, immunocytochemical and cytogenetical study.  Clin Endocrinol. 1994;  41 257-260
  • 4 Conlon J M, Grimelius L, Thim L. Structural characterization of a high-molecular-mass form of calcitonin [procalcitonin-(60-116)-peptide] and its corresponding N-terminal flanking peptide [procalcitonin-(1-57)-peptide] in a human medullary thyroid carcinoma.  Biochem J. 1988;  256 245-250
  • 5 De Herder W W. Insulinoma.  Neuroendocrinology. 2004;  80 20-22
  • 6 Findlay D M, Martin T J. Receptors of calcitropic homones.  Horm Metab Res. 1997;  29 128-134
  • 7 Fleury A, Flejou J F, Sauvanet A, Molas G, Vissuzaine C, Hammel P, Levy P, Belghiti J, Bernades P, Ruszniewski P. Calcitonin-secreting tumours of the pancreas: about six cases.  Pancreas. 1998;  16 545-550
  • 8 Gaulier A, Cahen J, Buisson J L, Perie G, Vacher G, Poulet B. Pancreatic insulinoma, adenomatosis of the Wirsung's duct and chronic pancreatitis. Apropos of a case.  Arch Anat Cyt Path. 1993;  41 245-250
  • 9 Insabato L, De Rosa G, Terracciano L M, Lupoli G, Montedoro D, Ravetto C. A calcitonin-producing neuroendocrine tumor of the larynx: a case report.  Tumori. 1993;  79 227-230
  • 10 Karges W, Dralle H, Raue F, Mann K, Reiners C, Grussendorf M, Hüfner M, Nierdle B, Brabant G. Calcitonin measurement to detect medullary thyroid carcinoma in nodular goiter: German evidence-based consensus recommendation.  Exp Clin Endocrinol Diab. 2004;  112 52-58
  • 11 King C M, Reznek R H, Dacie J E, Wass J A. Imaging islet cell tumours.  Clin Radiol. 1994;  49 295-303
  • 12 Leboulleux S, Baudin E, Young J, Caillou B, Lazar V, Pellegriti G, Ducreux M, Schaison G, Schlumberg M. Gastroenteropancreatic neuroendocrine tumour metastases to the thyroid gland: differential diagnosis with medullary thyroid carcinoma.  Eur J Endocrinol. 1999;  140 187-291
  • 13 Machens A, Haedecke J, Hinze R, Thomusch O, Schneyer U, Dralle H. Hypercalcitoninemia in a sporadic asymptomatic neuroendocrine tumor of the pancreatic tail.  Dig Surg. 2000;  17 522-524
  • 14 Matias-Guiu X, LaGuette J, Puras-Gil A M, Rosai J. Metastatic neuroendocrine tumor to the thyroid gland mimicking medullary carcinoma: a pathologic and immunohistochemical study of six cases.  Am J Surg Pathol. 1997;  21 757-762
  • 15 Ooi A, Nakanishi I, Kameya T, Funaki Y, Kobayashi K. Calcitonin-producing insulinoma. An immunohistochemical and electron microscopic study.  Acta Path Jap. 1986;  36 1897-1903
  • 16 Plöckinger U, Rindi G, Arnold R, Eriksson B, Krenning E P, Goede A, Caplin M, Wiedenmann B. Guidelines for the diagnosis and treatment of neuroendocrine gastrointestinal tumors.  Neuroendocrinology. 2004;  80 394-424
  • 17 Price D E, Absalom S R, Davidson K, Bolia A, Bell P R, Howlett T A. A case of multiple endocrine neoplasia: hyperparathyroidism, insulinoma, GRF‐oma, hypercalcitoninaemia and intractable peptic ulceration.  Clin Endocrinol. 1992;  37 187-188
  • 18 Rambaud J C, Jian R, Flourie B, Hautefeuille M, Salmeron M, Thuillier F, Ruskone A, Florent C, Chaoui F, Bernier J J. Pathophysiological study of diarrhoea in a patient with medullary thyroid carcinoma. Evidence against a secretory mechanism and for shortened colonic transit time.  Gut. 1988;  29 537-543
  • 19 Rindi G, Bordi C. Highlights of the biology of endocrine tumours of the gut and pancreas.  Endoc Rel Cancer. 2003;  10 427-436
  • 20 Rindi G, Kloppel G. Endocrine tumors of the gut and pancreas tumor biology and classification.  [Review] Neuroendocrinology. 2004;  80 12-15
  • 21 Service F J. Insulinoma and other islet-cells tumours.  Cancer Treat Res. 1997;  89 335-346
  • 22 Service F J, McMahon M M, O'Brien P C, Ballard D J. Functioning insulinoma: incidence, recurrence, and long-term survival of patients: a 60-year study.  Mayo Clin Proc. 1991;  66 711-719
  • 23 Singh R, Bibbo M, Cunnane M F, Carlson J A, de Papp A E. Metastatic cervical carcinoma with ectopic calcitonin production presenting as thyroid mass.  Endocr Pract. 2002;  8 50-53
  • 24 Sugimoto F, Sekiya T, Saito M, Tsuneo I, Suda K, Nozawa A, Nakazawa T, Ishizaki T, Ikarashi T. Calcitonin-producing pancreatic somatostatinoma: report of a case.  Jpn J Surg. 1998;  28 1279-1282
  • 25 Takahashi M, Hoshii Y, Kawano H, Setoguchi M, Gondo T, Yamashita Y, Nakayasu K, Kamei T, Ishihara T. Multihormone-producing islet cell tumor of the pancreas associated with somatostatin-immunoreactive amyloid: immunohistochemical and immunoelectron microscopic studies.  Am J Surg Pathol. 1998;  22 360-367
  • 26 Young A A, Wang M W, Gedulin B, Rink T J, Pittner R, Beaumont K. Diabetogenic effects of salmon calcitonin are attributable to amylin-like activity.  Metab Clin Exp. 1995;  44 1581-1589

MD Péter Pusztai

2nd Department of Medicine
Semmelweis University

1088, Szentkirályi u. 46.

Budapest

Hungary

Phone: + 36 1 266-0926 ext. 5577

Fax: + 36 1 266-0816

Email: peterpusztaimd@hotmail.com

    >