Download PDF
Exp Clin Endocrinol Diabetes 2002; 110(1): 43-49
DOI: 10.1055/s-2002-19994
Case Report

© Johann Ambrosius Barth

Renin producing neuroendocrine pancreatic carcinoma - a case report and review of the literature

P. Langer 1 , D. Bartsch 1 , B. Gerdes 1 , I. Schwetlick 2 , A. Wild 1 , B. Brehm 3 , C. Erley 3 , R. Lamberts 3
  • 1 Department of General Surgery, University Hospital, Philipps-University of Marburg, Germany
  • 2 Department of Pathology, University Hospital, Philipps-University of Marburg, Germany
  • 3 Department of Internal Medicine, University Hospital, Eberhard Karls University of Tübingen, Germany
Further Information

Publication History

received 09 May 2001 first decision 12 July 2001

received 31 August 2001

Publication Date:
07 February 2002 (online)

Also available at
    Permissions and Reprints

Summary

A 44 year old male patient presented with severe hypertension. The diagnostic work-up revealed elevated levels of plasma renin activity (about 10 times the upper limit of normal) in the presence of normal plasma aldosterone levels and serum potassium concentrations. Renovascular disease was excluded by angiography. Selective renal vein sampling did not show any renin gradient. CT-scans of the abdomen demonstrated normal morphology of the kidneys and adrenals but revealed a big mass in the pancreatic corpus and tail with infiltration of the splenic vein and the presence of enlarged local lymph nodes. The endocrine nature of the pancreatic mass was further supported by a positive octreotide scintigraphy scan. Surgical removal of the tumor by left sided pancreatectomy combined with splenectomy resulted in rapid normalization of elevated renin concentrations as well as blood pressure. Histological examination of the tumor tissue revealed the presence of a neuroendocrine pancreatic carcinoma. Highly (× 70) elevated renin levels were detected by radioimmunoassay in the tumor tissue.

To our knowledge this is the first renin-producing neuroendocrine pancreatic carcinoma described in the literature. The present paper describes the case in detail and reviews the available literature on clinical symptomatology, diagnosis and treatment of renin-producing tumors.

Key words:

Renin-producing endocrine tumors - Primary reninism - Pancreatic endocrine carcinoma - Hypertension - Ectopic hormone production

References

  • 1 Anderson P W, Macaulay L, Do Y S, Sherrod A, d'Ablaing G, Koss M, Shinagawa T, Tran B, Montz F J, Hsueh W A. Extrarenal renin-secreting tumors: insights into hypertension and ovarian renin production.  Medicine. 1989;  68 257-268
    PubMedGoogle Scholar
  • 2 Atlas S A, Hesson T E, Sealey J E, Dharmgrongartama B, Laragh J H, Ruddy M C, Aurell M. Characterization of inactive renin (“prorenin”) from renin-secreting tumors of nonrenal origin. Similarity to inactive renin from kidney and normal plasma.  J Clin Invest. 1984;  73 437-447
    PubMedGoogle Scholar
  • 3 Aurell M, Rudin A, Tisell L-E, Kindblom L G, Sandberg G. Captopril effect on hypertension in patient with renin-producing tumor.  Lancet. 1979;  2 (8134) 149-150
    PubMedGoogle Scholar
  • 4 Baruch D, Corvol P, Alhenc-Gelas F, Dufloux M A, Guyenne T T, Gaux J C, Raynaud A, Brisset J M, Duclos J M, Menard J. Diagnosis and treatment of renin-secreting tumors.  Hypertension. 1984;  6 760-766
    PubMedGoogle Scholar
  • 5 Bonnin J M, Hodge R L, Lumbers E R. A renin-secreting renal tumor associated with hypertension.  1972;  Aust New Zeal J Med. 2 78-181
    PubMedGoogle Scholar
  • 6 Brown L L, Lever A F, Robertson J IS. Hypertension and secondary hyperaldosteronism associated with a renin-secreting renal juxtaglomerular tumor.  Lancet. 1973;  2 1228-1231
    PubMedGoogle Scholar
  • 7 Chandrasekharappa S C, Guru S C, Manickam P, Olufemi S E, Collins F S, Emmert-Buck M R, Debelenko L V, Zhuang Z, Lubensky I A, Liotta L A, Crabtree J S, Wang Y, Roe B A, Weisemann J, Boguski M S, Agarwal S K, Kester M B, Kim Y S, Heppner C, Dong Q, Spiegel A M, Burns A L, Marx S J. Positional cloning of the gene for multiple endocrine neoplasia-type I.  Science. 1997;  276 404-407
    CrossrefPubMedGoogle Scholar
  • 8 Chappell M C, Millsted A, Diz D I, Brosnihan K B, Ferrario C M. Evidence for an intrinsic angiotensin system in the canine pancreas.  J Hypertens. 1991;  9 751-759
    PubMedGoogle Scholar
  • 9 Chiang H CV, O'Dorisio T M, Huang S C, Maton P N, Gardner J D, Jensen R T. Multiple hormone elevations in Zollinger-Ellison syndrome.  Gastroenterol. 1990;  99 1565-1575
    PubMedGoogle Scholar
  • 10 Conn J W, Cohen E L, Lucas C P, McDonald W J, Mayor G H, Blough W M, Eveland W C, Bookstein J J, Lapides J. Primary reninism. Hypertension, hyperreninemia, and secondary aldosteronism due to renin-producing jutaglomerular cell tumors.  Arch Intern Med. 1972;  130 682-696
    CrossrefPubMedGoogle Scholar
  • 11 Connor G, Bennett C M, Lindstrom R R, Brosman S A, Barajas L, Edelbaum D. Juxtaglomerular cell tumor.  Nephron. 1978;  21 325-333
    PubMedGoogle Scholar
  • 12 Corvol P, Pinet F, Plouin P F, Bruneval P, Menard J. Renin-secreting tumors.  Endocrinol Metab Clin North Am. 1994;  23 255-270
    PubMedGoogle Scholar
  • 13 Cox J N, Paunier L, Vallotton M B, Humbert J R, Rohner A. Epithelial liver hamartoma, systemic arterial hypertension and renin hypersecretion.  Virchows Arch A Path Anat Histol. 1975;  366 15-26
    PubMedGoogle Scholar
  • 14 Derkx F H, Schalekamp M A. Human prorenin: pathophysiology and clinical implications.  Clin Exp Hypertens A. 1988;  10 1213-1225
    PubMedGoogle Scholar
  • 15 Derkx F HM, Stuenkel C, Schalekamp M PA, Visser W, Huisveld J H, Schalekamp A DH. Immunoreactive renin, prorenin, and enzymatically active renin in plasma during pregnancy and in women taking oral contraceptives.  J Clin Endocrinol Metab. 1986;  63 1008-1015
    CrossrefPubMedGoogle Scholar
  • 16 Do Y S, Sherrod A, Lobo R A, Paulson R J, Shinagawa T, Chen S, Kjos S, Hsueh W A. Human ovarian theca cells are a source of renin.  Proc Natl Acad Sci USA. 1988;  85 1957-1961
    PubMedGoogle Scholar
  • 17 Eddy R L, Sanchez S A. Renin-secreting renal neoplasm and hypertension with hypokalemia.  Ann Int Med. 1971;  75 725-729
    PubMedGoogle Scholar
  • 18 Eriksson B, Arnberg H, Lindgren P G, Lorelius L E, Magnusson A, Lundqvist G, Skogseid B, Wide L, Wilander E, Öberg K. Neuroendocrine pancreatic tumors: clinical presentation, biochemical and histopathological findings in 84 patients.  J Int Med. 1990;  228 103-111
    PubMedGoogle Scholar
  • 19 Fukamizu A, Seo M S, Hatae T, Yokoyama M, Nomura T, Katsuki M, Murakami K. Tissue-specific expression of the human renin gene in transgenic mice.  Biochem Biophys Res Comm. 1989;  165 826-832
    CrossrefPubMedGoogle Scholar
  • 20 Ganguly A, Gribbler J, Tune B, Kempson R L, Luetscher J A. Renin-secreting Wilm's tumor with severe hypertension.  Ann Int Med. 1973;  79 835-837
    PubMedGoogle Scholar
  • 21 Genest J, Rojo-Ortega J M, Kuchel O, Boucher R, Nowaczynski W, Lefebvre R, Chretien M, Cantin J, Granger P. Malignant hypertension with hypokalemia in a patient with renin-producing pulmonary carcinoma.  Trans Assoc Am Physicians. 1975;  88 192-201
    PubMedGoogle Scholar
  • 22 Gordon R D, Tunny T J, Klemm S A, Finn W L, Hawkins P J, Hunyor S J, Norris J J. A renin-secreting tumour sensitive to changes in central blood volume (presumably via sympathetics) but not to circulating angiotensin II.  Clin Exp Pharmacol Physiol. 1990;  17 185-189
    PubMedGoogle Scholar
  • 23 Haab F, Guyenne T, Plouin P F, Corvol P. Renin secreting tumors: diagnosis, conservative surgical approach and long-term results.  J Urol. 153 ((6)) 1995;  1781-1784
    CrossrefPubMedGoogle Scholar
  • 24 Hauger-Klevenger J J. High plasma renin activity in an oat cell carcinoma.  Cancer. 1970;  26 1112-1114
    PubMedGoogle Scholar
  • 25 Hollifield J W, Page D L, Smith C, Michelakis A M, Staab E, Rhamy R. Renin-secreting clear cell carcinoma of the kidney.  Arch Intern Med. 1975;  135 859-864
    CrossrefPubMedGoogle Scholar
  • 26 Kawa S, Ueno T, Iijima A, Medorikawa T, Fujimori Y, Tokoo M, Oguchi H, Kiyosawa K, Imai Y, Kaneko G, Kuroda T, Hashizume K, Osamura R Y, Katakami H. Growth hormone-releasing hormone (GRH) producing pancreatic tumor with no evidence of multiple endocrine neoplasia type I.  Dig Dis Sci. 1997;  42 1480-1485
    CrossrefPubMedGoogle Scholar
  • 27 Kawai K, Fukamizu A, Kawakami Y. A case of renin producing leiomyosarcoma originating in the lung.  Endocrinol Jpn. 1991;  38 603-609
    PubMedGoogle Scholar
  • 28 Kihara I, Kitamura S, Hoshino T, Seida H, Watanabe T. A hitherto unreported vascular tumor of the kidney: a proposal of “juxtaglomerular cell tumor”.  Acta Path Jpn. 1968;  18 197
    PubMedGoogle Scholar
  • 29 Korzets A, Nouriel H, Steiner Z, Griffel B, Kraus L, Freund U, Klajman A. Resistant hypertension associated with a renin-producing ovarian Sertoli cell tumor.  Am J Clin Pathol. 1986;  85 242-247
    PubMedGoogle Scholar
  • 30 Krenning E P, Kwekkeboom D J, Bakker W H, Brzuman W A, Kooij P P, Oei H Y, van Hagen M, Postema P TE, de Jong M, Reubi J C, Visser T J, Reijs A EM, Hofland L J, Koper J W, Lamberts S WJ. Somatostatin receptor scintigraphy with (111In-DTPA-D-Phe1)- and (123J-Tyr3)-octreotide: the Rotterdam experience with more than 1000 patients.  Eur J Nucl Med. 1993;  20 716-731
    PubMedGoogle Scholar
  • 31 Leung P S, Chan H C, Wong P Y. Immunohistochemical localization of angiotensin II in the mouse pancreas.  Histochem J. 1998;  30 21-25
    PubMedGoogle Scholar
  • 32 Leung P S, Chan W P, Wong T P, Sernia C. Expression and localization of the renin-angiotensin system in the rat pancreas.  J Endocrinol. 1999;  160 13-19
    CrossrefPubMedGoogle Scholar
  • 33 Lopez G, Asenjo J A, Blanco-Gonzalez J, Ortega-Medina L, Sanz-Esponera J. Juxtaglomerular cell tumor of the kidney. Morphological, immunohistochemical and ultrastructural studies of a new case.  Pathol Res Pract. 1991;  187 354-359
    PubMedGoogle Scholar
  • 34 Mao C, El Attar A, Domenico D R, Kim K, Howard J M. Carcinoid tumors of the pancreas. Status report based on two cases and review of the world's literature.  Int J Pancreatol. 1998;  23 153-164
    CrossrefPubMedGoogle Scholar
  • 35 Martensson H, Böttcher G, Sundler F, Nobin A. Localization and peptide content of endocrine pancreatic tumors.  Ann Surg. 1990;  212 607-614
    PubMedGoogle Scholar
  • 36 Maurer C A, Glaser C, Reubi J C, Büchler M W. Carcinoid of the pancreas.  Digestion. 1997;  58 410-414
    CrossrefPubMedGoogle Scholar
  • 37 Metz D C. Multiple endocrine neoplasia type I.  Semin Gastrointest Dis. 1995;  6 56-66
    PubMedGoogle Scholar
  • 38 Miraliakbari B A, Asa S L, Boudreau S F. Parathyroid hormone-like peptide in pancreatic endocrine carcinoma and adenocarcinoma associated with hypercalcemia.  Hum Pathol. 1992;  23 884-887
    CrossrefPubMedGoogle Scholar
  • 39 Mitchell J D, Baxter T J, Blair-West J R, McCredie D A. Renin levels in nephroblastoma (Wilm's tumor): report of a renin-secreting tumor.  Arch Dis Child. 1970;  45 376-384
    PubMedGoogle Scholar
  • 40 Muller F B, Sealey J E, Case D B, Atlas S A, Pickering T G, Pecker M S, Preibisz J J, Laragh J H. The captopril test for identifying renovascular disease in hypertensive patients.  Am J Med. 1986;  80 633-644
    CrossrefPubMedGoogle Scholar
  • 41 Pedrinelli R, Graziadei L, Taddei S, Lenzi M, Magagna A, Bevilacqua G, Salvetti A. A renin-secreting tumor.  Nephron. 1987;  46 380-385
    PubMedGoogle Scholar
  • 42 Raynaud A, Chatellier G, Baruch D, Angel C, Menard J, Gaux J C. Radiologic features of renin-producing tumors. A report of two cases.  Ann Radiol Paris. 1985;  28 439-446
    PubMedGoogle Scholar
  • 43 Robertson P W. et al. . Hypertension due to a renin-secreting renal tumor.  Am J Med. 1967;  43 963-975
    CrossrefPubMedGoogle Scholar
  • 44 Roswell R H. Renin-secreting tumors.  J Okla State Med Assoc. 1990;  83 57-59
    PubMedGoogle Scholar
  • 45 Ruddy M C. et al. . Hypertension associated with a renin-secreting adenocarcinoma of the pancreas.  New Engl J Med. 1982;  307 993-997
    PubMedGoogle Scholar
  • 46 Schambelan M. et al. . Role of renin and aldosterone in hypertension due to a renin-secreting tumor.  Am J Med. 1973;  55 86-92
    CrossrefPubMedGoogle Scholar
  • 47 Sheth K J. et al. . Polydipsia, polyuria, and hypertension associated with a renin-secreting Wilms' tumor.  J Pediatr. 1978;  92 921-924
    PubMedGoogle Scholar
  • 48 Tahmasebi M. et al. . The tissue renin-angiotensin system in human pancreas.  J Endocrinol. 1999;  16 317-322
    PubMedGoogle Scholar
  • 49 Warshaw B L. et al. . Hypertension secondary to a renin-producing juxtaglomerular cell tumor.  J Pediatr. 1979;  94 247
    PubMedGoogle Scholar
  • 50 Zabernigg A. et al. . An unusual case of a renin-producing tumour of the fallopian tube.  Eur J Cancer. 1997;  33 1709
    CrossrefPubMedGoogle Scholar

Prof. Dr. med. Regina Lamberts

Department of Internal Medicine

Division of Gastroenterology, Hepatology and Infectious Diseases

Eberhard Karls University of Tübingen

Otfried-Müller-Str. 10

D-72076 Tübingen

Germany

Phone: +49-7071-2982722

Fax: +49-7071-292095

Email: regina.lamberts@uni-tuebingen.de