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Duodenum (Psammomatous) Somatostatinoma

Definition

  • Well differentiated duodenal neoplasm composed entirely of somatostatin producing cells, frequently containing psammoma bodies

Alternate/Historical Names

  • D cell tumor / neoplasm
  • Psammomatous somatostatinoma

Diagnostic Criteria

  • Nearly all intestinal cases located in or near ampulla of Vater
  • Uniform cells with bland round regular nuclei
    • Few mitotic figures
    • Slightly granular, pale eosinophilic cytoplasm
  • Tubular and glandular architectural pattern usually predominates
    • May also have insular and trabecular patterns
  • Psammoma bodies in glandular spaces in half of cases
  • Somatostatin stain diffusely positive
  • Somatostatinoma syndrome seen only in rare patients
    • Diabetes mellitus, steatorrhea, cholelithiasis
  • About half of cases associated with neurofibromatosis type 1
    • Pheochromocytoma frequently associated in such cases
    • Same behavior as sporadic cases
  • Rare cases reported associated with MEN1 syndrome
  • Invades locally and may metastasize
    • Duodenal wall, pancreas
    • Local node metastases (35%)
    • Metastases to liver (10%) and very rarely beyond
  • In spite of spread, after debulking, we are not aware of any reported cases of disease progression and/or death
  • Note that about half of somatostatinomas arise instead in the pancreas with a different biology
    • Frequently demonstrates somatostatinoma syndrome, metastases and mixed secretory products
    • Infrequently associated with neurofibromatosis type 1 or psammoma bodies
    • 50% 10 year survival
  • Rare cases reported elsewhere in and out of GI tract

Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342

Original posting/updates: 7/27/10, 12/24/11, 3/10/12

Supplemental studies

  • Somatostatin stain diffusely positive
    • (Scattered somatostatin positive cells may be seen in other neuroendocrine neoplasms)
    • May also have focal cells positive for calcitonin, pancreatic polypeptide, ACTH, gastrin, serotonin
    • Synaptophysin usually positive
      • Chromogranin variable, may be negative
    • Argyrophil variable, argentaffin negative
    • PASd stains luminal border and secretions but not cytoplasm

Grading

WHO 2010 recommends the following grading scheme, but it is not clear if it applies to somatostatinomas

    Proposed grading scale based on proliferation

    Grade Mitotic count per 10 hpf % of cells Ki67+
    G1 <2 ≤2
    G2 2-20 3-20
    G3 >20 >20
    • Mitotic counts based on 50 hpf
    • Ki67 % based on 500-2000 cells
    • If discrepant, use higher grade
    • Scale proposed by European Neuroendocrine Society (ENETS)
      • Carcinoids / neuroendocrine tumors may be G1 or G2
      • G3 is definitional for high grade neuroendocrine carcinoma
    • Progression from G1/2 to G3 is quite rare
      • G3 appears to be a separate process

    Grading/Staging References

    • Rindi G, Klöppel G, Couvelard A, Komminoth P, Körner M, Lopes JM, McNicol AM, Nilsson O, Perren A, Scarpa A, Scoazec JY, Wiedenmann B. TNM staging of midgut and hindgut (neuro) endocrine tumors: a consensus proposal including a grading system. Virchows Arch. 2007 Oct;451(4):757-62.
    • Rindi G, Klöppel G, Alhman H, Caplin M, Couvelard A, de Herder WW, Erikssson B, Falchetti A, Falconi M, Komminoth P, Körner M, Lopes JM, McNicol AM, Nilsson O, Perren A, Scarpa A, Scoazec JY, Wiedenmann B; and all other Frascati Consensus Conference participants; European Neuroendocrine Tumor Society (ENETS). TNM staging of foregut (neuro)endocrine tumors: a consensus proposal including a grading system. Virchows Arch. 2006 Oct;449(4):395-401.
  • Staging
    • Use TNM
  • Differential Diagnosis

    Duodenal (Psammomatous) Somatostatinoma Duodenal or Ampullary Adenocarcinoma
    Round regular nuclei Nuclear atypia and pleomorphism
    Very low mitotic rate Mitotic rate may be moderate to high, may have atypical forms
    May have insular or trabecular pattern Insular and trabecular patterns unusual
    Not associated with overlying adenoma May be associated with overlying adenoma
    50% have psammoma bodies Psammoma bodies not seen
    Somatostatin diffusely positive Somatostatin negative
    Synaptophysin usually positive, chromogranin may be positive Synaptophysin, chromogranin negative
    The frequent glandular pattern and invasion seen in somatostatinomas make this a more difficult distinction than for many other well differentiated gi endocrine neoplasms

    Lists

    Gastrointestinal Endocrine Cell Proliferations and Neoplasms

    Bibliography

    • Riddell RH, Petras RE, Williams GT, Sobin LH. Tumors of the Intestines, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 32, 2003.
    • Bosman FT, Carneiro F, Hruban RH, Thiese ND (Eds). WHO Classifiication of Tumors of the Digestive System, IARC, Lyon 2010
    • Burke AP, Sobin LH, Shekitka KM, Federspiel BH, Helwig EB. Somatostatin-producing duodenal carcinoids in patients with von Recklinghausen's neurofibromatosis. A predilection for black patients. Cancer. 1990 Apr 1;65(7):1591-5.
    • Williams GT. Endocrine tumours of the gastrointestinal tract-selected topics. Histopathology. 2007 Jan;50(1):30-41.
    • Dayal Y, Tallberg KA, Nunnemacher G, DeLellis RA, Wolfe HJ. Duodenal carcinoids in patients with and without neurofibromatosis. A comparative study. Am J Surg Pathol. 1986 May;10(5):348-57.
    • Green BT, Rockey DC. Duodenal somatostatinoma presenting with complete somatostatinoma syndrome. J Clin Gastroenterol. 2001 Nov-Dec;33(5):415-7.
    • Mao C, Shah A, Hanson DJ, Howard JM. Von Recklinghausen's disease associated with duodenal somatostatinoma: contrast of duodenal versus pancreatic somatostatinomas. J Surg Oncol. 1995 May;59(1):67-73.
    • Tanaka S, Yamasaki S, Matsushita H, Ozawa Y, Kurosaki A, Takeuchi K, Hoshihara Y, Doi T, Watanabe G, Kawaminami K. Duodenal somatostatinoma: a case report and review of 31 cases with special reference to the relationship between tumor size and metastasis. Pathol Int. 2000 Feb;50(2):146-52.
    • Bettini R, Falconi M, Crippa S, Capelli P, Boninsegna L, Pederzoli P. Ampullary somatostatinomas and jejunal gastrointestinal stromal tumor in a patient with Von Recklinghausen's disease. World J Gastroenterol. 2007 May 21;13(19):2761-3.
    • Patel VG, Henderson VJ, Fairweather DA, Fortson JK, Weaver WL, Martin DM, Lyons R, Hamami A. Malignant duodenal somatostatinoma presenting in association with von Recklinghausen disease. Am Surg. 2003 Dec;69(12):1077-82.
    • Soga J, Yakuwa Y. Somatostatinoma/inhibitory syndrome: a statistical evaluation of 173 reported cases as compared to other pancreatic endocrinomas. J Exp Clin Cancer Res. 1999 Mar;18(1):13-22.
    • Garbrecht N, Anlauf M, Schmitt A, Henopp T, Sipos B, Raffel A, Eisenberger CF, Knoefel WT, Pavel M, Fottner C, Musholt TJ, Rinke A, Arnold R, Berndt U, Plöckinger U, Wiedenmann B, Moch H, Heitz PU, Komminoth P, Perren A, Klöppel G. Somatostatin-producing neuroendocrine tumors of the duodenum and pancreas: incidence, types, biological behavior, association with inherited syndromes, and functional activity. Endocr Relat Cancer. 2008 Mar;15(1):229-41.
    • Nesi G, Marcucci T, Rubio CA, Brandi ML, Tonelli F. Somatostatinoma: clinico-pathological features of three cases and literature reviewed. J Gastroenterol Hepatol. 2008 Apr;23(4):521-6.
    • Srivastava A, Hornick JL. Immunohistochemical staining for CDX-2, PDX-1, NESP-55, and TTF-1 can help distinguish gastrointestinal carcinoid tumors from pancreatic endocrine and pulmonary carcinoid tumors. Am J Surg Pathol. 2009 Apr;33(4):626-32.
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