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Gangliocytic Paraganglioma

Definition

  • Triphasic tumor composed of epithelioid, ganglion and spindle cells, occurring in the ampulla of Vater

Alternate/Historical Names

  • Ganglioneuroma
  • Ganglioneuromatous paraganglioma
  • Non-chromaffin paraganglioma
  • Paraganglioneuroma

Diagnostic Criteria

  • Neoplasm formed by an intimate admixture of three cell types
    • Epithelioid cells
      • Polygonal to columnar
      • Pale eosinophilic finely granular cytoplasm
      • Bland oval nuclei with stippled chromatin
      • Form solid nests, trabeculae, tubules or pseudo-glands
    • Ganglion cells
      • Large vesicular nuclei with prominent nucleoli
      • Abundant cytoplasm with Nissl substance
      • Scattered singly or in clusters
    • Spindle cells
      • Bland round to elongate nuclei
      • Form broad fascicles and envelop epithelioid and ganglion cells
    • Atypia, pleomorphism, mitotic activity and necrosis are rare
  • Virtually restricted to ampulla of Vater
    • Rare cases reported in jejunum, pylorus, esophagus, pancreas, appendix, lung
      • One case arising in pancreas reported to have atypia, somatostatin serum elevation and metastasis to bone
    • Caution should be used in the diagnosis and prediction of clinical behavior for extra-ampullary gangliocytic paraganglioma
  • Extensively infiltrates smooth muscle and glands of ampulla
    • Centered in submucosa
    • Overlying mucosa not involved but may ulcerate
    • May infiltrate into head of pancreas
  • Infrequent cases of ampullary tumors with spread to local lymph nodes
    • About 7% of reported cases, but this may represent publication bias (Okubo 2011)
    • No reports of aggressive behavior or death following resection
    • Most node metastases are pure epithelioid cells but some cases report triphasic metastasis

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

Original posting/updates : 7/5/10, 11/13/11

Supplemental studies

Immunohistology

  • Immunohistologic stains show variable evidence of endocrine, neural and nerve sheath differentiation
    • Epithelioid cells
      • Keratin variably positive
      • Variably positive for synaptophysin, chromogranin, pancreatic polypeptide, somatostatin, calcitonin, glucagon and insulin
      • Occasionally neurofilament positive
    • Ganglion cells
      • Neurofilament and synaptophysin positive
      • Variably positive for pancreatic polypeptide, somatostatin, calcitonin
    • Spindle cells
      • S100, neurofilament positive

Differential Diagnosis

  • The distinctive triphasic appearance essentially excludes most other possibilities

Grading

  • Considered benign in AFIP classification
  • Because of several reports of local lymph node involvement it might better be considered either of uncertain malignant potential or low grade malignant

Classification/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.
  • Inai K, Kobuke T, Yonehara S, Tokuoka S. Duodenal gangliocytic paraganglioma with lymph node metastasis in a 17-year-old boy. Cancer. 1989 Jun 15;63(12):2540-5.
  • Perrone T, Sibley RK, Rosai J. Duodenal gangliocytic paraganglioma. An immunohistochemical and ultrastructuralstudy and a hypothesis concerning its origin. Am J Surg Pathol. 1985 Jan;9(1):31-41.
  • Witkiewicz A, Galler A, Yeo CJ, Gross SD. Gangliocytic paraganglioma: case report and review of the literature. J Gastrointest Surg. 2007 Oct;11(10):1351-4.
  • Sundararajan V, Robinson-Smith TM, Lowy AM. Duodenal gangliocytic paraganglioma with lymph node metastasis: a case report and review of the literature. Arch Pathol Lab Med. 2003 Mar;127(3):e139-41.
  • Henry C, Ghalel-Méchaoui H, Bottero N, Pradier T, Moindrot H. [Gangliocytic paraganglioma of the pancreas with bone metastasis] Ann Chir. 2003 Jun;128(5):336-8.
  • Okubo Y, Wakayama M, Nemoto T, Kitahara K, Nakayama H, Shibuya K, Yokose T, Yamada M, Shimodaira K, Sasai D, Ishiwatari T, Tsuchiya M, Hiruta N. Literature survey on epidemiology and pathology of gangliocytic paraganglioma. BMC Cancer. 2011 May 20;11:187
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