Gangliocytic Paraganglioma

Definition

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

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