Small bowel (small intestine) - Gangliocytic paraganglioma

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Small bowel (small intestine)

Benign tumors and tumor-like conditions

Gangliocytic paraganglioma

Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 19 December 2012, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

See also Ampulla"

General
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● Rare tumor of periampullary region and second portion (middle) of duodenum
● Rarely in jejunum or ileum
● May derive from endodermal-neuroectodermal complexes in the embryonic ventral pancreas, due to totipotential nature of adult stem cells that differentiate along different lineages

Clinical features
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● Presents with GI bleed, abdominal pain or anemia
● May be multiple or associated with neurofibromatosis 1 or carcinoid syndrome
● Usually benign, 7% had nodal metastases at presentation (BMC Cancer 2011;11:187), rarely has metastases of endocrine component

Case reports
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● 42 year old man with obstructive jaundice (Rev Col Bras Cir 2011;38:288)
● 56 year old man with melana (World J Surg Oncol 2010;8:42)
● 61 year old man with nodal metastasis (Diagn Pathol 2010;5:27)
● 67 year old man (Chin Med J (Engl) 2012;125:388)
● 67 year old woman with metastatic tumor in regional lymph nodes containing spindle cell, epithelial and ganglion components (Arch Pathol Lab Med 2003;127:e139)

Gross description
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● Usually 1-3 cm, sessile or polypoid

Clinical images
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Periampullary submucosal tumor and surface ulcer bleeding

Gross images
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Tumor near ampulla

Malignant tumor

Micro description
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● Submucosal lesion(s) with overlying ulcerations
● Organoid pattern
● Triphasic with endocrine (carcinoid like cells in compact nests and trabeculae), spindle cell (Schwann cell like), and ganglion type cells of varying proportions resembling carcinoid, paraganglioma and ganglioneuroma
● Variable stromal amyloid

Micro images
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Various images

Malignant tumor

Positive stains
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● Pancreatic polypeptide (in endocrine cells, not specific to this tumor)
● Keratin (epithelial cells)
● S100 (spindle cells)
● Neuron specific enolase (ganglion cells)

Differential diagnosis
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● Carcinoid tumor
● Carcinoma
● Ganglioneuroma
● Paraganglioma

End of Small bowel (small intestine) > Benign tumors and tumor-like conditions > Gangliocytic paraganglioma

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