Paraganglioma

 Progenitor neural crest cells


image Embryonic celiac ganglion

image Endodermally derived epithelial cells originating from ventral primordium of pancreas (hamartomatous proliferation)

image Pancreatic tumor composed of ganglion-islet cell complexes





Clinical Issues




• Uncommon

• 3rd-9th decades (mean age: 6th decade)

• Men slightly out number women

• Most commonly involve periampullary duodenum

• Clinically benign


Microscopic




• Epithelioid, spindled, and ganglion-like cells present in varying proportions

• Endocrine cells may also be present

• S100 may reveal sustentacular-like cells surrounding nests of epithelioid cells

• No mitoses or necrosis


Diagnostic Checklist




• Low-grade neuroendocrine tumor

• Ampullary carcinoma

• Ganglioneuroma

• Neurofibroma

• Gastrointestinal stromal tumor

image
Periampullary Gangliocytic Paraganglioma
Periampullary gangliocytic paragangliomas image often have an infiltrating pattern at the periphery as shown here at low-power magnification. The lesion is centered in the submucosa with extension into the overlying mucosa image .


image
Gangliocytic Paraganglioma
Gangliocytic paraganglioma (high-power magnification) consisting of a mixture of epithelioid cells, ganglion-like cells image, and nerve sheath elements image is shown.

image
Gangliocytic Paraganglioma
Ganglion-like cells image with dense cytoplasm, open chromatin, and prominent nucleoli are indistinguishable from normal ganglion cells. Nerve sheath elements image contain Schwann cells with elongated nuclei and vacuolated cytoplasm. Neural filaments are not well visualized.

image
Immunohsitochemistry for S100
As is typically seen, the Schwann cell component is positive for S100 in this gangliocytic paraganglioma.


TERMINOLOGY


Synonyms




• Gangliocytic paraganglioma

• Nonchromaffin paraganglioma


Definitions




• Neoplasm consisting of epithelioid, ganglion-like cells and nerve sheath elements
image Associated with neurofibromatosis type 1 in some cases

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Apr 20, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Paraganglioma

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