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Synchronous diffuse ganglioneuromatosis and multiple schwannomas of the colon: A case report and literature review.

Lu C, Qiu Y, Lu X, Li G, Bu H - Exp Ther Med (2015)

Bottom Line: Diffuse ganglioneuromatosis (DG) of the gastrointestinal tract is a rare condition that is closely associated with neurofibromatosis type 1 and multiple endocrine neoplasia type 2B.The present study describes the case of a 54-year-old male with indolent DG, principally involving the small intestine and colon, associated with multiple schwannomas in the subserosa.A brief overview of intestinal ganglioneuromatous lesions and the associated conditions is additionally presented.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P.R. China.

ABSTRACT

Diffuse ganglioneuromatosis (DG) of the gastrointestinal tract is a rare condition that is closely associated with neurofibromatosis type 1 and multiple endocrine neoplasia type 2B. The occurrence of DG with multiple schwannomas, which, of the GI tract, usually affect the stomach, is considerably more rare. The present study describes the case of a 54-year-old male with indolent DG, principally involving the small intestine and colon, associated with multiple schwannomas in the subserosa. The patient was treated with surgery. A brief overview of intestinal ganglioneuromatous lesions and the associated conditions is additionally presented.

No MeSH data available.


Related in: MedlinePlus

Schematic overview of DG and schwannomas. Ganglioneuromatous polyposis is characterized by aggregates of ganglion cells and nerve fibers within the colonic mucosa, while DG can be mucosal or transmural with a diffuse, band-like enlargement of the nerve fibers and ganglion cells of the submucosal and myenteric plexuses. The present case combined these two patterns of histological change together with involvement from the lamina propria to the muscularis propria. At the same time, >20 nodules of schwannomas without capsules, ranging in diameter from 0.1 to 1.5 cm, were found in the subserosa of the colon.
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f4-etm-09-03-0733: Schematic overview of DG and schwannomas. Ganglioneuromatous polyposis is characterized by aggregates of ganglion cells and nerve fibers within the colonic mucosa, while DG can be mucosal or transmural with a diffuse, band-like enlargement of the nerve fibers and ganglion cells of the submucosal and myenteric plexuses. The present case combined these two patterns of histological change together with involvement from the lamina propria to the muscularis propria. At the same time, >20 nodules of schwannomas without capsules, ranging in diameter from 0.1 to 1.5 cm, were found in the subserosa of the colon.

Mentions: GP is characterized by aggregates of ganglion cells and nerve fibers within the colonic mucosa, while DG can be mucosal or transmural with a diffuse, band-like enlargement of nerve fibers and ganglion cells of the submucosal and myenteric plexuses and more pronounced changes in the latter (1,7). Notably, the present case appeared to combine the two patterns of histological change together with involvement from the lamina propria to the muscularis propria (Fig. 4). The condition was classified as DG due to its transmural growth pattern and giant polyps. The intramural growth pattern and florid hyperplasia of the submucosal or myenteric plexus are distinct to DG and often occur with NF 1 and the first manifestations of MEN 2B.


Synchronous diffuse ganglioneuromatosis and multiple schwannomas of the colon: A case report and literature review.

Lu C, Qiu Y, Lu X, Li G, Bu H - Exp Ther Med (2015)

Schematic overview of DG and schwannomas. Ganglioneuromatous polyposis is characterized by aggregates of ganglion cells and nerve fibers within the colonic mucosa, while DG can be mucosal or transmural with a diffuse, band-like enlargement of the nerve fibers and ganglion cells of the submucosal and myenteric plexuses. The present case combined these two patterns of histological change together with involvement from the lamina propria to the muscularis propria. At the same time, >20 nodules of schwannomas without capsules, ranging in diameter from 0.1 to 1.5 cm, were found in the subserosa of the colon.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4316863&req=5

f4-etm-09-03-0733: Schematic overview of DG and schwannomas. Ganglioneuromatous polyposis is characterized by aggregates of ganglion cells and nerve fibers within the colonic mucosa, while DG can be mucosal or transmural with a diffuse, band-like enlargement of the nerve fibers and ganglion cells of the submucosal and myenteric plexuses. The present case combined these two patterns of histological change together with involvement from the lamina propria to the muscularis propria. At the same time, >20 nodules of schwannomas without capsules, ranging in diameter from 0.1 to 1.5 cm, were found in the subserosa of the colon.
Mentions: GP is characterized by aggregates of ganglion cells and nerve fibers within the colonic mucosa, while DG can be mucosal or transmural with a diffuse, band-like enlargement of nerve fibers and ganglion cells of the submucosal and myenteric plexuses and more pronounced changes in the latter (1,7). Notably, the present case appeared to combine the two patterns of histological change together with involvement from the lamina propria to the muscularis propria (Fig. 4). The condition was classified as DG due to its transmural growth pattern and giant polyps. The intramural growth pattern and florid hyperplasia of the submucosal or myenteric plexus are distinct to DG and often occur with NF 1 and the first manifestations of MEN 2B.

Bottom Line: Diffuse ganglioneuromatosis (DG) of the gastrointestinal tract is a rare condition that is closely associated with neurofibromatosis type 1 and multiple endocrine neoplasia type 2B.The present study describes the case of a 54-year-old male with indolent DG, principally involving the small intestine and colon, associated with multiple schwannomas in the subserosa.A brief overview of intestinal ganglioneuromatous lesions and the associated conditions is additionally presented.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P.R. China.

ABSTRACT

Diffuse ganglioneuromatosis (DG) of the gastrointestinal tract is a rare condition that is closely associated with neurofibromatosis type 1 and multiple endocrine neoplasia type 2B. The occurrence of DG with multiple schwannomas, which, of the GI tract, usually affect the stomach, is considerably more rare. The present study describes the case of a 54-year-old male with indolent DG, principally involving the small intestine and colon, associated with multiple schwannomas in the subserosa. The patient was treated with surgery. A brief overview of intestinal ganglioneuromatous lesions and the associated conditions is additionally presented.

No MeSH data available.


Related in: MedlinePlus