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A Case of Multiple Endocrine Neoplasia Type 2B and Gangliomatosis of Gastrointestinal Tract.

Shahnazari B, Aghamaleki A, Larijani B, Mohajeri Tehrani MR, Rafati H, Babamahmoodi A - Case Rep Med (2012)

Bottom Line: The underlying problem for all the MEN syndromes is failure of a tumour suppressor gene.The reported patient is an 18-year-old girl presented with long-term diarrhea and enterocutaneous fistula.Her thyroid nodules, marfanoid habitus and bumpy lips, were also highly suggestive for MEN2B.

View Article: PubMed Central - PubMed

Affiliation: Endocrinology and Metabolism Research Institute, Tehran University of Medical Sciences, Tehran, Iran.

ABSTRACT
Multiple endocrine neoplasia type 2 (MEN2) is a rare familial syndrome caused by mutations in the RET protooncogene and it is transmitted as an autosomal dominant trait. The underlying problem for all the MEN syndromes is failure of a tumour suppressor gene. The genetic defect in MEN2 is on chromosome 10 (10q11.2) and has also been identified both for MEN2A and MEN2B. The reported patient is an 18-year-old girl presented with long-term diarrhea and enterocutaneous fistula. Her thyroid nodules, marfanoid habitus and bumpy lips, were also highly suggestive for MEN2B.

No MeSH data available.


Related in: MedlinePlus

Spiral abdominopelvic CT scan (with contrast). The report was as follows. Multiple calcified and noncalcified lesions in liver are seen (metastasis should be considered). Some of the small bowel loops have thickened wall. Mild right side hydronephrosis is present. Anterior abdominal wall fistula is depicted. A few small paraaortic lymphnodes are seen. Mild left side pleural effusion and massive ascites were also noted.
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fig4: Spiral abdominopelvic CT scan (with contrast). The report was as follows. Multiple calcified and noncalcified lesions in liver are seen (metastasis should be considered). Some of the small bowel loops have thickened wall. Mild right side hydronephrosis is present. Anterior abdominal wall fistula is depicted. A few small paraaortic lymphnodes are seen. Mild left side pleural effusion and massive ascites were also noted.

Mentions: Abdominal CT scan revealed abscess formation in the site of previous surgery. Multiple calcified and noncalcified lesions were seen in the liver, suggestive of metastasis (Figure 4).


A Case of Multiple Endocrine Neoplasia Type 2B and Gangliomatosis of Gastrointestinal Tract.

Shahnazari B, Aghamaleki A, Larijani B, Mohajeri Tehrani MR, Rafati H, Babamahmoodi A - Case Rep Med (2012)

Spiral abdominopelvic CT scan (with contrast). The report was as follows. Multiple calcified and noncalcified lesions in liver are seen (metastasis should be considered). Some of the small bowel loops have thickened wall. Mild right side hydronephrosis is present. Anterior abdominal wall fistula is depicted. A few small paraaortic lymphnodes are seen. Mild left side pleural effusion and massive ascites were also noted.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3472613&req=5

fig4: Spiral abdominopelvic CT scan (with contrast). The report was as follows. Multiple calcified and noncalcified lesions in liver are seen (metastasis should be considered). Some of the small bowel loops have thickened wall. Mild right side hydronephrosis is present. Anterior abdominal wall fistula is depicted. A few small paraaortic lymphnodes are seen. Mild left side pleural effusion and massive ascites were also noted.
Mentions: Abdominal CT scan revealed abscess formation in the site of previous surgery. Multiple calcified and noncalcified lesions were seen in the liver, suggestive of metastasis (Figure 4).

Bottom Line: The underlying problem for all the MEN syndromes is failure of a tumour suppressor gene.The reported patient is an 18-year-old girl presented with long-term diarrhea and enterocutaneous fistula.Her thyroid nodules, marfanoid habitus and bumpy lips, were also highly suggestive for MEN2B.

View Article: PubMed Central - PubMed

Affiliation: Endocrinology and Metabolism Research Institute, Tehran University of Medical Sciences, Tehran, Iran.

ABSTRACT
Multiple endocrine neoplasia type 2 (MEN2) is a rare familial syndrome caused by mutations in the RET protooncogene and it is transmitted as an autosomal dominant trait. The underlying problem for all the MEN syndromes is failure of a tumour suppressor gene. The genetic defect in MEN2 is on chromosome 10 (10q11.2) and has also been identified both for MEN2A and MEN2B. The reported patient is an 18-year-old girl presented with long-term diarrhea and enterocutaneous fistula. Her thyroid nodules, marfanoid habitus and bumpy lips, were also highly suggestive for MEN2B.

No MeSH data available.


Related in: MedlinePlus