RAMB - Julho 2016

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2016; 62(6):485-489

lute the barium-based contrast, therefore limiting the

analysis of the mucosal lining.

2,3,7,11

USG is an affordable and non-invasive examination,

which can be used both in the initial investigation and to

assess disease progression. USG shows five layers of the

gastric wall: hyperechogenic layer (mucosa), hypoecho-

genic layer (muscularis mucosa), hyperechogenic layer

(submucosal), hypoechogenic layer (the muscularis pro-

pria), and another hyperechogenic layer (subserosal, se-

rous, and interface).

On CT scans, the enlarged folds protrude into the

gastric lumen and distort the mucosal surface. In gener-

al, the serosal surface remains smooth and the gastric

wall between folds remains normal or only slightly thick-

ened. After intravenous administration of contrast me-

dium, hyperenhancement of the folds is noted. CT find-

ings generally correspond to those of the UGE. Thus, TC,

less invasive and generally less costly and more affordable

than UGE, can be useful not only for diagnosis but also

for follow-up of patients with Ménétrier’s disease.

2,3,11

On UGE, the gastric mucosa shows marked irregu-

lar thickening, edema, and spongiform and tortuous ap-

pearance, with the folds resembling the brain. Gastric pH

is generally alkaline. Endoscopic ultrasound has been

used to evaluate gastric thickening. Five layers can be

identified in a normal endoscopic ultrasound, correspond-

ing to the mucosa, muscularis mucosa, submucosa, mus-

cularis propria, and serosa. Studies show that in Méné-

trier’s disease only the second layer (muscularis mucosa)

is thickened. The mucosa also shows increased echogenici-

ty, while the submucosa and the muscularis propria re-

main normal. Endoscopic ultrasound is useful in Méné-

trier’s disease to evaluate differential diagnoses to causes

of diffuse hypertrophy of the gastric folds, and especial-

ly due to the possibility to perform a secure biopsy.

2,3,12

Scintigraphy can help, indicating protein loss from

the stomach.

However, a certain and definitive diagno-

sis cannot be made, requiring confirmation by histolog-

ical analysis.

Histologically, hypertrophy of the gastric folds, more

evident in the body and fundus of the stomach in patients

withMénétrier’s disease, presents massive expansion of the

layer of mucous cells (foveolar hyperplasia), with a reduc-

tion in parietal cells, causing reduction of acid secretion.

Although in some cases the parietal cells are preserved, in

the vast majority there is a reduction in the number of these

cells specialized in acid secretion in the stomach.

It is im-

portant to note that, apparently, there are no inflammato-

ry cells, intestinal metaplasia or dysplasia.

2,7,8

The totality of clinical, imaging, laboratory, and his-

tological findings help differentiate Ménétrier’s disease

from other entities.

Therapeutic techniques include anticholinergic drugs,

prostaglandins, proton pump inhibitors, prednisone, and

histamine blockers, associated with a high-protein diet.

All treatments produced variable results.

It is known that Ménétrier’s disease increases the risk

for gastric cancer around 2 to 15% over a lifetime, but the

magnitude of this risk is uncertain because of the rarity

of cases. The exact risk of developing gastric cancer is un-

known and there is no consensus regarding screening

with UGE.

2,8,9

Nevertheless, CT findings are equivalent to

those of UGE for Ménétrier’s disease in children. There-

fore, CT, a less invasive examination with less costs and

more affordable than UGE, can be as useful as the UGE

for the follow-up of these patients.

Malignant diseases of the gastrointestinal tract are

very rare in children, accounting for approximately 1.2%

of all malignancies. Gastric adenocarcinoma (GAC) is ex-

tremely rare in children, accounting for about 0.5% of all

malignancies of the gastrointestinal tract.

13,14

It usually

affects patients between 50 and 70 years, and is uncom-

mon before the age of 40. The etiology of GAC is multi-

factorial, and risk factors include alcohol consumption,

smoking, high-sodium diet, few vegetables, foods with

nitrous components, and infections such as Epstein-Barr

and

H. pylori

. But all of these factors are unknown in chil-

dren, and only recently it was related to mutations in the

E-cadherin gene (CDH-1). GAC appears in children spo-

radically as a consequence of inherited syndromes or af-

ter treatment of gastric lymphomas.

13,14

Differential diagnoses for pediatric gastric tumors in-

clude stromal tumors, hemangioma, lymphoma, squamous

cell carcinoma, carcinoid tumors, fibroids, polyps from he-

reditary syndromes, leiomyosarcoma, and lipoma.

13,14

Symptoms vary according to the site of involvement

and the extent of the tumor. Tumors located in the cardia

lead to dysphagic symptoms, while tumors located distal

to the cardia gastric produce nonspecific symptoms such

as abdominal pain, loss of appetite, weight loss, vomiting,

anorexia, fatigue, bloating, hematemesis, and melena.

13-15

Barium-contrast radiography can be used for initial in-

vestigation as an alternative in locations without access to

more complex tests. The main findings are polyps, ulcers,

discontinuation and thickening of the folds, areas of bari-

umfilling defect, loss of gastric distension, and stenoses.

16,17

USG examination is easily accessible, inexpensive,

and does not use ionizing radiation, but it is operator-