Menetriers Disease : Often Heard But Seldom Seen !

Menetriers Disease : Often Heard
But Seldom Seen !
Authors : Shilpa Mishra,Rachana Chaturvedi,Maithili Gangurde ,Amita Joshi
Institution : Seth G.S. Medical College & K.E.M. Hospital, Parel, Mumbai
Background
Microscopy
An acquired premalignant disorder of unknown etiology, characterized by
giant hypertrophic rugal folds that involve the fundus but often spare the
antrum.
Listed by the Office of rare disease of the National institute of Health (USA),
indicating a prevalence of less than 1 in 200000 people.
Occurs in two forms
Childhood form - linked to CMV infection - resolves spontaneously.
Adult form - 4th to 6th decade - male predilection- over expression of TGFalpha- associated with H.Pylori infection.
Common symptoms - upper abdominal pain, nausea & vomiting. Other
symptoms - weight loss & bleeding due to erosions, diarrhoea, edema due
to excess mucus secretion & hypoproteinemia associated with
hypochlorhydria.
Case Histor y
A 50 year old male presented with a history of pain
in the epigastric region since 2 years
intermittent nausea/ vomiting since 1 ½ months.
On Investigations- hypoproteinemia & anemia
Endoscopy
Thickened mucosa with foveolar hyperplasia, cystic dilatation associated with
prominent eosinophils in lamina propria.
Thickened rugal folds from GE junction to fundus and body, sparing the antrum
Clinical impression : ? Menetriers disease, ? Infiltrative disorders
CECT
Elongated foveolae with characteristic “cork screw appearance”
& atrophic glands.
Stomach showing hypertrophied rugal folds resembling sulci and gyri of cerebrum.
Total Gastrectomy Specimen
5-6 cm
1b
Discussion & Conclusion
Diagnosis of Menetriers disease is based on characteristic gross &
microscopic features.
All classical features were seen in our case, however it was negative for H.
pylori
Differential diagnosis:
Zollinger Ellison syndrome
Infiltrative disorders
Hyperplastic polyps/ Polyposis syndrome
Treatment options:
High protein diet, albumins, plasma, diuretics, anticholinergic, PPI &
Monoclonal antibodies to EGFR ( Octreotide, Cetuximab)
Gastrectomy: Partial/ total
Indications for surgery: Dysplasia, Malignancy, High loss of proteins
and Recurrent bleeding.
Associated with increased risk of gastric adenocarcinoma (10-15%).
Adequate sampling with proper follow up is essential.
Menetriers disease is very rare in India. Exact incidence not known. Only
few case reports available, documenting association with trichobezoar and
primary pachydermoperiostosis.
References
1a
1c
Enlarged stomach showing markedly hypertrophied mucosal folds with
thickened wall ( 5-6 cm) and antral sparing (arrow ) .
1. Rich A, Zuluaga T. Distinguishing Menetriers disease from its mimics. Gut 2010; 59 :
1617 - 1624.
2. Brzacki V, Dragan M.Menetrier Disease – Case Report. Acta Fac. Med. Naiss. 2004;
21 (2):101-105.