5 -6 cm 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 B a c k g r o u n d 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 - 4 th to 6 th decade - male predilection- over expression of TGF- alpha- 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. M i c r o s c o p y C a s e H i s t o r 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 1b D i s c u s s i o n & C o n c l u s i o n 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. 1a R e f e r e n c e s 1c 1. Rich A, Zuluaga T. Distinguishing Menetriers disease from its mimics. Gut 2010; 59 : 1617 - 1624. Enlarged stomach showing markedly hypertrophied mucosal folds with thickened wall ( 5-6 cm) and antral sparing (arrow ) . 2. Brzacki V, Dragan M.Menetrier Disease – Case Report. Acta Fac. Med. Naiss. 2004; 21 (2):101-105.

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5-6

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

B a c k g r o u n d

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 - 4

th to 6

th decade - male predilection- over expression of TGF-

alpha- 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.

M i c r o s c o p y

C a s e H i s t o r 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

D i s c u s s i o n & C o n c l u s i o n

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.

1a R e f e r e n c e s 1c

1. Rich A, Zuluaga T. Distinguishing Menetriers disease from its mimics. Gut 2010; 59 : 1617 - 1624.

Enlarged stomach showing markedly hypertrophied mucosal folds with thickened wall ( 5-6 cm) and antral sparing (arrow ) .

2. Brzacki V, Dragan M.Menetrier Disease – Case Report. Acta Fac. Med. Naiss. 2004; 21 (2):101-105.