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Ménétrier’ s disease associated with gastric adenocarcinoma in a child – imaging aspect
Rev Assoc Med B ras 2016; 62(6):485-489 485
IMAGE IN MEDICINE
Ménétrier’s disease associated with gastric adenocarcinoma
in a child – imaging aspect
Pedro Henrique Ramos Quintino da Silva1, P aula Rigo 2, Renan Pedroso Batista3, Ricardo Katsuya T oma4,
Luiz Antonio Nunes de Oliveira5, Lisa Suzuki6*
1Resident Radiologist, Instituto de Radiologia (INRAD), Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo (HC-FMUSP), São Paulo, SP, Brazil
2Resident Gastroenterologist, Instituto da Criança, HC-FMUSP, São Paulo, SP, Brazil
3Resident Pathologist, HC-FMUSP, São Paulo, SP, Brazil
4PhD in Medicine from Universidade Federal de São Paulo. Head of the Service of Gastroenterology at Instituto da Criança, HC-FMUSP, São Paulo, SP, Brazil
5Assistant Physician, Service of Radiology, Instituto da Criança, HC-FMUSP, São Paulo, SP, Brazil
6PhD in Medicine from FMUSP. Head of the Service of Radiology at Instituto da Criança, HC-FMUSP, São Paulo, SP, Brazil
Study conducted at Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo (HC-FMUSP), São Paulo, SP, Brazil
Article received: 6/29/2016
Accepted for publication: 7/26/2016
*Correspondence:
Instituto da Criança
Address: Av. Dr. Enéas de Carvalho Aguiar, 647
São Paulo, SP – Brazil
Postal code: 05403-000
Case report
Female patient aged 18 years and 8 months, with diagno-
sis of hypertrophic gastritis since the age of 10, when she
started having sporadic vomiting, weight loss, and ane-
mia (Hb = 5.0 at diagnosis in August 2007).
On her first upper gastrointestinal endoscopy (UGE)
performed at the Instituto da Criança (ICr) at HC-FMUSP,
in August 2007, it was seem globally hypertrophied and
swollen gastric mucosa, with an infiltrative lesion with
ill-defined margins. A biopsy confirmed hyperplastic gas-
tritis with a pattern of Ménétrier’s disease.
Outpatient follow-up was initiated at Pediatric Gas-
troenterology Service of ICr in January 2008, with peri-
odic clinical evaluation and UGE. The patient progressed
in the following months with poor appetite and epi-
sodes of upper gastrointestinal bleeding and melena, re-
quiring hospitalization in the city of origin and trans-
fusions of blood concentrates. In the following years,
she showed less recurrent episodes of bleeding than as
seen in the first years after diagnosis, but continued un-
dergoing serial UGEs for disease control and malignant
surveillance, maintaining the same macroscopic and mi-
croscopic pattern. The patient has been submitted to
extensive research for Helicobacter pylori in gastric biop-
sies, all with negative results, the same occurring with
immunophenotyping for cytomegalovirus (CMV). Clin-
ically, she continued to show poor weight gain, but with
proper growth and no generalized edema at any time.
In 2009, a computed tomography (CT) with intrave-
nous contrast of the abdomen was performed, which showed
marked diffuse and symmetric thickening of the gastric
folds, with no evidence of nodular lesions (Figure 1).
In 2015, a control UGE with biopsies showed she
maintained the macroscopic appearance of intense hy-
pertrophy of gastric folds in the body and fundus with
a reddish pseudotumoral appearance, uneven and mul-
tilobulated surface. In the pathological examination,
signs of malignancy were found, a characteristic of mod-
erately differentiated invasive adenocarcinoma, of no
special type, negative for H. pylori (Figure 2). Two het-
erogeneous vegetative lesions were found (Figure 3) on
a new CT scan.
The patient underwent total gastrectomy in 2015,
with no need for adjuvant therapy.
Discussion
Ménétrier’s disease is considered rare by the Office of Rare
Diseases of the National Institutes of Health, which means
a prevalence of less than one in 200,000 individuals.2 The
disease was first described in 1888 by French pathologist
Pierre Ménétrier (1859–1935).1 It is a chronic and rare ac-
quired gastric disease of unknown cause, but has been as-
sociated with some gastric diseases, including bacterial
and viral infections: cultures and biopsies have shown an
association with CMV, H. pylori, herpes simplex, and My-
coplasma pneumoniae.3