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Liens et agrandissements sur : images et texte en bleu. Links - Zoom: pictures and highlighted text. |
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Patient psychiatrique de 55 ans, asthmatique, PMD depuis 30 ans sous traitement
( Anafranil, Tifranil, Seroxate ), chez qui on découvre une anémie
ferriprive il y a deux ans sur un bilan pour pneumonie.
Gastroscopie :
gastrite antrale rayon de roue. Lésions fundiques polypoïdes érodés.
A
noter que le patient est constipé depuis 30 ans, sans autres symptomatologie
digestive, avec apparition depuis deux mois de flush au visage,
survenant tous les 2-3 jours de 2-3h de durée. Une
gastrectomie totale est réalisée aprés confirmation tumorale biopsique. |
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Psychiatric patient
55 yo, with asthma treated, anemia for 2 years discovered during
a check-up for a pneumonia.
Gastroscopy discloses polypoid lesions
of the body, also noted on the CTScan.
The patient complained of constipation for 30 years, with no other digestive symptoms. Recently, facial flush every 2 to 3 days, for 2 to 3 hours. Total geatrectomy performed following biopsy confirmation. |
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La pièce de gastrectomie montre une muqueuse
fundique rugueuse, avec élargissement des plis, et la présence
de multiples nodules, indurés parfois érodés, variant de 0,7 1 cm de diamètre. |
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The gastrectomy specimen shows a granular
irregular mucosal surface, with enlargement of its foldings,
and the presence of multiple indurated
nodules, some eroded, varying from 0,7 to 1 cm in size. |
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La coupe incluant
plusieurs nodules montre la localisation
muqueuse et l'extension sous-muqueuse d'une prolifération cellulaire. Cette dernière est forme de cordons et de travées grèles, anastomotiques,
de cellules cuboïdes. Elles possèdent un noyau
arrondi faiblement nucléolé. |
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The section including multiple nodules demonstrates a neoplasm invading an eroded
mucosa, and extending superficially in the sub-mucosa.
The neoplasm is formed by anastomotic
thin cords of cuboid cells, with mild nuclear atypia. |
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Un autre nodule, érodant galement la muqueuse qui
montre quelques glandes dysplasiques, arbore une architecture
plus compacte, endocrinoïde, et s'accompagne d'un infiltrat
lymphocytaire périphérique, s'intriquant avec
des cordons cellulaires tumoraux. |
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Another nodule eroding the mucosa which shows rare
atypical glands, demonstrates a more dense, endocrinoid
pattern, surrounded by a heavy lymphoid
infiltrate. The latter is engulfed by cords
of tumor cells. |
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La muqueuse péri-tumorale est le siège de modification gastritique chronique atrophique de
type A, ou l'on observe une hyperplasie
diffuse des cellules neuro-endocrines, variable d'intensité,
et mise en évidence par l'immunomarquage la Chromogranine
A. Cette dernière est également positive dans les cellules tumorales. |
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The mucosa of the body of the stomach discloses
changes typical of Chronic Atrophic Gastritis,
type A . Immunohistochemistry demonstrates diffuse
hyperplasia of neuro-endocrine cells varying in intensity. The
marker (Chromogranin A) is also
positive in the tumor cells. |
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Diagnostic proposé
Tumeurs carcinoïdes multiples, bien différenciées, infiltrant la sous-muqueuse
dans un contexte de gastrite chronique atrophique, modérée sévère,
avec hyperplasie neuro-endocrine diffuse. |
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Proposed diagnosis
Multiple
carcinoid tumors, mucosal, in a context of chronic atrophic gastritis,
moderate to severe, with diffuse neuro-endocrine hyperplasia. |
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CARCINOID TUMORS
(ref, Ackerman's Surgical Pathology, 8th. Ed)
Grossly, gastric carcinoid tumors tend to be
small, sharply outlined, and covered by a flattened mucosa. They
may also have the appearance of gastric polyps.
Microscopically,
the predominant pattern of growth may be microglandular, trabecular,
orrarelyinsular. The nuclei are regular and normochromatic, mitoses
are scanty, necrosis is usually absent, and vascularization is florid.
Exceptionally, the cytoplasm has a clear appearance. Focal mucin
positivity may be present. Immunohistochemically, there is consistent
positivity for neuron-specific enolase, chromogranin, and keratin.
Ultrastructurally, dense-core secretory granules are found in the
cytoplasm, usually in abundance.
The rate of growth of these tumors
is very slow. Metastases are usually restricted to regional lymph
nodes and liver, and their presence does not rule out long-term
survival.It should be realized that carcinoid tumor is a generic term, which can be properly applied to any neoplasm
comprising any of several types of endocrine cells known to be present
in the normal human stomach.
This histogenetic diversity is already
suggested by the variety of products that these tumors have been
found to secrete, such as 5-hydroxytryptophan, serotonin, epinephrine,
norepinephrine, somatostatin, VIP, PP, YY peptide, ACTH, beta-MSH,
and alpha-1-antitrypsin.A combined morphologic, ultrastructural, and
immunohistochemical approach has allowed the identification of at
least two definite subtypes of gastric carcinoid.
The first is a
tumor composed of G-cells (gastrinoma), analogous
to its most common pancreatic or duodenal counterpart; this is usually
solitary, located in the antrum, non-argentaffin and non-argyrophilic,
immunoreactive for gastrin, and sometimes associated with peptic
ulcer. Parenthetically, gastric G-cell tumor should be clearly separated
from the disputed entity of primary G-cell hyperplasia, in which an increased number of G-cells in the antrum is accompanied
by hypergastrinemia and an ulcerogenic syndrome resembling the Zollinger-Ellison
syndrome.
The second form, which constitutes by far the
most common type of gastric carcinoid tumor, is composed of enterochromaffin-like
(ECL) cells. These lesions are usually multiple, often polypoid,
and distributed throughout the fundus. They are non-argentaffin
but strongly argyrophilic, nonreactive for any of the standard gastroduodenopancreatic
hormones, and accompanied by diffuse hyperplasia of similar argyrophilic
cells in the surrounding mucosa. Various types of smooth muscle
proliferation may be present, possibly as a response to the production
of basic fibroblastic growth factor. These tumors and hyperplasias
usually occur on a background of atrophic gastritis with intestinal
metaplasia, with or without pernicious anemia. The gastritis is
usually of type A (immune) with a low incidence of H. pylori colonization. The tumors have also been seen in association with
the Zollinger-Ellison syndrome, either alone or as a component of
multiple endocrine neoplasia. Since the common denominator for these
disorders is hypergastrinemia and since gastrin is known to have
a trophic influence on ECL cells, it has been postulated that these
tumors are the direct result of a continuous stimulation by gastrin
against a predisposing genetic background. Regression of these lesions
has been documented following the performance of antrectomy and
normalization of serum gastrin levels, suggesting that some of them
may represent nodular hyperplasias of ECL cells rather than true
neoplasms. Indeed, an almost continuous spectrum exists between
the clearly hyperplastic and the clearly neoplastic proliferation,
making the dividing line between the two processes an arbitrary
one.
The figure of 0.5 mm has been suggested as the dividing line
between carcinoid tumors and their precursors ("precarcinoid").Solitary carcinoid tumors composed of ECL-like
cells also occur; those associated with non-atrophic mucosa tend
to run a more aggressive course.Although no peptide secretory product has yet
been identified in ECL tumor cells, it is known that they store
histamine and that histamine-producing carcinoid tumors composed
of argyrophilic ECL-like cells are very common in the stomach of
the South African rodent, Mastomys (Praomys) natalensis.Carcinoid tumors with atypical histologic features
and adenocarcinomas with focal endocrine features are discussed
in the section on gastric carcinoma.
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