Gastrointestinal tract lymphomas: Rare types
Summary
Although, compared to carcinomas, the intestinal tract lymphomas are extremely rare, their incidence is increasing. This growth can be explained by an increase in the incidence of immunodeficiency-related disturbances, which are associated with an increase in the risk of developing gastrointestinal lymphomas. Gastrointestinal lymphomas are mainly large-cell diffuse B-type lymphomas and marginal-zone lymphomas. Despite the scarcity of other types of lymphoma such as mantle lymphomas, follicular lymphomas or T-lymphoma associated with enteropathy, the REAL classification as well as the recently published who classification, consider these lymphomas As separate entities based on their morphology, immunohistochemistry, cytogenetics and their clinical presentation.
Keywords
Helicobacter pylori Immunodeficiency lymphoma of Burkitt lymphomas of the marginal area diffuse type lymphoma to large cells B lymphoma of mantle cells follicular lymphoma T lymphomas associated with enteropathy celiac disease IBD Lymphomas Of the gastro-intestinal tract: Uncommon types
Summary
Although, in comparison to gastro-intestinal carcinomas, lymphomas of the gastrointestinal tract are extremely rare, the incidence of these tumours is rising. This rise may be explained by the increasing incidence of immunodeficiency disorders, as these disorders are associated with an increased risk for development of gastro-intestinal lymphomas. Gastro-intestinal lymphomas are largely diffuse large B-cell lymphomas and marginal zone cell lymphomas. Despite other types of lymphomas such as e.g. mantle cell lymphomas, follicular lymphomas or enteropathy-type T-cell lymphomas are uncommon, the REAL-classification as well as the recently published World Health Organization Classification of lymphoid Neoplasms consider these tumours as distinct entities, based on their morphology, immunohistochemistry, cytogenetics as well as on their different clinical presentation.
Lymphomas of the Gastro = intestinal tract: Uncommon types
RI ~ SUME
Although Compards to carcinomas, the intestinal tract lymphomas are extrfmement rare, their incidence is increasing. This
Growth can be explained by an increase in the incidence of disturbances li6es ~ immunod6ficience, they-m6mes associ6es h A
Increased risk of d6velopper of gastrointestinal lymphomas. Gastrointestinal lymphomas are mainly lymphomas
B Diffuse Type H large cells and lymphomas of the marginal zone. MALGR6 the Raret6 of other types of lymphoma such as
Mantle lymphomas, follicular lymphomas or T associ6s lymphomas has a ent6ropathie, the REAL classification as well as the
Classification publi6e r6cemment by I'OMS, consid6rent these lymphomas as distinct entit6s on the basis of their morphology,
Immunohistochemistry, Cytog6n6tique and their clinical pr6sentation.
SUMMARY
Although, in comparison to gastro-intestinal carcinomas, lymphomas of the gastro-intestinal tract are extremely rare, the incidence of these
Tumours is rising. This rise may be explained by the increasing incidence of immunodeficiency disorders, as these disorders are associated with an
Increased risk for development of gastro-intestinal lymphomas. Gastrointestinal lymphomas are largely diffuse large B-cell lymphomas and
Marginal zone cell lymphomas. Despite other types of lymphomas such as e.g. mantle cell lymphomas, follicular lymphomas or enteropathy-type
T-cell lymphomas are uncommon, the REAL-classification as well as the recently published World Health Organization Classification of
Lymphoid neoplasms Consider these tumours as distinct entities, based on their morphology, immunohistochemistry, cytogenetics as well as on
Their different clinical presentation.
Introd Introduction
Population 6tudes show an increase in
Of the incidence of gastrointestinal tract lymphomas
Which for the most part are lymphomas
Extraganglionnaires. This increase of about
50% could be the cons6quence of the Am61ioration
Diagnostics, particularly with regard to the
Concerns the pseudo lymphomas currently Red6finis
For most of them like lymphomas
of the marginal area. However, this increase
is also the r6sultat of the d6veloppement of
High grade lymphomas Associ6 h a immunod6ficience
[1-3]. Gastrointestinal lymphomas, D6finis
Like tumors lymphoid n6oplasiques
Confin6es to the digestive tract, constitute approximately
4% of non-Hodgkin lymphomas and
30% of Extraganglionnaires lymphomas [4]. The PR6
Valence of NonHodgkiniens Gastrointestinal lymphomas
varies depending on the location of the
Tumors with the highest percentages for the stomach
(55-65%), and a decrease in tr6quence to
As and? As we get closer to the distal pattie
of the intestine: 25 h 35% for the intestine gr ~ le, of
10 ~ 15% for C61on [5]. 5? At 10% of gastric tumors
and 30 ~ 50% of the tumors of the GR ~ have an origin
Lymphoid. These percentages are clearly
Sup6rieurs h those observ6s at the level of the 0esophage
Or C61on Oh the pr6valence is inf6rieure h i% [6].
In the past, the diagnosis of tract lymphomas
Gastrointestinal 6tait NETHERLANDS6 on the PROPOS6E classification
By Isaacs et al. [7]. R6cemment, this classification
A 6t6 remplac6e by the REAL classification
(1994) which considers the diff6rents types of lymphomas
As distinct clinical-pathological entit6s
Based on their morphology, the Ph6notype
Immunological, and their caract6ristiques g6n6-
Associ6es h of the separate clinical crits.
The purpose of this classification is to diff6rencier the
Types of lymphoma taking into account their 6quiTir6s
H part: D R Ann DRIESSEN, Department of Pathology, University Hospital Maastricht, P. Debyelaan 25, Postbus 5800, AZ6202
Maastricht (The Netherlands).
CL ~ s: Helicobacter pylori, immunodeficiency, Burkitt lymphoma, marginal zone lymphoma, diffuse lymphoma ~ t
Large B cells, mantle cell lymphoma, follicular lymphoma, T associ6s lymphoma H a ent6ropathie, celiac disease,
Ibd.
Key-Words: Burkitt lymphoma, coeliac disease, diffuse large B-cell lymphoma, enteropathy-type T-cell lymphoma, follicular lymphoma,
Helicobacter pylori, immunodeficiency, inflammatory bowel disease, mantle cell lymphoma, marginal zone cell lymphoma.
DIA GNOS TIC MORPHOL 0 iqm EU
Diff ~ Preferential diagnosis of lymphomas
Small B cells
In d6croissant order of Fr6quence, lymphomas
B H Small Gastrointestinal tract cells
The following are the lymphomas of the area
Marginal (20-50%), follicular lymphomas
(5-10%), mantle lymphomas (2-8%) and the
LLC (2%). C6t6 of these non-Hodgkin lymphomas
Primary, all types of ganglion lymphomas
Hodgkin can be observ6s
In the gastrointestinal tract, for example
The ganglion lymphomas of the mantle that are
Diss6min6s along the gastro-intestinal tract in 10
20% of patients [10-12]. Although the morphology
Of these diff6rents types of lymphomas B? A small cells
is well Ddfinie, the Diff6rentiel diagnosis can
Av6rer difficult because of the small size of the biopsy
Endoscopic. D6s during immunohistochemical analyses,
6ventuellement in association with the
Biology Mol6culaire, are n6cessaires 6tant donn6 the
Prognosis and the choice of treatment tr6s diff6rents of a
Type of lymphoma H the other. Previously, the 6tudes
Immunohistochemical n6cessitaient la cong61ation
of the fabric. Currently, several antibodies can be
~ Tre utilis6s on Mat6riel fix6 and enrob6? a paraffin.
An assortment of antibodies, cell markers
T and B, is n6cessaire to compare the composition
And the architecture of lymphomas ~ those of
Normal lymphoid cells belonging to MALT
(Tissue lymphoid associ6 to mucous membranes) and for
D6terminer The origin of lymphoid cells n6oplasiques
(Fig. 2) [13-15]. The distinction between these diff6-
Types of lymphoma B H small cells is
bas6e on the diff6rente expression of CD5, CD10,
CD23 and CD 43 (table II).
The lymphomas of the mantle can be d6velopper
D6part of the digestive tract but have a low tr6s
Pr6valence in the order of 2% [10, 12]. This tumor
Rare in middle-aged patients, mainly in the
males [16-19]. Abdominal pain
And diarrh6e are the clinical Sympt6mes
The most fr6quents. Although the tumor may
6tendre h The whole gastrointestinal tract,
With a diss6mination in the m6sent6 ganglia
Its 6picentre is most often localis6 in the
The R6gion il6o-C ~ East. The macroscopic examination
R6V61E of multiple lymphomateux polyps whose
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