1. INTRODUCTION
Multiple primary malignant tumors are defined as the detection of two or
more malignancies in an individual person [1,2] In particular,
synchronous primary malignancy is extremely rare [2-5]. The GI tract
is the most common site of extra-nodal lymphoma involvement. Primary GIL
is a heterogeneous entity and constitutes approximately 10-15% of all
NHL and estimates 30%-40% of all extra-nodal lymphomas [2,6,7].
Primary GIL accounts for 1% to 10% of all GI malignancies and up to
90% of which are B-cell non-Hodgkin lymphoma (NHL) [2.4.8.9]. In
majority of studies investigate that stomach is the commonly affected
site followed by small intestine. Lymphoma of the small intestine is the
most common part of the ileum, followed by the jejunum and duodenum
[10]. The most common identification site is the ileocolon region
[1,2,5,8,11-14]. The vast majority of GILs are NHLs, DLBCL,
mucosa-associated lymphoid tissue lymphomas (MALTomas), although Hodgkin
lymphoma about one-third of NHL has been reported. DLBCL is a
heterogeneous entity rarely causing acute bowel obstructive symptoms and
intussusceptions.2-4 Synchronous primary DLBCLs of the small intestine
is extremely rare and requires careful diagnosis is suspected [2,8].
Adult intussusception accounts for 5% of all intussusceptions. About
90% of cases of intussusception in children arise from unknown causes
[4,15], which include infections, anatomical factors and altered
motility. Only 10% of which is secondary to specific pathological
lesions such as Meckel’s diverticulum, polyps, and benign or malignant
tumor [1,8]. The most small bowel lead points are benign lesions,
and malignant lesions account for 30% of intussusception cases
[1-4].
Obstruction and perforation are rare and life-threatening complications
of GIL. Multiple intestinal intussusception caused by synchronous DLBCLs
of the jejunum is extremely rare [1,8,16,17]. Secondary lymphoma
pleural involvement (secondary pleural lymphoma) is common and occurs in
about 20% of lymphomas. Primary GI-DLBCL initially presenting with
pleural effusion [15], or with intestinal perforation is very
uncommon [4,6,16,18]. To our knowledge, we firstly report an 85-year
old male concerns an extremely rare manifestation of synchronous primary
DLBCLs of the jejunum with unexplained pleural effusion and
disseminating multiple lymph nodal involvement presenting as JJ
intussusception in clinically. The purpose of this report is to remind
clinicians that lymphoma should be considered when assessing the cause
of unexplained pleural effusion, especially when the available
examination data cannot be confirmed by clinical manifestations. To our
knowledge, this is the first confirmed case of synchronous primary
DLBCLs of the jejunum masquerading as JJ intussusception with lymph
nodal and pleural metastasis. Learning aims of this case report, we can
understand that its clinical manifestations, morphological
characteristics, immunophenotypes and molecular biological
characteristics are very different and important. It is indeed the
biggest challenge before surgery and is not to be ignored.