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.