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  • Discussion Mantle cell lymphoma is

    2019-06-24

    Discussion Mantle cell lymphoma is a distinct type of mature B-cell neoplasm that accounts for approximately 3–10% of non-Hodgkin\'s lymphoma. It is referred to several aliases, such as lymphocytic lymphoma of intermediate differentiation, mantle zone lymphoma, centrocytic lymphoma. Diffusion of nodular intermediate lymphocytic lymphoma tends to occur in middle-aged to older individuals with a higher incidence in males. Patients usually present with stage III or IV disease, and more than third-quarter of them have extranodal involvement, such as spleen, bone marrow, and gastrointestinal (GI) tract. Multiple lymphoid polyposis (MLP) was first described by Cornes et al. as a distinctive pattern of GI tract involvement as several long intestinal segments are infiltrated by white nodular or polypoid tumors. Most patients with MLP will have MCL, but this presentation may also occur in follicular lymphoma, marginal zone lymphoma, adult T-cell leukemia/lymphoma, and angioimmunoblastic T-cell lymphoma. The immunophenotype of the MCL cells is typically CD20 (+), CD5 (+), CD10 (−), CD23 (−), and cyclin-D1 (+) (also known as CCND1, BCL-1, B-cell lymphoma 1). Cyclin-D1 was due to a translocation of the cyclin-D1 gene on 11q13 to the promoter of the immunoglobulin heavy chain on 14q32. Overexpression of cyclin-D1 can be detected in the tumor cells by fluorescence in situ hybridization (FISH) and had been suggested as the highly specific marker of MCL. In various series, GI tract involvement may be seen in 10%–30% MCL cases, however, two recent prospective reports showed a much higher rate. The frequency of upper GI microscopic involvement was 43% and 77%, while lower GI microscopic involvement was 77% and 88%. Antonio et al. reported 92% of MCL patients had upper or lower GI tract infiltration in biopsy specimen while 63% had unremarkable gastroscopic examination and 71% had normal colonscopic appearance with microscopic evidence of MCL under biopsy. The ileocecal region is most commonly involved area, whereas the esophagus and JNJ-38877605 are rarely affected. Multiple lymphomatous polyposis was commonly seen in MCL patients during endoscopic examination of the gastrointestinal tract, however, it may also present endoscopically as inflammation, ulceration, mucosal thickening, and tumoral masses. Chung et al. reported MCL patients involving gastrointestinal tract presented bowel wall thickening or mass formation during computed tomography scan. Intussusception is rare in adults, and neoplasm usually presents as a lead point in most cases. Malignant adenocarcinoma, metastatic tumor, and benign neoplasms as lipoma or Peutz-Jegher adenoma were the most common causes of intussusception. Malignant lymphoma is an uncommon cause of intussusception and accounts for less than 1% of cases. To date, only four cases of intussusception have been reported as disease sequelae in previously diagnosed MCL patients, and three of these four patients were reported as a complication after chemotherapy. One patient had an intestinal lipoma as a lead point, which is irrelevant to the underlying disease. In this case, the unique point is that intussusception related abdominal pain was the initial presentation of MCL without other detectable lymphadenopathy during thorough examination. The whole segment of ileum, ileocecal valve, cecum, ascending colon, appendix and even gallbladder, have at least a microscopic level of disease. It also involved the regional lymph nodes. The evidence supported our hypothesis that the origin site was the intestinal mucosa. Gallbladder lymphoma or extrahepatic bile duct lymphoma presenting with symptoms of cholecystitis, cholelithiasis or jaundice are exceedingly rare. Only one case regarding metastatic MCL in the gallbladder was found in the literature. Haresh et al. reported 14 cases of gallbladder and extrahepatic bile duct lymphoma and reviewed an additional 52 cases in the literature. Extranodal marginal zone lymphoma was the most common lymphoma type. Other miscellaneous types include diffuse large B cell lymphoma, B-lymphoblastic lymphoma and follicular lymphoma, classical Hodgkin lymphoma, etc. In our case, the lesion at the tip of the gallbladder fundus was an accidental finding, and microscopically, the lymphoma cells involved JNJ-38877605 both subserosal lymph node and the gallbladder submucosal layer. The findings were compatible with previous reports as a microscopic invasion in macroscopical normal gallbladder mucosa.