Multiple Lymphomatous Polyposis Research Articles

Colonic lymphomatous polyposis mantle cell lymphoma: a case report and review of literature

IntroductionMantle cell lymphoma is a rare lymphoma of the gastrointestinal tract that may present as multiple lymphomatous polyposis. We report a case of lymphomatous polyposis with a review of the literature.Case reportA 56-year-old man of Black ethnicity and Ivorian nationality with no relevant past medical history, consulted for a sudden onset symptoms of gastrointestinal obstruction, which evolved over 2 days. Macroscopic examination revealed the presence of multiple polyploid formations of the colonic mucosa. Histology showed diffuse lymphomatous proliferation of submucosa consisting off small lymphoid cells with a hyperchromatic crenelated nucleus, suggesting lymphomatous polyposis. Immunohistochemical examination showed expression by the tumor cells of antibodies to CD20, CD5, Bcl2, and cyclin D1. They did not express antibodies to CD10 and CD23. The Ki67 proliferation index was 25%. We have thus retained the diagnosis of mantle cell lymphomatous polyposis.ConclusionMultiple lymphomatous polyposis is a rare entity characterized by the presence of numerous gastrointestinal polyploid lesions sometimes involving several segments of the gastrointestinal tract. Typical lymphoma presenting as lymphomatous polyposis is mantle cell lymphoma; although, other tumors may have this aspect.

S2509 Mantle Cell Lymphoma Presenting as Multiple Lymphomatous Polyposis: A Case Report

Introduction: Mantle Cell Lymphoma (MCL) typically presents with extensive lymphadenopathy, fevers, night sweats and unintentional weight loss. However, Multiple Lymphomatous Polyposis (MLP), MCL arising from the gastrointestinal tract, is an aggressive malignancy and is infrequently described. Case Description/Methods: A 68-year-old White man with atrial fibrillation and hypertension presented to the hospital with watery diarrhea, fatigue and intermittent epigastric pain for the past several weeks. He denied melena, hematochezia, heartburn, dysphagia, odynophagia, weight loss, loss of appetite, NSAID use, recent travel or sick contacts. There was no family history of gastrointestinal malignancy. No history of prior endoscopic evaluation. Physical exam was notable for brown stool on rectal exam. Further evaluation revealed Hgb 6.4, MCV 60.9, iron 43, TIBC 442 and iron saturation 10% consistent with iron deficiency anemia. Upper endoscopy was normal. Colonoscopy showed 8 6-16 mm polyps scattered throughout the colon, all of which were resected completely. Analysis showed a range of histology – 3 tubular adenomas, 1 serrated and 2 hyperplastic polyps. However, histology of 4 polypoid appearing lesions (Figure 1A) showed prominent nodular atypical submucosal lymphoid aggregates (Figure 1B) that were positive for CD20, cyclin D1, weak CD5, and negative for CD10 and CD23. This was consistent with the diagnosis of MCL. CT imaging showed diffuse lymphadenopathy, including bulky disease with a 16.6 x 10.6 cm small bowel mesenteric mass (Figure 1C), along with proximal small bowel and terminal ileal thickening. He was induced with 6 cycles of bendamustine-rituximab combination therapy and maintained on rituximab. A re-staging scan at 27 months showed no evidence of disease progression. Discussion: We present a case of MCL presenting as MLP with symptoms of anemia, diarrhea and epigastric pain. MCL is a rare B-cell non-Hodgkin's lymphoma that portends a poor prognosis, making early identification and diagnosis critical. Obstruction, GI bleeding, and perforation are common complications for MCL presenting as MLP. It is important to keep MLP on the differential when multiple small nodular or polypoid lesions are identified on colonoscopy. All different types of polyps should be resected or sampled during colonoscopy, and each evaluated by the pathologist to avoid missing clinically significant conditions. Early diagnosis is key to prevent morbidity and mortality in MLP as described in our case.Figure 1.: (A) polypoid lesion (B) nodular atypical submucosal lymphoid aggregates (C) 16.6 x 10.6 cm small bowel mesenteric mass.

S2063 Mantle Cell Lymphoma Presenting as Multiple Lymphomatous Polyposis of the Colon

Introduction: Mantle Cell Lymphoma (MCL), a type of non-Hodgkin lymphoma (NHL) is an aggressive neoplasm affecting the lymphatic system. Its incidence is 1–2 cases/105 people/year and accounts for only 5-6% of all cases of NHL. Multiple lymphomatous polyposis (MLP) is a rare presentation seen in MCL consisting of diffuse nodules or polyps covering multiple sites of the gastrointestinal tract. Case Description/Methods: A 68-year-old female with a past medical history of hypertension presented to the clinic with a one-year history of mild diffuse abdominal pain, change in bowel habits including loose stools, and significant involuntary weight loss. A review of systems, physical examination, and routine blood work was non-remarkable. The upper endoscopy was normal but a colonoscopy revealed numerous 1-10 mm submucosal nodules in the entire colon. Biopsies obtained from the nodules of the colon revealed colonic mucosa infiltrated with monotonous, small to medium-sized lymphocytes with irregular nuclear contour, and scant cytoplasm. Immunohistochemistry and immunophenotypic analysis were positive for CD20, CD5, Cyclin D1, BCL6, BCL 2, SOX 11, and negative for CD 10, and P 53. FISH testing revealed 11,14 translocations, consistent with Mantle Cell Lymphoma. CT scan of the abdomen showed pre-carinal, right hilar, extensive para-aortic, and mesenteric lymphadenopathy consistent with stage 4 Mantle Cell Lymphoma. She was subsequently started on Bendamustine and Rituximab combination therapy followed by Rituximab for maintenance. There was a significant improvement in her clinical status with progressive weight recovery. Follow-up upper and lower endoscopic examinations after 6 cycles revealed complete remission with the absence of all polypoid lesions in the GI tract (Figure 1). Discussion: The initial presentation of MCL can widely vary from being asymptomatic to having systemic symptoms of fever and weight loss to marked lymphadenopathy on physical examination. Although 15-30% of individuals with Mantle cell lymphoma (MCL) are reported with involvement in the gastrointestinal (GI) tract, MLP is seen only in less than 10%. It is important for clinicians to not only incorporate MCL as a differential in their workup when they come across diffuse polyps in the GI tract but also to differentiate it from other similar disease processes that present with multiple polyps in the colon. A definitive diagnosis of MLP requires biopsy and histological examination of the tissue with immunophenotypic and immunohistochemistry analysis.Figure 1.: Multiple sessile polypoid lesions of different sizes throughout the colon.

S3374 Asymptomatic Multiple Lymphomatous Polyposis: An Atypical Presentation of Mantle Cell Lymphoma

S3374 Asymptomatic Multiple Lymphomatous Polyposis: An Atypical Presentation of Mantle Cell Lymphoma

Multiple polypoid lesions with erosion of the gastric mucosa in adult T-cell lymphoma/leukemia superimposed on cytomegalovirus infection.

We report here unique endoscopic findings of cytomegalovirus (CMV) gastritis associated with adult T-cell leukemia/lymphoma (ATLL). A 67-year-old Japanese man presented to a hematologist with a month history of appetite loss. Physical examination showed blurred consciousness (GCS E4V2M5) with labored breathing and hypotension. Blood examination revealed hypercalcemia (adjusted Ca: 3.24 mmol/L), anemia (Hb: 70 g/L), and leukocytosis (white blood cell count: 21.3 × 109/L) with abnormal lymphocytes (7%). Human T-cell lymphotropic virus type 1 antibody and provirus DNA monoclonality were positive. A blood transfusion was given, and noradrenaline and denosumab were administered. Upper gastrointestinal tract endoscopy showed multiple polypoid lesions with redness and erosive surface (upper left; see Figure 1). A gastric-biopsy specimen showed the presence of CD3+CD4+CD8− T cells, compatible with the gastric involvement by ATLL, and CMV gastritis presenting with inclusion bodies positive for anti-CMV antibody by immunohistochemistry (upper right and lower left; see Figure 1). CMV antigenemia was present, and soluble interleukin-2 receptor was markedly elevated at 119,194 U/ml. CMV enteritis was also observed. He was treated with ganciclovir for CMV gastroenteritis. One-month after repeated endoscopy revealed a significant regression of redness and erosion of polypoid lesions (lower right; see Figure 1). Two months later, he has been undergoing chemotherapy for ATLL. Since gastric involvement by ATLL presenting as multiple lymphomatous polyposis could only been observed in advanced stage [1, 2], we seldom encounter with this lesion nowadays. Although CMV antigenemia is frequently observed in ATLL patients [3], we rarely treat ATLL patients with CMV infections, particularly gastritis. The present case illustrates the rare complication of ATLL in these days (see Figure 1). This study was conducted by the support of the staffs at gastrointestinal department in the Uji Tokushukai Medical Center. All the authors have no conflict of interest. The authors received no specific funding for this work. All procedures performed in this study involving the patient were in accordance with the ethical standards of our institutional and national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Conceptualization, Methodology, Investigation, Data curation, Visualization, Writing-Reviewing and Editing: Yutaka Shimazu. Investigation, Data curation and Visualization: Fumihiko Kouno. Investigation, Writing-Reviewing and Editing: Shinsaku Imashuku. Investigation, Writing-Reviewing and Editing: Keisuke Shindo. Investigation, Supervision and Writing-Reviewing and Editing: Takashi Miyoshi.

Endoscopic features of colorectal lymphoma according to histological type.

Background and AimThis study aimed to investigate the relationship between the histological type of colorectal lymphoma and its endoscopic features.MethodsWe retrospectively analyzed patients with primary colorectal lymphoma who were diagnosed using colonoscopy and biopsy specimens at the National Cancer Center Hospital, Tokyo, Japan. The lesions were macroscopically classified into the following types via colonoscopy: polypoid, ulcerative, multiple lymphomatous polyposis, diffuse, and mixed.ResultsA total of 117 lesions were identified in 90 patients enrolled in this study. Of these, 59 (50%) were located in the ileocecal region, 23 (20%) in the rectum, 9 (8%) in the transverse colon, 8 (7%) in the sigmoid colon, 7 (6%) in the descending colon, and 4 (3%) in the ascending colon. Moreover, the most common histological subtypes were diffuse large B‐cell lymphoma (DLBCL) in 39 patients (43%) and mantle cell lymphoma (MCL) in 23 patients (26%), followed by follicular lymphoma (FL; 17%), mucosa‐associated lymphoid tissue (MALT) lymphoma (9%), peripheral T‐cell lymphoma‐NOS (2%), monomorphic epitheliotropic intestinal T‐cell lymphoma (MEITL; 2%), and Burkitt lymphoma (1%). More than half of the DLBCL (52%), MCL (52%), and MALT (56%) lymphomas were macroscopically classified as polypoid types. In contrast, FL lesions showed various macroscopic types. The majority of DLBCL (62%) and FL (78%) lesions were distributed in the ileocecal region. MCL lesions tended to be widely spread in various sites of the large intestine.ConclusionsColorectal lymphomas showed macroscopically distinctive features depending on the histological type. Understanding the macroscopic classification of the lesions by colonoscopy and its distribution may be helpful in diagnosing the type of lymphoma and determining the malignant grade based on the histological types.

A Unique Case of Mantle Cell Lymphoma Masquerading as a Cecal Mass

Mantle cell lymphoma (MCL), a type of B-cell non-Hodgkin's lymphoma, is a rare and aggressive disease with a poor prognosis due to its advanced presentation at diagnosis. It is characterized by a translocation in the Bcl-1 gene, which results in overexpression of cyclin D1. MCL is frequently seen in the form of multiple lymphomatous polyposis (MLP) in which innumerable polyps are observed in the gastrointestinal (GI) tract. In rare instances, MCL presents a single mass. The most common presentation involves male patients in their sixties, with generalized lymphadenopathy, extranodal involvement, and B symptoms (night sweats, fever, and weight loss). Endoscopic findings of MLP include cerebroid folding of the gastric mucosa and innumerable polyps extending from the duodenum to the large intestine and are reported in approximately 9% of all GI lymphomas. Less commonly, only 2–4% of GI malignancies present as a primary GI MCL as a single mass, usually in the stomach and ileocecal region in the intestine. Radiologic findings include lymphadenopathy, splenomegaly, multiple polyposis, or wall thickening with ulceration or mass formation. In most instances, advanced disease is found at diagnosis, for which 5-year survival ranges only from 26 to 46%, even when appropriate treatment is initiated. High mitotic rate, or Ki-67 index, is of prognostic value and is associated with poor prognosis. Treatment involves conventional chemo-immunotherapy consisting of R CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) or RB (rituximab and bendamustine), with the latter being better tolerated and associated with longer progression-free survival. Surgical resection is usually limited to patients in which complications are seen such as bleeding, perforation, or bowel obstruction. We present a unique case of a 70-year-old male with nonbilious, nonbloody emesis, and symptomatic anemia who was found to have a cecal mass consistent with MCL.

A147 PRIMARY COLONIC MANTLE CELL LYMPHOMA: A RARE ENTITY

Abstract Background Mantle cell lymphoma (MCL) is an aggressive subtype of B-cell non-Hodgkin lymphoma (NHL), often diagnosed at later stages with secondary gastrointestinal (GI) involvement. Primary GI MCL is rare and is not often discussed in the literature. Aims To increase awareness of a rare condition that is likely to be encountered but can be challenging to diagnose. Methods Case report and review of the literature. Results Case Report A 78-year-old man with multiple untreated vascular risk factors including atrial fibrillation and type 2 diabetes presented with acute onset left hemiplegia, dysarthria, and imaging consistent with a left pontine stroke. As part of his workup he underwent a CT abdomen/pelvis identifying an 11 x 5 cm intraluminal mass in the transverse colon. Previous screening colonoscopies, for family history of colon cancer, were notable for tubular adenomas without high-grade dysplasia at 13, 12, 10, 7, and 2 years prior to admission. The patient had 16 pounds of weight loss without other constitutional symptoms, change in bowel habits or evidence of GI bleeding. Bloodwork was notable for microcytic anemia (Hemoglobin 91 g/L, MCV 75 fL), from a normal baseline one year prior, without other cytopenias. C-reactive protein (44 mg/L) and GGT (164 U/L) were elevated. Other liver enzymes, lactate dehydrogenase, and electrolytes were normal. Colonoscopy revealed numerous polypoid lesions throughout the entire colon and a large non-obstructive mass with submucosal appearance in the transverse colon. Biopsies were taken from the large mass and one of the smaller polypoid lesions. Histology showed a sheet-like infiltrate of small lymphocytes within the lamina propria. Immunohistochemical staining was positive for CD20, BCL2, Cyclin D1, equivocal for CD5, and negative for BCL6 and CD3. Ki67 index approached 30%. A diagnosis of colonic MCL was made. Literature Review Primary MCL of the GI tract is rare, accounting for only 1 to 4% of all GI malignancies. There is a male and Caucasian predominance with a median age of 68 years at diagnosis. Presenting complaints may include abdominal pain, anorexia, and GI bleeding. Typical endoscopic features are small nodular or polypoid tumors, between 2mm and 2 cm in size, along one or more segments of the GI tract referred to as multiple lymphomatous polyposis (MLP). A single colonic mass is infrequently seen, highlighting the importance of endoscopy for diagnosis, as subtle findings may be missed on radiographic evaluation. Biopsies for immunohistochemistry are essential to distinguish MCL from other NHLs, as almost all cases express cyclin D1. Despite aggressive immunochemotherapy, prognosis is often poor due to MCL’s rapid progression and early relapse. Conclusions Primary GI MCL is a rare entity. Awareness is essential as evaluation and management differ from lymphoma at other sites, and other GI malignancies. Funding Agencies None

Multiple Lymphomatous Polyposis Associated With Mucosa-Associated Lymphoid Tissue Lymphoma of the Colon.

Multiple Lymphomatous Polyposis Associated With Mucosa-Associated Lymphoid Tissue Lymphoma of the Colon.

Gastric mucosa-associated lymphoid tissue lymphoma in conjunction with multiple lymphomatous polyposis in the context of Helicobacter pylori and Helicobacter suis superinfection

A 53-year-old woman visited a doctor and complained of chest discomfort after meals. Esophagogastroduodenoscopy showed multiple granular elevations in the gastric body. After biopsies from the elevations, she was diagnosed with mucosa-associated lymphoid tissue (MALT) lymphoma. Polymerase chain reaction also detected Helicobacter pylori and H. suis. Treatment to eradicate H. pylori and H. suis was successful. Endoscopic examination after the bacterial eradication treatment showed that multiple granular elevations remained in the gastric body; however, no lymphoma cells were found during histopathological examination. Thus, we reported a case of H. pylori-positive gastric MALT lymphoma with a unique morphology associated with H. suis superinfection.

Mantle Cell Lymphoma Presenting as Multiple Lymphomatous Polyposis of the Colon

AbstractMantle cell lymphoma (MCL) is a subset of B-cell non-Hodgkin’s lymphoma with a tendency to involve the gastrointestinal (GI) tract and presents as multiple lymphomatous polyposis of one or multiple segments of the GI tract. Here, we report an unusual case of a 58-year-old female presenting with chief complaints of constipation, bleed per rectum, and unintentional weight loss of 15 kg over 6 months. Colonoscopy revealed the entire colon to be studded with polypoidal lesions of varying sizes which were biopsied. Histopathological examination and immunohistochemistry confirmed the polypoidal lesions to represent MCL. Computed tomography scan showed numerous variable-sized polypoidal lesions scattered throughout the entire colon along with paraaortic and mesenteric lymphadenopathy. Thus far, she has undergone three cycles of chemotherapy with R-CHOP resulting in significant tumor reduction and symptomatic improvement in her well-being.

GASTROINTESTINAL TRACT MULTIPLE LYMPHOMATOUS POLYPOSIS PRESENTED AS MANTLE CELL LYMPHOMA

Aims: Mantle cell lymphoma is a mature B cell non-Hodgkin lymphoma which may be presented with the involvement of the gastrointestinal tract as multiple lymphomatous polyposis. The aim of this case report is to increase the awareness of including lymphomatoid polyposis as an entity in the differential diagnosis of multiple polyposis of the gastrointestinal tract. Case Report: A 69-year-old male patient was admitted to the Trakya University Emergency Department with acute abdominal pain. His cli - nical findings were anorexia that started 3-4 months ago together with constipation and nausea causing him to lose 10-15 kg in 7-8 months, with denial of other parameters of B-symptoms (fevers and night sweats). Endoscopic biopsies that were taken from bulbus and duodenum were investigated and he was diagnosed with mantle cell lymphoma. The patient went through an ileoce - cal resection due to his intussusception that caused abdominal pain in the first place. Conclusion: Although being an infrequent disease, gastrointestinal lymphomatoid polyposis should be an entity comprised in differential diagnosis for multiple polyposis of the gastrointestinal tract. On the other hand, there is still not a therapeutic protocol with a definitive cure for gastrointestinal tract mantle cell lymphoma. Elderly patients in high risk group such as our patient should be given treatment by taking their conditions into consideration. Keywords: Mantle cell lymphoma, polyp, non-Hodgkin lymphoma

1640 Case Report: Diffuse Large B-Cell Lymphoma Presenting as Multiple Lymphomatous Polyposis

INTRODUCTION: Diffuse Large B Cell Lymphoma (DLBCL) is the most common type of Non-Hodgkin’s Lymphoma (NHL) in the U.S. accounting for one third of all newly diagnosed NHL’s. The gastrointestinal (GI) tract is the most common site of extra nodal involvement with mucosa-associated lymphoid tissue (MALT) lymphoma and DLBCL accounting for 70-95% of all GI lymphomas. Multiple lymphomatous polyposis (MLP) is a rare presentation of GI lymphoma characterized by diffuse proliferation of lymphocytes that present as multiple polypoid tumors involving long segments of the GI tract. MLP classically presents in mantle cell lymphoma, but can also present, although less frequently, in MALT and follicular lymphomas. We present a unique case of a patient that presented initially with lower GI bleed and was subsequently found to have extensive polypoid lesions in the entire colon. Histological evaluation revealed DLBCL, a rare etiology of MLP. CASE DESCRIPTION/METHODS: A 93-year-old woman with chronic thrombocytopenia suspicious for low-grade MDS and history of a large tubulovillous adenoma without high-grade dysplasia or cancer for which she had undergone a right hemicolectomy, presented to the emergency department with intermittent rectal bleeding, severe anemia, fatigue, decreased appetite, and a 17 pound weight loss over the past 2 months. Her hemoglobin level on admission was 6.5 g/dL (baseline Hgb 11 g/dL) and all other cell counts were otherwise normal. Her last colonoscopy ten years ago was normal and she was given the choice to continue or forego colon screening given her age. She had no family history of colon polyps or colorectal cancer. Colonoscopy revealed approximately 50-100 sesile polypoid lesions from the ileocolonic anastomosis to the distal rectum, ranging in size from 2-40 mm. The larger lesions were predominantly located in the proximal colon and were ulcerated and friable. Multiple biopsies were obtained and pathology results revealed diffuse large B-Cell lymphoma. Further studies revealed stage IV DLBCL without bone marrow involvement and she was started on mini R-CHOP chemotherapy. DISCUSSION: Although DLBCL is a rare cause of MLP, it should be included in the differential diagnosis in patients with multiple polypoid lesions throughout the colon that do not fit the typical phenotype for hereditary polyposis syndromes. Lymphomas associated with MLP include MALT and follicular lymphomas; however, DLBCL and other less common lymphomas should be considered when evaluating patients with this entity.

1913 A Unique Presentation of Asymptomatic Mantle Cell Lymphoma at Screening Colonoscopy: Beware of a Lumpy-bumpy Mucosa

INTRODUCTION: Mantle cell lymphoma (MCL) is a rare aggressive subtype of non-Hodgkin’s lymphoma. Most cases of MCL initially present with diffuse lymphadenopathy. The gastrointestinal (GI) tract is involved in 15–30% of cases and can manifest as a rare clinical entity called multiple lymphomatous polyposis (MLP). Because of its rarity, a clinical syndrome of MLP is not well defined and its presentation varies. Prior case reports describe the presence of GI symptoms in patients with MLP. We present an unusual case of an asymptomatic patient who was found to have MLP on screening colonoscopy and subsequently diagnosed with MCL. CASE DESCRIPTION/METHODS: A 55 year-old female presented for a screening colonoscopy with a family history of colon cancer. Her last screening colonoscopy 5 years prior was unremarkable. She denied any change in bowel movement, blood in her stool, abdominal pain, chest pain, or shortness of breath. Her CBC and CMP one year prior were unremarkable. At colonoscopy numerous small nodular, submucosal polypoid lesions with a prominent vascular pattern were found in the terminal ileum; the mucosa of sigmoid, descending, transverse, and ascending colon had a diffuse subtle nodular appearance. With narrow band imaging, numerous <1 mm nodules with atypical vascular pattern were visualized. Biopsies of all lesions were obtained for histology and flow cytometry, which confirmed a diagnosis of mantle cell lymphoma. Subsequent laboratory evaluation revealed a normal CBC and BMP, mildly elevated AST and ALT to 40–50s U/L, and an elevated LDH of 400 U/L. PET CT scan demonstrated left cervical lymphadenopathy (up to 1.9 cm), mesenteric lymphadenopathy (up to 1.2 cm), and uptake at the ileocecal junction. She was begun on a regimen of rituximab, cytarabine, and bendamustine by oncology. DISCUSSION: This case illustrates an unusual presentation of MLP in that it was discovered in an asymptomatic at time of her screening colonoscopy. It is important to recognize this rare endoscopic entity as early diagnosis and treatment improve the prognosis. The diagnosis of MLP should be considered whenever multiple submucosal polypoid lesions are encountered during endoscopy. Histopathology and flow cytometry are key to confirming the diagnosis; therefore, it is important to submit representative biopsies of these lesions for both histology and cytology to avoid delay in diagnosis, need for repeat procedures, and delay in treatment.

1510 Positive Fecal Immunochemical Testing in Detection of Mantle Cell Lymphoma With Gastrointestinal Involvement

INTRODUCTION: Current colorectal cancer (CRC) screening guidelines are directed at early detection of adenocarcinoma via stool testing or direct endoscopic or radiologic visualization. Compared to a colonoscopy, fecal immunochemical Testing (FIT), a non-invasive test that detects hemoglobin in stool by immunoassay, has similar CRC detection rates. We report a case of mantle cell lymphoma (MCL) involving the gastrointestinal tract (GIT) in a patient diagnosed through routine CRC screening. CASE DESCRIPTION/METHODS: A 70 year-old male with a history of diverticulitis was referred for urgent colonoscopy following a positive FIT. He reported a seven-month history of right upper quadrant abdominal pain, pruritus, and a 19 pound unintentional weight loss. He denied fever, night sweats, melena, hematochezia, and family history of malignancy. Physical exam did not reveal splenomegaly and lymphadenopathy. Notable labs included normocytic anemia and a normal LDH. Colonoscopy showed congested, nodular and thickened mucosal folds in the terminal ileum, ascending colon, and cecum. Random biopsies revealed diffuse atypical lymphoid infiltrates in a multiple lymphomatous polyposis pattern with cyclin D1, CD5 and CD20 immunohistochemical positivity consistent with MCL. PET-CT detected bone marrow and diffuse lymph node involvement. He tolerated six cycles of lymphoma-directed chemotherapy with complete metabolic response on imaging. DISCUSSION: Extranodal disease at primary presentation accounts for 25% of patients with MCL. Microscopic gastrointestinal involvement in MCL at presentation is widely underestimated and has been documented in up to 80% of cases at the time of diagnosis, suggesting that MCL has a propensity to home to the GIT. Clinically, most patients with MCL with GIT involvement are asymptomatic, but can also present with abdominal pain, altered bowel habits, and gastrointestinal bleeding. To date, annual FIT testing has been used solely as a screening test for CRC. The role of non-invasive stool testing such as FIT, and its detection of other primary malignancies involving the gastrointestinal tract besides CRC, has not been studied and requires further investigation.

Abstract B158: Clinical features and management of mantle cell lymphoma

Abstract Introduction: Mantle cell lymphoma (MCL) is a relatively uncommon subtype of lymphoid malignancy and represents 3%–10% of malignant lymphoma, and now appears as a biologic and therapeutic model in the understanding and treatment of hematologic malignancies. Diagnostic procedures include histomorphology, immunophenotype (CD5+, CD19/20+), Ki-67 staining and mandatory detection of cyclin D1 overexpression or t(11;14)(q13;q32). The treatment is very heterogenous and the evolution is marked by numerous relapses. Patients and Methods: During the period from January 2012 to January 2017, we conducted a retrospective and prospective study on the epidemiologic, clinical, prognostic and therapeutic profile of mantle lymphoma. This mono-centric study focused on 20 cases managed in the hematology department of the Annaba University Hospital. The diagnostic method was based on lymph node biopsy and immunophenotyping. The clinical characteristics (according to the international prognostic index of MCL [MIPI]), anatomopathologic (Ki67, blastoid appearance) and radiologic were collected, as well as the different treatment lines were reported. It should be noted that no patient has benefited from a cytogenetic study in search of t (11; 14). Results: Mean age was 63.5 years (range 44- 87). There was a predominance of male subjects with a sex ratio of 1.86. Nodal localization was found in 85%, followed by involvement of the spleen in 20%. The extranodal location is dominated by gastrointestinal involvement (multiple lymphomatous polyposis 25%) and ENT (10%). Almost all patients had ECOG 0-1. Patients had extensive stages (III and IV) of ANN ARBOR. Bone marrow infiltration was found in 35% of cases. The prognostic classification according to MIPI allowed us to classify our cohort into 3 groups as follows: high (n = 12), intermediate (n = 5) and low (n = 3) score. The high proliferation index (+ 50%) in 3 of our patients with the blastoid form in 2 cases were noted. Therapeutically, 19 patients received immuno-chemotherapy according to different protocols: alternating R-CHOP / R-DHAP (7 pts), R-CHOP (4 pts), VR-CAP (4 pts), R-bendamustine (2 pts) and R-mini CHOP (2 pts) with an average number of courses of all protocols 3 (1-6). Maintenance treatment with rituximab every 2 months for 2 years was indicated in 3 patients (2 of whom are still on treatment). The overall response rate is 79%; failure was found in 2 patients. After a median follow-up time of 21 months (5 days-63 months) of the 15 patients in response, 10 are alive in persistent response; late relapse occurred in 2 patients. We deplore 5 deaths including 2 toxic, versus progression of the disease for others. Conclusion: Mantle cell lymphoma is a heterogeneous pathology known to be aggressive and incurable. In spite of recent therapeutic advances, including the provision of immunotherapy (rituximab), the addition of aracytin to high-dose induction and therapeutic intensification followed by autologous-hematopoietic stem cell transplantation and the appearance of new therapies (bortezomib, lénalidomide, ibrutinib ...), the prognosis of the LCM remains poor with a median survival close to 5 years. Citation Format: Fatiha Grifi, Soraya Bougherira, Amel Djenouni. Clinical features and management of mantle cell lymphoma [abstract]. In: Proceedings of the Fourth CRI-CIMT-EATI-AACR International Cancer Immunotherapy Conference: Translating Science into Survival; Sept 30-Oct 3, 2018; New York, NY. Philadelphia (PA): AACR; Cancer Immunol Res 2019;7(2 Suppl):Abstract nr B158.

Mantle Cell Lymphoma of the Gastrointestinal Tract Detected by Endoscopy

Mantle cell lymphoma (MCL) of the gastrointestinal tract is a rare extra-nodal manifestation of malignant lymphomas. The endoscopic findings usually range from ulcers to infiltrative thickening to polyps. Extensive polyps, known as multiple lymphomatous polyposis (MLP), is seen in 0.5-2% of all cases of lymphoma, and should always be considered when there is a high clinical index of suspicion. Here, we present a rare case of MCL initially diagnosed with endoscopy. A 51 year old male, previously healthy without routine medical care, presented with abdominal bloating and constipation for 1 month. He had noted changes in the caliber of his stool, poor appetite, and 10 lb unintentional weight loss. He was prescribed a 2 week course of antibiotics due to concern for bacterial gastroenteritis which did not improve his symptoms. Examination was significant for generalized enlargement of cervical, axillary and inguinal lymph nodes, and mild abdominal distention. Blood work significant for ESR 42 CRP 82.2 Hb 7.9 MCV 77. CT scan of the abdomen had stomach wall thickening with diffuse extensive lymphadenopathy. Upper and lower endoscopy showed extensive involvement of the gastrointestinal tract with small to large polypoid lesions without any ulceration or surrounding erythema (Image 1,2). Tissue biopsy showed dense lymphoid infiltrate throughout lamina propria consistent with MCL (Image 3). Patient was started on the first cycle of R-CHOP therapy during this hospitalization. MCL involving the gastrointestinal tract must be differentiated from other causes of MLP, which include lymphoid nodular hyperplasia, adenomatous hereditary polyposis, lipomatosis, adenomas, carcinomas, and other lymphomas. Diagnosis is confirmed by tissue biopsy. Current guidelines for treatment of MCL is R-CHOP therapy and maintenance Rituximab. More intensive therapy involves aggressive chemotherapy followed by autologous stem-cell transplantation. MLP does not require separate treatment, other than symptom control with laxatives and high fiber diet. Monitoring for response after therapy will usually include repeat endoscopy to evaluate for disease regression. This case was unusual, as the gastrointestinal symptoms preceded the B symptoms typically associated with lymphomas. This prompted endoscopic evaluation, where the preliminary diagnosis of lymphoma was made by the finding of extensive polyps in the clinical context of diffuse lymphadenopathy.1880_A Figure 1. Esophagogastroduodenoscopy showing nodularity and cobblestone appearance throughout the stomach into the duodenal bulb.1880_B Figure 2. Colonoscopy showing cobblestone appearance and nodularity throughout the colon, with large ileocecal mass.1880_C Figure 3. H&E staining demonstrates colonic mucosa with a dense lymphoid infiltrate within the lamina propria.

Primary Small Bowel Diffuse Large B-Cell Lymphoma: Excellent Endoscopic Appearance

Primary gastrointestinal tract lymphoma is a rare entity and its occurrence primarily in the small intestine is even more uncommon. A 37-year-old female presented with abdominal pain ongoing for 3 months associated with nausea and vomiting. The pain was diffuse, radiating to back and it limited her oral intake. She endorsed night sweats and chills but did not report exposure to any sick contacts. She was HIV positive (CD4 count: 46 cells/mm3). CT abdomen and pelvis showed diffuse irregular small bowel wall thickening and submucosal edema, retroperitoneal and diffuse mesenteric lymphadenopathy. Endoscopy revealed multiple hard, friable nodules ranging from 5mm to 2 cm starting at the second portion of the duodenum and extending into the visualized jejunum (Image 1 and 2). Pathology confirmed the diagnosis of DLBCL (Image 3). Bone Marrow biopsy showed no evidence of lymphoma. The patient was started on chemotherapy with dose-adjusted rituximab with etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin. Primary gastrointestinal lymphomas are quite uncommon and constitute 5-10% of all gastrointestinal tumors. [1] In the small intestine, most commonly affected region is ileum followed by jejunum, duodenum (6-8%) and then other sites. [2] HIV, is a known risk factor for lymphoma [3]. With the involvement of small intestine, the symptoms are quite non-specific. [1] Diagnosis is made via biopsy. Endoscopy is used to identify suspicious lesions preceded by radiographical evaluation. In some rare cases, it can present as multiple lymphomatous polyposis (MLP). [3] No guidelines exist for treatment of DLBCL of the small intestine. Historically, in HIV patients chemotherapy combined with ART remains the first step in the management of aggressive lymphomas. [4] Radiotherapy, as a mono treatment modality, has not been shown to be beneficial in DLBCL of the small intestine. [1,2]2555_A Figure 1. Nodules seen endoscopically in the duodenum.2555_B Figure 2. Friable nodules seen in the small bowel2555_C Figure 3. Malignant lymphocytes with moderately abundant cytoplasm, and the nuclei are round to ovoid with prominent nucleoli and occasional mitoses. H&E Stain, 400x

Looks Can Be Deceiving: An Atypical Presentation of Mantle Cell Lymphoma

Introduction: Primary gastrointestinal(GI) mantle cell lymphoma (MCL) is a rare and aggressive malignancy. Endoscopy typically shows multiple lymphomatous polyposis (MLP) but other findings have been reported. We describe a case of primary GI MCL diagnosed during routine colon cancer screening. Case: A 61-year-old Hispanic female presented for a routine colonoscopy. Fifteen years prior, she underwent colonoscopy with polypectomy in El Salvador; report was not available. She denied fevers, chills, weight loss, diaphoresis, abdominal pain, melena, and hematochezia. Abdominal exam was benign and without lymphadenopathy. On colonoscopy, 3 sigmoidal polyps- all less than 5mm- were resected; the remaining mucosa was normal in appearance. Histology for 2 of the polyps showed lymphoid infiltrate with CD20+ B cells, reactive germinal centers, and expanded mantle zones, consistent with mantle cell lymphoma. The third polyp was an adenoma. Patient was referred to oncology and a workup of CT scans, FDG-PET CT, bone marrow biopsy and flow cytometry were normal. Patient declined chemotherapy and was observed. A colonoscopy 3 months later showed normal-appearing mucosa but biopsies from each colonic segment demonstrated diffuse MCL. Discussion: Primary GI MCL accounts for 1-4% of all GI lymphomas. The most common endoscopic finding is intestinal MLP but previous cases report ulcerated lesions, mucosal color changes or thickening, and frank masses. Our patient only had 3 small polyps on the initial screening and normal-appearing mucosa on the repeat endoscopy. Microscopic evidence of MCL is actually seen in two-thirds of cases with endoscopically normal mucosa but the significance of this on patient outcome is unclear. Primary GI MCL is often diagnosed in end stages and has a poor prognosis but the median survival remains to be studied. Chemotherapy regimens such as CHOP achieved high remission rates but for short time periods. More recently, the hyper-CVAD/MA regimen with rituximab has been established as an effective cytoreductive regimen with a high response rate. Our patient was offered bendamustine and rituximab, an effective regimen often utilized in older MCL patients. Specific regimens vary based on disease severity and patient's functional status. This case highlights an atypical presentation of a rare malignancy, the importance of timely screening colonoscopies, and the consideration of performing random colon biopsies on patients with MCL polyp pathology.

Extensive colorectal lymphomatous polyposis complicated by acute intestinal obstruction: a case report

BackgroundMultiple lymphomatous polyposis is a rare type of gastrointestinal lymphoma that extensively infiltrates the intestine. Multiple lymphomatous polyposis originates from the mantle zone of the lymphoma follicle and is considered to be a mantle cell lymphoma, which is a relatively aggressive type of B-cell non-Hodgkin’s lymphoma. We report an unusual case of a patient with multiple lymphomatous polyposis with extensive colorectal involvement and acute intestinal obstruction, an atypical complication of this rare disease. On the basis of this case study, the pitfalls in gastrointestinal tract lymphomatous polyposis diagnosis and prognosis, as well as the treatment options, are discussed.Case presentationOur patient was a 76-year-old white woman with asthenia, cramps, and swelling in the lower left quadrant of the abdomen, as well as weight loss within the previous 5 months. A colonoscopy revealed polyps in the rectum, sigmoid colon, descending colon, and right and left colic flexures. A biopsy revealed lymphomatous infiltration of the intestinal wall. Because of the large size of the polypoid masses, which narrowed the colonic lumen in multiple locations, the patient developed acute intestinal obstruction and was referred for laparotomy. She underwent a total proctocolectomy with a permanent ileostomy and a left salpingo-oophorectomy. Microscopic examination showed the presence of a multicentric, low-grade, small lymphocytic lymphoma. Immunohistochemical analysis revealed positive immunostaining for CD79a, CD20, and CD45. These results were consistent with the diagnosis of mantle cell lymphoma. Two weeks after surgery and prior to discharge, but before the beginning of chemotherapy, the patient’s general condition worsened as she experienced a severe and progressive respiratory tract infection, advanced respiratory insufficiency, and septic shock, and she ultimately died.ConclusionsMantle cell lymphoma develops as a progressive and aggressive disease with widespread polyposis of the gastrointestinal tract. The intensive chemotherapeutic regimens usually result in the regression of macroscopic and microscopic lesions; however, remissions are short in duration, and the median length of patient survival is 3–4 years. Mantle cell lymphoma is a rare disease that should be part of the differential diagnosis of polypoid diseases of the large intestine.