Introduction: Mantle cell lymphoma (MCL), a rare non-Hodgkin’s lymphoma, exhibits a genetic translocation causing CCND1 gene overexpression, affecting 5% of NHL cases, predominantly in males aged 60–70. Typically diagnosed with advanced symptoms, MCL involves widespread disease and organ spread, being aggressive and incurable with a 1.8–9.4-year average survival. Optimal treatment depends on disease aggressiveness and age. Multiple lymphomatous polyposis (MLP), a rare MCL subtype in the GI tract, is usually present with GI symptoms. Case Presentation: A 71-year-old woman was diagnosed with asymptomatic MLP during MCL staging who underwent thoracentesis due to large right pleural effusion and significant axillary lymphadenopathy and was treated with a chemotherapy regimen of rituximab/cytarabine and later transitioned to bendamustine/rituximab. This patient eventually underwent a bone marrow biopsy and later a bone marrow transplant. Conclusion: We present a unique case of asymptomatic MLP, emphasizing the importance of early detection for the poor prognosis of MLP with a mean survival of less than 3 years.

Mantle cell lymphoma (MCL) is a rare form of non-Hodgkin’s lymphoma, which has an incidence of 1 case per 200,000 people in the USA annually [1]. MCL is characterized by a genetic translocation (1;14) resulting in overexpression of CCND1 gene. MCL comprises 5% of all non-Hodgkin’s lymphomas and is more prevalent in a 3:1 ratio in males with a median age of 60–70 years old [1‒5].

MCL is often diagnosed when patients present with widespread disease, including extensive lymphadenopathy, fevers, chills, night sweats, and weight loss. Patients often have extranodal organ involvement with spread to the GI tract, spleen, and bone marrow [1‒3, 6]. MCL is aggressive in nature and noted to be incurable making the diagnosis grim. Average survival is noted to be between 1.8 and 9.4 years after diagnosis [1]. Choices of optimal treatment are dependent on aggressiveness of disease, age, and Mantle Cell International Prognostic Index (MIPI) scoring [1, 3, 4].

Patients with MCL usually present with symptoms, and it is noted that 70–80% of all patients present with aggressive advanced disease (stage III or stage IV) [3, 6]. It is rare for patients to present without any B-symptoms (unintentional weight loss, night sweats, or fevers).

Multiple lymphomatous polyposis (MLP) is a rare subtype of MCL that affects the GI tract. Multiple polyps in the GI tract suggest MLP [2]. Furthermore, MLP is a rare primary GI lymphoma characterized by polypoid lymphomatous tissue with a strong tendency to spread throughout the GI tract [7]. Patients with MLP present with generalized GI symptoms including abdominal pain, melena, and hematochezia [2, 6]. This case highlights an unusual presentation of a patient without GI symptoms who was found to have MLP affecting areas of the duodenum, colon, and rectum during an initial hospitalization for shortness of breath due to bilateral pleural effusions.

We present a 71-year-old female with a past medical history of irritable bowel syndrome, previous diverticulitis status post-sigmoidectomy, transverse myelitis, and non-insulin-dependent type 2 diabetes mellitus who presented with progressively worsening shortness of breath for 6 weeks. She denied any B-symptoms (fevers, night sweats, weight loss) at the time of admission. Subsequent laboratories did not identify any abnormalities. Computed tomography chest revealed a large right pleural effusion and significant lymphadenopathy in the axilla and supraclavicular regions bilaterally. A thoracentesis was completed and revealed cytology consistent with MCL. A computed tomography abdomen/pelvis demonstrated a mass in the pylorus and duodenal bulb, with a questionable mass in the sigmoid colon. A bidirectional endoscopy was performed which showed polypoid lesions in the cecum (Fig. 1), hepatic flexure (Fig. 2), sigmoid colon (Fig. 3), and rectosigmoid colon (Fig. 4). Biopsy of the polyps revealed MCL (CD5+, CD79a+, Cyclin D1+, p53−, and Ki-67 20%). These findings further confirmed with a diagnosis of MCL. A follow-up positron emission tomography scan revealed lymphadenopathy in the axillary, mediastinal, hilar, and retroperitoneal regions. There was also splenomegaly and FDG avidity in the gastrocolic ligament. The Mantle Cell Lymphoma International Prognostic Index (MIPI) score was 6.8, which denoted the patient to be high risk. The patient was initially treated with a chemotherapy regimen of rituximab/cytarabine and was later transitioned to bendamustine/rituximab. The patient tolerated the chemotherapy regimen and had no adverse effects. A bone marrow biopsy was completed and showed normocellular marrow without evidence of lymphoma. The patient received a bone marrow transplant, and follow-up care is ongoing.

Fig. 1.

Polypoid lesion noted on colonoscopy: cecum.

Fig. 1.

Polypoid lesion noted on colonoscopy: cecum.

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Fig. 2.

Polypoid lesion noted on colonoscopy: hepatic flexure.

Fig. 2.

Polypoid lesion noted on colonoscopy: hepatic flexure.

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Fig. 3.

Polypoid lesion noted on colonoscopy: sigmoid colon.

Fig. 3.

Polypoid lesion noted on colonoscopy: sigmoid colon.

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Fig. 4.

Polypoid lesion noted on colonoscopy: rectosigmoid junction.

Fig. 4.

Polypoid lesion noted on colonoscopy: rectosigmoid junction.

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MLP was first diagnosed in 1961 and is noted to be characterized by numerous GI polypoid lesions throughout the GI tract. Common clinical symptoms reported in patients with MLP include weight loss, nausea, vomiting, diarrhea, and intestinal malabsorption. Some cases have even reported chylous ascites and intestinal obstruction secondary to intussusception [8].

B-cell GI lymphomas are more common than T-cell GI lymphomas. These cancers originate from the mantle zone of the lymphoid follicle of the mucosa-associated lymphoid tissue [7, 8]. However, other GI tumors have similar characteristics and features, requiring biopsy and histopathological analysis as a definitive diagnosis. GI involvement of MCL tumors involves the ileocecal region (35.7%), ileum (20.3%), rectum (9.1%), and the duodenum (7.7%) [5]. Our case presentation highlights the presence of multiple masses, each noted in the pylorus, duodenal bulb, and sigmoid colon.

MLP should be considered when multiple submucosal polypoid lesions are found, and biopsies should be sent for histology and cytology [3]. Staging is completed via the Ann Arbor classification system, with an evaluation of Ki-67 antigen presentation, which is the most reliable method to evaluate for cell proliferation [2‒6]. MLP is an aggressive disease that responds poorly to conventional combination chemotherapy, with R-CHOP immunochemotherapy and maintenance of rituximab noted to be the “gold standard” therapy [1, 4‒6, 8].

Vignote et al. [7] explained that MLP should be treated as a high-grade lymphoma. Dawsey et al. [9] noted a unique presentation of a patient endorsing dyspnea upon hospitalization who was noted to have asymptomatic MLP. Herrera-Gonzalez et al. [10] highlighted a patient presenting with symptomatic anemia, nonbloody and nonbilious emesis, and weakness who was noted to have a cecal mass consistent with MCL. Although MCL is present oftentimes as notable B-symptoms (fevers, night sweats, and unintentional weight loss), occurrences of presentations without gastrointestinal symptoms are rare.

In summary, we report a unique clinical presentation of shortness of breath with high-risk MCL who had asymptomatic MLP identified only at staging bidirectional endoscopy. MLP has a poor prognosis and mean survival of less than 3 years. Clinicians can learn the importance of early detection and prompt treatment, which is imperative for increasing survival in patients [5]. The authors completed the CARE Checklist for this case report, attached as online supplementary material (for all online suppl. material, see https://doi.org/10.1159/000539288).

Ethical approval is not required for this study in accordance with local or national guidelines. Written informed consent was obtained from the patient for publication of the details of their medical case and any accompanying images.

There is no actual or potential conflict for financial support or competing interest.

No funding sources were received for any research relevant to this case study.

R.D. performed a literature review, designed and drafted the manuscript, and organized the figures. K.K., H.S., and P.M. also helped perform the literature review and assisted in the initial manuscript. N.R., A.K., and M.K. helped assist in the initial manuscript and performed revisions. A.Y. and T.S. performed the endoscopic procedures and edited the manuscript. All authors approved and read the final manuscript.

Additional Information

Article Guarantor: Tilemahos Spyratos, DO.

All data generated or analyzed during this study are included in this article and its online supplementary material. Further inquiries can be directed to the corresponding author.

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