A 72-year-old man previously presented with obstructive bowel symptoms. He was diagnosed with mantle cell lymphoma—a type of non-Hodgkin lymphoma—with involvement of the rectosigmoid junction and terminal ileum.
He underwent a left hemicolectomy and resection of the terminal ileum followed by chemotherapy (fludarabine, mitoxantrone, dexamethasone and rituximab). After treatment he went into prolonged remission and follow-up colonoscopies 5 years and 7 years later were both unremarkable. The findings of a gastroscopy and capsule endoscopy were also normal 7 years after treatment, at which time the patient was asymptomatic with normal blood results. His haematologist requested a surveillance FDG PET-CT scan, which demonstrated a hot spot in the descending colon (figure 1). A further colonoscopy was carried out and showed 2 polyps in the descending colon (figure 2; DC1 [15mm] and DC2 [50 mm]). The ileocolonic anastomosis and neoterminal ileum were both unremarkable.
a) Ignore them, as they are inflammatory.
b) Take biopsy samples only.
c) Perform standard snare polypectomy.
d) Organise an endoscopic mucosal resection (EMR).
e) Organise an endoscopic submucosal dissection (ESD).
Correct answer: b.
The polyps found in the case patient are likely to be recurrent gastrointestinal mantle cell lymphoma (MCL). To confirm the diagnosis, biopsy samples must be taken and immunohistochemistry performed. In this case immunohistochemistry indeed confirmed MCL. The polyps were lymphomatous submucosal lesions that were not suitable for endoscopic resection.
MCL is a type of mature B-cell non-Hodgkin lymphoma (NHL) that comprises 3–10% of NHLs in Europe and the USA.1,2 In MCL, the malignant cells are thought to originate from marginal-zone peripheral-blood memory B cells.3 The median age at presentation is 68 years and there is a male preponderance. In 75% of cases, patients with MCL present with lymphadenopathy, whilst 25% have extra-nodal disease. Common extra-nodal sites include the spleen, bone marrow, Waldeyer’s ring, gastrointestinal tract, breast, pleura and orbit.
MCL can involve any region of the gut and in one series of 31 cases, gut involvement was as follows: stomach (57%), duodenum (52%), jejunum/ileum (87%), colon (90%) and rectum (65%).4 In 28 of 31 cases lymphomatous submucosal nodules producing polypoid lesions were found in both the small and large bowel. These polypoid lesions may be seen at endoscopy (as was the case here) and measure from 2mm to several cm in diameter. Some of the polyps may be ulcerated. The polyps can involve more than one segment of the gut with or without normal intervening mucosa. Immunohistochemistry is essential to distinguish MCL from other small B-cell lymphoid proliferation.5 Peripheral and gastrointestinal MCL has a characteristic phenotype: CD20+, CD5+ and cyclin D1+ and CD10–.
Primary MCL of the gastrointestinal tract carries a poor prognosis. Combination chemotherapy and immunotherapy remains the main treatment modality with or without high-dose therapy with haematopoietic stem-cell transplantation.6 Surgery is usually not of benefit but may be necessary in patients with bowel obstruction or intractable bleeding. Radiotherapy is normally reserved for palliation. Therapy for MCL is not curative and virtually all patients will have refractory or recurrent disease. Treatment of recurrent MCL involves salvage chemotherapy, preferably in the context of a clinical trial.
Monzur Ahmed is a consultant gastroenterologist in the Department of Gastroenterology, Good Hope Hospital, Birmingham, UK. He graduated with BSc (Hons), MBChB in 1987 from the University of Birmingham, UK. He obtained an MD in 1997 for his research into viral hepatitis and was appointed as a consultant in 1999. He has an interest in advanced therapeutic endoscopy and leads the Trust’s Bowel Cancer Screening Programme.
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