Small bowel (small intestine) - Mantle cell lymphoma

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Small bowel (small intestine)

Lymphoma

Mantle cell lymphoma

Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 22 December 2012, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Uncommon in small bowel; often presents as multiple lymphomatoid polyposis (Arch Pathol Lab Med 2011;135:1283)
● Also involves stomach and colon

Clinical features
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● 88% males, mean age 61 years
● Weight loss, fatigue, diarrhea, abdominal pain, often iron deficiency
● Mean survival less than 3 years
● May begin as single lesion or with disseminated multiple mucosal involvement (Mod Pathol 2001;14:811)

Case reports
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● 38 year old man with intussusception (J Clin Oncol 2002;20:4397)
● 85 year old man with coexistent adenocarcinoma (Arch Pathol Lab Med 2003;127:E64)

Gross description
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● Nodular, sessile or polypoid lesions, widely spaced with confluent studded or cobblestone appearance
● Each polyp 2 mm to 2 cm
● May be dominant tumor mass in ileocecum

Gross images
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Mass of terminal ileum is partially necrotic

Micro description
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● Infiltrative mantle cells (small lymphocytes with cleaved irregular nuclei) with invasion of submucosa, sparing of mucosa
● Epithelial invasion and ulceration occurs late in disease
● Secondary intestinal dissemination by lymph node based mantle cell lymphoma is exceedingly common; has a lymphomatous polyposis pattern with involvement of colon (Am J Surg Pathol 1996;20:442)

Micro images
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Early tumors

Tumor of terminal ileum / clon (figures 2/3)

Left to right: H&E, cyclin D1, CD20

Various images

Positive stains
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● CD20, CD5, cyclin D1/BCL1 rearrangement with t(11;14)(q13;q32)

Negative stains
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● CD3, CD10, CD23

Differential diagnosis
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● Nodular lymphoid hyperplasia: benign, associated with common variable immunodeficiency syndrome or other infections
● Multiple lymphoid polyps: benign germinal centers in children, patients with Gardner’s syndrome

End of Small bowel (small intestine) > Lymphoma > Mantle cell lymphoma

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