Recurrent mantle cell lymphoma presenting as a solitary rectal mass

A 62-year-old man had been passing small stool for 2 months. He had been diagnosed as having mantle cell lymphoma of the spleen 8 years ago, and complete remission was achieved after chemotherapy. A computed tomography (CT) scan of the abdomen, taken to evaluate the intra-abdominal lymph nodes, revealed a rectal mass with perilesional lymphadenopathy, A primary rectal cancer was suspected ([Fig. 1], [2]). Colonoscopy revealed an ulcerative rectal mass with loss of rectal glandular structure confirmed with narrow-band imaging ([Fig. 3], [4]). Histological examination of the biopsy specimens showed numerous lymphocytic infiltrations ([Fig. 5], [6]), which were positive for cyclin D1 for B cells. Recurrent mantle cell lymphoma was diagnosed and the patient was treated with rituximab-based chemotherapy.

Fig. 1 Computed tomography (CT) scan (coronal section) showing a 4-cm intraluminal polypoid mass (arrow) at the left rectal wall, near the anorectal junction.

Fig. 2 Computed tomography (CT) scan (axial section) showing several enlarged lymph nodes (arrows) along the presacral space.

Fig. 3 A central ulcerative mass, 4 cm in diameter, at the posterior wall of rectum, at 3 cm from the anal verge, demonstrating contact bleeding.

Fig. 4 Narrow-band images demonstrating loss of rectal glandular structure with increased numbers of abnormal blood vessels.

Fig. 5 Abnormal dense lymphoid infiltration in the lamina propria. There was no proliferation in the center or lymphoepithelial lesion (hematoxylin and eosin stain).

Fig. 6 Small to medium sized lymphoid cells with hyperchromatic irregular nuclei and some scattered plasma cells. Note that the normal glandular structures have been destroyed.

A large solitary ulcerative rectal mass is a typical presentation of primary rectal cancer. Intestinal involvement of mantle cell lymphoma, in contrast, typically presents with multiple lymphomatous polyposis [1] [2]. The stomach is the favored location [2]. Rates of involvement as documented in previous endoscopy reports are: esophagus 6 %, stomach 74 %, duodenum 34 %, ileum 48 %, cecum 14 %, colon 57 %, and rectum 48 % [4]. Intestinal lesions of mantle cell lymphoma presented as multiple lesions in nearly 80 %, whereas a protruding mass was found in 18 % [2]. Narrow-band imaging of mantle cell lymphoma of the stomach has revealed loss of normal glandular structure and tree-like appearance of abnormal blood vessels [3]. The present rectal mantle lymphoma showed hypervascularity of the mucosa with loss of standard rectal glandular structure. Immunohistochemical staining provides a definite diagnosis, and infiltrates of small, atypical lymphocyte-like cells, which stain positive with pan B-cell marker, T-cell CD5, and cyclin D1, are characteristic of the disease. Typically the disease is aggressive and the median survival is 3–5 years despite aggressive chemotherapy [4].

Endoscopy_UCTN_Code_CCL_1AD_2AC

References

• 1 Rashid S, Pervez S, Khan M M. et al . Mantle cell lymphoma presenting as solitary polypoid colonic lesions.  Indian J Gastroenterol. 2001;  20 74-76
• 2 Iwamuro M, Okada H, Kawahara Y. et al . Endoscopic features and prognoses of mantle cell lymphoma with gastrointestinal involvement.  World J Gastroenterol. 2010;  16 4661-4669
• 3 Nonaka K, Ishikawa K, Arai S. et al . Magnifying endoscopic observation of mantle cell lymphoma in the stomach using the narrow-band imaging system.  Endoscopy. 2010;  42 (Suppl. 2) E94-E95
• 4 Ruskoné-Fourmestraux A, Delmer A, Lavergne A. et al . Multiple lymphomatous polyposis of the gastrointestinal tract: prospective clinicopathologic study of 31 cases. Groupe D' étude des Lymphomes Digestifs.  Gastroenterology. 1997;  112 7-16