Mantle cell lymphoma of the oral cavity: case series and comprehensive review of the literature

Abstract

Objective: Mantle cell lymphoma (MCL) is a rare B-cell neoplasm that has only recently been defined as a distinct entity. Because of its rarity and histologic similarities to other small cell lymphomas, the microscopic diagnosis of MCL may be challenging. This is particularly true within the oral cavity, where other lymphomas are more frequent. To date, few cases of MCL presenting within the oral cavity have been reported.

Study design: We present 2 new cases of MCL within the oral cavity and systematically review 7 other cases of MCL reported in the English-language literature. Historical cases were reviewed, and available data regarding morphology, special stains, demographics, clinical presentation, radiographic findings, management, and outcome were extracted. Data from our present series were then compared with the earlier published literature.

Results: To the best of our knowledge, this is the largest reviewed series of MCL within the oral cavity, totaling 9 cases. The features of our cases, including histology, clinical presentation, and outcome, are consistent with the 7 earlier reported cases. The majority of oral MCLs occur in an older male population, and a high proportion occur on the palate.

Conclusion: We conclude that MCL of the oral cavity is an uncommon diagnosis. Most oral MCLs occur in an elderly male population and have a possible predilection for the palate. The microscopic diagnosis can be challenging, given its similar appearance to other small cell lymphomas, requiring a comprehensive immunohistochemical panel for the accurate diagnosis. Like MCL occurring in other sites in the body, the prognosis and outcome of oral MCL appears to be poor.

Figure 1

Low power image of mantle cell lymphoma, classic variant (Case 2) showing a monomorphic population of small lymphoid cells in submucosa, (H&E 10x)

Figure 2

a: High power image of mantle cell lymphoma (Case 2) with small to medium-sized lymphoid cells (H&E 40x) b: Higher power image of mantle cell lymphoma (Case 2) showing detailed cytomorphology of moderately irregular nuclear contours, (H&E 100x oil)

Figure 3

a: High power image of mantle cell lymphoma, blastoid variant (Case 1) with larger, more atypical lymphoid cells (H&E 40x) b: High power image of mantle cell lymphoma, blastoid variant (Case 1) showing the prominent nucleoli found in this unusual variant (H&E 100x oil)

Figure 4

Comparative immunohistochemistry of classic variant of MCL (Case 2) showing positive staining for all of: CD20(A), CD5(B), CD43(C) and CCND1(D)