Mantle cell lymphoma: involvement of nodal and extranodal sites in the head and neck, with multifocal oral lesions

Abstract

Mantle cell lymphoma (MCL) is a malignant B-cell neoplasm, which comprises monomorphic and small- to medium-sized mantle zone-derived lymphoid cells. It is characterized by chromosomal translocation t(11;14)(q13;q32) and CCND1 truncation, resulting in cell cycle deregulation. It is an aggressive type of non-Hodgkin lymphoma with a propensity to present with extranodal involvement. This study shows the case of an 80-year-old Caucasian male who complained of a 2-month progressive swelling on the right side of his face. The magnetic resonance imaging exam showed multifocal involvement of the head and neck, including oral manifestations, bilateral parotid glands, palate, tongue, and floor of the mouth. An incisional biopsy of the tumor mass was performed. The morphological and immunophenotypic findings were consistent with the diagnosis of MCL. The patient died 4 months later, without any chance of undergoing a therapeutic approach. Although MCL is a rare condition, it should be subjected to a differential diagnosis when affecting the maxillofacial area. Imaging exams and both immunohistochemical and morphological analyses are needed to reach the correct diagnosis. Here, we present an unusual MCL with multifocal involvement of the head and neck.

Keywords: Head and Neck Neoplasms; Lymphoma, Mantle Cell; Magnetic Resonance Imaging, Salivary Glands; Oral Manifestations.

Figure 1. Clinical presentation of mantle cell lymphoma. A – The right parotid gland and submandibular regions are affected; B – A tumor mass is observed intraorally on the left side of the floor of the mouth.

Figure 2. Cervical MRI: Axial view of: A – T1W image; B – T2W image. (1) Superficial lobe of the right parotid gland affected by the MCL. (2) Nodular formations in the superficial lobe of the left parotid gland. (3) Expansive lesion in the palate; C – Sagittal view of T1W image showing a tumor mass affecting the palate and its continuity to the pharyngeal airspace; D – Coronal view of T2W image showing the involvement of cervical lymph nodes. MRI = magnetic resonance imaging; T1W = T1-weighted; T2W = T2-weighted.

Figure 3. Photomicrography of the biopsy specimen. A – Monotonous lymphoid proliferation of small- to medium-sized lymphocytes (H&E, 100X); B, C, and D – Immunohistochemical profile showing positivity for CD20, CD5, and cyclin D1 in the neoplastic cells (400X).