Palatine Tonsils Primary Presentation of Blastoid Variant of Mantle Cell Lymphoma: Case Report
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Palatine Tonsils Primary Presentation of Blastoid Variant of Mantle Cell Lymphoma: Case Report Joana A. S. Marques1,2 · Fátima Ferreira3 · Daniel P. Melo4,5 · Margarida Santos1 · Ricardo P. Vaz1,6,7 Received: 19 August 2020 / Accepted: 12 October 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Head and neck lymphomas can present with a wide range of symptoms. Timely and accurate diagnosis is often challenging. The blastoid variant of mantle cell lymphoma (MCL) accounts for less than one-third of all MCL cases. Isolated primary presentation on the palatine tonsils is rare, and prognosis and outcome are seemingly unfavorable. An 81-year-old man presented with persistent odynophagia, dysphagia, and obstructive hypertrophic palatine tonsils with purulent exudate. The signs and symptoms were non-responsive to antibiotic therapy, and the tonsils were biopsied. The cellular morphology, immunophenotype, and genotype supported a diagnosis of the blastoid variant of MCL. After staging, the patient underwent chemotherapy with Rituximab-Bendamustine (R-Benda). The patient is in clinical remission more than two years after therapy. We report an exceedingly rare case of blastoid MCL that is prone to be misdiagnosed as tonsillitis. We review the literature and discuss treatment options of this uncommon malignancy. Keywords Mantle cell lymphoma · Blastoid variant · Palatine tonsil lymphoma · Waldeyer’s ring · Head and neck lymphoma
Introduction Lymphomas represent the third most frequent malignancy of the head and neck region (12%) following squamous cell carcinoma (46%) and thyroid carcinoma (33%) [1]. They are * Joana A. S. Marques [email protected] 1
Department of Otorhinolaryngology, Centro Hospitalar Universitário S. João, EPE, Porto, Portugal
2
Unit of Otorhinolaryngology ‑ Department of Surgery and Physiology, Faculty of Medicine, University of Porto, Porto, Portugal
3
Department of Clinical Haematology, Centro Hospitalar Universitário S. João, EPE, Alameda Professor Hernâni Monteiro, 4200‑319 Porto, Portugal
4
Department of Anatomic Pathology, Centro Hospitalar Universitário S. João, EPE, Porto, Portugal
5
Department of Pathology, Faculty of Medicine, University of Porto, Porto, Portugal
6
Unit of Anatomy ‑ Department of Biomedicine, Faculty of Medicine, University of Porto, Porto, Portugal
7
Center for Health Technology and Services Research (CINTESIS), Porto, Portugal
traditionally divided into Hodgkin’s (HL) or non-Hodgkin (NHL) lymphomas, and the latter are the most prevalent of all head and neck lymphomas [2, 3]. Head and neck NHL can present in nodal or extranodal forms [4, 5]. The Waldeyer’s ring is the most common site of involvement, accounting for more than half of extranodal head and neck lymphomas reported in the literature [6]. The ocular adnexa, nasal fossae, paranasal sinuses, oral cavity, and salivary glands are additional extranodal sites that may be involved by NHL [6, 7]. We report a
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