Nasopharyngeal Mantle Cell Lymphoma: An Extremely Rare Entity

Abstract

Mantle cell lymphoma (MCL) is a rare, aggressive subtype of non-Hodgkin lymphoma with a poor prognosis and high recurrence rate. It seldom affects the Waldeyer’s ring let alone the nasopharynx. Patients usually present at late stages of the disease leading to poor failure-free and overall survival rates. Intensive chemotherapy regimes and autologous stem cell transplantation have reported increased survival rates. We report a relapsed case of nasopharyngeal MCL, which previously occurred in the gastrointestinal tract. The patient had undergone a hemicolectomy for colon intussusception secondary to the intraluminal lymphoma mass. He was unable to complete the treatment regime for MCL due to the adverse side effects. Oropharyngeal mass was discovered during routine outpatient follow-up, which was confirmed as nasopharyngeal MCL. We discuss the prognosis, disease progression, and possible treatments.

Fungating nasal mass as a rare presenting manifestation of relapsed mantle cell lymphoma

“…3 While cases reporting relapse of MCL involving the nasopharynx have been reported, the occurence of a fungating mass (5 cm × 3 cm) protruding out from external nare is an extremely rare presentation and highlights the extent of aggressive nature the cancer can develop. [4][5][6] Our patient had an early relapse within 2 months, which is associated with a poor prognosis. In a study by Bond et al, an inferior median overall survival of 1.3 years was seen in patients with early progression of disease within 6 months after both intensive and less-intensive frontline treatment.…”

“…While cases reporting relapse of MCL involving the nasopharynx have been reported, the occurence of a fungating mass (5 cm × 3 cm) protruding out from external nare is an extremely rare presentation and highlights the extent of aggressive nature the cancer can develop 4–6…”

Extranodal Head and Neck Mantle Cell Lymphoma: Characteristics, Treatment, and Survival

“…Due to the rarity of the disease entity, much of the literature on MCL in the head and neck consists of case reports or studies from a small number of institutions. 4,5 Although 1 national population-based study did evaluate overall survival (OS) in MCL, including cases with a primary site in the general head and neck region, our study is the first to characterize demographics, staging, and survival by subsites within the head and neck.…”