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

Abstract

Objectives: To describe disease characteristics and treatment and to analyze survival and mortality for extranodal mantle cell lymphoma (MCL) of the head and neck. Methods: Patients with extranodal MCL—excluding primary sites in the salivary glands, eye, and adnexa—were identified from the Surveillance, Epidemiology, and End Results (SEER) 18 Registries (2000-2015). Overall survival (OS) and cumulative incidence of MCL and non-MCL mortality were calculated. Factors associated with MCL and non-MCL mortality were analyzed with cause-specific hazard models. Results: Five hundred nine patients met criteria for descriptive analysis and 294 patients met criteria for survival analysis, with a median follow-up of 58 months. The most common sites for MCL were the oropharynx (66.0%), nasopharynx (19.1%), and oral cavity (8.4%). The most common treatment received was chemotherapy alone (48.9%), followed by chemoradiation therapy (16.9%), and radiation therapy alone (10.4%). The proportion of cases diagnosed as early-stage disease ranged from 31% of sinonasal MCLs to 83% of laryngeal MCLs. At 5 years, OS was 63% (95% CI: 57%-69%). There was no significant difference in OS ( P = .79), cumulative incidence of MCL mortality ( P = .76), or cumulative incidence of non-MCL mortality ( P = .98) by anatomic site. Comparing early-stage to late-stage disease, there was no significant difference in OS ( P = .38), cumulative incidence of MCL mortality ( P = .07), or cumulative incidence of non-MCL mortality ( P = .14). Multivariate analysis showed increased hazard of MCL mortality for patients that were older or that presented with stage III or stage IV disease. Conclusion: The oropharynx is the most common subsite of head and neck MCLs, followed by the nasopharynx. Primary head and neck MCLs appear to present at an earlier stage than MCLs of other regions. In particular, laryngeal and hypopharyngeal MCLs may present as stage I or II disease.

Clinical characteristics and prognostic analysis of primary extranodal non-Hodgkin lymphoma of the head and neck

“…Therefore, it is recommended that advanced patients participate in appropriate clinical trials, and those with relapsing or refractory diseases can be subjected to transplantation of hematopoietic stem cells if they meet the requirements [26]. Previous studies believed that radiotherapy is the main treatment for MALT lymphoma, FL, and other E-NHL, and can also be used as consolidation treatment, rescue treatment or palliative treatment after systemic treatment [37][38][39]. However, in our study, no statistical difference was observed in the impact of radiotherapy on prognosis, which may be related to the small sample size of patients subjected to radiotherapy.…”

Linfoma de células do manto na cavidade oral e região maxilofacial

“…Nodal MCLs usually have a worse prognosis compared to extranodal cases, with head and neck MCLs having a better survival rate compared to gastrointestinal cases. 11,18 In contrast, 12 failed to observe any significant differences among head and neck subsites. Moreover, the leukemic non-nodal MCL variant is associated with a better prognosis, 26 and the exceedingly rare in situ mantle cell neoplasia has an indolent course with excellent longterm survival.…”

“…Mantle cell lymphoma is a mature B-cell lymphoma with increasing trends in the USA and Europe. 3,[11][12][13][14] Lymph nodes are typically the most commonly affected sites for MCL. Extranodal involvement is rarer but may also be found, more usually in the gastrointestinal tract and the head and neck region, which account for 39.7% and 39.1% of all extranodal cases, respectively.…”

“…11 Recently, the oropharynx was demonstrated to be the most affected subsite (66%) in the head and neck, while the oral cavity was only involved in 8.4% of the cases. 12 The current original series further contributed to the understanding of oral and maxillofacial MCL by describing the clinicopathological features of primary lesions and manifestations of an already leukemic condition.…”

“…Clinical symptoms in the head and neck region may prompt patients to seek medical consultation timeously, resulting in earlier detection. 1,9,12 The clinical behavior of MCL is highly variable, with median survival ranging between three and five years. The survival rate ranges from 29.2% to 54.5%, with improvements following the introduction of rituximab and other novel biological agents.…”