Primary laryngeal lymphoma: a diagnostic challenge


  • Catarina Lombo Department of ENT, Hospital Senhora da Oliveira, Guimarães, Portugal
  • Carlos Matos Department of ENT, Hospital Senhora da Oliveira, Guimarães, Portugal
  • Rui Fonseca Department of ENT, Hospital Senhora da Oliveira, Guimarães, Portugal



Larynx, Diffuse B-cell lymphoma, Airway, Epstein-Barr


Lymphomas of head and neck constitute 5-15% of malignancies in this region. However, its primary occurrence at the larynx is exceedingly rare due to the paucity of lymphoid tissue. Here, we reported a case of a 41 year old male who presented with a 1 month history of hoarseness and odynophagia. The examination revealed right vocal cord palsy and an ipsilateral subglottic exudate, that was misdiagnosed as infectious disease. The lesion quickly progressed to airway obstruction, requiring a tracheotomy. Multiple biopsies under general anesthesia were needed before reaching the final diagnosis of diffuse large B-cell lymphoma, Epstein-Barr positive. Selected treatment modality included 3 cycles of chemotherapy followed by radiotherapy in moderate dose with complete remission after 2 years of follow up. The tracheotomy was removed, however, the patient did not recover vocal quality. This case highlighted the heterogeneous presentation of extra-nodal head and neck lymphomas and emphasized the need for suspicion of neoplasm when an infection doesn’t respond to maximal medical therapy.


Cooper JS, Porter K, Mallin K, Hoffman HT, Weber RS, Ang KK, et al. National cancer database report on cancer of the head and neck: 10-year update. Head Neck. 2009;31(6):748-58.

Kim KH, Kim RB, Woo SH. Individual participant data meta-analysis of primary laryngeal lymphoma: Focusing on the clinical characteristics and prognosis. Laryngoscope. 2015;125(12):2741-8.

Hong SA, Tajudeen BA, Choi S, Husain IA. Epidemiology and prognostic indicators in laryngeal lymphoma: a population-based analysis. Laryngoscope. 2018;128(9):2044-9.

Rahman B, Bilal J, Sipra QU, Riaz IB. All that wheezes is not asthma: a case of diffuse large B-cell lymphoma of the larynx. Case Rep Oncol Med. 2017;2017:7072615.

Markou K, Goudakos J, Constantinidis J, Kostopoulos I, Vital V, Nikolaou A. Primary laryngeal lymphoma: report of 3 cases and review of the literature. Head Neck. 2010;32(4):541-9.

Ansell SM, Habermann TM, Hoyer JD, Strickler JG, Chen MG, McDonald TJ. Primary laryngeal lymphoma. Laryngoscope. 1997;107:1502-6.

Azzopardi CP, Degaetano J, Betts A, Farrugia E, Magri C, Refalo N, et al. Laryngeal lymphoma: the high and low grades of rare lymphoma involvement sites. Case Rep Med. 2014;2014:284643.

Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 2016;127(20):2375-90.

Niemiec M, Stryjewska-Makuch G, Janik M, Kolebacz B, Lisowska G, Ścierski W. Head, and neck lymphomas-a retrospective ten-year observation. Contemp Oncol (Pozn). 2017;21(1):66-9.

Nicolae A, Pittaluga S, Abdullah S, Steinberg SM, Pham T, Davies-Hill T, et al. EBV-positive large B-cell lymphomas in young patients: a nodal lymphoma with evidence for a tolerogenic immune environment. Blood. 2015;126(7):863-72.

Cheson BD, Fisher RI, Barrington SF, Cavalli F, Schwartz L, Zucca E, et al. Recommendations for initial evaluation, staging, and response assessment of Hodgkin and non-Hodgkin lymphoma: the Lugano classification. J Clin Oncol. 2014;32(27):3059-68.

Sehn LH, Donaldson J, Chhanabhai M, Fitzgerald C, Gill K, Klasa R, et al. Introduction of combined CHOP plus rituximab therapy dramatically improved outcome of diffuse large B-cell lymphoma in British Columbia. J Clin Oncol. 2005;23(22):5027-33.

Tang Y, Li P, Cua D, Lai J. Laryngeal diffuse large b-cell lymphoma presenting as laryngeal stenosis. In Vivo. 2020;34(1):255-60.

Sehn LH, Berry B, Chhanabhai M, Fitzgerald C, Gill K, Hoskins P, et al. The revised International Prognostic Index (R-IPI) is a better predictor of outcome than the standard IPI for patients with diffuse large B-cell lymphoma treated with R-CHOP. Blood. 2007;109(5):1857-61.






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