Printer Friendly

Primary laryngeal lymphoma: case report.


Extranodal laryngeal lymphoma is extremely rare. We report a case of primary laryngeal lymphoma in a 76-year-old man who had presented with a 7-week history of progressive hoarseness. Laryngoscopy revealed asymmetry of the right false vocal fold. Pathology of a deep biopsy specimen identified a malignant, diffuse, CD20-positive, B-cell lymphoma. The stage 1E lymphoma completely resolved after treatment with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) and rituximab. Despite its relative rarity, the consequences of a missed diagnosis warrant vigilance for this type of laryngeal tumor.


Extranodal lymphoma involving the larynx is exceedingly rare, accounting for less than 1% of all primary laryngeal neoplasms. (1) Most lymphomas involving the larynx involve other sites as well, including the salivary glands, thyroid, nasopharynx, and tonsils.

Recognition of the clinical presentation of laryngeal lymphoma may prevent inappropriate management, particularly if definitive surgical decisions are made on the basis of frozen sections. The treatment of laryngeal lymphoma differs from that of other submucosal lesions such as laryngeal cysts, nenrofibromas, lipomas, myxolipomas, hemangiopericytomas, paragangliomas, laryngeal amyloidosis, neurilemomas, and Teflon granulomas. (2) Whereas these other lesions generally require resection, resection is contraindicated in laryngeal lymphoma.

We report this case of laryngeal lymphoma because, despite its relative rarity, the consequences of a missed diagnosis warrant awareness of and vigilance for this type of laryngeal tumor.

Case report

A 76-year-old man was referred to our Department of Otolaryngology-Head and Neck Surgery for evaluation of a 7-week history of progressive hoarseness. He denied any history of dyspnea, dysphagia, fever, night sweats, or weight loss. Indirect and flexible fiberoptic laryngoscopy revealed asymmetry of the false vocal fold on the right side, which made visualization of the true vocal told difficult. The rest of the larynx appeared to be normal. No palpable lymphadenopathy in the neck was identified. The initial presumptive diagnosis was either a possible early internal laryngocele or a ventricular cyst.

Findings on computed tomography (CT) of the neck were consistent with a right supraglottic tumor (figure 1). A soft-tissue mass measuring approximately 2.8 x 1.7 cm was present at the level of the false vocal fold on the right. The mass extended laterally into the paralaryngeal fat abutting the thyroid cartilage and posteriorly through the cricothyroid notch with effacement of the aerated portion of the right piriform sinus. No adenopathy was seen within the neck.


Microdirect laryngoscopy confirmed that a large, firm, smooth swelling had involved the supraglottic larynx on the right (figure 2). Frozen-section analysis of deep biopsies revealed that the mass was probably a lymphoma. Indeed, the final pathology identified it as a malignant, diffuse, CD20-positive, B-cell lymphoma--probably a large-cell variant (figure 3). After further workup, the tumor was staged as 1E.


The patient was treated with 8 cycles of CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) and rituximab weekly for 4 weeks. During treatment, he was also diagnosed with prostate carcinoma, which was treated with radiation implants. He also had a renal mass that was suspected of being a renal cell carcinoma, for which he was closely observed. His response to treatment of the laryngeal lymphoma was excellent, and follow-up 3 years later detected no evidence of recurrence.


In our review of 311 lymphomas involving the head and neck, we found that extranodal involvement occurred in 4% of patients with Hodgkin's disease and in 23% of those with non-Hodgkin's lymphoma. No case of primary laryngeal lymphoma was noted. The clinical features of our case are consistent with findings in other cases in the literature.

Lymphoma is one of the most common malignancies of the head and neck, second only to squamous cell carcinoma. Extranodal tumors in the head and neck are usually non-Hodgkin's lymphomas. (3,4) The larynx appears to be a rare site of extranodal lymphoma, accounting for less than 1% of all primary laryngeal neoplasms. (1) By 1976, only 14 cases had been reported in the English-language literature. (1) By 1989, 11 more cases of primary laryngeal non-Hodgkin's lymphoma had been added. (5-20) As of this writing, the total number of reported cases was approaching 90. (21) In these cases, however, the larynx was not always the only site of involvement, and probably fewer than 35 cases were true stage 1E tumors. (22) Our patient's disease was confined to the larynx.

Our review of the literature also revealed that the median age of patients with laryngeal lymphoma was 60 years (range: 14 to 81). The distribution between males and females was almost equal. The most common symptom at presentation was hoarseness, which had been present from 2 to 18 months. Other reported symptoms were dysphonia, dysphagia, stridor, and cough. Although these symptoms are indistinguishable from those of other laryngeal tumors, the macroscopic appearance of non-Hodgkin's lymphoma of the larynx may raise the suspicion of an attentive clinician.

Most laryngeal lymphomas present as a submucosal mass or a polypoid tumor; they are smooth, nonulcerated, and gray-white. They are usually located in the supraglottic region, and they have a particular tendency to involve the aryepiglottic folds, although some cases have been reported in the subglottis. While suggestive, none of these features is pathognomonic for a laryngeal lymphoma. Definitive diagnosis depends on histologic examination of a biopsy specimen.

Primary laryngeal lymphomas probably arise from specialized submucosal aggregates of lymphoid cells present in the lamina propria of the supraglottic area and epiglottis. (2,23,24) Tumor growth slowly expands the overlying mucosa, which remains intact, and this results in a benign-appearing mass. Squamous cell carcinomas, in contrast, arise in the squamous epithelium and present as an irregularity involving the free margin of the laryngeal structures.

It is characteristic of laryngeal non-Hodgkin's lymphomas to remain localized for long periods of time. They can, however, disseminate to distant sites years later, especially to other mucosal sites rather than to peripheral lymphoid tissue. (12,13,22,25) This predisposition for other mucosal sites is attributable to the homing properties of the mucosal B lymphocytes. (20,26) Recurrences in the gastric mucosa, lung, and orbit have been reported. (12,13)

A wide spectrum of histologic subtypes of laryngeal lymphomas has been reported. The great majority of laryngeal non-Hodgkin's lymphomas have been of B-cell lineage; very few T-cell immunophenotypes have been reported. (27-29) Using the working classification of these tumors, a high proportion were diffuse large-cell lymphomas. (20,22)

Historically, radiotherapy has been the primary modality of therapy for these tumors. Results have been fairly good, and long follow-ups have found few recurrences. (1,5,22) Considering the systemic nature of most cases of non-Hodgkin's lymphoma, we believe that chemotherapy has a role, especially in cases of low-grade lymphoma. A definitive diagnosis continues to rely on histologic examination of a biopsy specimen. Care in decision making should be exercised regarding frozen sections. There is little role for surgical resection.


The authors thank the Marshfield Clinic Research Foundation for providing assistance in the preparation of this article through the services of Grail Eldred, Linda Weis, and Alice Stargardt.


(1.) Anderson HA, Maisel RH, Cantrell RW. Isolated laryngeal lymphoma. Laryngoscope 1976:86:1251-7.

(2.) MacLennan KA, Schofield JB. Haemopoietic neoplasms. In: Ferlito A. ed. Neoplasms of the Larynx. New York: Churchill Livingstone, 1993:327-36.

(3.) Urquhart A, Berg R. Hodgkin's and non-Hodgkm's lymphonra of the head and neck. Laryngoscope 2001:111:1565-9.

(4.) Urquhart AC. Lymphomas of the head and neck. In: Alper CM, Myers EN, Eibling DE, eds. Decision-Making in Ear, Nose, and Throat Disorders. New York: W.B. Saunders, 2001:306-7.

(5.) Swerdlow JB, Med SA, Davey FR, et al. Non-Hodgkin's lymphoma limited to the larynx. Cancer 1984;53:2546-9.

(6.) MacKenty JE. Malignant disease of the larynx. Arch Otolaryngol 1934;20:297-328.

(7.) Clerf LH. Sarcoma of the larynx. Arch Otolaryngol 1946:44: 517-24.

(8.) Holmes GW, Schulz MD. Radiation treatment of localized malignant lymphoma. N Engl J Med 1946;235:789-91.

(9.) Lachmann J. Sarcoma of the larynx. AMA Arch Otolaryngol 1951:53:299-307.

(10.) DeSanto LW, Weiland LH. Malignant lymphoma of the larynx. Laryngoscope 1970:80:966-78.

(11.) Dickson R. Lymphoma of the larynx. Laryngoscope 1971;81: 578-85.

(12.) Podoshin L, Fradis M, Schalit M. Lymphosarcoma of the larynx. J Laryngol Otol 1971:85:1063-8.

(13.) Dogra TS. Lymphosarcoma of larynx. J Laryngol Otol 1972:86: 535-41.

(14.) Wang CC. Malignant lymphoma of the larynx. Laryngoscope 1972;82:97-100.

(15.) Fedito A, Carbone A. Volpe R. Diagnosis and assessment of non-Hodgkin's malignant lymphomas of the larynx. ORL J Otorhinolaryngol Relat Spec 1981:43:61-78.

(16.) Gregor RT. Laryngeal malignant lymphoma--An entity? J Laryngol Otol 1981:95:81-93.

(17.) Cohen SR, Landing BH, Byrne W J, et al. Primary lymphosarcoma of the larynx in a child. Ann Otol Rhinol Laryngol Suppl 1978;87(5 pt 2 suppl 52):20-4.

(18.) Bouttens F. Cuvelier C. Non-Hodgkin lymphoma presenting as a solitary laryngeal turnout. J Belge Radiol 1981;64:357-9.

(19.) Chen KT. Localized laryngeal lymphoma. J Surg Oncol 1984;26: 208-9.

(20.) Morgan K, MacLennan KA, Narula A, et al. Non-Hodgkin's lymphoma of the larynx (stage IE). Cancer 1989:64:1123-7.

(21.) Cavalot AL, Preti G, Vione N, et al. Isolated primary non-Hodgkin's malignant lymphoma of the larynx. J Laryngol Otol 2001;115: 324-6.

(22.) Ansell SM, Habennann TM, Hoyer JD, et al. Primary laryngeal lymphoma. Laryngoscope 1997;107:1502-6.

(23.) Ham AW, Cormack DH. Histology. 8th ed. Philadelphia: J.B. Lippincott, 1979:323-66.

(24.) Kutta H, Steven P, Tillmann BN. et al. Region-specific immunological response of the different laryngeal compartments: Significance of larynx-associated lymphoid tissue. Cell Tissue Res 2003;311: 365-71.

(25.) Hessan H, Houck J, Harvey H. Airway obstruction due to lymphoma of the larynx and trachea. Laryngoscope 1988:98:176-80.

(26.) Isaacson P, Wright DH. Extranodal malignant lymphoma arising from mucosa-associated lymphoid tissue. Cancer 1984;53: 2515-24.

(27.) Cheng CJ, Chen PR, Liu MC, et al. Primary malignant lymphoma of mucosa-associated lymphoid tissue of larynx. Otolaryngol Head Neck Surg 1999:121:661-2.

(28.) Mok JS, Pak MW, Chan KE et al. Unusual T- and T/NK-cell non-Hodgkin's lymphoma of the larynx: A diagnostic challenge for clinicians and pathologists. Head Neck 2001;23:625-8.

(29.) Fung EK, NeuhauserTS,Thompson LD. Hodgkin-like transformation of a marginal zone B-cell lymphoma of the larynx. Ann Diagn Pathol 2002;6:61-6.

Ronnie Word, MD; Andrew C. Urquhart, MD; Victor S. Ejercito, MD

From the Department of Otolaryngology Head and Neck Surgery, Marshfield Clinic, Marshfield, Wis.

Reprint requests: Andrew C. Urqubart, MD, Department of Otolaryngology Head and Neck Surgery. Marshfield Clinic, 1000 North Oak Ave., Marshfield, WI 54449. Phone: (715) 387-5271; fax: (715) 389-7622: e-mail:
COPYRIGHT 2006 Vendome Group LLC
No portion of this article can be reproduced without the express written permission from the copyright holder.
Copyright 2006, Gale Group. All rights reserved. Gale Group is a Thomson Corporation Company.

Article Details
Printer friendly Cite/link Email Feedback
Author:Ejercito, Victor S.
Publication:Ear, Nose and Throat Journal
Date:Feb 1, 2006
Previous Article:Kimura's disease of the parapharyngeal space.
Next Article:Unusual paratracheal masses presenting with vocal fold paralysis.

Related Articles
Leiomyosarcoma of the larynx: Diagnosis aided by advances in immunohistochemical staining.
First report of a case of osteoma of the larynx.
Malignancy of the larynx in a child.
Chondrosarcoma of the larynx: A therapeutic challenge.
A case of laryngeal carcinoma appearing as a goiter. (Original Article).
Options for preserving the larynx in patients with advanced laryngeal and hypopharyngeal cancer. (Original Article).
Salivary duct carcinoma of the larynx: report of a rare case.
Lower-extremity liposarcoma metastatic to the larynx: case report.
Coexistent cervical tuberculosis and metastatic squamous cell carcinoma in a single lymph node group: a diagnostic dilemma.

Terms of use | Privacy policy | Copyright © 2022 Farlex, Inc. | Feedback | For webmasters |