ORIGINALLYMPHOMA:
ARTICLE
PRIMARY LARYNGEAL
CASE REPORT
Primary laryngeal lymphoma:
Case report
Ronnie Word, MD; Andrew C. Urquhart, MD; Victor S. Ejercito, MD
Abstract
Extranodal laryngeal lymphoma is extremely rare. We
report a case of primary laryngeal lymphoma in a 76year-old man who had presented with a 7-week history of
progressivehoarseness.Laryngoscopyrevealedasymmetry
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.
Introduction
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, neurofibromas, 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.
From the Department of Otolaryngology–Head and Neck Surgery,
Marshfield Clinic, Marshfield, Wis.
Reprint requests: Andrew C. Urquhart, 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: [email protected]
Volume 85, Number 2
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 fold 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 × 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.
Discussion
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
109
WORD, URQUHART, EJERCITO
A
B
Figure 1. CT of the neck shows the supraglottic soft-tissue tumor
at the level of the false vocal fold on the right. The 2.8 × 1.7-cm
mass extends laterally into the paralaryngeal fat abutting the
thyroid cartilage and posteriorly with effacement of the aerated
portion of the right piriform sinus.
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,thetotalnumberofreportedcaseswasapproaching
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
presentationwashoarseness,whichhadbeenpresentfrom
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.
110
Figure 2. Microdirect laryngoscopy shows the large, firm,
smooth, nonulcerated, pale, submucosal tumor before (A) and
after (B) biopsy.
Mostlaryngeallymphomaspresentasasubmucosalmass
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
aryepiglotticfolds,althoughsomecaseshavebeenreported
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 lymphoENT-Ear, Nose & Throat Journal  February 2006
PRIMARY LARYNGEAL LYMPHOMA: CASE REPORT
Figure 3. Histopathology identifies the tumor as a malignant,
diffuse, CD20-positive, B-cell lymphoma––probably a largecell variant.
mas 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;
veryfewT-cellimmunophenotypeshavebeenreported.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 nonHodgkin’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.
Acknowledgment
The authors thank the Marshfield Clinic Research Foundation for providing assistance in the preparation of this
article through the services of Graig Eldred, Linda Weis,
and Alice Stargardt.
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