Acta Otorrinolaringol Esp. 2012;63(6):485---487

www.elsevier.es/otorrino

CASE STUDY

Laryngeal Manifestation of B-Cell Non-Hodgkin Lymphoma�

Jhonder Xavier Salazar Guilarte,∗ Manuela Sancho Mestre, Juan Ramón Gras Albert

Servicio de Otorrinolaringología, Hospital Universitario de Alicante, Alicante, Spain

Received 20 February 2011; accepted 27 April 2011

KEYWORDSLaryngeal tumour;B-cell lymphoma;Non-Hodgkinlymphoma

Abstract Laryngeal lymphomas are rare, involving less than 1% of all laryngeal tumours. Extra-nodal non-Hodgkin lymphomas generally appear in places where lymphoid tissue exists, suchas the small bowel or parotid; they are very unusual in organs with little or no presence oflymphoid tissue, such as the larynx. When advanced stages are established, initial lymphomamanifestation at the laryngeal level has rarely been documented. We present a case of asmall cell lymphocytic lymphoma at stage IV/E with initial laryngeal symptoms and indicateits management and follow up.© 2011 Elsevier España, S.L. All rights reserved.

PALABRAS CLAVETumores de laringe;Linfoma de células B;Linfoma no Hodgkin

Manifestación laríngea de linfoma no Hodgkin de células B

Resumen Los linfomas laríngeos primarios son raros, representando menos del 1% de ellos. Loslinfomas no Hodgkin (LNH) extranodales aparecen generalmente en lugares donde hay tejidolinfoide como el intestino delgado o la parótida, siendo muy raros en órganos con poca o

ausente cantidad de este tejido como la laringe. Cuando se encuentran en estadios avanza-dos, su debut laríngeo ha sido poco documentado. Presentamos el caso de un LNH linfocítico decélulas pequenas en estadio IV E con primera manifestación clínica a nivel laríngeo, así como

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Introduction

Primary haematological tumours are rare in the larynx,

with this presentation amounting to less than 1% of suchtumours.1 Primary non-Hodgkin lymphomas (NHL) are thesecond most common haematopoietic tumour in the larynx

� Please cite this article as: Salazar Guilarte JX, et al.Manifestación laríngea de linfoma no Hodgkin de células B. ActaOtorrinolaringol Esp. 2012;63:485---7.

∗ Corresponding author.E-mail address: jhoxasagui@hotmail.com

(J.X. Salazar Guilarte).

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2173-5735/$ – see front matter © 2011 Elsevier España, S.L. All rights re

ivo.os los derechos reservados.

fter plasmacytoma, with approximately 100 cases reportedn English-language publications, with very few of them indvanced stages.1---3

Timely recognition of which non-specific laryngealanifestations may correspond to a lymphoma prevents

nappropriate handling, particularly if decisions are basednly on intra-operative biopsies.2 The treatment of theseub-mucosal tumours varies depending on the other lesionsresent, as this type does not require surgical resection.4

ase Report

39-year-old male consulted due to persistent dysphoniand mild odynodysphagia lasting several months, with the

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486 J.X. Salazar Guilarte et al.

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igure 1 (A) Direct laryngoscopy of a left supraglottal growtrowth occupying the left laryngeal vestibule and the pre-epigl

oss of approximately 5 kilos in weight, without haemopt-sis, dyspnoea, fever or night sweats. His personal historyevealed that he smoked 35 packs/year and was a moder-te drinker, with no known allergies or metabolopathies; hisonsils were removed in infancy.

A fibrolaryngoscopic examination was performedFig. 1A) and revealed a smooth, unulcered, left lateralupraglottal growth under the mucosa, affecting the lingualace of the epiglottis, and the ipsilateral arytenoepiglottaland and fold, with good mobility of the vocal cords. Head no palpable laterocervical adenopathies. A CT scanFig. 1B) revealed a homogeneous supraglottal mass withhe same density as the muscle occupying the left part ofhe laryngeal vestibule with evident involvement of theeft pre-epiglottal space. Extension into the left pyriformecess was also observed without evidence of involvementt the level of the glottis. At chest level, a small peripheralpacity was noted as poorly defined on the upper right

obule of the lung and another larger one with attenuationn a triangular shape at the base of the pleura in the loweright lobule, adjacent to the cisura major, all non-specificndings.

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igure 2 (A) Proliferation of lymphoplasmocytic predominance

aryngeal growth following treatment.

truding into the ventricular band. (B) Cervical CT scan with a space.

The CT scan of the abdomen and pelvis revealed con-entric thickening of a segment of the colon suggestingymphomatous infiltration, without occlusion of its lumen,ausing an aneurysmatic dilatation.

Based on a suspected diagnosis of squamous-cell carci-oma, it was decided to carry out surgery to perform airect laryngoscopy with removal of a biopsy and endola-yngeal resection of the supraglottal growth using CO2 laser,ollowing confirmation of carcinomatous tissue in the intra-perative biopsy. However, the biopsy report showed it toe a malignant small-cell tumour and so resection was sus-ended to enable a better a posteriori study.

The pathology report (Fig. 2A) revealed an infiltrationy small-cell lymphocytic NHL, immunophenotype B, CD 20ositive, CD 3 negative, CD 79 A positive and Ki 67 positive.t was immunohistochemically positive for CD 45 ALC andytokeratin 18. In the extension study, normal haemogramnd proteinogram results were obtained, with ANA, HIV, EBV,

MV and HBV serologies all negative. No malignancy waseen in the biopsy of the bone marrow.

After confirmation of the diagnosis of a stage IV non-Hodgkin’s lymphoma by the Head and Neck

with occasional small cells. (B) Evident improvement in the

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Laryngeal Manifestation of B-Cell Non-Hodgkin Lymphoma

Oncology Committee, it was decided to apply sequentialradiochemotherapy. Seven cycles of chemotherapy (CHOP-R) were administered with cyclophosphamide, adriamycin,vincristin, prednisone and rituximab every 21 days followedby a PET-CT scan to assess response to the chemother-apy with the continued presence of marker uptake in theleft supraglottal region (SUVmax 3.4 g/mL) and bilateral hil-iar adenopathies (SUVmax 3.5 g/mL), meeting the criteriafor partial response, so cervical-mediastinic radiation ther-apy was continued for a total of 53.2 Gy in fractions of180 cGy/fraction/day over one month.

Thirty months after diagnosis, the patient remainsasymptomatic and with a complete response to treatment,as confirmed clinically and radiologically (Fig. 2B).

Discussion

Extranodal lymphomas in the head and neck region presentmost frequently in Waldeyer’s lymphatic ring and their loca-tion in the larynx is extremely rare.1---3 They are generallyB-cell type, appearing in the supraglottal region due to thepresence of scant lymphoid tissue in the lamina propria,but there are also a few cases of T/NK cells, at a ratio of6:1, with the latter predominating in HIV positive patients,without any predilection for a laryngeal location.4

In addition, they present as greyish non-ulcerous massesat the sub-mucosa level, without association of the cer-vical lymphatic ganglia. The age of this patient is belowaverage (50---60 years of age), but he does share some non-specific symptoms, such as progressive dysphonia, coughing,dysphagia or a foreign body sensation, that imitate thoseof squamous cell cancer, so its definitive diagnosis is byhistopathology.2

In this case, as the patient did not show any bone-marrowlesion and therefore did not present any cytopaenias, thereis a high probability that the lesion in the supraglottal lar-ynx could be considered primary, despite the discovery ofdissemination foci in infradiaphragmatic regions. AlthoughNHL generally remain localized for long periods of time,they can, however, metastasize to other mucosal regions,in which case the stage is advanced, with a more aggres-sive and disseminated disease, making the prognosis usuallysombre.5 The dilatation of the colon reflected the existenceof an aperistaltic segment due to lymphomatous infiltrationof the sub-mucosa and its muscle with destruction of themyoenteric nerve plexus.

For a correct differential diagnosis, the current study ofNHL must include immunophenotyping, immunohistochemi-cal typing and an extension study, such as a CT scan of thechest and abdomen, medullary aspiration and a PET-CT of

the whole body.2 Flow cytometry and kariotyping analysisare cellular and genetic analytical techniques aimed at thenovel identification of the lymphoma’s cell phenotype andsubsequent immune treatment.2

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There are few cases described in domesticiterature6---10,12 with the most outstanding feature beinghe good prognosis of the condition when detected aton-advanced stages as they tend to remain locally for aong time, the contrary to what happens when they recur,nvade other structures or present in HIV positive patients,here they are highly aggressive and atypical.

The most commonly used treatments are radiation andhemotherapy, generally when the stage is advanced.2,3

urgery is not contemplated as an option in these tumours.any publications highlight the statistical importance of

taging between stages I and IV for prognosis, and radia-ion therapy as the only successful treatment in early stages/II.11

onflicts of Interest

he authors have no conflict of interests to declare.

eferences

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2. Nayak JV, Cook JR, Molina JT, Branch MP, Branstetter IV BF, Fer-ris RL, et al. Primary lymphoma of the larynx: new diagnosticand therapeutic approaches. ORL J Otorhinolaryngol Relat Spec.2003;65:321---6.

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9. Fernández Liesa R, Maltrana García JA, Llorente Arenas E, Dam-borenea Tajada J, Mayayo Romeo P, Hernández Delgado G, et al.Neoplasia linfoide laríngea O.R.L. ARAGON. 2004;7:17---20.

0. Rosa Astacio F, Barberá Durbán R, Vaca González M, CobetaMarco I. Linfoma T/NK laringotraqueal: caso clínico. Acta Oto-rrinolaringol Esp. 2011;62:71---3.

1. Word R, Urquhart AC, Ejercito VS. Primary laryngeal lymphoma:case report. Ear Nose Throat J. 2006;85:109---11.

2. Tardío JC, Moreno A, Pérez C, Hernández-Rivas JA, López-Carreira M. Primary laryngeal T/NK-cell lymphoma, nasal-type:an unusual location for an aggressive subtype of extranodallymphoma. Eur Arch Otorhinolaryngol. 2008;265:705---8.