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arcoidosis is a chronic, multisystemic and granulomatous disease. Theetiology is unknown. Upper respiratory tract involvement is extremelyrare, seen approximately in 6% of systemic sarcoidosis.1,2 Diagnosis re-

quires exclusion of nasopharyngeal malignancy and other diseases with sim-ilar clinical manifestations and histologic properties such as amyloidosis,tuberculosis, histoplasmosis, and syphilis. This usually is a diagnosis of ex-clusion. Therefore the tissue samples should be taken from any suspiciousregion in the head and neck area and following a systematic scan. Treat-ment should initially be conservative and always individualized.3

In this article, we report the clinical features and imaging findings ofnasopharyngeal sarcoidosis.

CASE REPORT

A 31-year-old male patient was admitted to our clinic with nasal obstruc-tion. The patient had been well until about one year before admission, whenhe began complaining of poor appetite and weight-loss.

Nasopharyngeal Sarcoidosis:A Rare Location

AABBSSTTRRAACCTT Sarcoidosis is a multisystemic disease of unknown etiology; which rarely involves theupper respiratory tract. 2-18% of generalized sarcoidosis cases hold the ear-nose-throat system. Itcan affect the ear and temporal bones, sinonasal region, salivary glands, pharynx, tonsils and larynx.It may lead to hoarseness, dysphagia, laryngeal paralysis or upper airway obstruction according tolocation of the disease. The nasopharyngeal involvement is also very rare in sarcoidosis. Localisednasal forms can be treated with topical corticosteroids and intralesional injections. Surgery may bean alternative in obstructive cases, or those resistant to medical treatment. In this article, we reporta 31-year-old male with nasopharyngeal involvement of sarcoidosis.

KKeeyywwoorrddss:: Nasal obstruction; nasopharynx; sarcoidosis

ÖÖZZEETT Sarkoidoz nadiren üst solunum yolunu tutan etyolojisi bilinmeyen multisistemik bir has-talıktır. Tüm sarkoidoz vakalarının %2-18’i kulak burun boğaz bölgesini tutmaktadır. Sarkoidoz, ku-lağı ve temporal kemikleri, sinonazal bölgeyi, tükürük bezlerini, farenks, tonsilleri ve larinksietkileyebilir. Tutulum yaptığı bölgeye göre bulgu veren sarkoidoz ses kısıklığı, yutma güçlüğü, la-rengeal paralizi ve üst solunum yolunda tıkanıklığa yol açabilmektedir. Nazofaringeal tutulumu dasarkoidozda oldukça nadirdir. Lokalize nazal formlar topikal kortikosteroidlerle ya da intralez-yonal enjeksiyonlar ile tedavi edilebilir. Cerrahi tedavi, obstrüktif vakalarda ve tıbbi tedaviye di-rençli vakalarda alternatif olabilir. Biz bu yazıda, sarkoidoz nedeni ile nazofarenkste kitle tespitedilen 32 yaşında bir erkek hastayı rapor ettik.

AAnnaahhttaarr KKeelliimmeelleerr:: Nazal tıkanıklık; nazofarinks; sarkoidoz

Ozan EROLa,Ceren BAŞb,Levent N. ÖZLÜOĞLUb

aClinic of Otorhinolaryngology, Çankırı State Hospital, Çankırı, TURKEYbDepartment of Otorhinolaryngology,Başkent University Faculty of Medicine,Ankara, TURKEY

Re ce i ved: 13.01.2019Ac cep ted: 01.02.2019Available online: 18.02.2019

Cor res pon den ce:Ozan EROLÇankırı State Hospital, Clinic of Otorhinolaryngology, Çankırı,TURKEY/TÜRKİYEozzy.erol@gmail.com

This case report was presented as a poster at the 38th National Congress ofOtorhinolaryngology - Head and Neck Surgery, 26-30 October 2016, Antalya, Turkey.

Copyright © 2019 by Kulak Burun Boğazve Baş Boyun Cerrahisi Derneği

OLGU SUNUMU DOI: 10.24179/kbbbbc.2019-64848

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Bilateral inferior turbinate hypertrophy andobstructive mass in the nasopharynx are detectedon endoscopic nasal examination. Magnetic reso-nance imaging (MRI) of the nasopharynx revealedan obstructive mass in the nasopharynx (Figure1). Outfracturing of the inferior turbinates, na-sopharyngeal biopsy as well as adenoidectomy isplanned. Preoperative laboratory values were normal. Written informed consent for these pro-cedures was obtained from the patient. Ade-noidectomy and inferior turbinate outfracture wasperformed. Nasopharyngeal mass was sent forhistopathologic examination, which showed gran-ulomatous inflammation (Figure 2). Acid resistantbacteria (ARB) staining was negative for the spec-imen.

Further examination revealed normal bloodand urine calcium levels. Serum angiotensin converting enzyme level was slightly elevated.Thoracic computed tomography (CT) showed pres-ence of mediastinal lymphadenopaties. The patientwas referred to the Pulmonology Department forfurther investigation. Prednisolone (40 mg/day) hasbeen administered by pulmonology department asthere are systemic symptoms. The patient’s appetitewas restored, and his weight is currently stable. Al-though he suffers occasional low-grade fever andmalaise, his general condition has been good todate. At the 6th month follow-up, the nasopharyn-geal region was open. The ground-glass attenuation

of the lung and the bilateral hilar lymphadenopa-thy greatly reduced.

DISCUSSION

Primary involvement of the nasopharynx is extremely rare. Very few cases were reported inthe literature. Nasopharynx cancer, tuberculosis,amiloidosis, sarcoidosis, Tangier’s disease are cer-tain pathologies that may primarily involve na-sopharynx. The nasopharynx have no afferentlymph vessels, so metastases to the nasopharynx areextremely rare. In particular, renal cell carcinomasare known to metastasize to the nasopharynx.1,4,5

Sarcoidosis is a multisystem granulomatousdisorder of unknown cause. Presenting features ofsarcoidosis ranging from asymptomatic but abnor-mal findings on chest radiography in many patientsto progressive multiorgan failure. On chest radiog-raphy, pulmonary involvement with enlargementof the hilar lymph nodes and swelling of the pe-ripheral lymph nodes might be seen as early signs.However, virtually any organ or tissue maybe in-volved in cases with disseminated granulomas.Symptoms caused by pulmonary, cardiac, neural,gastrointestinal, hepatic, renal, cutaneous, oph-thalmic, and endocrine involvement manifest inlater stages of the disease.1,5

Sarcoidosis of the ear-nose-throat system isrelatively rare. It can affect the ear and temporal

FIGURE 1: MRI axial (A) and sagittal (B) section showing nasopharyngeal mass (yellow arrow).

A B

bones, sinonasal region, salivary glands, pharynx,tonsils and larynx. Among cases of ear-nose-throatsarcoidosis, nasal mucosal lesions are frequently de-tected (69%), while laryngeal and pharyngeal in-volvement are less frequent (15%).3 Nasal cavityinvolvement is present in all cases with laryngealand pharyngeal disease. The present case did nothave cutaneous involvement, in contrast to thehigh prevalence of skin invasion in reported cases(92%) of ear-nose-throat sarcoidosis.4

To date, nasopharyngeal involvement of sar-coidosis has been reported in a small number ofcases. However, the actual number of such casesmay be underestimated, because otorhinolaryngo-logic examination is not generally performed in pa-tients with systemic sarcoidosis. On the other hand,patients with sarcoidosis often initially seek treat-ment from an otolaryngologist, because the earliestsigns and symptoms of sarcoidosis may be identi-cal to those of other forms of chronic sinonasal in-flammation.1,5

Although the exact etiology of sarcoidosis re-mains obscure, its development is attributed to anexcessive, antigen-driven cellular immune re-sponse occuring within target organs, and promot-ing nonspecific systemic inflammation. Since thepharyngeal tonsil is exposed to antigens in inspiredair, immunologic activation may be occurring atthis site, subsequently leading to systemic sar-coidosis. In this respect, it merits emphasize that acareful and detailed otorhinolaryngologic exami-nation including biopsy of the nasopharynx should

be performed routinely in any patient suspected ofhaving systemic sarcoidosis. This way, the lesioncan be biopsied, and even excised without diffi-culty via transnasal approach. The patients shouldbe directed to related departments for the furtherinvestigation.1,5

There are few reports in the literature of pala-tine tonsillar and nasopharyngeal involvement dueto sarcoidosis. Saussez et al. and Yarington et al. re-ported a cases of incidentally identified tonsillarand adenoidal sarcoidosis.6,7 Gil Galero et al., ElBousaadani et al., Akin et al., Wilson et al. and Tu-grul et al. described sarcoidosis patients whichwere diagnosed after adenoidectomy.1,4,5,8,9

In an epidemiologic study at the Mayo Clinic,9% of all sarcoidosis patients had involvement ofhead and neck area. Another study reported thepercentage of patients with had head and neck in-volvement of sarcoidosis as 3% (n=736).10

James et al. reported that 36 patients of 818multisystem sarcoidosis patients exhibited involve-ment of the sinonasal mucosa.11 In nasal examina-tion may be seen yellow mucosal papules, crustingand granulomatous mass. In our patient the endo-scopic nasal examination was normal except for theinferior turbinate hypertrophy.

Epiglottis is the most commonly involvedstructure in laryngeal sarcoidosis. Involvement ofthe vocal cords and subglottic region are rare.These involvements can lead to stridor, dyspnea,dysphonia, dysphagia.9

Treatment depends on the severity of symp-toms associated with sinonasal disease and in-volvement of other organs. In topical treatment,nasal steroids, lubricant drops and nasal washing toreduce dryness and intralesional steroid injectionsin severe syptoms are applied. Systemic steroid ap-plications should be preferred in cases of more se-vere and systemic diseases with destructivechanges. Methotrexate, azothiopyrine, chlorokine,thalidomide, pentoxifylline or cyclophosphamideare the recommended drugs in cases where highdose steroid-resistant and high-dose steroids can-not be continued or where steroid doses should bereduced. Surgery may be an alternative in obstruc-

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FIGURE 2: Histopathologic section, showing respiratory epithelium with granulo-mas in lymphoid stromae (HE, x100).

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tive cases, or those resistant to medical treatment.Long-term and interdisciplinary follow-up is nec-essary because, despite long and aggressive treat-ment, relapse and chronicity are frequent afterreduction or discontinuation of corticosteroid ther-apy.12

The patients with nasopharyngeal involve-ment of sarcoidosis have nonspecific symptomssuch as nasal obstruction and snoring. In conclu-sion nasopharyngeal sarcoidosis is extremely rare,it should also be kept in mind in differential diag-nosis of nasopharyngeal lesions.

SSoouurrccee ooff FFiinnaannccee

During this study, no financial or spiritual support was receivedneither from any pharmaceutical company that has a directconnection with the research subject, nor from a company that

provides or produces medical instruments and materials whichmay negatively affect the evaluation process of this study.

CCoonnfflliicctt ooff IInntteerreesstt

No conflicts of interest between the authors and / or familymembers of the scientific and medical committee members ormembers of the potential conflicts of interest, counseling, ex-pertise, working conditions, share holding and similar situa-tions in any firm.

AAuutthhoorrsshhiipp CCoonnttrriibbuuttiioonnss

IIddeeaa//CCoonncceepptt:: Levent N. Özlüoğlu; DDeessiiggnn:: Ozan Erol, CerenBaş; CCoonnttrrooll//SSuuppeerrvviissiioonn:: Levent N. Özlüoğlu; DDaattaa CCoolllleeccttiioonnaanndd//oorr PPrroocceessssiinngg:: Ozan Erol; AAnnaallyyssiiss aanndd//oorr IInntteerrpprreettaattiioonn::Ozan Erol; LLiitteerraattuurree RReevviieeww:: Ceren Baş; WWrriittiinngg tthhee AArrttiiccllee::Ozan Erol, Ceren Baş; CCrriittiiccaall RReevviieeww:: Levent N. Özlüoğlu;RReeffeerreenncceess aanndd FFuunnddiinnggss:: Ozan Erol, Ceren Baş; MMaatteerriiaallss:: Lev-ent N. Özlüoğlu, Ozan Erol, Ceren Baş.

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2. Kleemann D, Nofz S, Schlottmann A, HöckerI, Stengel B. [Sinonasal sarcoidosis]. HNO.2007;55(12):956-60. [Crossref] [PubMed]

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9. Baughman RP, Teirstein AS, Judson MA,Rossman MD, Yeager H Jr, Bresnitz EA, et al.Clinical characteristics of patients in a casecontrol study of sarcoidosis. Am J Respir CritCare Med. 2001;164(10 Pt 1): 1885-9. [Cross-ref] [PubMed]

10. James DG, Barter S, Jash D, MacKinnon DM,Carstairs LS. Sarcoidosis of the upper respi-ratory tract (SURT). J Laryngol Otol.1982;96(8):711-8. [Crossref] [PubMed]

11. Tuğrul S, Göktaş SS, Özu ̈cer B, Sönmez FC,Özturan O. A clinically unsuspected nasopha-ryngeal sarcoidosis. Kulak Burun Bogaz IhtisDerg. 2016;26(3):169-71.

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