Journalof Voice Vol. 13, No. 2, pp. 240-245 © 1999 SingularPublishing Group, Inc.
Laryngeal Sarcoidosis Presenting as an Isolated Submucosal Vocal Fold Mass R o b e r t B. M c L a u g h l i n * , J o s e p h R. S p i e g e l , * t $ J e s s e Selber,-~ D e n i s e B. G o t s d i n e r , § R o b e r t T. S a t a l o f f * t $ 6 *Department of Otorhinolaryngology--Head and Neck Surgery, Hospitals" of the University of Pennsylvania, Philadelphia; ~Department of Otolaryngology--Head and Neck Surgery, Jefferson Medical College; and $Department of Otolaryngology--Head and Neck Surgery and #Department of Surgical Pathology, Allegheny University Hospitals, Graduate Hospital, Philadelphia, Pennsylvania, U.S.A.
Summary: Sarcoidosis can affect the larynx as a manifestation of systemic disease or as isolated laryngeal involvement. Classically, laryngeal involvement affects the supraglottis, and less commonly the subglottis, and true vocal fold involvement is rare. The clinical course is often highlighted by frequent exacerbations and remissions that, when associated with vague complaints and constitutional symptoms, are probably the greatest contributor to delayed presentation and diagnosis. We describe an unusual case of sarcoidosis that presented after a long and protracted clinical course as an isolated submucosal vocal fold mass requiring deep biopsy for diagnosis. A review of the literature with emphasis on diagnosis, appropriate airway management, and treatment is presented. Key Words: Laryngeal sarcoidosis--Vocal fold mass--Sarcoidosis--Microendoscopic surgery--Videolaryngoscopy--Systemic granulomatous disorder.
Sarcoidosis is a chronic granulomatous disease of unknown etiology that can affect multiple organ systems. 1 In addition to classic hilar and mediastinal lymph node involvement, involvement of nearly every part of the mucosa of the respiratory tract from the nasal cavity to the peripheral bronchioles has been described. 1 Granulomatous laryngitis more commonly presents in conjunction with generalized sarcoidosis, with a reported incidence of 0.6% to 1.3%. However, isolated laryngeal involvement is seen and was first described by Neel and McDon-
aid. 2,3 Laryngeal sarcoidosis classically involves the supraglottic region, and less commonly the subglottis, and true vocal fold involvement is considered rare. 1-6 Like generalized sarcoidosis, chronic granulomatous laryngitis has an unpredictable clinical course. This characteristic "waxing and waning" course associated with vague complaints and constitutional symptoms is probably the greatest contributor to delayed presentation and diagnosis.1 Patients with undiagnosed sarcoidosis that later resulted in impending upper airway obstruction requiring emergent intervention have been reported. 1,4 We describe an unusual case of sarcoidosis that presented after a long and protracted clinical course as an isolated submucosal vocal fold mass requiring deep biopsy for diagnosis.
Accepted for publication June 9, 1998. Address correspondence and reprint requests to Joseph R. Spiegel, MD, Associate Professor of Otolaryngology, Thomas Jefferson Medical College, 1721 Pine St., Philadelphia, PA 19103 U.S.A.
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CASE REPORT A 50-year-old Caucasian male presented with a 5year history of hoarseness. His earlier evaluations by outside otolaryngologists revealed abnormal findings isolated to the left vocal fold described as a polyp. He underwent 2 biopsies via a microscopic direct laryngoscopy approach at 2 and 3 years prior to presentation to the authors. These biopsies revealed inflammatory changes, but there was no mucosal atypia. Associated symptoms included a dry cough, dysphagia, and a 50-pound weight loss. He denied a temporal relationship between his hoarseness and the onset of dysphagia. Rather, his dysphagia began soon after he underwent his second anterior cervical fusion. It was more pronounced with solid foods than liquids, and he frequently required clear liquids to wash down his solids. A barium swallow performed 18 months after initial presentation revealed moderate pharyngeal and esophageal stage dysphagia. There was evidence of reduced pharyngeal wall contraction and reduced laryngeal elevation. A chest roentgenogram was noncontributory. His symptoms were only mildly improved after a short course of oral steroids and then recurred. He denied fevers, rashes, dyspnea, and shortness of breath. His past medical history was significant for coronary artery bypass surgery and C5 through C7 anterior cervical fusions performed at 7 and 11 years prior to presentation. He had a known history of hiatal hernia with gastroesophageal reflux disease and a 50-pack-year smoking history that was discontinued 7 years prior to presentation. His medications included prilosec, mevacor, and Sectral. Initial head and neck examination was unremarkable, with the exception of the larynx. Strobovideolaryngoscopic evaluation revealed a left submucosal vocal fold mass with dark-red discoloration and significantly reduced vibratory motion. Gross motion was normal. A videoendoscopic swallowing evaluation demonstrated delayed transit through the pharynx, with retention of a portion of both soft and liquid boluses in the hypopharynx. All retained material was eventually cleared without evidence of aspiration. Magnetic resonance imaging with gadolinium demonstrated a mildly enhancing 1.0 X 0.9-cm area of asymmetry involving the left vocal fold. There was no evidence of regional lymphadenopathy, and the salivary glands were unremarkable (Fig. 1).
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Microscopic direct laryngoscopy with biopsies of the left vocal fold showed chronic inflammation without evidence of neoplasia. His hoarseness improved slightly, but it recurred quickly to its preoperative level and persisted despite preoperative and postoperative voice therapy. The left vocal fold mass slowly became more prominent over the ensuing months, and a second microscopic direct laryngoscopy was performed 10 months after the authors' initial procedure. A mucosal flap was elevated, allowing sharp dissection for exploration to at least 1cm depth in the vocal fold and paraglottic space. Soft, irregular gray tissue was found replacing normal muscle at this level and was biopsied (Fig. 2). Final pathology revealed multiple noncaseating granulomas with occasional giant cells. Special stains for acid-fast bacilli and fungal organisms were negative (Fig. 3). Previous chest roentgenograms had been unrevealing; however, during the interval of apparent progression, just prior to his final biopsy, hilar adenopathy was noted on chest x-ray (Fig. 4). Subsequent management included a short course of oral steroids and voice therapy. In comparison to his last preoperative evaluation, at greater than 1 year of follow-up, his voice is stable and improved; however, he remains persistently hoarse. Postoperative videolaryngoscopic evaluation revealed resolution of his vocal fold fullness and improved (but persistently reduced) vibratory motion. DISCUSSION Since Poe described the first pathologically confirmed case in 1940, laryngeal sarcoidosis has become an accepted manifestation of systemic sarcoidosis. 7 In the Mayo Clinic's review of 2319 patients with sarcoidosis, 220 demonstrated head and neck manifestations, including 13 (6%) patients with laryngeal involvement.2 In addition to associated systemic involvement, Neel and McDonald described 6 patients exhibiting isolated laryngeal involvement. 1,2 Due to the unpredictable nature of laryngeal sarcoidosis, early diagnosis and management by an attentive otolaryngologist allow the best chance to prevent impending upper airway obstruction and potentially obviate the need for emergent surgical intervention. The most common complaints on clinical presentation include hoarseness, dyspnea, dysphagia, and chronic Journal of Voice, Vol. 13, No. 2, 1999
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A
B FIG. 1. MRI. A. Tl-weighted axial images of the neck demonstrating midleft vocal fold asymmetry. B. Tl-weighted axial image with Gd demonstrating enhancement.
cough), 2 Constitutional symptoms may include lowgrade fevers, fatigue, and weight loss. 3 When laryngeal sarcoidosis is suspected, communication with other physicians involved in the patient's care and collaboration with any necessary specialists, especially pulmonologists, ophthalmologists, and dermatologists, may be necessary to perform thorough evaluation for systemic involvement and management.2 When the diagnosis of sarcoidosis is suspected on the basis of laryngeal findings, biopsy is necessary to Journal of Voice, Vol. 13, No. 2, 1999
rule out malignant tumors, lymphoma, and rare cartilaginous tumors. Special stains and laboratory evaluation should evaluate for amyloidosis, and specific infections such as tuberculosis, syphilis, histoplasmosis, coccidiomycosis, actinomycosis, and blastomycosis.2, 8 The differential diagnosis of isolated involvement of the subglottis includes relapsing polychondritis, amyloidosis, and Wegener's granulomatosis. 2 The characteristic histologic findings in the larynx are similar to those found in other organs, pri-
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A
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B
FIG. 2. A. Left vocal fold incision. B. Elevation of a mucosal vocal fold flap revealing soft grayish mass for definitive deep biopsy.
FIG. 3. A characteristic sarcoid noncaseating granuloma made up of an aggregate of tightly clustered epithelioid cells, with occasional Langhans'-type giant cells.
marily noncaseating granulomas composed of epithelioid cells. Giant cells may be present but are not necessary for diagnosis. 2-4 Although any part of the larynx can be affected, sarcoidosis classically affects the supraglottic tissues and much less frequently the subglottis.l-6 Laryngoscopic examination can reveal a wide array of mucosal findings which include edema and erythema, punctate nodules, mass lesions, and ulcerations. 1,4
Typically supraglottic involvement has been described as pale, pink, diffuse induration and swelling described as "turbanlike thickening" of the supraglottic structures including the epiglottis, aryepiglottic folds, false vocal folds, and arytenoids.2-4, 9 Similar subglottic manifestations have been described; however, the true vocal folds are considered by most authors to be spared, presumably because of their lack of lymphatics. 1-6 On the other hand, in Bower's Journal of Voice, Vol. 13, No. 2, 1999
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FIG. 4. Chest roentgenogram revealing hilar lymphadenopathy.
1980 review of the literature, he reported 8 (24%) patients who underwent direct laryngoscopy exhibited mucosal abnormalities involving the true vocal cords; however, these lesions were not characterized. 2 In Neel and McDonald's series true vocal fold involvement was considered a reflection of more extensive subglottic disease. 4,9 This case has both unusual and typical features of laryngeal sarcoidosis. To our knowledge an isolated submucosal vocal fold involvement has never been reported as a presenting sign of laryngeal sarcoidosis. However, this patient's symptomatic presentation and diagnosis only after multiple biopsies are all too common in the literature. Early diagnosis and subsequent intervention are only possible by maintaining a high index of clinical suspicion when dealing with any and all submucosal lesions of the larynx.4 Overall, sarcoidosis is felt to have a tendency toward spontaneous regression, and treatment depends on the degree of symptoms. 10 However, due to the risk for possible future airway obstruction, any patient with laryngeal sarcoidosis should be followed closely.4 Systemic steroids are the mainstay of therapy for symptomatic laryngeal sarcoidosis. 1,4,11When laryngeal sarcoidosis fails to respond to systemic steroids, intralesional steroid injections, low dose radiation, and tracheostomy with local resection have all been advocated.l,2,4,5,11,12 Unfortunately, accurate assessment of these modalities is complicated by the frequent spontaneous exacerbations and remissions Journal of Voice, Vol. 13, No. 2, 1999
inherent in this disorder. 2 Although follow-up is limited, this patient has been successfully managed after endoscopic partial resection with speech therapy.
REFERENCES 1. Bower JS, Belen JE, Weg JG, Dantzker DR. Manifestations and treatment of laryngeal sarcoidosis. Am Rev Respir Dis. 1980; 122:325-332. 2. Neel BH, McDonald TJ. Laryngeal sarcoidosis: report of 13 patients. Ann Otol Rhinol Laryngol. 1982;91:359-362. 3. Benjamin B, Dalton C, Croxson G. Laryngoscopic diagnosis of laryngeal sarcoid. Ann Otol Rhinol Laryngol. 1995; 104:529-531. 4. Weisman RA, Canalis RE Powell WJ. Laryngeal sarcoidosis with airway obstruction. Ann Otol. 1980;89:58-61. 5. Fogel TD, Weissberg JB, Dobular K, Lirchner JA. Radiotherapy in sarcoidosis of the larynx: case report and review of.the literature, Laryngoscope. 1984;94:1223-1225. 6. Miglets AW, Viall JH, Kataria YP. Sarcoidosis of the head and neck. Laryngoscope. 1977;87:2038-2048. 7. Poe DL. Sarcoidosis of the larynx. Arch Otolaryngol. 1940;32:315-320. 8. Lebovics RS. Infectious and inflammatory disorders of the larynx. In: Rubin JS, Sataloff RT, Korovin GS, Gould WJ, eds. Diagnosis and Treatment of Voice Disorders. New York: Igaku-Shoin; 1995:176-202. 9. Gould WJ, Yanagisawa E. Benign vocal fold pathology through the eyes of the laryngologist. In: Rubin JS, Sataloft RT, Korovin GS, Gould WJ, eds. Diagnosis and Treatment of Voice Disorders. New York: Igaku-Shoin; 1995:137-151. 10. Gallivan GJ, Landis JN. Sarcoidosis of the larynx: preserving and restoring airway and professional voice. J Voice. 1993;7:81-94.
LARYNGEAL Krespi YP, Mitrani M, Husain S, Meltzer CJ. Treatment of laryngeal sarcoidosis with intralesional steroid injection, Ann Otol Rhinol Laryngol. 1987;96:713-715.
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12. Ruff T, Bellens EE. Sarcoidosis of the larynx treated with CO 2 laser. J Otolaryngol. 1985;14:245-247.
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