Ann Thorac Surg 2007;84:1756-1758. doi:10.1016/j.athoracsur.2007.05.075
© 2007 The Society of Thoracic Surgeons
Case Reports
Primary Lymphoma of the Subglottic Airway in a Patient With Sjogren’s Syndrome Mimicking High Laryngotracheal Stenosis
Robert J. Korst, MD*
Department of Surgery, Valley Health System, The Valley Hospital, Ridgewood, and The Blumenthal Cancer Center, Paramus, New Jersey
Accepted for publication May 29, 2007.
* Address correspondence to Dr Korst, Valley Health System, 1 Valley Health Plaza, Paramus, NJ 07652 (Email: korsro{at}valleyhealth.com).
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Abstract
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Primary B cell lymphoma of the central airways is a rare tumor. This report describes a case of primary B cell lymphoma of the subglottic airway in a patient with Sjogren’s syndrome, which resembled a benign, cicatricial subglottic stenosis. Successful management was achieved using surgical resection with laryngotracheal reconstruction.
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Introduction
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Laryngotracheal stenosis is an uncommon condition of the subglottic airway that is clinically manifested by increasing dyspnea progressing to stridor [1]. In the majority of cases, this process can be attributed to a variety of causes, which include trauma secondary to intubation or previous cricothyroidotomy, airway infections, burns or irradiation, amyloidosis, as well as collagen vascular diseases. However, in a minority of patients a specific cause is not identified, and the process is deemed to be idiopathic. This report describes a case of circumferential, high laryngotracheal stenosis in a patient with Sjogren’s syndrome treated with single-stage surgical resection and laryngotracheal reconstruction. Although the lesion resembled an inflammatory stenosis, the pathologic diagnosis was consistent with primary B cell lymphoma of the subglottic airway.
A 61-year-old woman presented to her primary care physician with the insidious onset of dyspnea on exertion, which had progressed to dyspnea at rest with stridor. Her past medical history was relevant for Sjogren’s syndrome, manifested by xerostomia and keratoconjunctivitis sicca, for which her only medication was artificial tears, taken on an as-needed basis. Initial bronchoscopy revealed a circumferential stenosis of the subglottic airway that appeared cicatricial, beginning 3 mm below the vocal folds and extending inferiorly for approximately 3 cm. These findings were confirmed by computed tomography (Figs 1A–1C). Biopsy was not performed. The patient’s dyspnea continued to progress and she was referred for surgical evaluation.
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Fig 1. Computed tomographic images of primary tracheal lymphoma resembling subglottic stenosis. (A) Virtual bronchoscopic image showing the vocal cords (arrows) and the tight stenosis in the immediate subglottic region. (B) Computed tomographic image demonstrating the level of stenosis. The thin arrow indicates the thyroid cartilage, whereas the wide arrow demonstrates the posterior cricoid plate. (C) Three-dimensional reconstruction of the lesion shown in A and B. The arrow represents the stenotic area, whereas the T designates the trachea and the L designates the apices of the lungs.
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After the diagnosis of Wegener’s granulomatosis was excluded by a negative serum anti-neutrophil cytoplasm antibody assay, the patient was taken to the operating room where subglottic resection was performed using the technique described by Grillo and colleagues [1]. Given that the lesion extended to within 3 mm of the vocal folds and into the inter-arytenoid space posteriorly, exposure was facilitated by the performance of a laryngofissure, as described in a report from Toronto General Hospital [2]. Reconstruction was then performed by fashioning a posterior flap of membranous trachea to provide mucosal coverage into the inter-arytenoid space. A protective tracheostomy was then placed three tracheal rings below the anastomosis.
The patient was extubated in the operating room and tolerated continuous capping of her tracheostomy from the first postoperative day and onward. Recovery was uneventful, and the patient was discharged on postoperative day 7 on a regular diet. She was decannulated two weeks after discharge, and her functional result was classified as good to excellent with no dyspnea, stridor, or aspiration, and only mild to moderate deepening of her voice.
Pathologic examination revealed an atypical lymphoid infiltrate that stained strongly for CD20 and demonstrated monotypic, cytoplasmic kappa immunoglobulin light chain restriction, consistent with mucosa-associated lymphoid tissue lymphoma [3]. The lesion was completely resected and a bronchoscopy was performed 4 months later, which revealed a widely patent subglottic space and no evidence of recurrent tumor.
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Comment
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Sjogren’s syndrome is an autoimmune disorder characterized by lymphocytic infiltration and destruction of the salivary and lacrimal glands by autoantibodies, resulting in xerostomia and keratoconjunctivitis sicca [3]. The most serious long-term complication of Sjogren’s syndrome is the development of non-Hodgkin’s lymphoma, especially mucosa-associated lymphoid tissue lymphoma. The risk of non-Hodgkin’s lymphoma in patients with Sjogren’s syndrome is believed to be approximately 44 times that seen in the general population [4]. Many lymphomas develop in organs affected by the lymphocytic infiltrates characteristic of Sjogren’s syndrome, including the salivary glands and ocular adnexa. The development of non-Hodgkin’s lymphoma in patients with Sjogren’s syndrome is believed to represent a multi-step process, ranging from benign lymphocytic infiltration to the relatively indolent mucosa-associated lymphoid tissue lymphoma, and finally to more aggressive histologic variants [3]. Mucosa-associated lymphoid tissue lymphomas, regardless of an association with Sjogren’s syndrome, tend to respond well to local treatment modalities, including both surgical resection and radiotherapy [5].
The case presented in this report is noteworthy in several regards. First, the location and appearance resembled that of a benign, circumferential, cicatricial stenosis of the subglottic airway, as opposed to an eccentrically positioned, exophytic tumor mass. The lesion was resected with the presumptive diagnosis of benign stenosis, and given its location and appearance, no preoperative biopsy was obtained. Alternatively, if a successful diagnostic biopsy had been preoperatively obtained, the management of this case could have consisted of primary radiotherapy, given the radiosensitivity of these lesions. As a result, the experience described herein suggests that preoperative biopsy of all subglottic stenoses should be obtained, if at all possible. Second, primary lymphoma of the central airways is extremely rare [6–8], with the present report representing a unique case of primary lymphoma of the high subglottic airway. Third, this report documents the very effective functional result that can be obtained by the surgical resection of such lesions, even when located high in the subglottic area. The outcome in this case echoes the experience of others involving surgical resection of primary tracheal lymphoma [8].
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References
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- Grillo HC, Mark EJ, Mathisen DJ, Wain JC. Idiopathic laryngotracheal stenosis and its management Ann Thorac Surg 1993;56:80-87.[Abstract]
- Maddeus MA, Toth JL, Gullane PJ, Pearson FG. Subglottic tracheal resection and synchronous laryngeal reconstruction J Thorac Cardiovasc Surg 1992;104:1443-1450.[Abstract]
- Royer B, Cazals-Hatem D, Sibilia J, et al. Lymphomas in patients with Sjogren’s syndrome are marginal zone B-cell neoplasms, arise in diverse extranodal and nodal sites, and are not associated with viruses Blood 1997;90:766-775.[Abstract/Free Full Text]
- Kassan SS, Thomas TL, Moutsopolous HM, et al. Increased risk of lymphoma in sicca syndrome Ann Intern Med 1978;89:888-892.[Abstract/Free Full Text]
- Zinzani PL, Magagnoli M, Galieni P, et al. Nongastrointestinal low-grade mucosa-associated lymphoid tissue lymphoma: analysis of 75 patients J Clin Oncol 1999;17:1254-1258.[Abstract/Free Full Text]
- Fidias P, Wright C, Harris NL, Urba W, Grossbard ML. Primary tracheal non-Hodgkin’s lymphoma: a case report and review of the literature Cancer 1996;77:2332-2338.[Medline]
- Johnson V, Burrows NJ, Ali NJ, Cox G. B cell non-Hodgkin’s lymphoma of the trachea mimicking COPD Thorax 2004;59:1100.[Free Full Text]
- Okubo K, Miyamoto N, Komaki C. Primary mucosa-associated lymphoid tissue (MALT) lymphoma of the trachea: a case of surgical resection and long term survival Thorax 2005;60:82-83.[Abstract/Free Full Text]
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Abstract
Introduction
