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Mucosa-associated Lymphoid Tissue Lymphoma of the Trachea

Mira-Avendano, Isabel MD; Cumbo-Nacheli, Gustavo MD; Parambil, Joseph MD

Journal of Bronchology & Interventional Pulmonology: January 2012 - Volume 19 - Issue 1 - p 44–46
doi: 10.1097/LBR.0b013e31823fadc2
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Primary malignant lymphoma of the trachea is rare. We report a case of a 60-year-old woman with a mucosa-associated lymphoid tissue lymphoma of the trachea and review the existing literature. This case highlights the importance of including intraluminal lesions in the differential diagnosis when a variable degree of obstruction is seen on pulmonary function testing.

Respiratory Institute, Cleveland Clinic Foundation, Cleveland, OH

Disclosure: There is no conflict of interest or other disclosures.

Reprints: Isabel Mira-Avendano, MD, Respiratory Institute, Cleveland Clinic Foundation, Cleveland, OH 44195 (e-mail: miraai@ccf.org).

Received May 12, 2011

Accepted June 10, 2011

Mucosa-associated lymphoid tissue (MALT) lymphoma arises in the context of preexisting prolonged lymphoid proliferation at mucosal sites. It is considered a low-grade extranodal B-cell neoplasm and may affect any organ in the body. Tracheal MALT lymphoma is rarely found in clinical situations, irrespective of dissemination status. We report a seventh case of tracheal MALT lymphoma to date and review the existing literature.

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CASE REPORT

A 60-year-old woman presented with a 2-year history of progressive shortness of breath. She had been diagnosed with chronic obstructive pulmonary disease and chronic sinusitis. Associated symptoms included dry cough, postnasal drip, intermittent wheezing, and hoarseness. The patient denied any relief of symptoms with over-the-counter decongestants, inhaled bronchodilators, oral steroids, and various antibiotics. She was a lifelong nonsmoker and denied high-risk behavior. Laboratory data were unremarkable. Outpatient workup included pulmonary function testing, which was indicative of a severe obstructive process. Her forced expiratory volume in 1 second was 22% of the predicted value, with a forced vital capacity of 84% of the predicted level. Spirometric flow-volume loop was suggestive of intrathoracic, variable upper airway obstruction (Fig. 1). Chest computed tomography (CT) revealed a 1×1.8-cm soft tissue mass along the right lateral aspect of the trachea, at the level of the aortic arch (Fig. 2). Computed tomogram of the abdomen and pelvis demonstrated a large soft tissue mass measuring 3.1×3.5×6.1 cm in the left para-aortic region. Bone marrow biopsy did not show infiltration. Her bronchoscopic examination was consistent with a large polypoid mass occluding 80% of the distal trachea (Fig. 3). Laser ablation was performed resulting in a near complete opening and visualization of the right mainstem bronchus. Cytopathology from the surgical specimen was positive for low-grade B-cell non-Hodgkin lymphoma with plasmacytic differentiation, compatible with extranodal marginal zone lymphoma of MALT type (Figs. 4, 5). The patient is currently receiving tracheal radiation therapy with possibility of adjuvant chemotherapy in the future.

FIGURE 1

FIGURE 1

FIGURE 2

FIGURE 2

FIGURE 3

FIGURE 3

FIGURE 4

FIGURE 4

FIGURE 5

FIGURE 5

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DISCUSSION

MALT lymphoma constitutes a group of low-grade extranodal B-cell neoplasms, described by Isaacson and Wright in 1983. It is a non-Hodgkin lymphoma that arises from marginal zone B-cells in the setting of a preexisting prolonged lymphoid proliferation in mucosal sites. It often remains localized for a prolonged period of time, frequently nonprogressing despite lack of treatment. When dissemination occurs, it is frequently found to affect various mucosal sites. Primary MALT lymphomas rarely transform to more aggressive lymphoma types.1

MALT lymphomas occur most commonly in the gastrointestinal tract. Other affectation sites include the ocular adnexa, salivary gland, thyroid, lung, thymus, and breast. The lung involvement of MALT lymphoma is usually seen along the bronchovascular bundles and the interlobular septae. Chest CT typically demonstrates single or multiple nodules. The upper respiratory tract is rarely involved. Tracheal involvement is exceedingly rare, with 6 reported cases to date in the medical literature.

Primary tracheal lymphoma was first described in 1975 by Pradhan et al.2 The second and third cases of lymphoma-associated tracheal stenosis were described by Maeda et al in 1981 and by Wiggins et al in 1988.3,4 These patients presented with tracheal lymphomas that required surgical resection and adjuvant radiotherapy. In these cases, similar symptoms were reported: shortness of breath, and intermittent wheezing refractory to noninvasive treatment. Pulmonary function testing was consistent with an obstructive pattern, and flow-volume curves displayed signs of fixed upper airway obstruction. Neither case had evidence of lymphoma dissemination. Survival for these patients was >20 months. These reports have scant documentation on histologic and immunophenotypic features, although they may possibly have represented MALT lymphomas of the trachea. Unfortunately, these diagnoses were not confirmed.

In 1992, Kaplan and colleagues reported the first case of tracheal MALT lymphoma. In this case, symptoms included shortness of breath and stridor. Neck magnetic resonance imaging demonstrated a mass in the trachea that subsequently required surgical resection and adjuvant radiotherapy. There were no signs of dissemination, and the patient was disease free for more than 12 months.5 A second confirmed case of MALT lymphoma was reported in 1999. An Italian group reported a series of 75 nongastrointestinal MALT lymphoma cases, with a single patient presenting the trachea as the affected organ.6 A third case presented as an incidental tracheal polypoid lesion, found on gastroendoscopy. Laser photoresection followed by local ethanol injection was the therapeutic approach of choice.7 In 2005, there was a report of tracheal MALT that was amenable to surgical intervention. The patient underwent tracheal resection and reconstruction.8

In 2008, there were 2 cases reported for tracheal MALT. The first case was reported in Spain in a 65-year-old woman with a 4-week history of worsening dyspnea. Spirometry showed signs of mild obstruction, and chest CT demonstrated an intratracheal mass. Bronchoscopy was consistent with a 70% tracheal stenosis caused by lymphoma-type MALT. The patient underwent chemoradiation therapy and displayed no evidence of relapse after 5 months.9 The second reported case of tracheal MALT lymphoma described in 2008 displayed signs of disseminated disease. Lesions were found in the trachea, bronchi, and lungs. In addition, CT of the neck and abdomen demonstrated multiple, variable-sized lymph nodes with unusual characteristics in the cervical area and left pelvic cavity. The patient received chemotherapy with good response. The patient survived for more than 18 months.10

We report a seventh case of proved MALT lymphoma in the trachea and the second one with evidence of dissemination. These reports indicate that MALT lymphoma affects women aged 40 to 80 years more frequently (Table 1). Common spirometry findings include an obstructive process with flow-volume loop indicating a variable obstructive component. This last finding may prompt the physician to include endotracheobronchial lesions in the differential diagnosis of obstructive tracheobronchial diseases.

TABLE 1

TABLE 1

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REFERENCES

1. Cohen SM, Petryk M, Varma M, et al. Non-Hodgkin’s lymphoma of mucosa-associated lymphoid tissue. Oncologist. 2006;11:1100–1117
2. Pradhan D, Rabuzzi D, Meyer J. Primary solitary lymphoma of the trachea. J Thorac Cardiovasc Surg. 1975;70:938–940
3. Maeda M, Kotake Y, Monden Y, et al. Primary malignant lymphoma of the trachea. J Thorac Cardiovasc Surg. 1981;81:835–839
4. Wiggins J, Sheffield E, Green M. Primary B cell malignant lymphoma of the trachea. Thorax. 1988;43:497–498
5. Kaplan M, Pettit C, Zukerberg L, et al. Primary lymphoma of the trachea with morphologic and immunophenotypic characteristics of low-grade B-cell lymphoma of mucosa-associated lymphoid tissue. Am J Surg Pathol. 1992;16:71–75
6. Zinzani P, Magagnoli M, Galienei P, et al. Nongastrointestinal low-grade mucosa-associated lymphoid tissue lymphoma: analysis of 75 patients. J Clin Oncol. 1999;17:1254–1258
7. Tsurutani J, Kinoshita A, Hideyuki K, et al. Bronchoscipic therapy for mucosa-associated lymphoid tissue lymphoma of the trachea. Intern Med. 1999;38:276–278
8. 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
9. García-García J, López García F, Carratalá Torregrosa JA, et al. Tracheal MALT lymphoma: first case report from Spain. Med Clin (Barc). 2008;131:478–479
10. Kang J, Park H, Lee K, et al. Extranodal marginal zone lymphoma occurring along the trachea and central airway. Yonsei Med J. 2008;49:860–863
Keywords:

malt lymphoma; trachea; variable obstruction

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