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Journal of Thoracic Oncology:
doi: 10.1097/JTO.0b013e3181cee464
Case Reports

Primary Non-Hodgkin's Lymphoma of the Trachea

Fernandez, Felix G. MD*; Denlinger, Chadrick E. MD†; Crabtree, Traves D. MD‡

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*Division of Cardiothoracic Surgery, Emory University, Atlanta, Georgia; †Division of Cardiothoracic Surgery, Medical University of South Carolina, Charleston, South Carolina; and ‡Division of Cardiothoracic Surgery, Washington University School of Medicine, St. Louis, Missouri.

Disclosure: The authors declare no conflict of interest.

Address for correspondence: Traves D. Crabtree, MD, Washington University School of Medicine, Suite 3108, #1 Barnes Jewish Plaza, St. Louis, MO. E-mail: crabtreet@wustl.edu

A 41-year-old woman developed progressive dyspnea during a 3-month period. She was initially diagnosed with asthma and treated medically without significant relief. Chest x-ray was unremarkable. She reported feeling like her “throat was closing up.” Pulmonary function demonstrated a severe obstructive pattern. Her dyspnea continued to progress and eventually the patient reportedly became apneic at home and required resuscitation. A computed tomography scan demonstrated a near obstructing lesion of the distal trachea (Figure 1). She was emergently transferred to our institution. Bronchoscopy revealed a near obstructing polypoid mass 7 cm from the vocal cords (Figure 2). The lesion was detached from the tracheal wall with a snare cautery. The mass was located 3 cm proximal to the carina and 7 cm distal to the vocal cords. The mass measured 2 × 1.8 × 1.3 cm and frozen section analysis revealed a poorly differentiated carcinoma. Five days later, she underwent a sternotomy with resection of 2 cm of trachea, 3 cm proximal to the carina. Subsequently, final pathology demonstrated an anaplastic lymphoma kinase positive large B-cell lymphoma. Resection margins were free of tumor. Immunohistochemistry was positive for anaplastic lymphoma kinase-1, CD138, and MUM1. Ebstein-Barr encoded RNA was negative as was CD30 and CD20. One paratracheal lymph node was involved out of 17 sampled. Tumor was stage II by the Ann Arbor staging system. Six cycles of cyclophosphamide, doxorubicin, vincristine, and prednisolone chemotherapy were administered postoperatively. At 4-month follow-up, the patient is alive and well without clinical or radiographic evidence of recurrent disease.

Figure 1
Figure 1
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Figure 2
Figure 2
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Primary tumors of the trachea represent about 0.1% of all malignancies.1 Primary non-Hodgkin's lymphoma of the trachea is even more uncommon, representing 0.23% of all tracheal tumors.2 The most comprehensive review of the literature identified only 28 primary tracheal lymphomas from 1973 to 2000.3 Median age of presentation was 44 years (range of 4–81). Common presenting symptoms include dyspnea, cough, stridor, and wheezing. Hemoptysis is uncommon given the submucosal location of tracheal lymphomas. Patients are frequently treated erroneously for asthma or bronchitis on initial presentation. Symptomatic progression leads to bronchoscopy or computed tomography scan. Tracheal lymphomas may also present with impending airway obstruction as seen in our patient. Airway obstruction has been observed in 87% of patients with 54% requiring an emergent intervention.3

The first management priority is establishment of an airway by coring out endoluminal tumor. This also establishes a diagnosis. Given the rarity of primary tracheal lymphomas, management remains anecdotal. Surgery, chemotherapy, and radiation have all been used either alone or in combination. Combined chemotherapy and radiation is generally administered for localized non-Hodgkin's lymphoma. However, surgery is often undertaken for early stage disease in locations such as the small intestine and epidural space, either alone or in combination with chemotherapy. A primary surgical approach, as undertaken in this case, has been used successfully in treatment of primary tracheal lymphomas.2–4 Others have undergone successful treatment with chemotherapy.3,5 Outcomes are generally favorable, however, 22% of patients die of disease progression with histologic subtype possibly impacting prognosis.4

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REFERENCES

1. Macchiarini P. Primary tracheal tumors. Lancet Oncol 2006;7:83–91.

2. Fidas P, Wright C, Harris LH, et al. Primary tracheal non-Hodgkin's lymphoma: a case report and review of the literature. Cancer 1996;77:83–91.

3. Takimi A, Okumura H, Maeda Y, et al. Primary tracheal lymphoma: case report and literature review. Int J Hematol 2005;82:338–342.

4. Cai C, Jiang RC, Li ZB, et al. Two-stage tracheal reconstruction of primary tracheal non-Hodgkin's lymphoma with nitinol mesh stent and cervical myocutaneous flap. Ann Thorac Surg 2008;85:e17–e19.

5. Tan DSW, Eng PTC, Thye LS, et al. Primary tracheal lymphoma causing respiratory failure. J Thorac Oncol 2008;3:929–930.

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© 2010International Association for the Study of Lung Cancer

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