A long-standing endobronchial non-Hodgkin's lymphoma: Designed to be cured.

Sir,

Primary pulmonary lymphoma (PPL) itself is a rare occurrence representing about <1% of all non-Hodgkin's lymphoma (NHL) cases and 0.5–1% of primary pulmonary malignancies.[1] Endobronchial involvement in NHL is a rare entity. The endobronchial location of the lesion causes postobstructive intrinsic collapse which in its initial phases may be mistaken to be a consolidation. Isolated occurrence of chest symptoms such as cough, chest pain, and dyspnea without any systemic manifestation may be even more misleading. The first case of endobronchial lymphoma was reported in 1955[2] and a few cases have been reported since then. Less than five cases have been reported from India after the first case reported by Barthwal et al.[3]

A 17-year-old male presented with right-sided chest pain, breathlessness, and nonproductive bouts of cough. X-ray of the chest showed collapse of the right middle and lower lobe [Figure 1], which was further confirmed by computed tomography (CT) scan [Figure 2]. Bronchoscopy showed an intraluminal pearly white growth in the intermediate lobe just within 2 cm of the carina [Figure 3]. Bronchoscopic resection of the mass lesion was carried out using rigid bronchoscope, and this was followed by chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisolone 6 cycles every 21 days. The patient showed a very good response to therapy, and CT scan performed also showed clearing of the lesion [Figure 4]. Repeat bronchoscopy was done to see the endoluminal growth and showed clearing of the lesion.

Figure 1

Chest skiagram showing collapse of the right middle lobe and segmental collapse of the right lower lobe

Figure 2

Computed tomography scan showing collapse of the right middle lobe due to obstruction of the right main bronchus

Figure 3

Endobronchial growth in the right intermediate bronchus

Figure 4

Computed tomography scan of the thorax showing clearing of the lesion in the right intermediate lobe bronchus

The lymphoid tissue associated with respiratory epithelium is called bronchus-associated lymphoid tissue (BALT). These lymphoid cells may show uncontrolled proliferations in certain conditions and grow into lymphomas. The larger airways can be involved by direct invasion by parenchymal or mediastinal mass, lymphatic or hematogenous spread or even by aspiration of tumor emboli, direct invasion from mediastinal structures, and lymphatic spread being the most common.[4] These lymphomas are usually of B-cell type, but in our case, it was of the T-cell variety. They remain localized for a long period and have good prognosis as in our case. Our patient remained asymptomatic for 1½ years with episodes of nonproductive cough which never bothered him much. He could carry out his activities without much distress. His symptoms had aggravated only in a month's time before presenting to the physician. However, on the other hand, presentation may be aggressive with unfavorable outcomes.

Depending on the presentation and pattern of involvement of the bronchial walls, NHL may be classified into two types. Type 1 refers to lesions presenting as diffuse mucosal infiltrates derived from lymphatic or hematogenous spread as a part of systemic involvement, and Type 2 lesions are those that remain localized and have no evidence of systemic disease.[5]

Looking at the indolent course of the disease and its localized behavior, our case is categorized at Type 1. Immunohistochemical analysis helps in diagnosing the type of BALT PPL and deciding further management. The presence of CD3 indicates small T-cell lymphocytes as in our case whereas the presence of CD19 and CD20 indicates B-cell origin of lymphoma. The presence of CD5 and CD10 indicates high-grade lymphoma.[6]

In the case presented, the tissue was found to be strongly positive for leukocyte-specific antigen, CD3, CD5 and negative for CD20. Hence, the patient was managed with excision of the endobronchial lesion using rigid bronchoscopy which was followed by chemotherapy using CHOP regimen. The patient responded very well to treatment, and his symptoms improved as well as lesions vanished by the end of treatment.

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Conflicts of interest

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