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Endobronchial non-Hodgkin's lymphoma presenting as an isolated endobronchial mass
Clinical Radiology (2006) 61, 202–205
CASE REPORT
Endobronchial non-Hodgkin’s lymphoma presenting as an isolated endobronchial mass M. Janga, Y.W. Choia,*, S.C. Jeona, C. Parka, H.J. Yoonb Departments of aDiagnostic Radiology, and bPulmonary Medicine, College of Medicine, Hanyang University, Seoul 133-792, South Korea
Introduction Lymphomatous involvement of the thorax may be present in up to 43% of patients with non-Hodgkin’s lymphoma (NHL).1 Mediastinal adenopathy is the most frequent manifestation of NHL, but involvement of the pulmonary parenchyma and/or tracheo-bronchial tree is recognized. Endobronchial involvement with NHL is very rare and usually occurs in the setting of widely disseminated disease.2–4 Although some cases have reported NHL mostly confined to the major bronchi,1–7 it is difficult to call them cases of primary endobronchial NHL, i.e., cases in which NHL originated from the airway, as only a few described the computed tomography (CT) features.1,4 We report a case of isolated primary endobronchial NHL presenting as an endobronchial mass on CT examination in which systemic evaluation did not reveal any additional involvement. Our institutional review board does not require the patient’s informed consent for this kind of retrospective study.
Case Report A 62-year-old woman was admitted with multiple joint pains. She did not have any pulmonary symptoms. She had had systemic lupus erythematosus (SLE) for 10 years. Laboratory studies on admission showed a thrombocytopenia (platelet count of 12,000/mm3) and a normal white blood cell (WBC) count (7800/ml) with neutrophilia (85%) and lymphopenia (11%). The blood chemistry profile was within normal limits. Pulmonary function tests * Correspondent: Y.W. Choi, Department of Diagnostic Radiology, Hanyang University Hospital, 17 Haengdang-Dong, Sungdong-Gu, Seoul 133-792, South Korea. Tel.: C82 2 2290 9161; fax: C82 2 2293 2111. E-mail address: [email protected] (Y.W. Choi).
showed a mild restrictive defect. A chest radiograph showed cardiomegaly and bilateral reticular shadowing in the lower zones (Fig. 1(a)). The reticular shadowing was detected 6 years before, and had progressed slowly since then. Eight days later, collapse of the right upper lobe was noted on a follow-up chest radiograph (Fig. 1(b)). A chest CT examination was performed. This showed a slightly enhancing, endobronchial mass causing bronchial obstruction and near total collapse of the right upper lobe. Enlarged lymph nodes of 1–1.2 cm in diameter were noted in the right lower paratracheal area (Fig. 1(c) and (d)). Scattered areas of ground glass and honeycombing opacities and traction bronchiectasis were noted at the lung bases (Fig. 1(e)). Multiple CT studies, obtained since 6 years before, showed slow progression of the lung lesions. At bronchoscopy, a hypervascular mass totally occluding the right upper lobar bronchus was visualized. Histopathological analysis of the bronchoscopic biopsy material revealed diffuse large B-cell lymphoma infiltrating the bronchial submucosa. A number of staging investigations, including abdominal and pelvic CT, bone scintigraphy, and bone marrow biopsy did not reveal any additional lymphomatous involvement. The patient responded to CHOP (cyclophosphamide, adriamycin, vincristine, and prednisone) chemotherapy. Four months after treatment, there was complete resolution of the endobronchial mass on follow-up CT. The right lower paratracheal lymphadenopathy and pulmonary abnormalities remained unchanged (Fig. 1(f) and (g)). One year later she remains free of disease.
Discussion The radiological features of pulmonary NHL have been classified into bronchovascular/lymphangitic, pulmonary nodular, pneumonic/alveolar, and
0009-9260/$ - see front matter Q 2005 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.
Endobronchial non-Hodgkin’s lymphoma presenting as an isolated endobronchial mass
Figure 1 (continued )
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Figure 1. Endobronchial NHL in 62-year-old woman. (a) Initial chest radiograph shows cardiomegaly and reticular shadowing in both lower lung zones. (b) Chest radiograph obtained 8 days later shows new collapse of the right upper lobe. Contrast-enhanced CT images at the level of the aortic arch (C) and carina (D), obtained on the same day as (b), demonstrate lymph node in the right paratracheal area (arrow, c), and endobronchial mass obstructing the right upper lobar bronchus (arrow, d). (e) Chest CT at the level of the lung base, obtained on the same day as (b) with the patient in the prone position, shows reticular shadowing and traction bronchiectasis in the right lower lobe. (f) Contrast-enhanced CT at a level similar to (c), obtained 4 months after treatment, reveals a persistent paratracheal lymph node (black arrow) unchanged in size. Note unenhanced azygos vein (white arrow). The endobronchial mass had resolved completely after chemotherapy (not shown). (g) Chest CT at a level similar to (e), obtained on the same day as (f) with the patient in the supine position, shows persistent reticular shadowing and traction bronchiectasis in the right lower lobe.
military-haematogenous patterns.8 Endobronchial lymphoma refers to either secondary invasion of a bronchus from adjacent nodes or a discrete nodule arising from within the bronchus8 and is a subtype of bronchovascular-lymphangitic NHL. It is thought to be the rarest intrathoracic manifestation of NHL. Endobronchial NHL is usually associated with systemic involvement.2–4 Although cases of NHL mostly confined to the major bronchi have been rarely reported,1–7 it is uncertain whether they were endobronchial in origin, in the strictest sense of the word. For cases to be endobronchial in origin, no additional lymphomatous involvement should be demonstrated by systemic investigations, including CT of the chest, abdomen, and pelvis, bone scintigraphy, and bone marrow biopsy. However, this was not the case in previously reported cases: regional lymph nodes were also involved;4,6 chest CT was not performed, which might have revealed
M. Jang et al.
coexisting lymph nodes inconspicuous on chest radiographs;2,3,5–7 or coexisting lesions were not mentioned.1 The present case of NHL presented as an endobronchial mass obstructing the right upper lobar bronchus on CT. Although lymph nodes were observed in the right paratracheal area, they were unlikely to have been affected by lymphoma because they did not change in size in conjunction with the resolution of the endobronchial lesion after chemotherapy. The case also showed ground glass opacities, traction bronchiectasis, and honeycombing at bilateral basal lung areas. However, these lung lesions were also not considered to lymphomatous, because they had been present and progressed slowly for 6 years until the diagnosis of endobronchial lymphoma. The lung lesions also remained unchanged 4 months after chemotherapy that resulted in the resolution of the endobronchial lesion. In addition, honeycombing and traction bronchiectasis suggest the presence of pulmonary fibrosis, unusual in untreated lymphoma. These findings suggest that the pulmonary abnormalities were SLE-associated interstitial lung disease. Staging work-up, including CT of the chest and abdomen and bone marrow examinations, did not disclose any additional lymphomatous involvement. To the authors’ knowledge, this is the first definitive case of primary endobronchial NHL confined to the tracheobronchial tree. Rose et al.6 classified endobronchial NHL into two types on the basis of the clinical and radiological appearances. The more common form, type 1, occurs in the presence of systemic lymphoma and shows diffuse submucosal infiltrates. The rarer form, type 2, occurs in the absence of clinically apparent systemic lymphoma and shows central airway involvement by a solitary mass. The present case would be categorized as type 2. Endobronchial lymphoma may originate from direct bronchial invasion from parenchymal disease or mediastinal tumour, lymphatic spread, transbronchial aspiration of tumour emboli, or direct haematogenous metastasis.6 The most common mechanisms probably involve direct bronchial invasion and lymphatic spread. However, all of these mechanisms seem unlikely in view of the absence of any detectable lesions elsewhere. A more plausible explanation would be the de novo formation of a NHL in the bronchus-associated lymphoid tissue (BALT).6 This tissue consists of lymphoid aggregates and is localized at airway bifurcations. These BALT lymphomas, mostly B-cell in type, tend to remain localized and respond favourably to therapy. We can only speculate a relationship to BALT in this case.
Endobronchial non-Hodgkin’s lymphoma presenting as an isolated endobronchial mass
Endobronchial lymphoma may mimic lung cancer radiologically and cannot be differentiated from it using CT. The differential diagnoses of a focal endobronchial mass, as depicted in this case, should include many other diseases, including carcinoid tumour, mucoepidermoid tumour, endobronchial metastasis, inflammatory pseudotumour, Hodgkin’s disease, and fungal infection. Bronchoscopic examination with biopsy is mandatory for definitive diagnosis. In summary, although endobronchial NHL is usually associated with disseminated disease, NHL may present as a solitary endobronchial mass in the central airway without lymphomatous involvement elsewhere. In these cases, bronchoscopic biopsy is essential for diagnosis.
References 1. Mason AC, White CS. CT appearance of endobronchial nonHodgkin lymphoma. J Comput Assist Tomogr 1994;18:559—61.
205
2. Spapen H, Eeckhout E, Volckaert A, Dehou MF, Schandevyl W, van Camp B. Primary endobronchial non-Hodgkin’s lymphoma: report of two cases and review of the literature. Neth J Med 1988;33:86—90. 3. Xaubet A, Santabarbara P, Llebaria C, et al. Endobronchial involvement in non-Hodgkin’s lymphoma. Eur J Respir Dis 1986;69:57—60. 4. Hardy K, Nicholson DP, Schaefer RF, Hsu SM. Bilateral endobronchial non-Hodgkin’s lymphoma. South Med J 1995; 88:367—70. 5. Maeda M, Kotake Y, Monden Y, Nakahara K, Kawashima Y, Kitamura H. Primary malignant lymphoma of the trachea. Report of a case successfully treated by primary end-to-end anastomosis after circumferential resection of the trachea. J Thorac Cardiovasc Surg 1981;81:835—9. 6. Rose RM, Grigas D, Strattemeir E, Harris NL, Linggood RM. Endobronchial involvement with non-Hodgkin’s lymphoma. A clinical–radiologic analysis. Cancer 1986;57:1750—5. 7. Spapen H, Impens N, Eeckhout E, Vandermoten G, Schandevyl W. Bronchial obstruction caused by a solitary endobronchial non-Hodgkin lymphoma. Eur J Respir Dis 1987; 71:130—1. 8. Gollub MJ, Castellino RA. Diffuse endobronchial non-Hodgkin’s lymphoma: CT demonstration. AJR Am J Roentgenol 1995;164: 1093—4.
CASE REPORT
Endobronchial non-Hodgkin’s lymphoma presenting as an isolated endobronchial mass M. Janga, Y.W. Choia,*, S.C. Jeona, C. Parka, H.J. Yoonb Departments of aDiagnostic Radiology, and bPulmonary Medicine, College of Medicine, Hanyang University, Seoul 133-792, South Korea
Introduction Lymphomatous involvement of the thorax may be present in up to 43% of patients with non-Hodgkin’s lymphoma (NHL).1 Mediastinal adenopathy is the most frequent manifestation of NHL, but involvement of the pulmonary parenchyma and/or tracheo-bronchial tree is recognized. Endobronchial involvement with NHL is very rare and usually occurs in the setting of widely disseminated disease.2–4 Although some cases have reported NHL mostly confined to the major bronchi,1–7 it is difficult to call them cases of primary endobronchial NHL, i.e., cases in which NHL originated from the airway, as only a few described the computed tomography (CT) features.1,4 We report a case of isolated primary endobronchial NHL presenting as an endobronchial mass on CT examination in which systemic evaluation did not reveal any additional involvement. Our institutional review board does not require the patient’s informed consent for this kind of retrospective study.
Case Report A 62-year-old woman was admitted with multiple joint pains. She did not have any pulmonary symptoms. She had had systemic lupus erythematosus (SLE) for 10 years. Laboratory studies on admission showed a thrombocytopenia (platelet count of 12,000/mm3) and a normal white blood cell (WBC) count (7800/ml) with neutrophilia (85%) and lymphopenia (11%). The blood chemistry profile was within normal limits. Pulmonary function tests * Correspondent: Y.W. Choi, Department of Diagnostic Radiology, Hanyang University Hospital, 17 Haengdang-Dong, Sungdong-Gu, Seoul 133-792, South Korea. Tel.: C82 2 2290 9161; fax: C82 2 2293 2111. E-mail address: [email protected] (Y.W. Choi).
showed a mild restrictive defect. A chest radiograph showed cardiomegaly and bilateral reticular shadowing in the lower zones (Fig. 1(a)). The reticular shadowing was detected 6 years before, and had progressed slowly since then. Eight days later, collapse of the right upper lobe was noted on a follow-up chest radiograph (Fig. 1(b)). A chest CT examination was performed. This showed a slightly enhancing, endobronchial mass causing bronchial obstruction and near total collapse of the right upper lobe. Enlarged lymph nodes of 1–1.2 cm in diameter were noted in the right lower paratracheal area (Fig. 1(c) and (d)). Scattered areas of ground glass and honeycombing opacities and traction bronchiectasis were noted at the lung bases (Fig. 1(e)). Multiple CT studies, obtained since 6 years before, showed slow progression of the lung lesions. At bronchoscopy, a hypervascular mass totally occluding the right upper lobar bronchus was visualized. Histopathological analysis of the bronchoscopic biopsy material revealed diffuse large B-cell lymphoma infiltrating the bronchial submucosa. A number of staging investigations, including abdominal and pelvic CT, bone scintigraphy, and bone marrow biopsy did not reveal any additional lymphomatous involvement. The patient responded to CHOP (cyclophosphamide, adriamycin, vincristine, and prednisone) chemotherapy. Four months after treatment, there was complete resolution of the endobronchial mass on follow-up CT. The right lower paratracheal lymphadenopathy and pulmonary abnormalities remained unchanged (Fig. 1(f) and (g)). One year later she remains free of disease.
Discussion The radiological features of pulmonary NHL have been classified into bronchovascular/lymphangitic, pulmonary nodular, pneumonic/alveolar, and
0009-9260/$ - see front matter Q 2005 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.
Endobronchial non-Hodgkin’s lymphoma presenting as an isolated endobronchial mass
Figure 1 (continued )
203
204
Figure 1. Endobronchial NHL in 62-year-old woman. (a) Initial chest radiograph shows cardiomegaly and reticular shadowing in both lower lung zones. (b) Chest radiograph obtained 8 days later shows new collapse of the right upper lobe. Contrast-enhanced CT images at the level of the aortic arch (C) and carina (D), obtained on the same day as (b), demonstrate lymph node in the right paratracheal area (arrow, c), and endobronchial mass obstructing the right upper lobar bronchus (arrow, d). (e) Chest CT at the level of the lung base, obtained on the same day as (b) with the patient in the prone position, shows reticular shadowing and traction bronchiectasis in the right lower lobe. (f) Contrast-enhanced CT at a level similar to (c), obtained 4 months after treatment, reveals a persistent paratracheal lymph node (black arrow) unchanged in size. Note unenhanced azygos vein (white arrow). The endobronchial mass had resolved completely after chemotherapy (not shown). (g) Chest CT at a level similar to (e), obtained on the same day as (f) with the patient in the supine position, shows persistent reticular shadowing and traction bronchiectasis in the right lower lobe.
military-haematogenous patterns.8 Endobronchial lymphoma refers to either secondary invasion of a bronchus from adjacent nodes or a discrete nodule arising from within the bronchus8 and is a subtype of bronchovascular-lymphangitic NHL. It is thought to be the rarest intrathoracic manifestation of NHL. Endobronchial NHL is usually associated with systemic involvement.2–4 Although cases of NHL mostly confined to the major bronchi have been rarely reported,1–7 it is uncertain whether they were endobronchial in origin, in the strictest sense of the word. For cases to be endobronchial in origin, no additional lymphomatous involvement should be demonstrated by systemic investigations, including CT of the chest, abdomen, and pelvis, bone scintigraphy, and bone marrow biopsy. However, this was not the case in previously reported cases: regional lymph nodes were also involved;4,6 chest CT was not performed, which might have revealed
M. Jang et al.
coexisting lymph nodes inconspicuous on chest radiographs;2,3,5–7 or coexisting lesions were not mentioned.1 The present case of NHL presented as an endobronchial mass obstructing the right upper lobar bronchus on CT. Although lymph nodes were observed in the right paratracheal area, they were unlikely to have been affected by lymphoma because they did not change in size in conjunction with the resolution of the endobronchial lesion after chemotherapy. The case also showed ground glass opacities, traction bronchiectasis, and honeycombing at bilateral basal lung areas. However, these lung lesions were also not considered to lymphomatous, because they had been present and progressed slowly for 6 years until the diagnosis of endobronchial lymphoma. The lung lesions also remained unchanged 4 months after chemotherapy that resulted in the resolution of the endobronchial lesion. In addition, honeycombing and traction bronchiectasis suggest the presence of pulmonary fibrosis, unusual in untreated lymphoma. These findings suggest that the pulmonary abnormalities were SLE-associated interstitial lung disease. Staging work-up, including CT of the chest and abdomen and bone marrow examinations, did not disclose any additional lymphomatous involvement. To the authors’ knowledge, this is the first definitive case of primary endobronchial NHL confined to the tracheobronchial tree. Rose et al.6 classified endobronchial NHL into two types on the basis of the clinical and radiological appearances. The more common form, type 1, occurs in the presence of systemic lymphoma and shows diffuse submucosal infiltrates. The rarer form, type 2, occurs in the absence of clinically apparent systemic lymphoma and shows central airway involvement by a solitary mass. The present case would be categorized as type 2. Endobronchial lymphoma may originate from direct bronchial invasion from parenchymal disease or mediastinal tumour, lymphatic spread, transbronchial aspiration of tumour emboli, or direct haematogenous metastasis.6 The most common mechanisms probably involve direct bronchial invasion and lymphatic spread. However, all of these mechanisms seem unlikely in view of the absence of any detectable lesions elsewhere. A more plausible explanation would be the de novo formation of a NHL in the bronchus-associated lymphoid tissue (BALT).6 This tissue consists of lymphoid aggregates and is localized at airway bifurcations. These BALT lymphomas, mostly B-cell in type, tend to remain localized and respond favourably to therapy. We can only speculate a relationship to BALT in this case.
Endobronchial non-Hodgkin’s lymphoma presenting as an isolated endobronchial mass
Endobronchial lymphoma may mimic lung cancer radiologically and cannot be differentiated from it using CT. The differential diagnoses of a focal endobronchial mass, as depicted in this case, should include many other diseases, including carcinoid tumour, mucoepidermoid tumour, endobronchial metastasis, inflammatory pseudotumour, Hodgkin’s disease, and fungal infection. Bronchoscopic examination with biopsy is mandatory for definitive diagnosis. In summary, although endobronchial NHL is usually associated with disseminated disease, NHL may present as a solitary endobronchial mass in the central airway without lymphomatous involvement elsewhere. In these cases, bronchoscopic biopsy is essential for diagnosis.
References 1. Mason AC, White CS. CT appearance of endobronchial nonHodgkin lymphoma. J Comput Assist Tomogr 1994;18:559—61.
205
2. Spapen H, Eeckhout E, Volckaert A, Dehou MF, Schandevyl W, van Camp B. Primary endobronchial non-Hodgkin’s lymphoma: report of two cases and review of the literature. Neth J Med 1988;33:86—90. 3. Xaubet A, Santabarbara P, Llebaria C, et al. Endobronchial involvement in non-Hodgkin’s lymphoma. Eur J Respir Dis 1986;69:57—60. 4. Hardy K, Nicholson DP, Schaefer RF, Hsu SM. Bilateral endobronchial non-Hodgkin’s lymphoma. South Med J 1995; 88:367—70. 5. Maeda M, Kotake Y, Monden Y, Nakahara K, Kawashima Y, Kitamura H. Primary malignant lymphoma of the trachea. Report of a case successfully treated by primary end-to-end anastomosis after circumferential resection of the trachea. J Thorac Cardiovasc Surg 1981;81:835—9. 6. Rose RM, Grigas D, Strattemeir E, Harris NL, Linggood RM. Endobronchial involvement with non-Hodgkin’s lymphoma. A clinical–radiologic analysis. Cancer 1986;57:1750—5. 7. Spapen H, Impens N, Eeckhout E, Vandermoten G, Schandevyl W. Bronchial obstruction caused by a solitary endobronchial non-Hodgkin lymphoma. Eur J Respir Dis 1987; 71:130—1. 8. Gollub MJ, Castellino RA. Diffuse endobronchial non-Hodgkin’s lymphoma: CT demonstration. AJR Am J Roentgenol 1995;164: 1093—4.