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A 63-yr-old-woman presented with pulmonary infiltrates and macroglobinemia. Open lung biopsy revealed an interstitial plasma cell infiltrate. Immunocytochemical staining to determine clonality of the infiltrate was negative for both kappa and lambda light chains and therefore was not helpful. Polymerase chain reaction (PCR) amplification of the CDR-III region of the immunoglobulin heavy chain gene revealed that the plasma cells were either monoclonal or biclonal, thus suggesting the diagnosis of a plasmacytoid pulmonary lymphoma. The advantages and limitations of the PCR technique over the traditional method of detecting gene rearrangements, i.e., restriction-digestion of DNA and Southern blotting, are discussed. To our knowledge this patient represents the first reported application of the PCR technique for detecting gene rearrangements to determine the clonality of a lymphoid infiltrate in the lung. This technique can also be applied to determine the clonality of lymphocytes obtained by bronchoalveolar lavage or needle biopsy, or from a pleural effusion.

We studied 70 patients with biopsy-proven pulmonary non-Hodgkin's lymphomas without extrathoracic involvement or mediastinal adenopathy to determine the clinical, imaging, and endoscopic features of this condition in a homogeneous series. In low-grade (LG) lymphomas, symptoms were cough, dyspnea, chest pain, hemoptysis. Imaging features consisted of localized alveolar opacities, infiltrative diffuse opacities, atelectasis, and pleural effusions. Inflammatory changes of the mucosa were present in some patients, leading to bronchial stenosis in 7; biopsies showed lymphomatous infiltration in 12. Prognosis of LG lymphomas was excellent, with 93.6 percent survival at five years. High-grade lymphomas differed from LG lymphomas principally by a more aggressive course and a worse survival. Inflammatory changes occurred in seven of nine cases leading to stenosis in two, and biopsies showed lymphomatous involvement in five. The profile of primary pulmonary lymphomas in this study could help clinicians consider this condition and prompt them to evaluate new diagnostic tools.

症例1は48歳の女性. 職場検診で胸部異常陰影を指摘されたため来院した. 右肺中葉にair bronchogramを伴った浸潤影がみられ, 気管支鏡下生検でリンパ腫が疑われた. 平成3年4月18日, 右肺中葉切除および縦隔リンパ節郭清術を行った. 病理診断は非ポジキンリンパ腫 (びまん性, 小細胞型) であった. 補助療法は行わずに退院し, 術後1年9カ月の現在再発の徴候なく経過観察中である.症例2は38歳の男性. 感冒で来院した際, 左舌区に辺縁が鮮明で, air bronchogramを伴わない腫瘤陰影を指摘され入院となった. 術前検査では確定診断はえられず, 平成3年10月17日肺腫瘍の疑いで左肺上葉切除および縦隔リンパ節郭清術を行った. 病理診断は非ポジキンリンパ腫 (びまん性, 小細胞型) であったが, 症例1に比べ細胞の異型性が強く, 肺内リンパ節にも腫瘍性病変がみられたため, 術後CHOP療法を2クール施行した. 術後1年3カ月の現在再発の徴候なく外来通院中である.本疾患は稀な疾患ではあるが, その報告例は最近増加してきており文献的考察を加え報告する

A 69-year-old woman was examined due to abnormal pulmonary shadows on her chest roentgenogram. Although small lymphocyte proliferation was revealed in specimens by transbronchial lung biopsy (TBLB), immunoglobulin light chain restriction could not be seen. We attempted to verify the B-cell clonality by broncho-alveolar lavage (BAL) and a new sensitive method called kappa-lambda imaging (KLI), which was available for the detection of monoclonal B cells. Therefore, B-cell monoclonality was found. Thus, the patient was diagnosed as having primary pulmonary lymphoma (PPL). PPL may be differentiated from benign lymphoproliferative disorders such as pseudolymphoma and lymphocytic interstitial pneumonia by KLI of BAL-derived lymphocytes.

We report a case of primary pulmonary classical Hodgkin lymphoma (CHL) in a 58-year-old woman. Twelve years ago, the patient complained of slight fever and weight loss. A mass of about 5 cm in diameter was seen in the right lung on radiography and computed tomography (CT). Right total pneumonectomy and resection of mediastinal lymph nodes were performed. A pathological examination led to a strong suspicion of Hodgkin disease (HD) (now referred to as CHL), but a definite diagnosis could not be made at the time. Six years later, a chest CT showed a tumor around the ascending aorta, which was treated successfully by radiation therapy. Six years later, the chest CT revealed a tumor in the anterior mediastinum. CHL was diagnosed based on an immunohistochemical re-examination of lung specimens resected 12 years earlier and CT-guided fine needle tumor biopsy specimens of the second recurrent tumor in the anterior mediastinum were compatible with the recurrence of CHL. Therefore, we diagnosed this case as primary pulmonary CHL that later relapsed in the mediastinum. The tumor size was reduced by radiation therapy and the patient is currently under observation as an outpatient.

Sjogren syndrome is a slowly progressing autoimmune disease. Pulmonary manifestations are frequent in primary Sjogren syndrome but often not clinically significant; the most common are xerotrachea, interstitial lung diseases, and small airway obstruction. Pulmonary manifestations in Sjogren syndrome have a slow progression and favorable prognosis, with the exception of primary pulmonary lymphoma and pulmonary hypertension.

Primary pulmonary lymphoma (PPL) is an uncommon tumour arising from mucosa-associated lymphoid tissue (MALT). It develops from B lymphocytes predominantly and shows light chain restriction. Six cases are presented. Surgery is a method of treatment but local recurrence is a problem with limited resection. Chemotherapy and radiotherapy have a role to play in residual or recurrent disease.

Mucosa-associated lymphoid tissue-derived (MALT) lymphoma, a low grade B-cell extranodal lymphoma, is the most frequent subset of primary pulmonary lymphoma. Our objective was to evaluate the initial extent of disease and to analyse the characteristics and long-term outcome of these patients. All chest and pathological departments of teaching hospitals in Paris were contacted in order to identify patients with a histological diagnosis of primary pulmonary lymphoma of the MALT subtype. 63 cases were identified. The median age was 60 yrs. 36% of cases had no symptoms at diagnosis. 46% of patients had at least one extrapulmonary location of lymphoma. The estimated 5- and 10-yr overall survival rates were 90% and 72%, respectively. Only two of the nine observed deaths were related to lymphoma. Age and performance status were the only two adverse prognostic factors for survival. Extrapulmonary location of lymphoma was not a prognostic factor for overall survival or for progression-free survival. Treatment with cyclophosphamide or anthracycline was associated with shorter progression-free survival, when compared with chlorambucil. The survival data confirm the indolent nature of pulmonary MALT lymphoma. Better progression-free survival was observed with chlorambucil when compared with cyclophosphamide or anthracycline.

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Non-Hodgkin's lymphoma (NHL) involving the endobronchial tree is uncommon, and the initial presentation of NHL as an endobronchial tumor is extremely rare. In a series of 441 patients with newly diagnosed non-Hodgkin's lymphoma over a 7-year period, we reviewed the clinical features of eight patients who presented with an endobronchial tumor. All patients had local pulmonary disease without extrathoracic involvement. The major presenting symptoms were dyspnea, chest pain, cough, and hoarseness. None of the patients had systemic symptoms. Radiographs revealed lobar collapse in all cases. Five patients had mediastinal masses and three had isolated endobronchial lesions. Although MALT lymphoma is the most common primary pulmonary lymphoma, it was present in only one of our patients, while seven patients had aggressive lymphoma. All patients received chemotherapy. Six of the eight patients responded favorably to treatment with complete remission. The prognosis of patients with isolated endobronchial lymphoma is not worse than other local presentations of lymphoma. Bronchoscopic examination with biopsy is essential to differentiate these lesions from primary bronchongenic carcinoma.

The incidence of synchronous lung tumors is rare, as reported in various clinical series, ranging from 0.2% to 8%. Most reported cases of synchronous tumors were shown to have the same histologic types of lung cancer. Among possible combinations, squamous cell carcinoma was by far the most common. Primary pulmonary lymphoma (PPL) is very rare in clinics accounting for only 0.5-1% of primary lung tumors. There is no report about synchronous primary pulmonary adenocarcinoma presenting with lung lymphoma. It can be easily misdiagnosed or missed. Although the treatment of PPL and synchronous pulmonary tumors has controversial, surgery with/without postoperative adjuvant radio-chemotherapy are used for most patients in present. We describe a case of synchronous primary lung tumors presenting with lymphoma and adenocarcinoma, in which expression of the cell surface antigens were evaluated immunohistochemically. By taking into consideration of the reported experiences, the author discusses the clinical features, prognostic criteria and therapeutic management of synchronous lung cancer and PPL.

Background The purpose of this study was to define the role of surgery in the treatment of primary pulmonary lymphoma (PPL). Methods We reviewed all patients presenting with a pathologic diagnosis of PPL in our institution during the past 20 years. We analyzed the outcome and determined the impact of complete versus incomplete surgical resection on survival. Results The study included 17 patients with PPL confined to the pulmonary parenchyma. Pathologic diagnosis was low-grade B-cell PPL in 14 patients, high-grade B-cell PPL in 2, and lymphomatoid granulomatosis in 1. A complete resection was performed in 8 patients presenting with low-grade B-cell PPL and in 2 patients with high-grade B-cell PPL. The overall survival of patients presenting with low-grade B-cell PPL was 63% at 10 years; however, survival at 10 year tended to be better when a complete resection was performed (87.5% vs 25%, respectively; p = 0.08). Gender, bilateral disease, or adjuvant therapy did not affect survival. Both patients presenting with high-grade B-cell PPL are alive and free of disease 22 and 36 months after the surgery, respectively. The patient presenting with lymphomatoid granulomatosis is alive after 2 years of follow-up. Conclusions PPL is a rare disease that may be localized or diffuse in one or both lungs. Surgery should be the treatment of choice in the localized form of PPL if complete resection can be achieved. A complete resection is associated with an excellent long-term survival of almost 90%. If the lesions are diffuse or involve both lungs, medical therapy should then be the treatment of choice.

Primary pulmonary lymphoma (PPL) is a rare disease with a favorable prognosis compared with lung cancer. Although a number of histologic variables of B- and T-cell lymphoma were reported as PPL, marginal zone lymphoma of the mucosa-associated lymphoid tissue type is by far the most frequent diagnosis. This review summarizes the present knowledge of histopathology, molecular biology, diagnosis, prognosis, and treatment of this heterogeneous entity.

Bronchial-associated lymphoid tissue (BALT) lymphoma is a distinct subgroup of low-grade B-cell extranodal non-Hodgkin's lymphoma, classified as marginal-zone lymphoma. This study was performed in order to assess the natural history of this rare entity. We evaluated retrospectively the clinical data of 22 patients with biopsy-proven BALT lymphoma at two tertiary-care institutions from 1996 to 2002. Immunophenotyping was done to confirm the abnormal populations of B-lymphoid cells in all cases, and clonality was determined by flow cytometry or molecular studies. There were 11 men and 11 women in the sample, median age 61 years (range 21-80 years); nine were asymptomatic at diagnosis. All 13 symptomatic patients had non-specific pulmonary complaints. On computed tomographic examination of the chest, 11 patients had bilateral disease, 12 had lung nodules, and 10 had a mass or air-space consolidation. In all but one case the disease was localised to the lung at diagnosis and none had peripheral blood or bone marrow involvement. Out of 22 patients, 20 received treatment in various combinations, 12 had chemotherapy and/or rituximab, six had surgery, and two received radiation therapy as primary treatment. A complete response (CR) was achieved in nine patients and a partial response was obtained in 10 patients. Seven of 10 patients who had unilateral disease achieved a CR. The estimated progression-free survival was 53 months. All patients were alive during the median follow-up period of 36 months (range 12-76 months). It appears that BALT lymphoma tends to be localised to lung at the time of diagnosis, responds well to local or systemic therapy, and has a favourable prognosis.

F 34-year-old woman was referred for evaluation of a chest roentgenogram abnormality. The patient, a nonsmoker, as immunocompetent with no risk behavior for HIV. She eported a sudden cough that developed, followed by pain in er left anterior chest radiating to the shoulder. The cough was ccompanied by subsequent development of bloody mucus. er symptoms persisted for 1 week with no prior history of imilar episodes. On physical examination, diminished breath sounds were uscultated over the left chest. Chest roentgenogram (Fig 1) emonstrated a large left upper lobe opacification with poserior displacement of the major fissure. Subsequent workup ncluded computed tomographic imaging of the thorax (Fig 2) nd a bronchoscopy, which did not provide the diagnosis. Due o the large size of the mass, a tissue biopsy was not pursued ecause it would not impact the plan for thoracotomy and esection. The patient underwent a left thoracotomy, revealing a large, olid mass filling the hemithorax from hilum to apex. An ntraoperative frozen section evaluation of the specimen sugested lymphoma. A pneumonectomy was performed. Sevral factors were judged to prohibit completion of a lesser peration (lobectomy and wedge resection). Calcific granuloatous disease involved the hilum and fissure, causing fusion f the two lobes. In addition, the primary lesion was considrable in size and was accompanied by a satellite nodule. In act, resection of portions of the pericardium and left phrenic erve were required in conjunction with resection of the pecimen. Individual nodes from the inferior pulmonary ligig 1.

Primary pulmonary lymphoma arising from mucosa- or bronchus-associated lymphoid tissue (MALT or BALT) is a rare disease.1,2 We report a 30 year old man with low grade B cell BALT lymphoma who presented with a bulky mass in the lung and was treated successfully with cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) combined chemotherapy. The patient was admitted with a dry cough, shortness of breath, back pain, and progressive infiltrates on chest radiographs. He had no …

Three distinct entities are now covered by the definition of primary pulmonary clonal lymphoid proliferation. The aim of this review is to describe the pathophysiological, diagnostic, prognostic and therapeutic aspects of these three entities. Low-grade pulmonary B-cell lymphoma is the most frequent form of primary pulmonary clonal lymphoid proliferation. It arises from mucosa-associated lymphoid tissue. It is usually indolent and appears in the form of a chronic alveolar opacity. The prognosis is excellent, but treatment is controversial (simple monitoring, surgery or single-agent chemotherapy). High-grade pulmonary B-cell lymphoma is far rarer and usually occurs in individuals with an underlying disorder (e.g. immunodeficiency). The prognosis is poor and therapeutic options depend on the underlying disorder. The inclusion of lymphomatoid granulomatosis in the definition of primary pulmonary lymphomas is controversial. The clonal nature of the proliferation is very rarely demonstrated and extrapulmonary involvement is frequent (upper airways, skin, kidneys, central nervous system, etc.). The prognosis is extremely variable, with some authors reporting complete remission with steroids and cyclophosphamide and others reporting failure of combination chemotherapy.

Los linfomas no Hodgkin anaplásicos de células grandes Ki-1 positivo son una entidad recientemente reconocida, poco frecuentes y caracterizados por la expresión del antígeno CD30. Su forma mas frecuente de presentación es la de adenopatías periféricas con afectación mediastínica, acompañada en aproximadamente la mitad de los casos de afectación extranodal, siendo la piel el lugar mas común; y siendo infrecuente que se presenten con afectación del pulmón, la médula ósea y el sistema nervioso central. Por tanto los linfomas no Hodgkin pulmonares primarios anaplásicos de células grandes Ki-l (+) son una entidad clínica rara. Se clasifican y clínicamente se comportan como linfomas de alto grado de malignidad, que en la mayoría de casos se encuentran en estadios clínicos avanzados en el momento del diagnóstico. Se presentan con mayor incidencia en gente joven, donde el pronóstico es mas favorable. Se describen las características clínicas, morfológicas e inmunofenotípicas de este tipo de linfomas. Ki-l positive anaplastic large cell non-Hodgkin´s lymphomas are a recently recognized entity, unusual and characterized by the expression of CD30 antigen.The most usual clinical feature is peripheral lymphadenopathy with mediastinal sparing and extranodal disease which occurs in approximately half of the cases, with the skin as the most common site; lung, bone marrow and central nervous system involvement are uncommon. Therefore primary pulmonary Ki-l positive anaplastic large cell non-Hodgkin´s lymphomas are an uncommon clinical entity. Their classification and clinical behaviour look like high grade malignancy lymphomas, that in the most cases are presented in advanced stages disease to the diagnostic. They are presented with higher incidence in young people, where prognostic is more favourable. Clinical, morphologic and inmunophenotypic features of this lymphoma type are reported.

We reviewed pathologic, phenotypic, and clinical features of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) type primarily involving lung to address unresolved questions regarding behavior and pathologic features of unambiguously diagnosed pulmonary MALT lymphoma. Lung specimens from 50 patients were reviewed. Forty-one had low-grade MALT lymphoma. Nine had low-grade MALT lymphoma and diffuse large B-cell lymphoma. The patients included 32 women and 18 men with a median age of 68 years (range 34-88 years). Half of the patients were asymptomatic at the time lymphoma was diagnosed. Radiographic abnormalities were more commonly unilateral (37 patients) than bilateral (12 patients). Localized masses or nodules occurred in 39 patients. Associated autoimmune disorders (29%) and monoclonal gammopathies (43%) were common. Low-grade lymphomas formed intraparenchymal masses composed of centrocyte-like cells, plasmacytoid lymphocytes, and plasma cells that formed lymphoepithelial lesions and exhibited a lymphangitic growth pattern. Mediastinal lymph nodes were involved histologically in 44% of cases. Lymphoma-specific survival was 71.7% at 10 years, and overall survival was significantly worse than age-and gender-matched control patients. None of the following features predicted those patients who had an adverse outcome: systemic symptoms, presence of autoimmune disorders or paraproteinemia, anatomic distribution and number of pulmonary lesions, lymph node involvement, or presence of anthracycline-treated large B-cell lymphoma.

A 68-year-old man was admitted to our hospital for cough and dyspnea. Laboratory data showed positive human T-lymphotropic virus type 1 (HTLV-1) antibody and elevated level of soluble interleukin-2 receptor (4225 U/ml). Thoracic computed tomography showed emphysema, reticular shadow, ground-glass attenuation, and subpleural consolidation (Figure 1). Bronchofiberscopy was carried out and cytologic examination of bronchoalveolar lavage fluid revealed atypical lymphocytes. These cells showed positive reaction for CD2, CD3, CD4, CD5, and CD25 and negative for CD8 in flow cytometry. Specimens of transbronchial lung biopsy showed massive infiltration of malignant lymphocytes, which were

A 55-year-old woman with primary pulmonary lymphoma is presented. Contrast enhanced CT through the right lower lobe mass demonstrated the "CT angiogram sign" previously reported to be highly specific for bronchioloalveolar carcinoma.

Nodular amyloidomas (NA) of the lung are non-neoplastic inflammatory nodules containing eosinophilic amyloid deposits and a lymphoplasmacytic infiltrate. In some instances, the extensive amyloid deposits may obscure an underlying lymphoproliferative disorder. The histologic and immunohistologic features that discriminate these two differential diagnostic possibilities were studied in this series of six cases of NA and five cases of primary low-grade malignant lymphomas of lung with secondary amyloid deposits (ML). Two of lymphoma cases showed histopathologic and immunophenotypic features of B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (B-cell CLL/SLL), and three cases were low-grade B-cell lymphoma derived from mucosa associated lymphoid tissue (MALT lymphoma). Key discriminating morphologic features between NA and ML included lymphatic tracking of the cellular infiltrate (3/5 ML; 1/6 NA), pleural infiltration (3/5 ML; 0/6 NA), sheet-like masses of plasma cells (5/5 ML; 0/6 NA) and reactive follicles (4/5 ML; 1/6 NA). Lesional circumscription, vascular and bronchial destruction, lymphoepithelial lesions, and granulomas were not helpful discriminators. Immunohistochemical features indicating a dominant CD20+, CD79a+ B-cell population (5/5 ML; 0/6 NA), light chain restriction (4/5 ML; 0/6 NA), and aberrant antigen expression of CD20/CD43 (2/5 ML; 0/6 NA) were helpful. Amyloid tumors with a reactive lymphoplasmacytic infiltrate can be separated from low-grade malignant lymphomas utilizing both histologic and immunohistochemical features.

Background . Primary non-Hodgkin's lymphoma of the lung is a rare entity. Although the prognosis is favorable, clinical features, prognostic factors, and patient management have not been clearly defined. Methods . We reviewed retrospectively the records of 48 patients operated on for primary pulmonary non-Hodgkin's lymphoma. The study group consisted of 21 male (44%) and 27 female (56%) patients with a mean age of 61.8 years. Thirty-seven and a half percent of patients were asymptomatic, and 62.5% were seen with pulmonary symptoms, systemic symptoms, or both. A definitive diagnosis was obtained by thoracotomy in 90% of patients, thoracoscopy in 8%, and anterior mediastinotomy in 2%. Results . Complete surgical resection was possible in 19 patients (40%). A mucosa-associated lymphoid tissue lymphoma (MALT) was found in 35 patients and lymphoma that was not of this type, in 13. The 1-year, 5-year, and 10-year survival rates were 91%, 68%, and 53%, respectively in the group with mucosa-associated lymphoid tissue lymphoma and 85%, 65%, and 64% in the group with lymphoma that was not of the mucosa-associated lymphoid tissue type. None of the prognostic factors studied (mode of presentation, smoking history, bilateral disease, postoperative stage, complete resection, adjuvant chemotherapy, histology) significantly influenced patient survival. Conclusions . Primary non-Hodgkin's lymphoma of the lung occurs with nonspecific clinical features. Although patient survival is good, prognostic factors could not be identified.

A 46-year-male smoker presented with cough and fever to a chest physician, who on the basis of chest X-ray started him on antitubercular treatment. However, further evaluation suggested that the patient was suffering from a rare disease, i.e. Anaplastic large cell lymphoma of lung. Although lung involvement in cases of lymphoma is observed in as high as 40% of cases, in autopsy series, the exact clinical incidence is not known. One of the largest lymphoma groups reported it to be around 25%. However, primary pulmonary lymphomas have been extremely rare (0.4%), and whenever present they are of (Mucosa Associate Lymphoid tissue) MALT type, with occasional diffuse large cell lymphomas. The anaplastic variant is extremely uncommon. Usually the treatment results are satisfactory with more than 80% of the cases surviving even after 3 years. Here we report the case of anaplastic primary nonHodgkin′s lymphoma of lung and review the literature.

We present a patient with primary pulmonary lymphoma studied by magnetic resonance (MR), whose MR appearance was considered to be suggestive of the disorder. On T1-weighted MR images the tumour was seen as a large mass with homogeneous signal intensity, replacing the entire left lower lobe, while on T2-weighted images it was demonstrated as a homogeneous mass with a surrounding thin layer of greater signal intensity. This discrimination was not possible with computed tomography.

Because of the rarity of its occurrence, there is no established treatment for early stage primary pulmonary lymphoma. We retrospectively examined five patients with early stage primary pulmonary lymphoma to elucidate the optimal treatment for this unusual disease. All patients underwent complete resection with regional lymph node dissection. Lymphatic and blood vessel invasion were pathologically detected in all cases. All four patients with postoperative chemotherapy are alive without recurrence. One patient who did not undergo chemotherapy had an extrapulmonary recurrence 21 months after operation. The combined therapy of complete resection and postoperative chemotherapy may be the treatment of choice for this unusual disease.

In HIV-infected patients, non Hodgkin’s (NHL) and Hodgkin’s lymphomas (HD) are increased by a factor of 30–60 (NHL) and 5 (HD) in comparison to the HIV-negative population (Beral et al., 1991; Hessol et al., 1992; Rabkin et al., 1992). HIV-associated NHLs represent the second most common opportunistic tumor after Kaposi’s sarcoma and are listed, together with invasive cervical carcinoma, as AIDS-defining conditions (Centers for Disease Control, 1992). The association of Hodgkin’s disease with HIV-infection is well established but its role as AIDS-defining manifestation is still under discussion (Reynolds et al., 1993).

We report a retrospective study of the pathological features in 69 primary pulmonary non-Hodgkin's lymphomas which have previously been clinically reviewed. The tumours consisted of 61 (88%) low-grade and eight (12%) high-grade malignant lymphomas. Fifty-four of the low-grade malignant lymphomas were MALT lymphomas. Lymphoepithelial lesions were observed in bronchial, bronchiolar and alveolar lining. All tumours were composed of nodules, forming a lymphangitic pattern at the periphery and a confluent central mass. Invasion of pleura and vessels was often seen but this without any consequence on survival. Granulomas were found in 20% of cases. Six of the eight high-grade tumours were centroblastic and another two were B-cell lymphomas of undetermined type. In four cases, associated areas of low-grade malignant lymphoma with lympho-epithelial lesions indicated a preexisting MALT lymphoma. Clinical data suggest that limited surgery or non-aggressive chemotherapy can provide long-term survival in patients with such slowly developing neoplasms. However, non-invasive diagnostic methods need to be developed.

Abstract 880 Pulmonary marginal zone lymphoma is a distinct subgroup of B-cell non-Hodgkin's lymphomas originating from the bronchial-associated lymphoid tissue (BALT) and characterized by an indolent clinical course. Here we present the data of the IELSG-28 study of the International Extranodal Lymphoma Study Group (IELSG) on primary pulmonary lymphoma patients. Clinical records and histological material of 186 patients diagnosed with pulmonary marginal zone lymphoma from December 1986 to June 2009 were collected. The pathology was reviewed centrally and the diagnosis was confirmed in 180 (96%) patients. The median age at diagnosis was 62 (30 – 88). The majority of the patients (149/180) had localized disease at diagnosis with a good performance status (0–1) and low prognostic index (IPI 0–2). Eighty-one patients (45%) received local treatment, including surgery (74%) or radiotherapy (4%). Ninety-nine patients with advanced stage disease or incomplete surgical resection received an additional systemic treatment. Most of these patients (66; 66%) received an alkylating containing regimen, 22 (22%) patients were treated with an anthracycline containing regimen and 29 (29%) received the anti-CD20 monoclonal antibody in combination with chemotherapy. With a median follow up of 67 (range 1–256) months, 47/180 patients (26%) experienced disease progression. (median time to progression 30 months, range 0–109). Progression free survival analysis showed a trend in favour of patients treated with immuno-chemotherapy versus those receiving chemotherapy alone (HR 0.53–95%CI: 0.21, 1.33), while no difference in terms of OS and PFS was observed between patients receiving systemic anthracycline or alkylating containing regimens. In twenty five cases a specific PCR-assay was carried out for detecting the presence of Achromobacter (Alcaligenes) xylosoxidans. The presence of Achromobacter was detected in 50% of patients. Our results confirm that local therapy (surgery or radiotherapy) is the treatment of choice for patients with localized disease. Chemotherapy has to be considered in case of relapse, incomplete surgical excision or advanced disease. In this setting, alkylating containing regimens plus rituximab is the most effective option. Further studies are ongoing to confirm the relevance of infection/colonization with Achromobacter (Alcaligenes) xylosoxidans for pathogenesis of BALT lymphoma. Disclosures: No relevant conflicts of interest to declare.

Abstract 5203 AIDS-related primary pulmonary lymphoma (PPL) is defined as exclusive involvement of the lung by lymphoma with no evidence of extrapulmonary disease at the time of diagnosis and three months thereafter. It is an aggressive disease with a median survival time of four months. A 46 year old male patient with a seven year history of HIV/AIDS and CD4 count of 200/ul was hospitalized because of flank pain, hematuria and hemoptysis. Abdominal CT scan incidentally revealed multiple nodules in the lower lobes of both lungs. Serial chest CT scans demonstrated multiple nodular opacities in all lobes of the lungs that enlarged rapidly and coalesced together. Clinicians favored opportunistic infection due to rapid disease progression but included Wegener's granulomatosis in the differential diagnosis because of hemoptysis. Open lung biopsy showed diffuse large B cell lymphoma (DLBCL), NOS, with an activated B cell phenotype (CD10−/BCL6−/MUM1+) and high proliferative index (Ki67 90%). CD79A expression confirmed B cell lineage. The diagnostic criteria for PPL were fulfilled because staging failed to detect extrapulmonary disease. Infarction due to the angiodestructive nature of the PPL and short doubling time accounted for hemoptysis and rapid disease progression, respectively. The patient died 45 days after admission because of shock, presumably due to superimposed infection. Unlike prior reports of AIDS-related PPL, our case was negative for CD19, CD20 and Epstein-Barr virus (EBV). Although PPL is a rare disease, it deserves consideration in the differential diagnosis of rapidly progressive lung nodules in AIDS patients. To the best of our knowledge, this is the first reported case of EBV-negative PPL. Disclosures: No relevant conflicts of interest to declare.

【Background: Primary non-Hodgkin's lymphoma of the lung is a rare entity. It is represented commonly as marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type. Although there have been a few reviews of this lymphoma, clinical features, radiologic findings, management and prognosis have not been well defined. Methods: We reviewed the medical records of 24 patients with primary pulmonary lymphoma between January 1995 and September 2008; all diagnoses had been confirmed based on pathology. Results: The median follow-up time was 42.3 months (range, 0.1~131.2 months). Five (20.8%) patients were asymptomatic, 17 (70.8%) patients had pulmonary symptoms, and the remaining 2 (8.3%) patients presented with constitutional symptoms. There were 16 (66.7%) patients with MALT lymphoma, 4 (16.7%) patients with diffuse large B-cell lymphoma and 4 (16.7%) patients with lymphoma that had not received a WHO classification. Radiologic findings of primary pulmonary lymphoma were diverse and multiple nodule or consolidation was the most common finding regardless of pathologic lymphoma type. PET scan was carried out in 13 (54.2%) patients and all lesions showed notable FDG uptake. MALT lymphoma showed a trend of better prognosis (3-year survival, 78.8% vs. 70.0%; 5-year survival, 78.8% vs. 52.5%; p=0.310) than non-MALT lymphoma. Conclusion: Primary non-Hodgkin's lymphoma of the lung occurs with nonspecific clinical features and radiologic findings. MALT lymphoma is the most common pathologic type of primary pulmonary lymphoma. This entity of lymphoma appears to have a good prognosis and in this study, there was a trend of better outcome than non-MALT lymphoma.】

Endobronchial involvement by non-Hodgkin's lymphoma (NHL) is uncommon and usually occurs in the presence of more generalised disease. Solitary endobronchial lymphoma in the absence of disease elsewhere is extremely rare. In this report we describe a patient with an obstructing endobronchial mass which was the initial manifestation of NHL. The patient was treated initially with radiotherapy followed by cyclic combination chemotherapy with initial complete resolution of the endobronchial disease. However, he subsequently developed widespread lymphoma. This unusual presentation of NHL is discussed and the relevant literature is reviewed.

The CT angiogram sign has been described as a finding in the lobar form of bronchoalveolar cell carcinoma (1). Bronchoalveolar cell carcinoma represents a subgroup of adenocarcinoma and accounts for approximately 5% of all bronchogenic carcinomas in most series, although a recent increase in its prevalence to as high as 20.3% has been reported (2). Histopathologically, bronchoalveolar cell carcinoma can be separated into two basic morphologic types—mucinous and Clara cell or type II pneumocyte—and two spread pattern types—tumors with aerogenous spread and those without aerogenous spread. The mucinous tumors account for approximately 20%–30% of all bronchoalveolar cell carcinomas (3,4) and are characterized by tall, mucus-filled columnar cells. The mucinous tumors tend to spread aerogenously, infiltrating along the preexisting normal framework of the lung (ie, lepidic growth). Abundant secretion of mucin positive for periodic acid–Schiff reagent that fills the alveoli and small airways is more characteristic of the mucinous tumors; however, minimal amounts of this mucin can be seen in the nonmucinous tumors. Four clinical manifestations have been reported: (a) a single nodule, (b) multiple nodules, (c) a single consolidation, and (d) multiple lobar consolidations. Single and multiple lobar consolidations are more common in the mucinous tumors. Bronchoalveolar cell carcinoma in a patient with alveolar consolidation remains difficult to diagnose clinically and radiologically. Bronchorrhea, with up to liters of mucus discharged per day in some cases, was once considered a standard clinical finding; however, it is seen in a minority of patients. Bronchorrhea was found in 5% of the patients with bronchoalveolar cell carcinoma examined by Miller et al in 1978 (5). Some patients present for radiologic evaluation of a nonresolving alveolar consolidation that was initially diagnosed as pneumonia. Without underlying causes of hemorrhage, edema, or infection, persistent alveolar consolidation is suggestive of bronchoalveolar cell carcinoma. An incidental asymptomatic mass is another common manifestation. The CT angiogram sign was initially described as a specific sign of lobar bronchoalveolar cell carcinoma; specificity can be as high as 92.3% (1). Recently, the results of several retrospective studies have challenged the specificity of the CT angiogram sign; the CT angiogram sign was reported to be seen in both benign and malignant entities, including bronchoalveolar cell carcinoma, pneumonia, pulmonary edema, obstructive pneumonitis due to central lung tumors, lymphoma, and metastasis from gastrointestinal carcinomas (6–14). In cases of obstructive pneumonitis and primary pulmonary lymphoma, investigators have suggested that it is the relative difference between the attenuation of the pulmonary vessels

Sixty-two cases of primary malignant lymphoma of the lung were investigated. Fifty-eight lymphomas were of B- and two of T-cell type. Two cases of high-grade lymphoma could not be further classified. The largest group (43 cases) consisted of low-grade B-cell lymphoma of the bronchus-associated lymphoid tissue. These showed features similar to low-grade B-cell lymphomas of the mucosa-associated lymphoid tissue of the stomach. The low-grade lymphomas showed a peak occurrence in the sixth decade, the high-grade lymphomas in the seventh decade. Males predominated slightly. Three-quarters of the patients with low-grade B-cell lymphoma of the bronchus-associated lymphoid tissue showed solitary or multiple sharply defined nodules of the lung. The prognosis of the B-cell-derived lung lymphomas without constitutional symptoms was relatively favourable, regardless of whether they were of low- or high-grade malignancy, whereas patients with constitutional symptoms and the two patients with T-cell lymphomas showed a bad prognosis. However, recurrences and metastases in the lung, stomach, lymph nodes and salivary glands were seen in about 46% of the cases of low-grade B-cell lymphoma of the bronchus-associated lymphoid tissue.

Primary pulmonary lymphoma is rare, especially lymphomas arising in and limited to the tracheal wall without pulmonary parenchymal involvement are extremely rare. Bronchus-associated lymphoid tissue (BALT) lymphoma accounts for the majority of tracheal lymphomas. BALT lymphoma reveals distinct clinicopathologic features and remains localized for prolonged periods. The diagnosis is made histopathologically. Optimal management of these rare lesions has not been established. But, chemotherapy and radiation therapy all have been tried with favorable short-term results. The prognosis of BALT lymphoma is relatively good. We present here a case of BALT lymphoma of the tracheal wall which had responded to bronchoscopic ND-YAG laser therapy and local radiation therapy. (Tuberculosis and Respiratory Diseases 2003, 55:198-205)

A primary pulmonary malignant lymphoma is a rare disease. It is thought to be a category of non-Hodgkin's lymphoma arising from the bronchous-associated lymphoid tissue (BALT). The majority of primary pulmonary lymphomas are low-grade, small B-cell lymphomas, which are associated with Sjogren's syndrome and similar autoimmune disorders. A case of primary pulmonary low-grade B-cell lymphoma arising from the BALT was encountered in a patient with systemic lupus erythematosus. A 54-year-old man was admitted to the hospital for the evaluation of left pleuritic chest pain and multiple joint pain in both hands. Serologic tests for collagen vascular disease were performed. The results of ANA and anti-ds-DNA were all positive. The computed tomography of the chest showed patchy consolidations in the left lower lobe with a pleural effusion and a video-assisted thoracoscopic biopsy was performed. Here we report a case of a low-grade B-cell lymphoma of BALT in a patient with systemic lupus erythematosus with a review of the relevant literatures.

A rare association between primary pulmonary lymphoma and pulmonary nodular amyloidosis in a 56 year old man is described.