Primary pleural lymphomas

Abstract

Two patients are presented with primary low grade pleural B cell lymphomas with no history of a pyothorax.P leural disease in non-Hodgkin's lymphoma is well documented and commonly presents with pleural effusions in 20% of patients. 1 However, solid pleural involvement is less common and is usually a secondary event. Primary pleural lymphomas are extremely rare and, in a series reported by Burgener and Hamlin, pleural plaques were seen in less then 4% of cases. 2 Two types of primary pleural lymphomas have been described-the body cavity based lymphoma in patients with HIV and the pyothorax associated pleural lymphoma in those with tuberculosis. Primary pleural non-Hodgkin's lymphoma in an immunocompetent patient without a history of chronic pyothorax is extremely rare. CASE HISTORY 1A 59 year old man presented with a 4 month history of gradually increasing shortness of breath and left sided chest pain. He was a non-smoker with a history of occupational exposure to asbestos (he had worked in power stations for over 30 years). On examination, air entry over the left side of his chest was diminished.Investigations showed a normal full blood count, urea and electrolytes. His chest radiograph revealed a left sided pleural effusion. A chest drain was therefore inserted and approximately 7 litres of serous fluid were drained. A computed tomographic (CT) scan of the thorax showed a left pleural effusion with irregular thickening of the adjacent parietal pleura, extending medially to displace the aorta. Bilateral pleural calcification suggestive of asbestos exposure was also seen. No significant lymphadenopathy was noted. A bone marrow biopsy performed during this admission did not show any evidence of lymphoma. Video assisted thoracoscopy showed diffuse involvement of the parietal pleura from which biopsy samples were taken. Light microscopy showed lymphoid infiltration with a hint of nodularity. Isolated reactive germinal centres were buried within the infiltrate of medium sized lymphoid cells and mononuclear blasts (fig 1). Mitotic figures were easily visible. Immunohistochemical staining confirmed the dominant B cell nature of the infiltrate. The phenotype of the B cells was confirmed as CD43, CD23 and BCl-2 positive, and CD5 and CD10 negative. Follicular dendritic cells in lymphoid nodules were highlighted with CD23 and CD21, confirming follicular colonisation. Ki67 labelling was observed in 30% of the tumour population. These findings supported a diagnosis of low grade marginal zone lymphoma of the pleura. Also present in the sample were hyalinised plaques infiltrated by lymphoma on one surface.DNA was extracted from paraffin embedded material and PCR was performed by standard methods using primers for the FR2 and FR3 regions of immunoglobulin heavy chain.This showed a band, in keeping with the monoclonal nature of the disease. Pleurodesis with 5-fluorouracil was performed and the chest drain was eventually removed. The patient was treated with chlorambucil. Follow up at 18 months showed no evidence of lymphadenopa...

Rare Cases of Primary Pleural Hodgkin and Non-Hodgkin Lymphomas

“…Also, he had an extranodal follicular NHL, which has not been reported so far. In adult patients without chronic pyothorax, Ahmad et al [5] reported 2 cases of low-grade marginal zone lymphoma and Kano et al [6] reported cases of diffuse large B cell lymphoma. Only one case in a pediatric patient, a 2.5-year-old girl with lymphoblastic lymphoma, has been reported by Karadeniz et al [7].…”

Primary mucosa-associated lymphoid tissue lymphoma as a pleural mass

“…Both are clinically aggressive and have well-characterised clinical and pathological features. Various mechanisms have been implicated for the development of primary pleural lymphoma, including chronic stimulation of B cells by longstanding chronic pleural disease, which occurs in different diseases such as tuberculous pleuritis, Sjö gren's syndrome, rheumatoid arthritis, malignant lymphoma, chronic lymphocytic thyroiditis, thyroid lymphomas and Epstein-Barr virus (EBV) infection [3,8]. PEL has been reported to be strongly associated with human herpesvirus-8 (HHV-8) infection and occasionally with EBV infection [4].…”

“…The histological subtype in these few cases referred to in the literature was extranodal marginal zone lymphoma [4,5]. The radiological findings of these rare cases were pleural effusion associated with a pleural mass, consolidation or inhomogeneous pleural thickening [3][4][5].…”

“…However, an extremely rare type of primary pleural lymphoma has been described in immunocompetent patients with no history of chronic tuberculous pyothorax. The radiological findings consisted of pleural effusion associated with a pleural mass, consolidation or inhomogeneous pleural thickening [3][4][5][6]. Although pleural effusion is a common manifestation of both secondary and primary pleural lymphoma, in this case there was no pleural effusion and the diagnosis was initially obtained by CT-guided fine needle aspiration cytology (FNAC) of an extraparenchymatous mass and confirmed by a CT-guided tru-cut biopsy.…”

Lymphoproliferative Conditions of the Serosa

“…Primary pleural diffuse large B-cell lymphoma and marginal zone lymphoma have been reported. 28,29 Lymphomatoid granulomatosis is recognized as an angiocentric and angiodestructive disease arising in a variety of extranodal sites. Pathologically it is best classified as an EBV-induced T cell-rich B-cell lymphoma.…”