|Year : 2014 | Volume
| Issue : 2 | Page : 118-120
T lymphoblastic lymphoma of pleura: A report of two cases with review of literature
UG Vandana1, SS Parinitha1, Aneel Myageri1, Venkatesh Annigeri2, US Dinesh1
1 Department of Pathology, SDM College of Medical Sciences and Hospital, Dharwad, Karnataka, India
2 Department of Paediatric Surgery, SDM College of Medical Sciences and Hospital, Dharwad, Karnataka, India
|Date of Web Publication||20-Jun-2014|
U G Vandana
Department of Pathology, SDM College of Medical Sciences and Hospital, Dharwad, Karnataka
Source of Support: None, Conflict of Interest: None
Primary or secondary non-Hodgkin's lymphomas of the pleura are well known. Although secondary are more frequent to involve the pleura; primary pleural lymphomas [PPLs] are relatively rare in occurrence. Two types are discussed in literature: Primary effusion lymphoma in patients with human immunodeficiency virus (HIV) infection and pyothorax associated lymphoma. Primary pleural lymphoma can be also seen in immunocompetent patients with no history of pyothorax or HIV infection. Primary T lymphoblastic lymphomas of pleura that too occurring in children are quite rare. One should keep in mind the differential diagnosis of PPL with unusual presentation and rare location in immunocompetent patients not only in adults but also in children. Herewith we discuss two cases of T lymphoblastic lymphoma in immunocompetent children who presented with pleural effusion and extensive thickening of the pleura but were not associated with pyothorax.
Keywords: Lymphoma, pleura, primary, T lymphoblastic
|How to cite this article:|
Vandana U G, Parinitha S S, Myageri A, Annigeri V, Dinesh U S. T lymphoblastic lymphoma of pleura: A report of two cases with review of literature. Saudi J Health Sci 2014;3:118-20
|How to cite this URL:|
Vandana U G, Parinitha S S, Myageri A, Annigeri V, Dinesh U S. T lymphoblastic lymphoma of pleura: A report of two cases with review of literature. Saudi J Health Sci [serial online] 2014 [cited 2021 Oct 28];3:118-20. Available from: https://www.saudijhealthsci.org/text.asp?2014/3/2/118/134866
| Introduction|| |
Primary or secondary non-Hodgkin's lymphomas of the pleura are well known. Although secondary are more frequent to involve the pleura; primary pleural lymphomas [PPLs] are relatively rare in occurrence. PPLs encompass 7% of all lymphomas , and 2.4% of chest wall tumors. , Herewith; we discuss two cases of T-lymphoblastic lymphoma (T-LBL) in immunocompetent children who presented with pleural effusion and extensive thickening of the pleura.
| Case reports|| |
A 12-year-old female complained of low-grade fever and breathlessness for three months duration. On examination, there was right shift of trachea with absent breath sounds and stony dullness all over left lung fields. Computed tomography chest showed massive left pleural effusion and 9mm thick enhancing parietal pleura with complete collapse of left lung [Figure 1]a. Pleural fluid analysis showed an exudate with 80% small mature lymphocytes and occasional atypical cells suspicious of malignancy.
|Figure 1: (a) Computed tomography chest showing massive left pleural effusion with thick enhancing parietal pleura in case 1, (b) Chest x-ray with massive left pleural effusion in case 2|
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Another 11-year-old girl was admitted in our hospital after treatment by local hospital with ATT and antibiotics. On examination, left side breath sounds were reduced. Chest x-ray revealed massive fluid collection in left hemithorax with right mediastinal shift and inversion of diaphragm [Figure 1]b. Pleural fluid study showed an exudate with 60% small mature lymphocytes with no evidence of malignant cells.
There was no organomegaly or lymphadenopathy in both cases. Cultures of both samples of pleural fluid failed to grow any infectious agents while Ziehl-Neelsen stain for acid-fast bacilli and polymerase chain reaction for Mycobacterium tuberculosis re negative. In both cases, because of severe respiratory distress left-sided decortication of pleura was done with video-assisted thoracic surgery. Intra-operatively, thick effusion fluid with necrosis of base of left lower lobe of lung was observed in both.
Microscopy of grossly thickened pleura revealed infiltration with monomorphous small round tumour cells arranged in sheets between radiating vessels and large areas of necrosis. Tumor cells were larger than small lymphocytes with round hyperchromatic nuclei with inconspicuous nucleoli, scanty cytoplasm and high mitotic rate [Figure 2]a. In case 2, amidst tumour cells were seen uniformly distributed reactive histiocytes with phagocytosed nuclear debris giving "starry sky" appearance [Figure 2]b so a differential of Burkitt's lymphoma was also considered but few cases of T-LBL can also show this pattern.
Immunohistochemical study with CD45, TdT, CD3 [Figure 3]a and CD99 [Figure 3]b showed positivity in tumour cells in both cases and negative staining for CD20 and CD34. These findings were consistent with T-LBL. Bone marrow aspiration was within normal limits in both the cases.
|Figure 2: (a) Monomorphous small round tumour cells with hyperchromatic nuclei and inconspicuous nucleoli case 1 (H and E, ×40) (b) "Starry sky" appearance in case 2, amidst tumour cells were seen uniformly distributed reactive histiocytes with phagocytosed nuclear debris|
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|Figure 3: (a) Immunohistochemical study showing positivity for CD3 (b) Immunohistochemical study showing positivity for CD99|
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In case 1, induction and consolidation therapy was given with NHL-BFM (Berlin-Frankfurt-Munster)-95 protocol as per advice of hemato-oncologist and complete clinical remission was achieved. Patient is being followed for 18 months and is doing well. But case 2 succumbed to death in immediate postoperative period due to sepsis in spite of all feasible medical supportive treatment.
| Discussion|| |
Pleural involvement in patients with lymphomas is frequent and almost 30% of the tumors may present malignant pleural effusion during the course of the disease.  Primary pleural lymphomas (PPLs) are extremely rare to occur and in a series reported by Burgener and Hamlin pleural plaques were seen in less than 4% of cases. ,, PPLs reported so far in adulthood are mostly diffuse large B-cell lymphomas.  T-LBL of pleura that too occurring in children are quite rare and very few cases are documented in the literature. ,, T-LBL usually affects men in 3 rd decade with mediastinal mass or cervical lymphadenopathy with early infiltration of bone marrow leading to leukemia. The clinical course is aggressive with frequent relapses and short survival. 
Two types of PPLs have been discussed: Primary effusion lymphoma (PEL) in patients with human immunodeficiency virus (HIV) infection and pyothorax associated lymphoma (PAL).  PEL has also been reported to be strongly associated with human herpesvirus-8 (HHV-8) and occasionally with Epstein-Barr virus (EBV) infection. PAL is seen in patients with chronic pleural inflammation due to chronic pyothorax and previous artificial pneumothorax to treat tuberculosis. , In a review of literature, PAL occurred in 2.2% of patients with chronic pyothorax due to chronic tuberculosis mainly documented in Japanese people. There are very few reports from Western countries because treating lung tuberculosis with artificial pneumothorax was a trend frequently observed in Japan.  In PEL, pleural effusion without identifiable pleural mass is a predominant feature while PAL comprises homogenous or in-homogenous non-enhancing soft tissue mass.  PPLs can be seen in immunocompetent patients with no history of pyothorax or HIV, HHV or EBV infection. The radiological findings of these rare non-PAL/PEL PPLs are described as pleural effusion with a pleural mass, homogenous or in-homogenous thickening or consolidation. ,, In our two case studies, there was no history of previous pyothorax or autoimmune disease and investigations for mycobacterium species and HIV were negative.
Pleural effusions in patients with lymphoma may be due to lymphatic obstruction or direct involvement of pleura by tumour.  The positive cytology of pleural fluid is the first sign of underlying malignancy. About 10% of malignant pleural effusions are due to lymphomas and in lymphomatous effusions, positive cytology is reported in 14-88% of patients.  One may have negative cytology probably due to sparse malignant cells in pleural specimens or difficulty in differentiating lymphoma cells from reactive lymphocytes. , Probably some lymphoma types involving the pleura may have a limited predisposition to seed into the pleural fluid, so the combination of cytologic and histologic examination seems to be the most practical approach for diagnosing pleural lymphomas.  Presence of pleural effusion if cytologically positive is associated with poor survival rate. , With repeated thoracocentesis more specific results can be accomplished.  PPLs require early precise diagnosis and complete surgical resection with adjuvant radio/chemotherapy. 
| Conclusion|| |
Primary pleural lymphomas in immunocompetent children without a history of chronic pyothorax or autoimmune disease are rare. PPLs can present with pleural effusion with thickened pleura. Pleural cytology alone may not yield the diagnosis but histopathology and immunostaining are crucial to establish the diagnosis and facilitate optimal treatment. One should be aware of this unusual location and presentation of PPL in immunocompetent patients not only in adults but also in children.
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[Figure 1], [Figure 2], [Figure 3]