Primary lung lymphoma involving the superior vena cava
© Wei et al.; licensee BioMed Central Ltd. 2012
Received: 8 February 2012
Accepted: 3 June 2012
Published: 30 June 2012
Primary lung lymphoma (PLL) presenting as a primary pulmonary lesion is rare and usually affects elderly people. Here we describe a 25-year-old Chinese man diagnosed with primary lung lymphoma, which presented as a huge lung tumor mimicking a primary lung cancer and involving the superior vena cava. He underwent double-sleeve reconstructions of bronchus and pulmonary arteries with right upper- and middle-lobe lobectomy along with replacement of the superior vena cava with a graft, and was then given standard chemotherapy of CHOP plus Rituximab. The patient has been well, showing no local recurrence or distal metastasis during a 27-month follow-up.
Primary lung lymphoma (PLL) is a rare distinct entity (0.4% of all lymphomas) that usually affects elderly patients [1–3]. Generally, these cases are difficult to diagnose accurately because of a nonspecific clinical and radiological presentation. On the other hand, they have relatively satisfactory outcomes, especially in cases amenable to surgical resection. Owing to the rarity of PLL, standard treatment protocols have not yet been optimized, and there is no guideline as to when surgery is indicated.
Herein, we present a young patient with a rare primary pulmonary lymphoma (diffuse large B cell lymphoma, DLBCL) mimicking a primary lung cancer and involving the superior vena cava. He was treated by double-sleeve reconstructions of bronchus and pulmonary arteries with right upper and middle lobe lobectomy, and replacement of the superior vena cava with a graft.
A 25-year-old Chinese male was admitted because of respiratory symptoms and a large mass on the computed tomography (CT) scan suggestive of primary lung carcinoma. This patient presented with 45 days of an irritating dry cough and right chest pain without other abnormal findings such as fever, wheezing, hemoptysis, or superior vena cava syndrome. He had no smoking history and no family history of lung cancer. A review of systems was noncontributory. On admission, peripheral blood count, serum chemistry, and urinalysis were normal.
Extranodal lymphomas account for 24% to 48% of NHL, and primary pulmonary lymphomas are infrequent (about 3.6% among all extranodal lymphomas) . Primary pulmonary lymphoma (PPL) is defined as a clonal lymphoid proliferation affecting one or both lungs (parenchyma and/or bronchi) in a patient with no detectable extrapulmonary involvement at diagnosis or during the subsequent 3 months [6, 7]. According to the World Health Organization’s classification system, the most common histological subtypes of primary pulmonary lymphoma are low-grade lymphomas (75-88%), and the second most frequent histological type of lymphoma to involve the lung is diffuse large B-cell lymphoma (DLBCL), which represents only about 5-20% of primary pulmonary lymphomas . The high-grade B-cell PPLs usually spread rapidly into mediastinal and extra-thoracic locations. The case of DLBCL we reported here presented as a huge lung tumor mimicking a primary lung cancer involving the superior vena cava.
Primary pulmonary lymphoma is most commonly seen in between the 5th and 7th decades of life, with a mean age of 50 years and a male-to-female ratio of 1.07:1 . The present case was a young (25-year-old) man with nonspecific symptoms of irritating dry cough and right chest pain. As reported previously, PPLs can present with nonspecific symptoms . Since PPLs can present with different radiological manifestations, an accurate diagnosis is often not easy. In our case, the images were suggestive of locally advanced lung cancer, but the final diagnosis turned out to be a primary pulmonary lymphoma. This further emphasizes the importance of histological diagnosis of lung lesions, although this tumor was even misdiagnosed as lung cancer before operation based on pathological findings from CT-guided fine-needle aspiration biopsy. In the literature, preoperative CT-guided fine-needle aspiration biopsy allows diagnosis of PPL in only 25% of patients . Thus, in order to avoid the mistake of the misdiagnosis of this curable disease, it is important to keep in mind that any radiological abnormality of the lung parenchyma may be a lymphoproliferative disorder, which sometimes has a good prognosis and has a very different management plan from epithelial neoplasia of the lung, especially when the lung is primarily affected .
High-grade pulmonary B-cell lymphoma is far rare and usually occurs in individuals with an underlying disorder (e.g., immunodeficiency). The prognosis is poor compared with low-grade lymphoma, and therapeutic options depend on the underlying disorder. Because of its rarity, relatively little is known about the biological characteristics of DLBCL. Treatment of these rare tumors is poorly standardized. Several treatment options are available, including tumor resection, surgery with adjuvant chemotherapy, or chemotherapy alone. The optimal treatment remains controversial, but the prognosis can be relatively excellent . One large series of 70 patients reported a 94% survival at 5 years for low-grade primary pulmonary lymphoma, and a median survival of 3 years for high-grade disease . Another series with 48 patients reported a 68% 5-year survival for primary pulmonary MALT lymphoma and a 65% 5-year survival for non-MALT lymphoma .
We believe surgery should be the treatment of choice if a complete resection can be achieved. The potential surgical candidate could be any patient with locally resectable tumor up to stage IIE. Lymph node involvement does not appear to be a contraindication to surgery . To our knowledge, few patients with pulmonary DLBCL involving the superior vena cava have received radical surgical treatment, so this is an extremely uncommon case. Although some authors have recommended a pneumonectomy for multiple lesions of low-grade PPL involving one lung, in our view this option may be too aggressive because of the indolent course of the disease. One patient reported by Frederic et al.  is still alive after a 36-month follow-up.
Treatment after surgical resection is often based on a combination chemotherapy regimen. Diffuse large B cell lymphoma is frequently treated by CHOP (cyclophosphamide, Adriamycin, vincristine and prednisone), and Rituximab may improve the response to CHOP treatment, as has been shown in systemic diffuse large B-cell lymphoma. Although survival is worse for patients with high-grade PPL than for those with low-grade PPL, published reports show that more than half of patients with high-grade PPL can achieve survivals of 8 to 10 years .
In conclusion, surgery should be the treatment of choice in cases of localized PPL when complete resection can be achieved.
Written informed consent was obtained from the patient for publication of this Case report and any accompanying images.
This study was partly supported by grants from the Science and Technology Support Key Program of Tianjin (09ZCZDSF04100, 09ZCZDSF04000) and the project of Ministry of Education for New Century Excellent Talents (NCET-10-0956).
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