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Presented by Chad McCall, M.D., Ph.D. and prepared by Doreen Nguyen, M.D.
Case 3: This is a 68 year old man with a 2 cm left upper lobe lung mass, found incidentally on CT scan.
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Week 569: Case 3
This is a 68 year old man with a 2 cm left upper lobe lung mass, found incidentally on CT scan.images/D Nguyen/11-4-13/case 3/BALToma 2X_450pixels.jpg
images/D Nguyen/11-4-13/case 3/BALToma 4X_450pixels.jpg
images/D Nguyen/11-4-13/case 3/BALToma 20X_450pixels.jpg
images/D Nguyen/11-4-13/case 3/BALToma 20X plasma cells_450pixels.jpgCorrect
Answer: Extranodal marginal zone lymphoma of bronchus-associated lymphoid tissue
Histology: A dense lymphoid infiltrate is seen in this lung wedge resection. The infiltrate is largely composed of monotonous, small- to medium-sized lymphocytes with minimally irregular nuclei with coarse chromatin. Abundant clear cytoplasm results in a monocytoid appearance. These cells infiltrate around residual germinal centers. Necrosis and vascular invasion are not present. In focal areas, sheets of plasma cells are found, which are lambda-restricted by immunohistochemistry.
Discussion: Extranodal marginal zone lymphoma of bronchus-associated lymphoid tissue (“BALToma”) is the most common primary pulmonary lymphoma, accounting for around 75% of cases. Half of cases are asymptomatic and found incidentally on chest imaging. Symptoms, if present, are non-specific, such as cough, chest pain, dyspnea, and fatigue. One-third of cases have an associated monoclonal gammopathy, which is usually low in concentration and IgM. While an indolent B-cell lymphoma, rare cases do transform to diffuse large B-cell lymphoma. Histologically, one-third of cases, such as this one, have areas of monoclonal plasma cell differentiation, which can help to differentiate from lymphoid interstitial pneumonia.
Also in the differential diagnosis is lymphomatoid granulomatosis, a B-cell lymphoma which usually presents as necrotic lesions with vascular invasion; the cells are larger and have prominent nucleoli; and, Epstein-Barr virus is positive. Secondary involvement by an extrapulmonary lymphoma must also be considered, such as chronic lymphocytic leukemia/small lymphocytic lymphoma, mantle cell lymphoma, and follicular lymphoma. Cellular morphology and immunohistochemistry are useful to make this distinction: BALToma is typically negative for CD5, CD10, CD23, and Cyclin D1 and positive for BCL-2. CD43 is positive in a significant minority of cases and not in most other B-cell lymphomas. CLL/SLL would be positive for CD5 and CD23; follicular lymphoma is usually positive for CD10, BCL-6, and BCL-2; mantle cell lymphoma is positive for CD5 and Cyclin D1.
Incorrect
Answer: Extranodal marginal zone lymphoma of bronchus-associated lymphoid tissue
Histology: A dense lymphoid infiltrate is seen in this lung wedge resection. The infiltrate is largely composed of monotonous, small- to medium-sized lymphocytes with minimally irregular nuclei with coarse chromatin. Abundant clear cytoplasm results in a monocytoid appearance. These cells infiltrate around residual germinal centers. Necrosis and vascular invasion are not present. In focal areas, sheets of plasma cells are found, which are lambda-restricted by immunohistochemistry.
Discussion: Extranodal marginal zone lymphoma of bronchus-associated lymphoid tissue (“BALToma”) is the most common primary pulmonary lymphoma, accounting for around 75% of cases. Half of cases are asymptomatic and found incidentally on chest imaging. Symptoms, if present, are non-specific, such as cough, chest pain, dyspnea, and fatigue. One-third of cases have an associated monoclonal gammopathy, which is usually low in concentration and IgM. While an indolent B-cell lymphoma, rare cases do transform to diffuse large B-cell lymphoma. Histologically, one-third of cases, such as this one, have areas of monoclonal plasma cell differentiation, which can help to differentiate from lymphoid interstitial pneumonia.
Also in the differential diagnosis is lymphomatoid granulomatosis, a B-cell lymphoma which usually presents as necrotic lesions with vascular invasion; the cells are larger and have prominent nucleoli; and, Epstein-Barr virus is positive. Secondary involvement by an extrapulmonary lymphoma must also be considered, such as chronic lymphocytic leukemia/small lymphocytic lymphoma, mantle cell lymphoma, and follicular lymphoma. Cellular morphology and immunohistochemistry are useful to make this distinction: BALToma is typically negative for CD5, CD10, CD23, and Cyclin D1 and positive for BCL-2. CD43 is positive in a significant minority of cases and not in most other B-cell lymphomas. CLL/SLL would be positive for CD5 and CD23; follicular lymphoma is usually positive for CD10, BCL-6, and BCL-2; mantle cell lymphoma is positive for CD5 and Cyclin D1.
