Weekly Chest CasesArchive of Old Cases

Case No : 403 Date 2005-07-18

  • Courtesy of Tae Hoon Kim M.D., Sang Jin Kim M.D. / Yongdong Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
  • Age/Sex 71 / M
  • Chief ComplaintDyspnea for two months
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4

Diagnosis With Brief Discussion

Diagnosis
Mantle Cell Lymphoma
Radiologic Findings
Chest radiograph (Fig. 1) showed bronchovascular thickening and diffused nodular infiltrates in both lungs. Lung window and high resolution CT scans (Figs. 2 and 3) revealed peribronchovascular thickening and nodular infiltration along the perivascular, peribronchial, and subpleural interstitium. Mediastinal window CT scan (Fig. 4) showed multiple lymphadenopathies in the mediastinum and bilateral axillas. Photomicroscopes showed monotonous proliferation of atypical lymphocytes with B cell monoclonality at the axillary lymph node (x100, HE stain) (Fig. 5) and patchy peribronchial and perivascular infiltration of atypical lymphocytes with B cell monoclonality in the lung (x40, HE stain) (Fig. 6). The results of immunohistochemical stain are as follows: CD3 (-), CD20/79a (+), and cyclin D1 (+).
Brief Review
Lymphoid follicle in bronchus-associated lymphoid tissue consists of germinal center (follicular center), mantle zone, and marginal zone. Mantle cell lymphoma (MCL) is a malignant neoplasm of virgin B cells that closely resemble normal mantle zone B cells surrounding germinal centers. They occur in older adults with a distinct male predominance. MCL is usually widespread at diagnosis and frequently involves, apart from lymph nodes, the spleen, bone marrow, and gastrointestinal tract. Pathologically, it is characterized by an infiltrate of small to medium-sized cells with folded nuclei and scanty cytoplasm. The diagnosis of MCL was confirmed by histological assessment and immunohistochemical evaluation, including cyclin D1. On a genetic level, many cases of MCL have the t(11;14)(q13;q32) that causes overexpression of cyclin-D1. The survival is short, with over one-third of the patients dying within a year despite the administration of aggressive combination chemotherapy. The median survival ranges from 3 to 5 years. The radiological appearances were rarely known for mantle cell lymphoma. Differential diagnoses include lymphocytic interstitial pneumonitis, lymphangitic carcinomatosis, and sometimes sarcoidosis.
References
1. Harris NL, Jaffe ES, Stein H, et al. A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group. Blood. 1994;84:1361-1392.

2. Kurtin PJ. Mantle cell lymphoma. Adv Anat Pathol. 1998;5:376-398.

3. King LJ, Padley SP, Wotherspoon AC, Nicholson AG. Pulmonary MALT lymphoma: imaging findings in 24 cases. Eur Radiol. 2000;10:1932-1938.
Keywords
Lung, Mediastinum, Lymphproliferative disorder, Lymphoma,

No. of Applicants : 23

▶ Correct Answer : 7/23,  30.4%
  • - Ewha Womans University Mokdong Hospital, Korea Yookyung Kim
  • - Asan Medical Center, Seoul, Korea Hyun-Joo Kim
  • - Hangang SacredHeart Hospital, Korea Eil Seong Lee
  • - Ping Tung Christian Hospital,Taiwan Jun-Jun Yeh
  • - Annemasse, Polyclinique de Savoie, France Gay-Depassier
  • - Max Hospital, New Delhi, India Vickrant Malhotra
  • - Ev. Krkhs. Hubertus, Berlin, Germany Michael Weber
▶ Correct Answer as Differential Diagnosis : 8/23,  34.8%
  • - Incheon Sarang Hospital, Incheon, Korea Jung Hee Kim
  • - Radiologie Guiton, La Rochelle, France Denis Chabassiere
  • - Monaldi Hospital, Naples, Italy Gaetano Rea
  • - ASL Bologna, Maggiore Hospital, Bologna, Italy Marcellino Burzi
  • - Social Security Hospital, Ankara, Turkey Meric Tuzun
  • - -- Ram Prakash Galwa
  • - Homs National Hospital, Homs, Syria Rami Abou Zalaf
  • - Hanyang University Hospital, Seoul, Korea Yo Won Choi
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