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Weekly Chest CasesArchive of Old Cases

Case No : 1019 Date 2017-05-08

  • Courtesy of Su Yeon Ahn , Kum Ju Chae, Soon Ho Yoon, Jin Mo Goo / Seoul National University Hospital
  • Age/Sex 49 / F
  • Chief ComplaintIncidental radiologic abnormality, dry mouth
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6

Diagnosis With Brief Discussion

Diagnosis
MALT lymphoma
Radiologic Findings
Chest CT demonstrated an anterior mediastinal mass having focal low attenuation. On lung window settings, there were multiple thin-walled cysts in both lungs. In addition, a few solid nodules were shown in RML and RLL. A nodule in the RML contained calcification. The lesions were hypermetabolic on PET/CT scan. The patient underwent total thymectomy and wedge resection for multiple nodules in both lungs. The pathologic diagnosis of anterior mediastinal mass was thymic extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) with extensive plasma cell differentiation. Lung nodules were confirmed as lung involvement of thymic extranodal marginal zone B-cell lymphoma and amyloid deposit.
Brief Review
Primary low-grade B-cell MALT lymphoma is a rare tumor in the thymus. Most patients are Asians and associated with autoimmune disease, especially Sjögren syndrome.
Thymic MALT lymphomas are often accompanied with macroscopic or microscopic cyst formation. Cyst formation may be related to the tendency for cystic transformation of medullary duct-epithelium-derived structures (including Hassall corpuscles) when tumor grows in the thymic gland. Thymic MALT lymphoma needs to be included in the differential diagnosis for thymic masses accompanied by cystic changes and autoimmune diseases in Asian patients.
Sjögren syndrome is a systemic chronic inflammatory disorder but is also recognized as a lymphoproliferative disease with varying presentation from polyclonal lymphocytic infiltration of the salivary glands to oligo- or monoclonal B-cell proliferation resulting in clonally derived lymphoproliferative disorders such as monoclonal gammopathy, light-chain amyloidosis, and malignant lymphoma. In the study on primary Sjögren syndrome, Baqir et.al. found that pulmonary amyloidosis was associated with cystic lesions and nodules in the lung parenchyma. The mechanism of cyst formation in Sjögren syndrome is not fully understood. Some authors have proposed the presence of inflammatory cells or amyloid exhibiting ball-valve mechanism resulting from bronchiolar obstruction.
References
1. Kang, L.-Y., Ho, S.-P., & Chou, Y.-P. Primary thymic mucosa-associated lymphoid tissue lymphoma with multiple thin walled lung cysts: case report and literature review. Chinese Journal of Cancer Research 2013;25:354–357.
2. Shimizu K, Yoshida J, Kakegawa S, et al. Primary thymic mucosa-associated lymphoid tissue lymphoma: diagnostic tips. J Thorac Oncol 2010;5:117-121.
3. Baqir, Misbah, et al. Amyloid-associated cystic lung disease in primary Sjögren syndrome. Respiratory medicine 2013;107:616-621.
Keywords
Thymus, MALT lymphoma, Sjogren syndrome with Lymphoproliferative disease ,

No. of Applicants : 107

▶ Correct Answer : 25/107,  23.4%
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - Showa university Northern Yokohama Hospital , Japan KOTA WATANABE
  • - The University of Tokyo Hospital , Japan Akifumi Hagiwara
  • - Chonbuk National University Hospital , Korea (South) MINGI SHIN
  • - IRSA LA ROCHELLE , France, Metropolitan BIGOT
  • - Chonnam national university hospital , Korea (South) GYEONG IL MOON
  • - Busan National University Hospital , Korea (South) ILWAN SON
  • - Seoul National University Hospital , Korea (South) SIWON JANG
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - Beaulieu clinic Geneva , Switzerland gilles GENIN
  • - Chonnam National University Hospital , Korea (South) GUNSU KIM
  • - HHS , Canada S LEE
  • - Narayana Multispeciality Hospital Jaipur Rajasthan , India JAINENDRA JAIN
  • - Asan Medical Center , Korea (South) JOOAE CHOE
  • - Busan National University Hospital , Korea (South) KIM JEONG MYEONG
  • - Samsung medical center , Korea (South) Ran Kim
  • - Samsung Medical Center , Korea (South) KYOWON GU
  • - Chungbuk National University Hospital , Korea (South) LEE JUNG HWAN
  • - The University of Tokyo Hospital , Japan TAKU TAJIMA
  • - Korea university medical center, guro hospital , Korea (South) Lee kyu chong
  • - Teikyo University Hospital , Japan KAORU SUMIDA
  • - Pusan national university hospital , Korea (South) Jin Joo Kim
  • - University of Utah , United States AKIHIKO SAKATA
  • - Pusan national university hospital , Korea (South) So min Park
  • - Chungbuk National University Hospital , Korea (South) HYEONMI RYU
▶ Correct Answer as Differential Diagnosis : 33/107,  30.8%
  • - dayanand , India REENA GUPTA
  • - Niigata University , Japan ATSUSHI UEHARA
  • - Shiga University of Medical Science , Japan AKITOSHI INOUE
  • - NIMS, Hyderabad , India AMIT DADHANIA
  • - Korea University Guro Hospital , Korea (South) Bit Na Park
  • - Korea University Guro Hospital , Korea (South) PARK SOYEON
  • - The University of Tokyo Hospital , Japan Yusuke Watanabe
  • - Chungbuk National University Hospital , Korea (South) SOOJUNG LEE
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - Other , Korea (South) SEONG SU KANG
  • - Amagasaki General Medical Center , Japan GENKI FUKUMOTO
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - Chonnam National University Hospital , Korea (South) JONG HYEON KIM
  • - Chung buk national university hospital , Korea (South) hong gwon Byun
  • - Chungbuk National University Hospital , Korea (South) JUNGHWAN KIM
  • - The University of Tokyo Hospital , Japan RYO KUROKAWA
  • - Chungbuk national university , Korea (South) Yong Hun Kim
  • - Mallinckrodt Institute of Radiology , United States Naganathan BS Mani
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey MERIC TUZUN
  • - Other , Korea (South) HAYEON LEE
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - Kyeongpook National University Hospital , Korea (South) SUNGJUN MOON
  • - Chungbuk national university hospital , Korea (South) Miran Yeon Yeon
  • - Azienda Ospedaliera di Cremona , Italy Pietro Sergio
  • - Yujin Yamazaki Hospital , Japan MASASHI TAKAHASHI
  • - Fortis hospital , Mohali , India SHALEEN RANA
  • - Korea University Guro Hospital , Korea (South) SUN HONG
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - The University of Tokyo Hospital , Japan HIROSHI TAKUMIDA
  • - Private sector , Greece VASILIOS TZILAS
  • - Diagnose.me (BV) / Royal Perth Hospital , Australia YURANGA WEERAKKODY
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
▶ Semi-Correct Answer : 34/107,  31.8%
  • - Ishikawa Matto Central Hospital , Japan MANABU AKIMOTO
  • - Pamukkale University, Faculty of Medicine, Dept. of Radiology , Turkey Furkan Ufuk
  • - ZIGONG TCM HOSPITAL OF CHINA , China Cao Cunyou
  • - Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
  • - Showa University Fujigaoka Hospital , Japan KYOKO NAGAI
  • - King Abdulaziz University Hospital , Saudi Arabia Amr M. Ajlan
  • - NIMS, HYDERABAD , India BHASKAR K
  • - , Korea (South) BO RAM KIM
  • - Yonsei University,Severance Hospital , Korea (South) CHAE JUNG PARK
  • - Myongji Hospital , Korea (South) KYUNGHO KIM
  • - PRINCE SATTAM IBN ABDULAZIZ UNIVERSITY HOSPITAL , Saudi Arabia ELBAGIR MOHAMED NASSIR
  • - Asan Medical Center , Korea (South) HYUN JUNG KOO
  • - Union Hospital ,Tongji medical college , China JIE YU
  • - university of montreal , Canada Andrei Bogdan Gorgos I
  • - Seoul National University Hospital , Korea (South) HYOUNG IN CHOI
  • - Asan Medical Center , Korea (South) HONG SANG HYUP
  • - McGill University / University College London , Canada Ilan Azuelos
  • - Asan Medical Center , Korea (South) JIHYE KWON
  • - Okayama Red Cross Hospital , Japan KAZUAKI MUNETOMO
  • - Dong-A University, College of Medicine , Korea (South) KI-NAM LEE
  • - , United Kingdom KRISHNA PRASAD PREMNATH BELLAM
  • - Chonbuk National University Hospital , Korea (South) WON HYEONG IM
  • - Other , Korea (South) MINKYU KWAK
  • - Avrasya Hospital/ISTANBUL , Turkey MURAT ULUSOY
  • - Soonchunhyang University Hospital Seoul , Korea (South) BODA NAM
  • - Chonnam National University Hospital, Radiology department , Korea (South) Park byung jin
  • - , India TEKWANI PARMANAND HOLARAM
  • - Multimagem Diagn泥˜sticos , Brazil PEDRO PAULO TEIXEIRA E SILVA TORRES
  • - GHICL , France MANUEL TOLEDANO
  • - MBAL BURGAS , Bulgaria VLADISLAV BOYANOV RUSINOV
  • - Korea University Guro Hospital , Korea (South) JINWOO HAN
  • - Other , Korea (South) IN CHUL NAM
  • - Seoul National University Hospital , Korea (South) SUNKYUNG JEON
  • - Korea University Ansan Hospital , Korea (South) CHERRY KIM
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