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Weekly Chest CasesArchive of Old Cases

Case No : 1251 Date 2021-10-11

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  • Courtesy of Seung-Jin Yoo, Yo Won Choi, Sang Hyun Paik / Hanyang University Medical Center
  • Age/Sex 64 / F
  • Chief ComplaintHistory of anterior mediastinal mass excision – IgG4 related disease (Figure 1)
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6
  • Figure 7

preop initial CT

Diagnosis With Brief Discussion

Diagnosis
Lymphoma occurred in IgG4-related disease
Radiologic Findings
Fig. 1. Enhanced chest computed tomography (CT) scan showing a 5.5-cm soft-tissue mass in the anterior mediastinum with large amount of right pleural effusion. A pigtail catheter is inserted into the right hemithorax.
Figs. 2
Brief Review
Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that affects multiple organs. Infiltration of organ systems by IgG4-positive plasma cells and lymphocytes leads to fibrosis and tumefactive lesions. The classic histopathologic features include dense lymphocytic infiltration, storiform fibrosis, and obliterative phlebitis with abundant IgG4-positive cells. In computed tomography scans, IgG4-related mediastinal disease appears as lymphadenopathy or anterior mediastinal mass, which can be confused with lymphoma.
Multiple cases in which lymphoma occurred in patients with IgG4-RD have been reported. However, the underlying pathophysiologic mechanisms that may potentially contribute to lymphomagenesis in IgG4-RD are not well defined. In a case series of eight lymphomas occurring in patients with IgG4-RD, the tumors were mostly low-grade B-cell lymphomas with predilection for extranodal sites.
In the present case, anterior mediastinal mass excision was performed, and IgG4-RD was diagnosed in May 2019. The patient had been treated with intravenous steroids (prednisone) since May 2019. Azathioprine was added in November 2019. Despite the treatment, new soft-tissue masses and enlarged lymph nodes developed in the thorax. Core needle biopsy of the right axillary lymph node and excisional biopsy of the right paratracheal lymph node revealed T-lymphoblastic lymphoma. Excisional biopsy of the mass in the right internal mammary chain was also performed, and atypical lymphocyte infiltrations were observed, which were suggestive of T-lymphoblastic lymphoma.
Azathioprine is associated with an increased risk of lymphoma development. However, considering that azathioprine was used for < 2 months, it is presumed that azathioprine use was not the cause of lymphoma in this case.
References
References
1. Bledsoe, J. R., Wallace, Z. S., Stone, J. H., Deshpande, V., & Ferry, J. A. (2018). Lymphomas in IgG4-related disease: clinicopathologic features in a Western population. Virchows Archiv, 472(5), 839-852.
2. Mart
Keywords

No. of Applicants : 72

▶ Correct Answer : 29/72,  40.3%
  • - ZIGONG TCM HOSPITAL OF CHINA , China CAO CUN YOU
  • - Mayo Clinic , United States AKITOSHI INOUE
  • - Other , Korea (South) HYEJIN YANG
  • - , Korea (South) HYEYOUNG CHOI
  • - Other , Korea (South) MINGI SHIN
  • - Osaka Red Cross Hospital , Japan YUSUKE UTSUNOMIYA
  • - Chungbuk National University Hospital , Korea (South) HOSIG JANG
  • - Tiger Gate Hospital , Japan SHIN-ICHI CHO
  • - Ichinomiya Nishi Hospital , Japan CHIHIRO KIGUCHI
  • - Chungbuk National University Hospital , Korea (South) JIYUN KANG
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - Other , Korea (South) SEONGSU KANG
  • - , Korea (South) HYEWON CHOI
  • - Seoul National University Bundang Hospital , Korea (South) SUNG HYUN YOON
  • - Narayana Multispeciality Hospital Jaipur Rajasthan , India JAINENDRA JAIN
  • - Yeungnam University Medical Center , Korea (South) JONGSOO PARK
  • - Gifu University Hospital , Japan Yo Kaneko
  • - Osaka City General Hospital , Japan SHU MATSUSHITA
  • - Chungbuk National University Hospital , Korea (South) MIHYEON PARK
  • - Other , Korea (South) MINSU KIM
  • - Avrasya Hospital/ISTANBUL , Turkey MURAT ULUSOY
  • - Columbia asia hospital Hebbal ,Bengaluru , India PRAVIN KUMAR M
  • - Other , Korea (South) KYU-CHONG LEE
  • - Samsung Medical Center , Korea (South) SUHO KIM
  • - Nishida Hospital , Japan SHOJI OKUDA
  • - Toranomon Hospital , Japan KAORU SUMIDA
  • - , Korea (South) WONJU HONG
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Correct Answer as Differential Diagnosis : 26/72,  36.1%
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - , Japan HIROAKI ARAKAWA
  • - Korea University Guro Hospital , Korea (South) SEOKYOUNG LEE
  • - Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
  • - Showa General Hospital , Japan MASAFUMI KAIUME
  • - , United Kingdom SAMSON KADE
  • - , Italy PAOLO BALDASSARI
  • - university of montreal , Canada Andrei Bogdan Gorgos I
  • - Chonbuk National University Hospital , Korea (South) JUNGHWAN KIM
  • - Shimada General Medical Center , Japan HAYATO NOZAWA
  • - National Center for Global Health and Medicine , Japan MASATOSHI HOTTA
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - Kyoto University Hospital , Japan YASUHISA KURATA
  • - The University of Tokyo Hospital , Japan RYO KUROKAWA
  • - Mallinckrodt Institute of Radiology , United States NAGANATHAN MANI
  • - AIMS , India NITHYA HARIDAS MALIYAM
  • - , Korea (South) YEJIN JEON
  • - Dokkyo Medical University , Japan HIROAKI ARAKAWA
  • - Chungbuk National University Hospital , Korea (South) PARK JUN HYEON
  • - Kyoto University Hospital , Japan SATOSHI IKEDA
  • - Chonnam National University Hospital , Korea (South) SOMIN LEE
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - The University of Tokyo Hospital , Japan HIROSHI TAKUMIDA
  • - , Japan KAZUMA TERAUCHI
  • - Chonnam National University Hospital , Korea (South) SEUNG WAN KANG
  • - Private sector , Greece VASILIOS TZILAS
▶ Semi-Correct Answer : 2/72,  2.8%
  • - HHS , Canada S LEE
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
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