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Weekly Chest CasesArchive of Old Cases

Case No : 1251 Date 2021-10-11

  • 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. 2–3. Chest CT scans showing new soft-tissue lesions in the left and right internal mammary chain and the right posterior chest wall. The right posterior chest wall mass shows a sandwich sign.
Figs. 4–7. Follow-up chest computed tomography (CT) scans showing increased size of soft-tissue lesions in the left and right internal mammary chain. Multiple enlarged lymph nodes are seen in the left supraclavicular area, left and right axillae, and mediastinum. The right posterior chest wall mass with a sandwich sign is unchanged.
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. Martínez-de-Alegría, A., Baleato-González, S., García-Figueiras, R., Bermúdez-Naveira, A., Abdulkader-Nallib, I., Díaz-Peromingo, J. A., & Villalba-Martín, C. (2015). IgG4-related disease from head to toe. Radiographics, 35(7), 2007-2025.
3. Kandiel, A., Fraser, A. G., Korelitz, B. I., Brensinger, C., & Lewis, J. (2005). Increased risk of lymphoma among inflammatory bowel disease patients treated with azathioprine and 6-mercaptopurine. Gut, 54(8), 1121-1125.
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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