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Weekly Chest CasesArchive of Old Cases

Case No : 1082 Date 2018-07-23

  • Courtesy of Jae Sup Jun, Sarah Kyongmin Beck / Seoul St. Mary's hospital
  • Age/Sex 66 / M
  • Chief ComplaintDyspnea and weight loss during 3 weeks
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5

Diagnosis With Brief Discussion

Diagnosis
Intravascular lymphomatosis
Radiologic Findings
Figs 1. Chest PA shows diffuse faint nodular increased opacity in both lungs.
Fig 2-4. Lung window setting of the chest CT scan shows diffuse GGO and subtle centrilobular GGO nodules in both lungs.
Fig 5. FDG-PET image shows diffuse elevated FDG uptake at the corresponding areas of abnormality on CT images.
Brief Review
Intravascular lymphomatosis (IVL), also known as malignant endotheliomatosis or angiotropic large-cell lymphoma, is a rare and distinctive form of malignant lymphoma characterized by the proliferation of neoplastic lymphoid cells, mostly of B-cell origin, in the vascular lumina. The disease can affect vessels in any organ.
The clinical features of IVL are nonspecific, but IVL most commonly manifests as fever of unknown origin, weight loss, night sweats, dementia, and cutaneous nodules or plaques. With vascular occlusion of various organs and attendant diverse and confusing clinical presentations, the diagnosis of IVL is usually deferred. IVL has been confused with other possible conditions including vasculitis, dementia, or infarction, occult neoplasm, and infection. When the lungs are involved, patients experience shortness of breath, hypoxemia, and, rarely, pulmonary hypertension, and the disease may simulate various lung diseases, particularly diffuse interstitial lung disease or a subacute or chronic infectious condition. Moreover, due to the rarity of this condition, ready diagnosis of the disease is difficult, and lung lesions in patients with IVL are often detected at autopsy.
To date, there have been several reports about the radiologic findings of lung IVLs. Although the main computed tomographic (CT) finding of lung IVLs is diffuse ground-glass opacity (GGO), the disease also may demonstrate subpleural patchy areas of consolidation and reticulonodular lesions. These CT manifestations of IVL are nonspecific and can be seen in patients with diverse diseases, which may cause delayed diagnosis of this rapidly progressive and diffuse vascular tumorous condition.
Consequently, chest physicians and radiologists should recognize this unusual vascular lymphoma as a possibility in the differential diagnosis of diffuse lung diseases.
References
1. Pulmonary Intravascular Lymphomatosis: Clinical, CT, and PET Findings, Correlation of CT and Pathologic Results, and Survival Outcome. Min Jae Cha, Kyung Soo Lee, Hye Sun Hwang, Tae Jung Kim, Tae Sung Kim, Byung-Tae Kim, Young-Hyeh Ko, and Young Mog Shim. Radiology 2016 280:2, 602-610
Keywords
Lung, Neoplasm_Malignant,

No. of Applicants : 78

▶ Correct Answer : 34/78,  43.6%
  • - The University of Kyoto Hospital , Japan TOMOAKI OTANI
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
  • - Showa University Fujigaoka Hospital , Japan KYOKO NAGAI
  • - Higashi-Ohmi General Medical Center , Japan AKITOSHI INOUE
  • - The University of Tokyo Hospital , Japan Akifumi Hagiwara
  • - Other , Korea (South) SEONGSU KANG
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - The Jikei university , Japan TAKU GOMI
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - Fujieda Municipal General Hospital , Japan HAYATO NOZAWA
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - Gifu University Hospital , Japan Yo Kaneko
  • - Samsung Medical Center , Korea (South) MIN YEONG KIM
  • - The University of Tokyo Hospital , Japan RYO KUROKAWA
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey MERIC TUZUN
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - Soonchunhyang University Hospital Seoul , Korea (South) BODA NAM
  • - , Japan NAOMI YUASA
  • - Kyoto Prefectural University of Medicine , Japan YOSHIAKI OTA
  • - Chonbuk National University Hospital , Korea (South) KUM JU CHAE
  • - Multimagem Diagn泥˜sticos , Brazil PEDRO PAULO TEIXEIRA E SILVA TORRES
  • - Wuhan Union Hospital , China QIGUANG CHENG
  • - Kizawa Memorial Hospital , Japan Shoji Okuda
  • - University of Tsukuba Hospital , Japan SODAI HOSHIAI
  • - Teikyo University Hospital , Japan KAORU SUMIDA
  • - Kyoto Prefectural University of Medicine , Japan TADASHI TANAKA
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - National Center for Global Health and Medicine , Japan HIROSHI TAKUMIDA
  • - , Japan YUMI MAEHARA
  • - Hamamatsu University Hospital , Japan YUKI HAYASHI
  • - Chonnam National University Hwasun Hospital , Korea (South) JONG EUN LEE
  • - Seoul Veterans Hospital , Korea (South) HYUN JUNG YOON
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Correct Answer as Differential Diagnosis : 2/78,  2.6%
  • - Showa university Northern Yokohama Hospital , Japan KOTA WATANABE
  • - The University of Tokyo Hospital , Japan TAKU TAJIMA
▶ Semi-Correct Answer : 8/78,  10.3%
  • - IRSA LA ROCHEELLE , France JEAN LUC BIGOT
  • - Seoul National University Hospital , Korea (South) Ju Gang Nam
  • - Avrasya Hospital/ISTANBUL , Turkey MURAT ULUSOY
  • - eskisehir devlet hastanesi , Turkey MURAT SAHIN
  • - Escola Paulista de Medicina , Brazil RENATO MASSON DE ALMEIDA PRADO
  • - TB centre kasaragod. , India rikhy krishnan
  • - Korea University Guro Hospital , Korea (South) KYU-CHONG LEE
  • - , Korea (South) JONGSUN LEE
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