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A perplexing airspace: peace of mind now or later

Ismini Kourouni, Caroline M. Abramovich, Stephen W. Tamarkin, Joseph F. Tomashefski Jr., Edward D. Sivak
Breathe 2021 17: 210017; DOI: 10.1183/20734735.0017-2021
Ismini Kourouni
1Division of Pulmonary, Critical Care and Sleep Medicine, Case Western Reserve University (MetroHealth), Cleveland, OH, USA
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  • For correspondence: ikourouni@metrohealth.org
Caroline M. Abramovich
2Dept of Pathology, Case Western Reserve University (MetroHealth), Cleveland, OH, USA
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Stephen W. Tamarkin
3Dept of Radiology, Case Western Reserve University (MetroHealth), Cleveland, OH, USA
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Joseph F. Tomashefski Jr.
2Dept of Pathology, Case Western Reserve University (MetroHealth), Cleveland, OH, USA
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Edward D. Sivak
1Division of Pulmonary, Critical Care and Sleep Medicine, Case Western Reserve University (MetroHealth), Cleveland, OH, USA
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Figures

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  • Figure 1
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    Figure 1

    a–c) CT images demonstrating the mostly thin-walled, pleural-based, cavitary lesion in the periphery of the RML.

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    Figure 2

    CT demonstrating the low density enlarged right hilar lymph node. Similar appearing nodes were also present elsewhere in the mediastinum.

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    Figure 3

    Lack of FDG activity in the cavitary lesion on a fused PET/CT image.

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    Figure 4

    Pathology slide from pulmonary resection showing multiple, cystically dilated lymphatic spaces surrounded by a reactive myofibroblastic proliferation, abundant haemo­siderin and scattered granulomas. Large, abnormal collateral pleural vessels were present, intermixed with the lymphangioma and resulting in a sizable pleural adhesion to the chest wall.

  • Figure 5
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    Figure 5

    a–c) Immunostaining for CD31 and D2-40 confirmed the lymphatic nature of the cystic spaces, and the surrounding myofibroblastic reaction was negative for HMB-45, excluding lymphangioleiomyomatosis.

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    Figure 6

    Lymph node resection depicting lymphangioma within the lymph node.

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    Figure 7

    a–c) Convex probe EBUS images of subcarinal station showing ill-defined margins, heterogeneous consistency and absence of central blood vessels. Note the absence of Doppler flow signal.

Tables

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  • Table 1

    Clinical characteristics of lymphangiomas and differential diagnosis

    Age at presentationChildhood (90% <2 years-old)
    AetiologyCongenital
    Acquired when in the context of chronic lymphatic obstruction due to chronic infection, radiation therapy or trauma
    Sex predilectionNone in early diagnosis, female when diagnosed in adults
    Thoracic manifestationsIntrapulmonary mass
    Mediastinal mass (equal distribution among compartments)
    Chylous pleural or chylous pericardial effusion
    Extrathoracic manifestationsHead, neck, axilla, abdomen, bones
    ClassificationCapillary or simple, cavernous, cystic
    Natural historyNo spontaneous resolution
    Secondary infections may occur
    Differential diagnosis [9]Lymphangiectasis (primary or secondary)
    Lymphangiomatosis
    Lymphatic dysplasia syndromes (e.g. lymphedema, yellow nail syndrome)
    Lymphangiosarcoma
    Acquired lymphatic injuries (e.g. traumatic)
    Other lymphatic abnormalities (e.g. lymphangioleiomyomatosis, lymphangiolipomas, haemangiolymphangiomas)
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A perplexing airspace: peace of mind now or later
Ismini Kourouni, Caroline M. Abramovich, Stephen W. Tamarkin, Joseph F. Tomashefski, Edward D. Sivak
Breathe Mar 2021, 17 (1) 210017; DOI: 10.1183/20734735.0017-2021

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A perplexing airspace: peace of mind now or later
Ismini Kourouni, Caroline M. Abramovich, Stephen W. Tamarkin, Joseph F. Tomashefski, Edward D. Sivak
Breathe Mar 2021, 17 (1) 210017; DOI: 10.1183/20734735.0017-2021
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