PMC10164843_01
Coronal chest (A) and abdominal (B) T2-weighted MRI images show extensive and infiltrative cervical, mediastinal, abdominal, and retroperitoneal lesion, with bilateral hilar, pleural, mesenteric, and perirectal involvement.
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Dynamic contrast-enhancing magnetic resonance imaging (MRI) confirmed an extensive cervical, mediastinal, abdominal, and retroperitoneal lesion, with bilateral hilar, pleural, mesenteric, and perirectal involvement, with fluid signal and some foci of late enhancement (Figure 1).
Thoracic (A), abdominal (B), and pelvic (C) intranodal lymphangiography reveal delay in contrast progression and deviation to vascular malformation's ectatic lymphatic channels.
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Intranodal lymphangiography revealed delay in contrast progression and deviation to vascular malformation's ectatic lymphatic channels at abdominal, thoracic, and cervical levels (Figure 2).
The histologic analyses show remarkable presence of dilated lymphatic channels (podoplanin positive). The immunostain for actin displays the irregularity of the vessel wall, due to the irregular proliferation of the muscular layer.
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Histology second look revealed remarkable presence of dilated lymphatic channels (podoplanin positive), confirming a lymphatic-venous malformative vascular anomaly (Figure 3).