Rare cancer with primary pleural epithelioid hemangioendothelioma diagnosed by thoracoscopic biopsy achieving disease control after 16 months: case report and literature review

Figures

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  FIGURE 1. Preoperative computed tomography (CT) scans without intravenous contrast due to suspicion of renal disease. Note the presence of the large right-side pleural effusion (enclosed by the dotted circle) along with localized nodularity at the posterior costal arches and limited pleural fluid in the interlobar fissure after percutaneous drainage.

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  FIGURE 2. Histological evaluations employing hematoxylin–eosin staining with (A) low and (B) high magnifications revealed pleural infiltration by epithelioid cells with mild nuclear atypia and moderate eosinophilic cytoplasm with occasional intracytoplasmic vacuoles arranged in a myxohyaline stroma. The arrowheads in the inset image on the bottom left indicate the intracytoplasmic vacuoles. Scale bar: (A) 100 μm and (B) 20 μm. These representative images (with the dashed rectangles indicating the areas enlarged in the inset images shown on the bottom left) demonstrate neoplastic cells exhibiting diffuse strong nuclear positive immunostaining for calmodulin binding transcription activator 1 (CAMTA1) with intense immunopositivity for the vascular markers CD31, CD34, and ERG. Scale bar: (C–F) 100 μm.

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  FIGURE 3. (A) Timeline showing the clinical course of the patient for pleural epithelioid hemangioendothelioma (pEHE) and post-diagnosis management. (B) Computed tomography (CT) scan obtained 9 months after commencement of first-line therapy.