Role of FDG-PET scan in staging of pulmonary epithelioid hemangioendothelioma

Abstract

In this report we describe a case of pulmonary epithelioid hemangioendothelioma (PEH) in a young woman. The neoplasm manifested with dry cough, chest pain, finger clubbing, and multiple bilateral pulmonary nodules on chest x-ray and computed tomographic (CT) scan. She underwent thoracoscopy, and the histological features of the lung biopsies were initially interpreted as consistent with a not-well-defined interstitial lung disease. Our patient was clinically and radiologically stable over a period of four years, after which the disease progressed to involve not only the lung but also mediastinal lymph nodes, liver and bone. Fiberoptic bronchoscopy showed subtotal occlusion of the right middle and lower lobe bronchi. The histologic examination of bronchial biopsies revealed a poorly differentiated neoplasm immunohistochemically positive for vimentin and vascular markers CD31, CD34 and Factor VIII. A diagnosis of malignant hemangioendothelioma was made. Positron emission tomography (PET) is more sensitive than CT scan and bone scintigraphy in detecting PEH metastases. Furthermore, 18-fluorodeoxyglucose (FDG) uptake seems to be related to the grade of malignancy of PEH lesions. Therefore, we suggest that FDG-PET should be included in the staging system and follow-up of PEH.

Keywords: CT-scan; FDG-PET; lung cancer staging; pulmonary epithelioid hemangioendothelioma.

Figure 1

CT scan of the thorax. A: multiple bilateral centimetric and subcentimetric pulmonary. B: extensive areas of consolidation in the right middle and lower lobes, and bilateral centimetric pulmonary nodular opacities.

Figure 2

. Top: Bronchial biopsy showing PPA. A: Diffuse submuco-sal infiltration by anaplastic, large cells (hematoxylineosin, original × 25); B: Neoplastic cells stained positively for CD31 (original × 25). Bottom: Lung biopsy showing PEH; C: nodule with a fibrotic core and a micropolypoid structure at their perifery (hematoxylin-eosin, original × 250); D: Rare CD34 positive cells (original × 400).

Figure 3

18-FDG-PET showing abnormal accumulation throughout the right middle and lower lobe, and in mediastinal lymph nodes, in the left iliac crest (arrow) and in a left rib (arrow).