Primary splenic epithelioid hemangioendothelioma with diffuse metastases revealed by FDG PET/CT imaging: A case report.

RATIONALE

Epithelioid hemangioendothelioma (EHE) is a rare low-to-intermediate grade malignant vascular neoplasm. We report a primary splenic EHE with diffused metastasis who underwent 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT). Our case emphasizes that EHE should be considered a differential diagnose of 18F-FDG-avid splenic malignancies.

PATIENT CONCERNS

A 39-year-old man presented with abdominal distension and chest distress for 20 days and lumbago for 2 days. Transthoracic echocardiography suggested a large amount of pericardial effusion. Contrast-enhanced CT imaging showed splenomegaly with multiple low-density nodules with ring enhancement. A large irregular mass was also found in the right superior mediastinum with heterogeneous density and enhancement. 18F-FDG PET/CT imaging revealed splenomegaly, filled with intense hypermetabolic nodules and masses. And multiple regions of increased 18F-FDG uptake were observed in the mediastinum, left pleura, and bones.

DIAGNOSIS

EHE of the spleen.

INTERVENTIONS

Half a month after the diagnosis was confirmed, the patient then underwent chemotherapy, Docetaxel combined with carboplatin, and Endu were administrated every 3 weeks.

OUTCOMES

During the 6-month follow-up period, the patient has finished 4 cycles of chemotherapy combined with 2 months of targeted drug. Efficacy assessment is partial remission through CT imaging, and clinical symptoms of patient improved significantly.

LESSONS

Primary splenic EHE is extremely rare, especially with diffuse systemic metastasis. Our report suggested that EHE should be considered a differential diagnosis of 18F-FDG-avid splenic malignancies. Furthermore, 18F-FDG PET/CT plays critical role in staging and accessing disease extent of EHE.