INTRODUCTION: Epithelioid Hemangioendothelioma (EHE) is a vascular tumor that rarely presents of pulmonary origin. CREST Syndrome (calcinosis cutis, Raynaud phenomenon, esophageal motility disorder sclerodactyly, and telangiectasia) is associated with a modest increase in risk of lung cancer. We present a case of a patient who presents with concomitant EHE and CREST syndrome.
CASE PRESENTATION: Patient is a 59 year old Caucasian female with a past medical history of CREST syndrome, pulmonary fibrosis, pulmonary hypertension, and hypothyroidism, who presented for further workup of multiple pulmonary nodules that were found on a CT scan of the chest. Her initial diagnosis of CREST was made in 2007 during the workup of her severe pulmonary hypertension. She also was found to have pulmonary fibrosis that was considered to be most likely secondary to the CREST syndrome. During her follow up, multiple nodules were followed with an outpatient PET scan which showed the largest lesions to be hypermetabolic. Subsequently, the patient underwent VATS which histologically showed EHE. The patient was given a brief trial of Sunitinib by medical oncology, but the medication was eventually discontinued due to side effects of diarrhea and worsening of her pulmonary hypertension. Ultimately, the patient expired from complications of her pulmonary hypertension and respiratory failure.
DISCUSSION: EHE is rare cancer that was first described in 1975 and it was initially thought to be an aggressive variant of bronchoalveolar cell carcinoma. EHE can present with a variable clinical course: it can be asymptomatic, indolent with chronic cough, regress spontaneously, or be rapidly fatal with similar CT findings. It is a tumor that is usually located in soft tissue, bone or liver, but it can rarely be of primary pulmonary origin. The radiographic appearance of EHE can be easily mistaken for metastatic disease. EHE is a vascular tumor and is considered a sarcoma that does not have a well-described natural history. The literature has documented cases that were stable without progression over a decade or cases that were rapidly fatal in months even with similar CT scan findings. The clinical presentation of EHE favors women over men and is half of the patients are less than 40 years old, but EHE can present in men and women of any age. Chemotherapy and radiotherapy are generally considered ineffective and curative surgery is often not feasible since EHE often presents as multifocal bilateral lesions. In the pulmonary form of EHE, half of the cases die due to respiratory failure. Case reports and small phase 2 clinical trials have reported clinical response or regression to chemotherapy regimens that include medications that block vascular endothelial growth factor (VEGFR) such as bevacizumab, or a VEGFR Tyrosine kinase inhibitor (sunitinib, sorafenib, or pazopanib). There have also been a few case reports that describe clinical response to treatment with regimens of carboplatin plus etoposide or interferon. CREST syndrome has been associated with a modest increase in risk for certain malignancies, especially lung cancers, but no previous case reports have reported EHE in a patient with CREST syndrome.
CONCLUSIONS: To our knowledge, this is the first reported case of EHE in the English literature that presents concomitantly in a patient with the CREST syndrome.
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Reference #2 Park MS, Ravi V, Araujo DM Inhibiting the VEGF-VEGFR pathway in angiosarcoma, epithelioid hemangioendothelioma, and hemangiopericytoma/solitary fibrous tumor. Curr Opin Oncol. 2010 Jul;22(4):351-5.
DISCLOSURE: The following authors have nothing to disclose: Jason Henderson, Mark Lega, Marvin Balaan
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