Case Presentation: Our patient is a 48-year-old female with medical history significant for hypertension who presented multiple times with recurrent intractable chest wall pain. Her history was significant for loss of appetite and weight loss of about 20 lbs in the last 2 months She had an unremarkable laboratory and imaging work up and eventually had thoracentesis performed, which was significant for an exudative, non-malignant pleural effusion. She had a bronchoscopy with biopsy performed which was negative for malignancy. Due to recurrent symptoms a PET scan revealed a hypermetabolic 1.9 cm x 3.9 cm left lingular pulmonary mass and metastatic nodular pleural thickening in the left hemithorax with small left pleural effusion concerning for a lung malignancy with pleural metastatic disease. A pleural biopsy revealed epithelioid hemangioendothelioma (EHE). Due to the unrespectable tumor, she was started on palliative radiotherapy for five sessions and was later started on adriamycin, ifosfamide and mesna. Unfortunately our patient passed away.
Discussion: Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor with multiple subtypes. Only 96 cases of EHE have been reported, of which 5 presented with pleural effusions. This tumor can arise in the bone, liver, soft tissue, and pleura. Although etiology is not well understood, it has been reported that over 90% of EHEs express oncogenic WWTR1-CAMTA1 fusion. This results in a transcriptional program that is resistant to anoikis and oncogenic transformation. We would like other internists to be aware of this diagnosis: presentation, poor prognosis, treatment.
Conclusions: Common things being common, a general internist should never anchor on a diagnosis. A patient’s symptoms should always be re-evaluated with a non-biased perspective. In addition, patients with a new medical diagnosis are usually nervous and scared, especially patients with a rare medical condition known to have a poor prognosis. It is our duty to find the time to ensure the patient has the adequate support and resources on discharge.
