SESSION TYPE: Cancer Cases I
PRESENTED ON: Monday, October 22, 2012 at 01:45 PM - 03:00 PM
INTRODUCTION: Pulmonary Epithelioid Hemangioendothelioma (PEHE) is a very rare vascular tumor, only about 100 cases have been reported worldwide. PEHE is often diagnosed incidentally or with minor symptoms of cough, SOB or hemoptysis. Here we are reporting a case who presented to us with complaints of intermittent hemoptysis for 5 years and extensive negative work up.
CASE PRESENTATION: A 43 year old male, ex-smoker came for second opinion to our clinic regarding cough and hemoptysis for 5 years. Previous CT chest showed bilateral multiple nodules with surrounding ground glass opacities(GGO) and no pleural effusion. Subsequent bronchoscopy was negative for cultures and cytology, however BAL showed hemosiderin laden macrophages. Transbronchial biopsy was also negative for vasculitis, granulomas or malignancy. Patient was HIV-ve and PPD+ve. Rheumatological work up was also negative. Repeat CT scan showed new large prominent geogrphic areas of GGO and bilateral nodules. Decision was made for VATS biopsy due to persistent symptoms and pervious negative work up. Biopsy showed vascular lymphoid infiltration, scattered neoplastic cells, embedded in eosinophilic matrix. Immunohistochemical staining was diagnostic of PEHE. Patient was referred to oncologist, however due to lack of an accurate sense of the pace of the progression; decision was made to monitor the disease for now.
DISCUSSION: Pulmonary epithelioid hemangioendothelioma(PEHE) are usually asymptomatic, however persistent cough and dyspnea are present in few patients. Typical radiologic finding are nodular opacities in both lungs. In this case, geographic GGO areas and bilateral nodular opacities were the predominant findings on the chest CT scan. Definitive diagnosis is made by surgical lung biopsy and immunohistochemical staining. In our patient there was a significant delay before he underwent surgical biopsy because of prior negative transbronchial biopsy. Review of literature shows that there are case reports of being diagnosed with transbronchial biopsy but eventually had an open lung biopsy done. No standard treatment is available because of its rarity. As our patient had no progression with minimal symptoms we decided to proceed with close observation.
CONCLUSIONS: Typical radiological findings in PEHE are multiple lung nodules. Our case emphasizes the unusual radiological presentation of this rare disease with geographic areas of GGO with multiple nodules. Therefore early work up with VATS or open lung biopsy should be considered.
1) Mukundan et al PEHE: Atypical Radiologic Findings of a Rare Tumor with Pathologic Correlation JCATomography. September/October 2000.
2) Cronin P,et al: An usual case and a review of literature: Chest 2004
DISCLOSURE: The following authors have nothing to disclose: Ravindra Ramakrishna, Amitesh Agarwal, Laurence Smolley, Julia Diacovo
No Product/Research Disclosure InformationCleveland Clinic Florida, Weston, FL