Nathan W Blessing1, J Antonio Bermudez-Magner2, Maria P Fernandez3, Andrew E Rosenberg4, Sander R Dubovy3,5, Thomas E Johnson1. 1. Oculofacial Plastic & Reconstructive Surgery, Dean McGee Eye Institute, University of Oklahoma Department of Ophthalmology, Oklahoma City, Oklahoma, U.S.A. 2. Ocular Pathology, Department of Ophthalmology, Instituto de Oftalmologia Conde de Valenciana, Mexico City, Mexico. 3. Ocular Pathology, Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, Florida, U.S.A. 4. Division of Anatomic Pathology, University of Miami Miller School of Medicine, Miami, Florida, U.S.A. 5. Florida Lions Eye Bank, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, Florida, U.S.A.
Abstract
PURPOSE: To retrospectively describe the clinical characteristics, management, and outcomes of a series of patients with solitary fibrous tumor (SFT) of the orbit and to evaluate signal transducer and activator of transcription 6 (STAT6) as a diagnostic marker. METHODS: Review of a retrospective, noncomparative, consecutive series of patients treated at a single institution with a histopathologic diagnosis of SFT. Demographic, clinical, and imaging data were collected, and paraffin-embedded tissue sections were stained to evaluate for the presence of STAT6 and other pertinent markers. RESULTS: Twenty-one patients were identified. Most presented with painless progressive proptosis or eyelid swelling for less than 6 months. Imaging revealed well-circumscribed, firm, variably vascular contrast-enhancing lesions with low to medium reflectivity on ultrasound. Four tumors were histopathologically malignant. All tumors were primarily excised, and 1 patient required exenteration. Two patients were treated with adjuvant radiation therapy. Six patients had recurrent disease of which 3 underwent repeat excision, and 2 were observed. No metastatic disease or attributable deaths were observed. All lesions with available tissue stained positively for both CD34 and STAT6. CONCLUSION: This is the largest single institution case series of orbital SFT with clinicopathologic correlation and the largest series to confirm the presence of STAT6 in orbital lesions. The management of SFT remains challenging due to unpredictable tumor behavior, and complete excision is the generally recommended treatment. It remains unclear whether a subset of asymptomatic patients with histopathologically benign disease can be durably observed without negative sequelae.
PURPOSE: To retrospectively describe the clinical characteristics, management, and outcomes of a series of patients with solitary fibrous tumor (SFT) of the orbit and to evaluate signal transducer and activator of transcription 6 (STAT6) as a diagnostic marker. METHODS: Review of a retrospective, noncomparative, consecutive series of patients treated at a single institution with a histopathologic diagnosis of SFT. Demographic, clinical, and imaging data were collected, and paraffin-embedded tissue sections were stained to evaluate for the presence of STAT6 and other pertinent markers. RESULTS: Twenty-one patients were identified. Most presented with painless progressive proptosis or eyelid swelling for less than 6 months. Imaging revealed well-circumscribed, firm, variably vascular contrast-enhancing lesions with low to medium reflectivity on ultrasound. Four tumors were histopathologically malignant. All tumors were primarily excised, and 1 patient required exenteration. Two patients were treated with adjuvant radiation therapy. Six patients had recurrent disease of which 3 underwent repeat excision, and 2 were observed. No metastatic disease or attributable deaths were observed. All lesions with available tissue stained positively for both CD34 and STAT6. CONCLUSION: This is the largest single institution case series of orbital SFT with clinicopathologic correlation and the largest series to confirm the presence of STAT6 in orbital lesions. The management of SFT remains challenging due to unpredictable tumor behavior, and complete excision is the generally recommended treatment. It remains unclear whether a subset of asymptomatic patients with histopathologically benign disease can be durably observed without negative sequelae.
Authors: Leona A Doyle; Marina Vivero; Christopher Dm Fletcher; Fredrik Mertens; Jason L Hornick Journal: Mod Pathol Date: 2013-09-13 Impact factor: 7.842
Authors: Dan R Robinson; Yi-Mi Wu; Shanker Kalyana-Sundaram; Xuhong Cao; Robert J Lonigro; Yun-Shao Sung; Chun-Liang Chen; Lei Zhang; Rui Wang; Fengyun Su; Matthew K Iyer; Sameek Roychowdhury; Javed Siddiqui; Kenneth J Pienta; Lakshmi P Kunju; Moshe Talpaz; Juan Miguel Mosquera; Samuel Singer; Scott M Schuetze; Cristina R Antonescu; Arul M Chinnaiyan Journal: Nat Genet Date: 2013-01-13 Impact factor: 38.330