Orbital hemangiopericytoma in an Asian population
Journal
Journal of the Formosan Medical Association
Journal Volume
113
Journal Issue
6
Pages
356-363
Date Issued
2014
Author(s)
Abstract
Background/Purpose: Hemangiopericytoma is a very rare orbital tumor. The purpose of this study was to report the clinical and histopathological features of six cases of orbital hemangiopericytoma in an Asian population. Methods: Clinical and histopathological features were reviewed in six patients who were histopathologically confirmed as having primary orbital hemangiopericytoma in National Taiwan University Hospital between May 2001 and December 2010. Results: Among the six cases who were diagnosed as having primary orbital hemangiopericytoma, all lesions were reported as vascular tumors and featured branching "staghorn appearance" vessels. All patients, including one male and five females, presented with progressive proptosis and some associated symptoms such as extraocular motility limitation with diplopia, displacement of the globe, afferent pupillary defect, congested vessels of conjunctiva, or decreased visual acuity. On computed tomography, the orbital tumors tended to manifest as circumscribed masses with homogeneous medium-to-high enhancement with contrast studies. All six patients received surgical treatments, and four of them had additional radiotherapy. Three patients had recurrence after surgeries, and one of them had multiple metastases to lung and liver. All patients were still alive after a follow-up period of 5-10 years. Conclusion: Orbital hemangiopericytoma has malignant potential, which may lead to local recurrence and/or metastasis. Histopathological findings alone are insufficient to predict the behavior of this tumor. Therefore, both clinical and histopathological findings are important to evaluate the treatment outcomes. Total excision accompanied with radiotherapy is suggested and long-term follow-up is required. ? 2012.
Subjects
Asian population; Orbital hemangio-pericytoma
SDGs
Other Subjects
adult; afferent pupillary defect; article; Asian; cancer radiotherapy; cancer surgery; clinical article; clinical feature; computer assisted tomography; conjunctiva disease; differential diagnosis; diplopia; enucleation; exophthalmos; eye examination; female; hemangiopericytoma; histopathology; human; human tissue; immunohistochemistry; intensity modulated radiation therapy; liver metastasis; liver resection; lung metastasis; male; middle aged; orbit tumor; treatment outcome; tumor recurrence; video assisted thoracoscopic surgery; visual acuity; hemangiopericytoma; Orbital Neoplasms; pathology; retrospective study; Adult; Female; Hemangiopericytoma; Humans; Male; Middle Aged; Neoplasm Recurrence, Local; Orbital Neoplasms; Retrospective Studies
Publisher
Elsevier Ltd
Type
journal article