We reviewed the world literature on solitary fibrous tumors of the central nervous system from August 1996 to July 2011, focusing on both clinicopathological features and diagnostic findings. The anatomical distribution of the 220 cases reported so far reveals that most are intracranial and just over one-fifth are intraspinal. In decreasing frequency, intracranial tumors involve the supratentorial and infratentorial compartments, the pontocerebellar angle, the sellar and parasellar regions, and the cranial nerves. Intraspinal tumors are mainly located in the thoracic and cervical segments. Although most solitary fibrous tumors of the central nervous system are dural based, a small subset presents as subpial, intraparenchymal, intraventricular, or as tumors involving the nerve rootlets with no dural connection. Preoperative imaging and intraoperative findings suggest meningioma, schwannoma or neurofibroma, hemangiopericytoma, or pituitary tumors. Immunohistochemistry is critical to establish a definitive histopathological diagnosis. Vimentin, CD34, BCL2, and CD99 are the most consistently positive markers. The usual histologic type generally behaves in a benign manner if complete removal is achieved. Recurrence is anticipated when resection is subtotal or when the tumor exhibits atypical histology. The proliferative index as assessed by MIB1 labeling is of prognostic significance. Occasionally, tumors featuring conventional morphology may recur, perhaps because of minimal residual disease left behind during surgical extirpation. Rare extracranial metastases and tumor-related deaths are on record. Surgery is the treatment of choice. Stereotactic and external beam radiation therapy may be indicated for postsurgical tumor remnants and for unresectable recurrences. Long-term active surveillance of the patients is mandatory.
*Departments of Pathology
§Neurosciences, IRCCS “Casa Sollievo della Sofferenza” Hospital, San Giovanni Rotondo (FG), Italy
†Department of Pathology, Cook Children's Medical Center, Fort Worth, TX
∥Department of Pathology and Laboratory Medicine, “S. Maria della Misericordia” General Hospital, Udine, Italy
¶Unit of Anatomic Pathology, IRCCS-CROB, Referral Cancer Center of Basilicata, Rionero in Vulture (PZ), Italy
#Department of Hematology, Oncology and Clinical Pathology, Policlinico S. Orsola, University of Bologna, Bologna, Italy
**Department of Pathology, University of Catania, Catania, Italy
††Department of Pathology, Barzilai Medical Center, Ashkelon, Israel
The authors have no funding or conflicts of interest to disclose.
Reprints: Michele Bisceglia, MD, Department of Pathology, Division of Anatomic Pathology, IRCSS “Casa Sollievo della Sofferenza” Hospital, V.le Cappuccini, 71013 San Giovanni Rotondo, Italy (e-mail: firstname.lastname@example.org).All figures can be viewed online in color at http://http://www.anatomicpathology.com.