Solitary Fibrous Tumor of the Central Nervous System

Abstract

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.

NAB2–STAT6 fusion types account for clinicopathological variations in solitary fibrous tumors

“…In univariate analysis, only the meningeal location showed a marginal trend toward association with worse disease-free survival, which might be attributable to the difficulty in achieving complete resection of solitary fibrous tumors in this anatomical region. 34 However, the possible intrinsic biological effect of the preponderant NAB2ex6-STAT6ex16/17 fusions (17 of 19) in meningeal solitary fibrous tumors could not be totally excluded at this point. Therefore, it is of potential interest to elucidate the prognostic relevance of NAB2-STAT6 gene fusions in a future large-scale study comprising prospectively accrued solitary fibrous tumors with molecular characterization at diagnsosis, balanced proportions of tumors from various anatomic regions, and longer subsequent follow-up.…”

Intramedullary spinal cord solitary fibrous tumor: A rare clinical entity

“…Additionally, a "ying-yang" sign ("patchy black and white" pattern with areas of hyperintensity and hypointensity on T2 sequences) may suggest SFT. This sign occurs because of staghorn vascularity and variations in cellularity [1,8].…”

“…Solitary Fibrous Tumors (SFT) are rare and typically arise from visceral pleura, first described in 1931 using the term "localized fibrous mesothelioma" [1]. This tumor can also occur in non-serosal sites including the spinal cord.…”

“…Of the intraspinal SFTs, the majority are dural-based and extra-medullary, with a small number being subpial, intraparenchymal, or involving nerve rootlets with no dural connection [1,4].…”

A Technical Problem in Diagnosis Clarity of Solitary Spinal Cord Neurofibroma in an Eleven-month-old Boy

“…Immunohistochemistry is critical to establish a correct diagnosis. 8 In our case, it was a real diagnostic challenge for an 11-month period. The discrete atypical symptoms and the fl uctuating fever with unknown etiology were the cause of a lot of laboratory examinations and treatment with different antibiotics in the hospital.…”