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Solitary fibrous tumors of the central nervous system: report of five cases with unusual clinicopathological and outcome patterns

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Abstract

Background

This is a retrospective study of 11 patients harboring a solitary fibrous tumor (SFT) of the central nervous system (CNS), with special emphasis on unusual clinicopathological and outcomes patterns.

Method

Between 2000 and 2008, 11 patients harboring CNS SFTs were treated at our institution. Patient charts were retrospectively reviewed and tumor location, clinical presentation, imaging characteristics, extent of resection, dural origin, pathological features, adjuvant treatment, and follow-up data were collected, focusing on five atypical cases (four intracranial and one within the spine).

Findings

One intracranial SFT arose from the sella turcica and relapsed threefold during the 6 years following partial removal. Disease progressed as successive isolated local recurrences treated by subsequent surgical interventions and gamma-knife radiosurgery. The MiB-1 labeling index analysis showed a steady increase in these sequential recurrences (ranging from less than 3% up to 6%) without obvious malignant transformation. The second SFT occurred in the cerebellopontine angle and exhibited a high MiB-1 index (10%) without noticeable features of malignancy. It relapsed twice during the 5 years following gross total resection without demonstrating a more aggressive histological pattern. The third SFT arose from the cerebellar tentorium, widely invaded the lateral sinus and adjacent bone, had a low MiB-1 index, and has not recurred within the 2 years after incomplete resection. The two remaining SFTs presented with unusual clinicoradiological features. We described a extremely rare case of intraventricular SFT, and a case of extradural SFT of the thoracic spine (T8–T9) radiologically consistent with a schwannoma. Immunohistochemistry confirmed that all tumors were SFTs.

Conclusions

These atypical presentations gave us the opportunity to provide further information about the variability of the clinicoradiological patterns and natural histological course of CNS SFTs.

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Abbreviations

CNS:

Central nervous system

CPA:

Cerebellopontine angle

CT:

Computed tomography

EMA:

Epithelial membrane antigen

GKRS:

Gamma-knife radiosurgey

GTR:

Gross total removal

HPC:

Hemangiopericytoma

HPF:

High-power fields

ICH:

Intracranial hypertension

MRI:

Magnetic resonance imaging

PS100:

S100 protein

SFT:

Solitary fibrous tumor

STR:

Subtotal removal

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Conflicts of interest

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Authors and Affiliations

  1. Department of Neurosurgery, North Hospital, University Hospital of Saint-Etienne, Saint-Etienne, France

    François Vassal, Romain Manet & Christophe Nuti

  2. Department of Pathology, North Hospital, University Hospital of Saint-Etienne, Saint-Etienne, France

    Fabien Forest & Michel Péoc’h

  3. Department of Neurology, North Hospital, University Hospital of Saint-Etienne, Saint-Etienne, France

    Jean Phillipe Camdessanche

  4. Service de Neurochirurgie, Hôpital Nord, Avenue Albert Raimond, 42270, Saint-Priest en Jarez, France

    François Vassal

Authors
  1. François Vassal
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  2. Romain Manet
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  3. Fabien Forest
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  4. Jean Phillipe Camdessanche
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  5. Michel Péoc’h
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  6. Christophe Nuti
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Correspondence to François Vassal.

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Comment

This reviewer during his career encountered only one case of histologically verified solitary fibrous tumor (located in the orbit), and read with interest this review of the author's experience. Although this contribution does not contain new information to the existing literature, both the rarity and the atypical presentation of some of the cases illustrated here yet warrant this other publication. I fully agree that the real incidence of such neoplasms may be underestimated.

Domenico d'Avella

Padova, Italy

MRI shows an intensively enhancing and sharply demarcated intracranial tumor:

1. Dural attachment—meningioma/metastasis/malignant glioma ?—but also a hoard of unusual to very rare tumors, including mesenchymal ones (hemangiopericytoma/firbrosarcoma/solitary fibrous tumor or SRT/et al.) and histiocytic ones (Langerhans/Erdham-Chester/Rosai-Dorfman/et al.).

2. Parenchymal or intraventricular—metastasis ?—but also a stock of rare WHO grade I (II) gliomas characterized by intense enhancing and sharp borders other than pilocytic astrocytoma, ganglioglioma, or DNT.

Rare CNS tumors carry an aura of unpredictability. An important part of neurooncology group practice is the recognition of unusual to very rare CNS tumors and the tailoring of optimal therapy and follow-up for their carriers—based on more or less scarce published data. It is axiomatic—never trust the previous diagnosis but have it re-evaluated by your neuropathologist (more than one if in doubt). Neurooncosurgeons are grateful when distinguished colleagues see the trouble of presenting their rare tumors from the last 10 to 20 years, even more so when a critical review of the published cases follows—a practice that should be re-invented in the era of genomics.

The French colleagues do that favor to the readers of Acta Neurochirurgica by presenting their 11 cases of solitary fibrous tumor.

Juha Jaaskelainen

Kuopio, Finland

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Vassal, F., Manet, R., Forest, F. et al. Solitary fibrous tumors of the central nervous system: report of five cases with unusual clinicopathological and outcome patterns. Acta Neurochir 153, 377–384 (2011). https://doi.org/10.1007/s00701-010-0866-4

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