- Neurosurgery Department, Centre Hospitalier Universitaire Vaudois (CHUV), Rue du Bugnon 46, Switzerland
- University Institute of Pathology, Centre Hospitalier Universitaire Vaudois (CHUV), Rue du Bugnon 25, 1011 Lausanne, Switzerland
- Rudolf Magnus Institute of Neuroscience, Department of Neurosurgery, University Medical Center Utrecht, Heidelberglaan 100, 3508 GA Utrecht, Netherlands
Rudolf Magnus Institute of Neuroscience, Department of Neurosurgery, University Medical Center Utrecht, Heidelberglaan 100, 3508 GA Utrecht, Netherlands
DOI:10.4103/2152-7806.66852© 2010 Benoit M This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Benoit M, Janzer R, Regli L. Bifrontal solitary fibrous tumor of the meninges. Surg Neurol Int 29-Jul-2010;1:35
How to cite this URL: Benoit M, Janzer R, Regli L. Bifrontal solitary fibrous tumor of the meninges. Surg Neurol Int 29-Jul-2010;1:35. Available from: http://sni.wpengine.com/surgicalint_articles/bifrontal-solitary-fibrous-tumor-of-the-meninges/
Background:We report the case of a bifrontal solitary fibrous tumor (SFT) arising from the meninges. The points of interest in this case report are the particular imaging appearance, the immunohistochemical findings and the surgical features.
Case Description:A 53-year-old Caucasian male presented with a 1-year history of behavioral changes, attention disorders and anterograde memory disorders. Magnetic resonance imaging revealed a bifrontal heterogeneous lesion attached to the anterior falx cerebri with a prominent multicompartmental cystic part. The patient underwent craniotomy for a sub-total resection of the tumor. At surgery, the multicystic component was highly vascularized and encased the anterior cerebral arteries. Neuropathological findings were consistent with a solitary fibrous tumor. Despite the absence of malignant features, there was a focal expression of p53.
Conclusion:SFT is a pathological entity with specific immunohistochemical features; it has frequently been misdiagnosed in the past. The multicystic imaging appearance of this SFT and the particular p53 immunohistochemical staining are features that should be added to the growing data on intracranial SFTs. The surgical features described (high vascularization and partial vessel encasement) may help improve surgical planning.
Keywords: CNS tumors, meningeal tumors, p53, solitary fibrous tumor
Solitary fibrous tumors (SFTs) were first described in the visceral pleura by Klemperer and Rabin [
SFTs in the CNS were first reported in 1996 by Carneiro et al.,[
The subject of this case report is an unusual dura -based SFT arising from the falx cerebri, invading both frontal lobes. What makes this report interesting is the particular imaging appearance due to a large multicystic component, and the immunohistochemical staining. In addition, the highly vascularized structures and the arterial encasement are important features of this SFT that help improve surgical planning.
The patient was a 53-year-old right-handed Caucasian male who presented with a 1-year history of behavioral changes characterized by a severe frontal syndrome (apathy, abulia, lack of energy, anosognosia, familiarity and disinhibition), as well as attention disorders and anterograde memory deficits. He also described persistent bifrontal headaches for the same period of time, which were relieved by simple analgesia.
General physical examination was unremarkable, although recent-onset obesity (BMI, 34) was noted.
Neurological examination revealed a cooperative and oriented patient with no cranial nerve deficiencies. No focal sensory and motor disabilities were noted, except for a right plantar reflex in extension.
The MRI examination revealed a large bifrontal extra-axial lesion with an attachment to the anterior falx cerebri. The lesion was heterogeneous, presenting two distinct aspects: a smaller, fleshy, nodular part attached to the meninges (23 mm in diameter) and a large (41 × 87 × 68 mm) multi-compartmented cystic part with thick septa and fleshy parts. The lesion was markedly hyperintense on T2 images and had two distinct contrast enhancement patterns on T1 images, with a homogeneous enhancement of the fleshy part and heterogeneous enhancement limited to the septa in the cystic part [
Magnetic resonance imaging of a large frontal interhemispheric heterogeneous lesion; (a) sagittal T1-weighted scan; (b) axial and coronal T2-weighted images showing the important mass-effect and the partial inclusion of both anterior cerebral arteries and their branches in the tumor (arrows); (c) and (d) axial and sagittal gadolinium-enhanced T1-weighted images showing both the anterior solid (asterisk) and the multicystic components of the lesion with the homogenous enhancement of the solid part, as well as the enhancement of the thick cystic walls
An important point to mention is that the lesion encased both A2 segments of the anterior cerebral arteries [ACAs] and their branches.
The patient underwent a bifrontal craniotomy and a subtotal resection of the tumor. The preoperative plan was to access the tumor interhemispherically, remove the solid, fleshy part and then resect the multicystic part. The encasement of both ACAs in the tumor appeared to present a major challenge.
The tumor was firm and relatively avascular in its anterior nodular part. Microsurgical dissection identified a clear arachnoidal plane. Resection of the anterior nodular part was easily performed. Once the multicystic portion was reached, the arachnoidal plane of dissection disappeared and despite meticulous microsurgical dissection, the plan had to be subpial. The intralesional consistency also changed once the multicystic component was reached. The multiple septa were thick and extremely vascular. Hemostasis was tedious despite abundant bipolar electrocoagulation. The surgical impression was similar to a hemangiopericytoma. In line with the absent tumor-brain interface, the ACAs were adherent to the tumor capsule and encased within the tumor in its multicystic part. Tumor resection was subtotal (99%), with only tiny tumor remnants on the anterior cerebral artery branches.
The postoperative recovery was uneventful, and the patient showed complete remission of headaches and normalization of behavioral deficits. He returned to normal weight and was able to return to work.
At the 1-year follow-up, the patient was found to be symptom free. The MRI revealed only minimal tumoral residue on the right ACA, measuring 2 × 3 mm, with no evidence of growth, when compared to the condition at the 3-month follow-up [
The compact tumor showed irregular borders with invasion of the dura and the subarachnoid space but no signs of brain invasion. The tumor was mostly composed of spindle-shaped cells disposed in a fascicular arrangement and separated by prominent eosinophilic bands of collagen [
Histology and immunohistochemical profile of resected tumor (all original magnification at ×200; a+d at ×400): a) Tumor mostly containing spindle cells arranged in fascicles. Hematoxilyn and eosin staining coloration; b) Expression of CD34 in most tumor cells; c) Expression of Bcl-2 in most tumor cells; d) Nuclear expression of p53 in about half of the tumor cells
There was strong cytoplasmic expression of vimentin, CD34, Bcl-2, CD99; and a focal nuclear expression of the progesterone receptor. Stainings for Epithelial Membrane Antigen (EMA), cytokeratins, protein S-100, CD117 and estrogen receptor were negative. The proliferation index indicated by an MIB-1 staining was low (less than 1%), and there were no more than 2 mitoses per 10 high-power fields. These morphological and immunohistochemical findings are in keeping with an SFT [
Solitary fibrous tumor (SFT) is an uncommon spindle cell tumor that typically arises from the visceral pleura.[
The imaging characteristics described in this case report are quite atypical compared to the classical features of SFTs reported in the literature. Typically, SFTs present themselves as well-defined masses with low signal intensity on T2-weighted images and homogeneous enhancement on post-contrast T1-weighted images, leading to an initial radiographic impression of the more common meningiomas.[
Histologically, SFTs are tumors composed of spindle cells growing in fascicles separated by thick collagen bundles. More compact areas or irregular arrangements may be present. The vascular stroma may resemble the stag-horn pattern seen in hemangiopericytoma.[
Immunohistochemically, SFTs are typically positive for vimentin, CD34, CD99 and Bcl-2. Protein S-100, EMA and CD117 are usually negative; as well as vascular, neural crest and muscle immunological markers also are negative.[
Expression of p53 has only been reported in SFTs located outside the CNS and in SFTs with histologically malignant features.[
SFTs are considered as benign tumors with a slow, indolent and nonaggressive course. Recurrence and metastasis are rare.[
SFTs represent a unique pathological entity that is more often recognized today due to clear immunohistochemical markers. Prior to the development of these markers, these tumors were often misdiagnosed. Until more data is gathered on the management of SFTs, the current literature suggests complete resection of the tumor and careful radiological follow-up as the optimal treatment program for all patients with SFTs. It should be kept in mind, however, that recurrence and metastasis are possible; even if the postoperative course is benign, as in most cases.
This case report is of interest because of the multicystic appearance, which adds new imaging features of CNS SFTs. It also emphasizes the importance of careful pre-surgical planning, because of the “hemangiopericytoma-like” vascularization pattern of the tumor and possible encasement in the tumor of arterial branches “en passage.” Finally, it also reports the positive p53 staining inside the CNS without malignant histological features.
We would like to thank Dr. P. Maeder and Dr. A. Abdelmoumene for the imaging material and their advice, as well as Dr. John McManus for language-related advice.
1. Alvarez-Fernandez E, Diez-Nau MD. Malignant fibrosarcomatous mesothelioma and benign peural fi-broma (localized fibrous mesothelioma) in tissue culture. Cancer. 1979. 43: 1658-63
2. Bongiovanni M, Viberti L, Pecchioni C, Papotti M, Thonhofer R, Sapino A. Steroid hormone receptor in pleural solitary fibrous tumours and CD34+ progenitor stromal cells. J Pathol. 2002. 198: 252-7
3. Brunori A, Cerasoli S, Donati R, Gingaspero F, Chiapetta F. Solitary fibrous tumour of the meninges: Two new cases and review of the literature. Surg Neurol. 1999. 51: 636-40
4. Burger PC, Scheithuaer BW, Vogel FS.editors. Solitary fibrous tumour. Surgical Pathology of the Nervous System and its Coverings. Churchill-Livingstone; 2002. p. 71-3
5. Cameselle-Teijeiro J, Varela-Duran J, Fonseca E, Villanueva JP, Sobrinho-Simoes M. Solitary fibrous tumour of the thyroid. Am J Clin Pathol. 1994. 101: 535-8
6. Carneiro SS, Scheithauer BW, Nascimento AG, Hirose T, Davis DH. Solitary fibrous tumour of the meninges: A lesion distinct from fibrous meningioma.A clinicopathologic and immunohistochemical study. Am J Clin Pathol. 1996. 106: 217-24
7. Caroli E, Salvati M, Epimenio RO, Lenzi J, Santoro A, Giangaspero F. Solitary fibrous tumour of the meninges: Report of four cases and literature review. Neurosurg Rev. 2004. 27: 246-51
8. Carretta A, Bandiera A, Melloni G, Ciriaco P, Arrigoni G, Zannini P. Solitary fibrous tumours of the pleura: Immunohistochemical analysis and evaluation of prognostic factors after surgical treatment. J Surg Oncol. 2006. 94: 40-4
9. El-Naggar AK, Ro JY, Ayala AG, Ward R, Ordonez NG. Localized fibrous tumour of the serosal cavities. Am J Clin Pathol. 1989. 92: 561-5
10. Flint A, Weiss SW. CD34 and keratin expression distinguishes solitary fibrous tumour (fibrous mesothelioma) of pleura from desmoplastic mesothelioma. Hum Pathol. 1995. 26: 428-31
11. Gentil Perret A, Mosnier JF, Duthel R, Brunon J, Barral F, Boucheron S. Solitary fibrous tumour of the meninges. Ann Pathol. 1999. 19: 532-5
12. Kim KA, Ganzalez I, McComb JG, Giannotta SL. Unusual presentation of cerebral solitary fibrous tumour: Report of four cases. Neurosurgery. 2004. 54: 1004-9
13. Kleihues P, Cavanee KW. Pathology and genetics of the tumours of the nervous system (WHO). Lyon: International Agency for Research in Cancer. 2000. p.
14. Klemperer P, Rabin CB. Primary neoplasms of the pleura. Arch Pathol. 1931. 11: 385-412
15. Koçak A, Cayli SR, Saraç K, Aydin NE. Intraventricular solitary fibrous tumour.An unusual tumour with radiological, ultra-structural and immunhistochemical evalutation: Case report. Neurosurgery. 2004. 54: 213-7
16. Kottke-Marchant K, Hart WR, Broughton T. Localized fibrous tumour (localized fibrous mesothelioma) of the liver. Cancer. 1989. 64: 1096-102
17. Martin AJ, Fisher C, Igbaseimokumo U, Jarosz JM, Dean AF. Solitary fibrous tumours of the meninges: Case series and literature review. J Neurooncol. 2001. 54: 57-69
18. Ng NK, Choi PC, Wong CW, To KF, Poon WS. Metastatic solitary fibrous tumour of the meninges.Case report. J Neurosurg. 2000. 93: 490-3
19. Pakasa NM, Pasquier B, Chambonnière ML, Morrison AL, Khaddage A, Peoc’h M. Atypical presentation of solitary fibrous tumours of the central nervous system: An analysis of unusual clinicopathological and outcome patterns in three new cases with a review of the literature. Virchows Arch. 2005. 447: 81-6
20. Parveen T, Fleischman J, Petrelli M. Benign fibrous tumour of the tunica vaginalis testes.Report of a case with light, electron microscopy, and review of the literature. Arch Pathol Lab Med. 1992. 116: 277-80
21. de Ribeaupierre S, Meagher-Villemure K, Agazzi S, Rilliet B. Meningeal solitary fibrous tumour in a child. Childs Nerv Syst. 2006. 22: 619-22
22. Suster S, Nascimento AG, Miettinen M, Nickel JZ, Moran C. Solitary fibrous tumours of soft tissue. Am J Surg Pathol. 1995. 19: 1257-66
23. Tihan T, Viglione M, Rosenblum MK, Olivi A, Burger PC. Solitary fibrous tumours in the central nervous system.A clinicopathologic review of 18 cases and comparison to meningeal hemangiopericytomas. Arch Pathol Lab Med. 2003. 127: 432-9
24. Van de Rijm M, Lombard CM, Rouse RV. Expression of CD34 by solitary fibrous tumors of the pleura, mediastinum, and lung. Am J Surg Pathol. 1994. 18: 814-20
25. Witkin GB, Rosai J. Solitary fibrous tumour of the mediastinum: A report of 14 cases. Am J Surg Pathol. 1989. 13: 547-57
26. Witkin GB, Rosai J. Solitary fibrous tumour of the upper respiratory tract: A report of six cases. Am J Surg Pathol. 1991. 15: 842-8
27. Yokoi T, Tsuzuki T, Yatabe Y, Suzuki M, Kurumaya H, Kakudo K. Solitary fibrous tumour: Significance of p53 and CD34 immunoreactivity in its malignant transformation. Histopathology. 1998. 32: 423-32
28. Young RH, Clement PB, McCaughei WT. Solitary fibrous tumours (“fibrous mesotheliomas”") of the peritoneum. Arch Pathol Lab Med. 1990. 114: 493-5
29. Yousem SA, Flynn SD. Intrapulmonary localized fibrous tumour. Am J Clin Pathol. 1988. 89: 365-9