Case report

Distinguishing Falcine Chondrosarcomas from Their Mimics and Management

Sana Omezine1, Sofiene Bouali1, Marouen Taallah1, Alia Zehani2, Jalel Kallel1, Hafedh Jemel1

Key words - BACKGROUND: Primary intracranial chondrosarcoma is an extremely rare

- Chondroma malignant tumor of the central nervous system, which accounts for <0.16% of all
- Falx chondrosarcoma
- Intracranial tumor
primary intracranial tumors. This rare tumor has a high associated morbidity
- Meningioma from the tumor itself as well as from treatment modalities.
- Mesenchymal chondrosarcoma
- CASE DESCRIPTION: A 33-year-old man presented with a diffuse headache of
Abbreviations and Acronyms 3 months’ duration. He was admitted to our department with weakness in the
CT: Computed tomography right extremities that had persisted for more than a month. Findings of the
MRI: Magnetic resonance imaging
neurologic examination revealed right hemiparesis. Cranial magnetic resonance
From the 1Departments of Neurosurgery, National Institute imaging demonstrated a well-demarcated, parasagittal left frontal mass, which
of Neurology Tunis, Tunisia Faculty of Medicine and compressed to the lateral ventricle. It was hypointense on T1-weighted and
2
Department of Histopathology, la Rabta, Faculty of
Medicine, University of Tunis El Manar, Tunis, Tunisia hyperintense on T2-weighted images without creating edema in the surrounding
To whom correspondence should be addressed: tissue. A left frontoparietal craniotomy with complete excision of the mass was
Sana Omezine, M.D. performed. The postoperative period was uneventful, and patient was dis-
[E-mail: s.omezine@yahoo.fr] charged on the fourth postoperative day without any neurologic deficit. Histo-
Citation: World Neurosurg. (2018) 118:279-283. pathology showed a morphology that was in favor of chondrosarcoma grade 1.
https://doi.org/10.1016/j.wneu.2018.06.164
Journal homepage: www.WORLDNEUROSURGERY.org - CONCLUSIONS: Dural chondrosarcoma is a possible entity in the differential
Available online: www.sciencedirect.com diagnosis of a presumed meningioma, particularly when atypical features are
1878-8750/$ - see front matter ª 2018 Elsevier Inc. All present. We report a grade 1 intracranial chondrosarcoma of the classical
rights reserved. subtype without any neurologic problems after complete surgical excision. The
patient did not receive any adjuvant therapy and at 26 months’ follow-up showed
no recurrence.

INTRODUCTION
Primary intracranial chondrosarcoma is an
extremely rare malignant tumor of the
central nervous system, which accounts
for <0.16% of all primary intracranial
tumors and is associated with high
morbidity.1 Although the pathogenesis of
these tumors remains unclear, it has
been proposed that intracranial
chondrosarcomas develop from the
chondrocytes within rests of
endochondral cartilage present within the
skull base.2
A review of literature regarding intra-
cranial chondrosarcomas shows that only
18 cases have been reported. In light of
this, we report a new clinical observation
to enforce knowledge about this malig-
nant tumor. In this study, we explore the
current state of intracranial falcine
chondrosarcoma to provide a compre-
hensive quantitative review, improve clin-
ical characterization, and discuss potential
“pitfalls” in the management of patients
with this unusual pathologic entity.
STUDY SELECTION
A comprehensive literature search for this
review was conducted on PubMed, MED-
LINE, and Google Scholar. There were no
limitations on the date, type, or language
of the publication. The first search was
conducted using the term “falx chon-
drosarcoma” followed by “intracranial
chondrosarcoma,” and “skull base chon-
drosarcoma.” The titles and abstracts were
reviewed, and only 18 publications were
selected relating to falx chondrosarcoma.
These cases are reviewed in Table 1.
CASE DESCRIPTION
A 33-year-old man presented with a diffuse
headache of 3 months’ duration. He was
admitted to our department with weak-
ness in the right extremities that had
persisted for more than a month. Findings
of the neurologic examination revealed
right hemiparesis. All laboratory tests
(including routine blood count tests and
assessments of liver and kidney, immune
and blood coagulation functions) were
within the normal ranges. Cranial mag-
netic resonance imaging (MRI) demon-
strated a well-demarcated, parasagittal left
frontal mass. It was hypointense on
T1-weighted and hyperintense on
T2-weighted images (Figure 1). The
preoperative diagnosis radiologically was
a meningioma.
The patient underwent a left-sided
frontal craniotomy for tumor resection.
During excision, the tumor was well cir-
cumscribed, grayishewhite in color, firm
to hard in consistency, and relatively
avascular. Total resection of the tumor was
performed.
On histopathology, sections showed a
circumscribed lobulated growth pattern
with areas having hyaline cartilage. The
lobules were variable in size. The capsule
was moderately thickened and showed
mild lymphocytic infiltrate. The tumor had
low-to-moderate cellularity and nuclear

WORLD NEUROSURGERY 118: 279-283, OCTOBER 2018 www.WORLDNEUROSURGERY.org 279

 CASE REPORT

SANA OMEZINE ET AL. DISTINGUISHING FALCINE CHONDROSARCOMAS

Table 1. Summary of the Reported Cases of Falx Chondrosarcoma

Article Age, Years/Sex Location Pathology Follow-Up Recurrence Alive/Dead

Cybulski et al., 19853 58/M Anterior falx Low-grade chondrosarcoma 18 months No Alive
4
Salcman et al., 1992 28/F Middle falx Low-grade chondrosarcoma 20 months Yes Alive
Gerstzen et al., 19975 12/F Middle falx Low-grade chondrosarcoma 9 months No Alive
6
Forbes et al., 1998 19/F Anterior falx Non-mesenchymal chondrosarcoma 2 years No Alive
7
Oruckaptan et al., 2001 56/F Anterior falx Low-grade classical chondrosarcoma 3 years No Alive
Chen et al., 20048 13/F Anterior falx Mesenchymal chondrosarcoma 30 months No Unknown
Korthary et al., 20039 28/F Middle falx Low-grade chondrosarcoma No follow-up No follow-up No follow-up
Salvati et al., 200510 30/F Middle falx Low grade chondrosarcoma 18 months No Unknown
11
Kathiravel et al., 2008 32/F Anterior falx Mesenchymal chondrosarcoma 15 months No Unknown
12
Boccardo et al., 2009 32/F Anterior falx Low-grade chondrosarcoma Unknown Unknown Unknown
Gunes et al., 200913 25/M Posterior falx Low-grade chondrosarcoma No follow-up No follow-up No follow-up
Saito et al., 201014 31/F Posterior falx Mesenchymal chondrosarcoma 20 months No Unknown
Safe et al., 201115 23/F Anterior falx Grade 1 classical chondrosarcoma 16 months No Alive
16
Urgun et al., 2015 35/F Anterior falx Myxoid chondrosarcoma 9 months Yes Alive
17
Sadashiva et al., 2016 42/F Anterior falx Mesenchymal chondrosarcoma 66 months No Alive
Sadashiva et al., 201617 7/M Anterior falx Mesenchymal chondrosarcoma 21 months No Alive
17
Sadashiva et al., 2016 52/F Posterior falx Mesenchymal chondrosarcoma 16 months Yes Alive
18
Murrone et al., 2017 55/F Posterior falx Meningothelial chondrosarcoma No follow-up Unknown Unknown

pleomorphism. Predominantly the lacunae
were mononucleated; however, binucle-
ated and multinucleated lacunae also were
seen. Focal areas of calcification and
fibromyxoid changes also were seen.
Overall morphology pointed to a grade 1
chondrosarcoma (Figure 2).
The postoperative period was uneventful,
and patient was discharged on the fourth
postoperative day without any neurologic
deficit. No radiotherapy or chemotherapy
was administered, and we decided to
observe the evolution with repeated
imaging.
Follow-up of the patient with cranial
MRI at 26 months after the surgery did not
show any evidence of tumor recurrence.
Long-term follow-up is still in progress.
DISCUSSION
Chondrosarcomas account for 6% of skull
base neoplasms and 0.15% of all intra-
cranial tumors.19 Primary intracranial
chondrosarcomas were first described by
Dahlin and Henderson in 1964.20 To date,
18 cases of falcine chondrosarcomas have
been reported in the literature (Table 1).
In the reported cases, the mean patient
age was 30 years (range, 7e58 years), with
a female predominance (4 males and 15
females, a ratio of almost 1:3) (Table 1).
The reason for the observed sexual
dimorphism between males and females
is poorly understood.
A total of 9 studies reported that frontal
compartment was the most common
location for falcine chondrosarcoma. The
posterior location was reported in 4 cases,
and the middle location in 4 cases. His-
tologically, 3 variants of chondrosarcoma
have been described: myxoid, mesen-
chymal, and classic chondrosarcoma.21-23
Among intracranial chondrosarcomas,
the conventional and mesenchymal sub-
types are the predominant histologic
patterns described in the literature. The
grading system for chondrosarcomas con-
sists of 3 categories: grade I (well differen-
tiated), grade II (moderately differentiated),
and grade III (poorly differentiated).19 The
chondrosarcoma described in this report is
of the classical type.
The classic cranial and intracranial
chondrosarcoma usually arise at the
skull base and most frequently affect
adults24 and was associated with a
better prognosis.14 Mesenchymal
chondrosarcoma usually is observed in
the frontoparietal region; this entity is
highly vascular25 and it is the most
aggressive subtype, with a tendency for
recurrence and metastasis.4,24
A few hypotheses have been proposed
regarding the pathogenesis of the disease.
The dura itself does not normally contain
cartilage. However, the meninges contain
primitive multipotential mesenchymal
cells that might explain the development
of dural chondrosarcoma.25-27
An alternative explanation for their
origin is aberrant embryonal rests,28
supported by the discovery of an ectopic
nodule of cartilage in the falx of a
kitten.29 The symptoms vary among
patients, although a long-standing his-
tory of headaches and signs associated
with increased intracranial pressure are
the main symptoms. Furthermore, dizzi-
ness, tinnitus, sensory disturbances of the
face, and decreased visual acuity have
been reported in some cases.30
Cranial computed tomography (CT) and
MRI can aid in the diagnosis of these

280 www.SCIENCEDIRECT.com WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2018.06.164

 CASE REPORT

SANA OMEZINE ET AL. DISTINGUISHING FALCINE CHONDROSARCOMAS

Figure 1. Preoperative magnetic resonance imaging
(MRI): axial T1 native (A), axial (B), sagittal (C), and
coronal section (D) T2 showing extra-axial multilobular
and heterogeneous frontal mass, abutting the left falx
with associated extensive tumor related edema and 6
mm left to right midline shift. Postoperative MRI:
sagittal (E) and axial (F) postcontrast-weighted imaging.
At 2-year follow-up, there was no evidence of local
recurrence of tumor or other focal areas that were
abnormal.

tumors, although a clinical pathologic chondrosarcoma on CT and MRI is not They are isointense to hypointense on
diagnosis is the gold standard. The pathognomonic but should suggest a T1-weighted images and hyperintense on
appearance of intracranial dural relatively slow-growing extra-axial tumor. T2-weighted images and have strong

WORLD NEUROSURGERY 118: 279-283, OCTOBER 2018 www.WORLDNEUROSURGERY.org 281


SANA OMEZINE ET AL.
Figure 2. (A) Hematoxylin and eosin (H&E) 10: Low-grade chondrosarcoma with abundant myxoid matrix. (B) HE 40:
Chondrocytes with mild nuclear atypia.
enhancement after gadolinium enhance-
ment. The mesenchymal chon-
drosarcomas, the most malignant form,
are usually hypervascular angiographically
and often attach to the meninges in the
frontoparietal region, especially in the falx
cerebri.31 The lesions to be differentiated
from dural chondrosarcoma vary
depending on the location of tumor.
The differential diagnosis for such
lesions along the floor of the cranial fossa
or in the parasellar region includes me-
ningioma, chondroma, neuroma, carotid
aneurysm, and metastases.28 The main
entity in the differential diagnosis of
falcial chondrosarcoma is the more
frequently occurring meningioma, as is
the case with such lesions along the
floor of the cranium. Classically,
meningioma presents on CT as a
hyperdense, solidly enhancing extra-axial
mass with adjacent reactive bone
changes. Atypical CT features such as cyst
formation, necrosis, or lipomatous infil-
tration may be confusing,25,32 but often
the arteriogram will demonstrate classic
tumor hypervascularity and enlarged
feeding dural arteries characteristic of
meningioma.
However, we think that certain features
can be used prospectively to differentiate a
classic chondrosarcoma from a meningi-
oma. These include greater signal in-
tensity on the T2- weighted images than
that of a meningioma, islands of low
signal intensity despite lack of gross
calcification on all sequences due to well-
differentiated cartilage, frequent preser-
vation of the pial barrier, “honeycomb”
enhancement and, most importantly, lack
of perfusion.33
282 www.SCIENCEDIRECT.com
DISTINGUISHING FALCINE CHONDROSARCOMAS
In addition to these features, the relative
lack of edema also can be used to distin-
guish it from tumors such as glioblas-
tomas multiforme and metastases. Lack of
vascularity and absence of flow voids
differentiate it from a vascular malforma-
tion or the mesenchymal variety.34
Because of their low frequency, a standard
treatment for intracranial chondrosarcoma
has not been established yet. According
to the literature, complete surgical resection
is recommended.4 After resection,
radiotherapy seems to improve local control
rates in high-grade chondrosarcomas and
should be applied as adjuvant therapy.
Standard fractionated radiotherapy is com-
mon for most cases.35,36 However, the ten-
dency for the tumor to recur locally is quite
high.37 It has been suggested that recurrence
does not necessarily dictate shorter life span,
as it does in cases of extracranial
mesenchymal chondrosarcoma.38
On rare occasions, extracranial metas-
tasis also can be observed.39 To prevent
local recurrence, adjuvant radiotherapy
also can be used, especially when total
excision is not possible.35,36
Adjuvant therapies, including brachy-
therapy and proton beam treatment, have
been found to improve patient outcomes
for this rare tumor.4,22,40 However there
have been no reports on the advantages of
adjuvant chemotherapy for patients with
intracranial extraskeletal myxoid chon-
drosarcomas to date. Factors such as
pathologic pattern, previous treatment
(surgery or radiation therapy), degree of
tumor resection, and adjuvant post-
operative radiation therapy have all been
implicated in the prognosis of patients
with chondrosarcoma.
WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2018.06.164
CASE REPORT
CONCLUSIONS
Intracranial falcine chondrosarcoma is a
rare form of this entity and it should be
considered in the differential diagnosis of
intracranial brain tumors. In the absence
of such guidelines, the role of adjuvant
therapy remains unclear. Future studies
should use our results combined with the
other variables mentioned to enforce
knowledge about pathology, diagnosis,
and management of this rare tumor.
REFERENCES
1. Berkmen YM, Blatt ES. Cranial and intracranial
cartilaginous tumors. Clin Radiol. 1968;19:327-333.
2. Grossman RI, Davis KR. Cranial computed
tomographic appearance of chondrosarcoma of
the base of skull. Radiology. 1981;141:403-408.
3. Cybulski GR, Russell EJ, D’Angelo CM, Bailey OT.
Falcine chondrosarcoma: case report and litera-
ture review. Neurosurgery. 1985;16:412-415.
4. Salcman M, Scholtz H, Kristt D, Numaguchi Y.
Extraskeletal myxoid chondrosarcoma of the falx.
Neurosurgery. 1992;31:344-348.
5. Gerszten PC, Pollack IF, Hamilton RL. Primary
parafalcine chondrosarcoma in a child. Acta Neu-
ropathol (Berl). 1998;95:111-114.
6. Forbes RB, Eljamel MS. Meningeal chon-
drosarcomas, a review of 31 patients. Br J Neuro-
surg. 1998;12:461-464.
7. Oruckaptan HH, Berker M, Soylemezoglu F. Par-
afalcine chondrosarcoma: an unusual localization
for a classical variant: case report and review of
the literature. Surg Neurol. 2001;55:174-179.
8. Chen Y, Wang X, Guo L. Radiological features
and pathology of extraskeletal mesenchymal
chondrosarcoma. Clin Imaging. 2012;36:365-370.
9. Kothary N, Law M, Cha S, Zagzag D. Conven-
tional and perfusion MR imaging of parafalcine
chondrosarcoma. AJNR Am J Neuroradiol. 2003;24:
245-248.

SANA OMEZINE ET AL.
10. Salvati M, Caroli E, Frati A, Piccirilli M, Agrillo A,
Brogna C, Occhiogrosso G, Giangaspero F. Cen-
tral nervous system mesenchymal chon-
drosarcoma. J Exp Clin Cancer Res. 2005;24:317-324.
11. Kathiravel Y, Finnis ND. Primary falcine chon-
drosarcoma. J Clin Neurosci. 2008;15:1406-1409.
12. Boccardo M, Bavaresco E, Sola S, Vitali A. Paraf-
alcine chondrosarcoma: report of a case and re-
view of the literature. J Neurosurg Sci. 2009;53:
137-140.
13. Güneş M, Günaldi O, Tugcu B, Tanriverdi O,
Güler AK, Cöllüo glu B. Intracranial chon-
drosarcoma: a case report and review of the
literature. Minim Invasive Neurosurg. 2009;52:
238-241.
14. Saito Y, Takemura S, Sakurada K, Sato S,
Kayama T. Intracranial extraskeletal mesenchymal
chondrosarcoma arising from falx: a case report
and literature review. No Shinkei Geka. 2010;38:
441-448.
15. Safaee M, Clark AJ, Tihan T, Parsa AT, Bloch O.
Falcine and parasagittal chondrosarcomas. J Clin
Neurosci. 2013;20:1232-1236.
16. Akakin A, Urgun K, Eksi MS, Yilmaz B,
Yapicier Ö, Mestanoglu M. Falcine myxoid chon-
drosarcoma: a rare aggressive case. Asian J Neuro-
surg. 2018;13:68-71.
17. Sadashiva N, Sharma A, Rajalakshmi P,
Mahadevan A, Shukla D, Devi BI. Intracranial
extraskeletal mesenchymal chondrosarcoma.
World Neurosurg. 2016;95:618.e1-618.e6.
18. Murrone D, De Paulis D, di Norcia V, Di
Vitantonio H, Galzio RJ. Surgical management of
falcine meningiomas: experience of 95 patients. J
Clin Neurosci. 2017;37:25-30.
19. Cianfriglia F, Pompili A, Occhipinti E. Intracranial
malignant cartilaginous tumours. Report of two
cases and review of literature. Acta Neurochir (Wien).
1978;45:163-175.
20. De Dahlin, Henderson ED. Mesenchymal chon-
drosarcoma. Further observations on a new entity.
Cancer. 1962;15:410-417.
WORLD NEUROSURGERY 118: 279-283, OCTOBER 2018
DISTINGUISHING FALCINE CHONDROSARCOMAS
21. Chandler JP, Yashar P, Laskin WB, Russell EJ.
Intracranial chondrosarcoma: a case report and
review of the literature. J Neuoro-Oncol. 2004;68:
33-39.
22. Korten AG, ter Berg HJ, Spincemaille GH, van der
Laan RT, van de Wel AM. Intracranial chon-
drosarcoma: review of the literature and report of
15 cases. J Neurol Neurosurg Psychiatry. 1998;65:
88-92.
23. Bourgouin PM, Tampieri D, Robitaille Y,
Robert F, Bergeron D, del Carpio R, et al. Low-
grade myxoid chondrosarcoma of the base of the
skull: CT, MR, and histopathology. J Comput Assist
Tomogr. 1992;16:268-273.
24. Im SH, Kim DG, Park IA, Chi JG. Primary intra-
cranial myxoid chondrosarcoma: report of a case
and review of the literature. J Korean Med Sci. 2003;
18:301-307.
25. Dahlin DC, Unni KK. In: Bone Tumors. 4th ed.
Springfield, IL: Charles C. Thomas; 1986:227-230.
26. Kepes JL, Kepes M, Slowik F. Fibrous xanthomas
and xanthosarcomas of the meninges of the brain.
Acta Neuropathol (Berl). 1973;23:187-199.
27. Lam RM, Colak SA. Atypical fibrous histiocytoma
with myxoid stroma. A rare lesion arising from
dura mater of the brain. Cancer. 1979;43:237-245.
28. Wolf A, Echlin F. Osteochondrosarcoma of the
falx invading the frontal lobes of the cerebrum.
Bull Neurol lnst NY. 1936;5:515-525.
29. Russell OS. Meningeal tumours. A review. J Clin
Pathol. 1950;3:191-211.
30. Gay E, Sekhar LN, Rubinstein E, Wright DC,
Sen C, Janecka IP, et al. Chordomas and chon-
drosarcomas of the cranial base: results and
follow-up of 60 patients. Neurosurgery. 1995;36:
887-897.
31. Bingaman KD, Alleyne CH Jr, Olson JJ. Intracra-
nial extraskeletal mesenchymal chondrosarcoma:
case report. Neurosurgery. 2000;46:207-211.
32. Russell EJ, George AE, Kricheff II, Budzilovich G.
Atypical computed tomographic features of intra-
cranial meningioma. Radiology. 1980;135:673-682.
www.WORLDNEUROSURGERY.org
CASE REPORT
33. Kothary N, Law M, Cha S, Zagzag D. Conventional
and perfusion MR imaging of parafalcine chon-
drosarcoma. AJNR Am J Neuroradiol. 2003;24:
245-248.
34. Cho BK, Chi JG, Wang KC, Chang KH, Choi KS.
Intracranial mesenchymal chondrosarcoma: a
case report and literature review. Childs Nerv Syst.
1993;9:295-299.
35. Harsh GR, Wilson CB. Central nervous system
mesenchymal chondrosarcoma. J Neurosurg. 1984;
61:375-381.
36. Harwood AR, Krajbieh JI, Fornasier VL. Mesen-
chymal chondrosarcoma. A report of 17 cases. Clin
Orthop. 1981;158:144-148.
37. Seheithauer BW, Rubinstein LJ. Meningeal
mesenchymal chondrosarcoma. Report of 8 eases
with review of the literature. Cancer. 1978;42:
2744-2752.
38. Nokes SR, Dauito R, Murtagh FR, Love LC,
Arrington JA. Intracranial mesenchymal chon-
drosarcoma. AJNR Am J Neuroradiol. 1987;8:
1137-1138.
39. Waga S, Matsushima M, Ando K, Morii S. Intra-
cranial chondrosarcoma with extracranial metas-
tasis. J Neurosurg. 1972;36:790-794.
40. González-Lois C, Cuevas C, Abdullah O, Ricoy JR.
Intracranial extraskeletal myxoid chon-
drosarcoma: case report and review of the litera-
ture. Acta Neurochir (Wien). 2002;144:735-740.
Conflict of interest statement: The authors declare that the
article content was composed in the absence of any
commercial or financial relationships that could be construed
as a potential conflict of interest.
Received 13 March 2018; accepted 20 June 2018
Citation: World Neurosurg. (2018) 118:279-283.
https://doi.org/10.1016/j.wneu.2018.06.164
Journal homepage: www.WORLDNEUROSURGERY.org
Available online: www.sciencedirect.com
1878-8750/$ - see front matter ª 2018 Elsevier Inc. All
rights reserved.
283