British Journal of Neurosurgery, December 2012; 26(6): 912–914
© 2012 The Neurosurgical Foundation
ISSN: 0268-8697 print / ISSN 1360-046X online
DOI: 10.3109/02688697.2012.697219
SHORT REPORT
Intracranial mesenchymal chondrosarcoma: case report and
literature review
Zhisheng Kan, Hao Li, Ji Zhang & Chao You
Br J Neurosurg Downloaded from informahealthcare.com by SUNY State University of New York at Stony Brook on 10/25/14
Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, China
Abstract
Background. Mesenchymal chondrosarcoma is a very rare
malignant cartilaginous forming tumour in central nervous
system (CNS), which is rarely encountered in clinical practice
and generally occurs in young adults. This article describes a
case of primary intracranial mesenchymal chondrosarcoma
in a 31-year-old woman and reviews the literature on its
manifestations and management. Case report. This patient
had suffered from severe headache, intermittent nausea and
vomiting for 1 week. Systemic examination was unremarkable.
Magnetic resonance imaging (MRI) demonstrated a giant,
heterogeneous, intensely enhancing mass of 6 ⴛ 5 ⴛ 4 cm,
For personal use only.
occupying the bilateral frontal and based on the anterior falx
cerebri, which was initially thought to be a simply meningioma.
The patient underwent a bicoronal craniotomy and gross total
resection of the tumour. Pathologic examination revealed
the mesenchymal chondrosarcoma. Conclusion. Intracranial
mesenchymal chondrosarcoma is an extreme rare neoplasm,
which should be considered in the differential diagnosis
of intracranial mass like a meningioma. We emphasize the
importance of surgical intervention and combination of
microsurgical resection and radiotherapy, it should be the
therapeutical choice of the future.
Keywords: falx cerebri; mesenchymal chondrosarcoma;
radiotherapy
Introduction
Mesenchymal chondrosarcoma of the central nervous sys-
tem is uncommon neoplasms, which account for < 0.16% of
all intracranial tumours and constantly arise from the basal
synchondroses and can also arise from the meninges along
the falx cerebri, tentorium and convexity.1 This chondro-
genic lesion is a malignant small round cell neoplasm with
focal cartilaginous differentiation. We present a patient with
intracranial mesenchymal chondrosarcoma, which was ini-
tially thought to be a simply meningioma. This rare tumour
should be considered in the differential diagnosis of young
adults with an aggressive-appearing intracranial lesion.
Correspondence: Hao Li, MD, PhD, Department of Neurosurgery, West China hospital, Sichuan University, Chengdu 610041, PR China. Tel: ⫹ 86-13882279538.
E-mail: Neurosurgery_lh@hotmail.com.
Received for publication 26 March 2012; accepted 12 May 2012
912
Case report
A 31-year-old female patient was admitted to our department
with a history of progressive headache and dizziness asso-
ciated with intermittent nausea and vomiting for 1 month.
She was an alert, well-oriented patient with normal physical
findings, no recent generalized seizure and normalities in
the high grade neurological activity on her initial evaluation.
Neurological examination found no hemiparesis and sen-
sory disturbance. Her past medical history was unremark-
able. Computed tomography (CT) scan showed a bilateral
frontal lobes, high-density lesion along the falx cerebri, with
severe mass effect, perifocal edema and calcification sur-
rounded by tumour. The collapse of the left lateral ventricle
was also noted, suggesting a simply meningioma. Magnetic
resonance imaging (MRI) demonstrated a well-demarcated
parafalx mass with a 7.2 ⫻ 4.8 ⫻ 6.7 cm in size, occupying
the bilateral frontal lobes. The lesion has a signal intensity
higher than grey matter on T1-weighted images and slightly
higher than grey matter on T2-weighted images. When
gadolinium-diethylene triamine pentaacetic acid contrast
was injected, obvious enhancement in non-calcified portions
were detected (Fig. 1). Her preoperative symptom quickly
disappeared through infusing mannitol after admission.
Bicoronal craniotomy was performed with total resection
of the tumour arising from falx cerebri and having no exten-
sion into sagittal sinus. The mass which was reddish-brown
and rubbery soft was enucleated piece by piece from the left
side to the right through opening falx cerebri. The postopera-
tive course of the patient was eventful without neurosurgi-
cal deficit. The patient was discharged 5 days after surgery
without postoperative adjuvant radiotherapy and then
went back to her job as a lawyer. Microscopic examination
of the tumour specimen revealed undifferentiated round
or spindle-shaped cells and islets of cartilaginous tissue
(hematoxylin & eosin, ⫻ 200) (Fig. 2). Immunohistochemical
studies demonstrated positivity for S-100 (Fig. 3), which is
consistent with the diagnosis of mesenchymal chondrosar-
coma. Postoperative serial MRI studies showed no evidence
of recurrence 3 months.

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Fig. 1. Preoperative MRI demonstrating a bifrontal mass with strongly
intense enhancement, obvious mass effect, perifocal edema, the
collapse of the lateral ventricle.
Discussion
Since the first report of extraskeletal intracranial chond-
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rosarcoma was reported in 1962, only a limited number of
intracranial chondrosarcomas arising away from the base
of skull have been reported in the literature.2 Mesenchymal
chondrosarcoma is a high-grade malignant tumour with a
very high local recurrence and distant metastasis. Mesen-
chymal chondrosarcomas of the falx cerebri, which account
for approximately 1% of all chondrosarcomas, are character-
ized as mesenchymal, classic and myxoid variants, and are
extremely rare.
Chondrosarcomas which arise at the skull base from the
cartilaginous synchondroses, are usually located in the para-
sellar region, the cerebellopontine and petrous bone region,
Fig. 2. Histological sections of mesenchymal chondrosarcoma
showing primitive undifferentiated cells possessed round or ovoid
hyperchromatic nuclei and scanty, poorly outlined, eosinophilic
cytoplasm (Hemotoxylin and eosin, ⫻ 200).
Intracranial mesenchymal chondrosarcoma 913
Fig. 3. The cartilaginous portion of the tumour was positive for S-100
protein (⫻ 200).
but occasionally from the meninges along the falx cerebri,
tentorium and cerebral convexity. The peak age of incidence
ranges between the second and third decades of life. Mes-
enchymal, classic and myxoid chondrosarcomas differ from
each other in terms of cytoarchitecture, natural history and
prognosis. To date the origin of chondrosarcoma is still not
clear. The embryonal cartilaginous rests in the cranial bones
and dura, originate from the meningeal fibroblasts, or from
the multipotent mesenchymal cells in the dura or arachnoid.
Radiological examination including CT, MRI and angiography
is essential for any intracranial lesion. CT scans usually show
slightly hyperdense or isodense lesions with heterogeneous
enhancement and frequent calcification in patients with chon-
drosarcomas. Calcification in the centre of the tumour along
cerebral falx is remarkable on CT in our patient. MRI which can
define a possible neurovascular involvement and the extent of
mass has an absolute significance on preoperative evaluation.
Meningioma, oligodendroglioma and hemangiopericytoma
should be involved in preoperative presumptive diagnosis.
The treatment strategy is the radical removal of these les-
sions, regardless of anatomic site, especially having a mar-
gin with neurovascular structures. The majority of reported
parafalx chondrosarcomas are mesenchymal type tumours,
which have usually been described as highly vascular lesions,
but the present case stood out from these as a poorly vascu-
larized tumour. Total excision of the tumour was achieved
without any neurological deficit after surgery.
Postoperative adjuvant radiotherapy is a preferred treat-
ment for the remnant of the lesion. When the invasive char-
acter of chondrosarcoma was considered, some authors
recommend adjuvant radiotherapy even after the success-
ful radical resection.3 Similar treatment of other sarcomas
are available, postoperative adjuvant radiotherapy has been
standard practice for mesenchymal chondrosarcoma.4,5
The benefits of chemotherapy still remain to be not clear,
because there is no current reliable evidence for its effec-
tiveness. Generally, benefit from chemotherapy and/or
radiation therapy is under investigation, but chemotherapy
has been recommended by some authors for mesenchy-
mal chondrosarcoma.6,7 The factors of prognosis include
the use of postoperative adjuvant radiation therapy,
 914 Z. Kan et al.
pattern, previous treatment (surgery or radiation therapy)
and extent of tumour removal. The overall 5 and 10-year
survival for patients with mesenchymal chondrosarcoma,
when considering all sites, is 55% and 27%, respectively.8
Among the reported cases in parasagittal region, three
patients died 3 days to 5 years after surgery. Two patients
had recurrence after surgery. Our patient experienced
no evidence of recurrence for at least 3 months after sur-
gery and neurologic deficit. Her performance in job was
the same as before. The mass in our patient was resected
radically without radio-chemotherapy. Some authors rec-
ommend that radiotherapy with or without concomitant
Br J Neurosurg Downloaded from informahealthcare.com by SUNY State University of New York at Stony Brook on 10/25/14
chemotherapy should be considered for treatment of
patients with unresectable mesenchymal chondrosarcoma
and for those whom the surgical margins are inadequate.
However, the potential benefits of radiotherapy and che-
motherapy was under uncertainty because of the rarity of
the tumour. Future development in the therapy will benefit
from better knowledge of the biology of the tumour.
Conclusion
Intracranial mesenchymal chondrosarcoma of the falx is
an extreme rare neoplasm, with a strong tendency to local
aggression and distant metastasis. Differential diagnosis is
very difficult because of its rarity and atypical imagine. The
For personal use only.
treatment strategy is radical resection with close postopera-
tive follow-up, because it offers the greatest opportunity for a
long-term, recurrence-free period for patients, although the
long-term outcomes may be disappointing. Radiotherapy
and chemotherapy may be considered to reduce the risk
of recurrence. We emphasize the importance of surgical
intervention and combination of microsurgical resection
and radiotherapy should be the therapeutical choice of the
future.
Declaration of interest: The authors report no conflicts of
interest. The authors alone are responsible for the content
and writing of the paper.
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