S. Tsutsumi et al.

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Chondrosarcoma With Atypical Clinical Presentation
Treated by Gamma Knife Radiosurgery
for Multiple Brain Metastases
—Case Report—
Satoshi TSUTSUMI, Yukimasa YASUMOTO,
Hideki OIZUMI*, and Masanori ITO
Departments of Neurological Surgery and *Neurology,
Juntendo University Urayasu Hospital, Urayasu, Chiba

Abstract
A 60-year-old male was first treated for World Health Organization (WHO) grade II chondrosarcoma arising from the ring finger manifesting as painful swelling. Four years later, the patient presented with
cerebral infarction. Echocardiography revealed a tumor occupying the left atrium. He underwent open
heart surgery and the tumor was identified as metastatic chondrosarcoma with malignant transformation to WHO grade III lesion. Five months following the cardiac surgery, the patient suffered generalized seizure. Cerebral magnetic resonance imaging revealed multiple parenchymal lesions. Surgical
tumor extirpation confirmed the histological diagnosis as metastatic grade III chondrosarcoma. Gamma knife radiosurgery (GKS) performed postoperatively controlled the parenchymal lesions for more
than 10 months without relapse. GKS may be effective for the treatment of brain metastasis from high
grade chondrosarcoma.
Key words:

Received

chondrosarcoma,

August 20, 2009;

Accepted

cardiac metastasis,

brain metastasis,

radiosurgery

December 2, 2009

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Introduction
Chondrosarcoma is a malignant type of mesenchymal
tumor arising from the bone and soft tissue, and can be
classified into World Health Organization (WHO) grade I
benign to grade III malignant subtypes according to the
histological appearance. Grade III chondrosarcoma is a
dedifferentiated type with high metastatic potential and is
associated with a dismal prognosis, as adjuvant
chemoradiation therapy is not effective.6,12,13) Distant
metastases account for 10% of grade II and 71% of grade
III chondrosarcomas, and can occur in the lung, humerus,
femur, sternum, liver, pleura, and ureter. Survival in
patients with metastasis is significantly reduced, with a 5year survival rate of only 18%.2,12) Heart and brain
metastases, especially the latter, are exceptionally rare and
scarcely documented.1,2,4,5,9–11) Although rare, chondrosarcoma can arise from the hand.8,10,12)
Here we describe a case of chondrosarcoma originating
from the ring finger, followed by metastasis to the heart
manifesting as cerebral infarction, and presented with
multiple brain metastases which were successfully controlled by gamma knife radiosurgery (GKS).

Fig. 1 Preoperative axial (A) and sagittal (B) T1-weighted magnetic resonance images after gadolinium infusion demonstrating subcortically located, heterogeneously enhanced, multiple
tumors in the bilateral frontoparietal and occipital lobes (arrows), with a lesion located in the right frontal lobe (arrowhead).

Case Report
A 60-year-old male presented with a painful swelling in
the left ring finger in 2003. He underwent a biopsy which
established the diagnosis of WHO grade II chondrosarcoma, followed by amputation of the affected finger. Systemic examination revealed no metastatic foci and the patient
did not undergo additional therapy. He remained asymptomatic for more than 4 years, but then suffered cerebral
infarction in the right frontal lobe manifesting as left
hemiparesis in October 2007.
Echocardiography revealed a mixed-echoic mass occupying the left atrium with to and fro movements between
the atrium and ventricle, without symptoms of heart
failure. Systemic examination revealed tumorous lesions,
3.5 cm in diameter, in the hilum of the left lung, separate
from the mass in the heart, and spleen. He was a nonsmoker and the serum tumor markers were within normal
limits. Cerebral magnetic resonance (MR) imaging with
contrast medium showed no metastatic lesion other than
small infarcts in the right frontal lobe and ipsilateral cerebellar hemisphere in the distribution of the posterior inferior cerebellar artery. MR angiography revealed no vascular lesions. As the cardiac tumor was considered to be
the origin for brain infarction and threatened sudden
heart failure, he underwent open heart surgery. The tumor
was totally resected and histological examination identified WHO grade III chondrosarcoma with exaggerated
cell atypia compared to the primary histology. Postoperatively, the patient underwent three courses of cisplatinbased chemotherapy, after which the lung tumor showed
no growth, but the splenic lesion demonstrated additional
asymptomatic enlargement. The patient suffered generalized seizure in March 2008 and was referred to our department.
On admission, the patient presented with mild left

Neurol Med Chir (Tokyo) 50, June, 2010

Fig. 2 Photomicrograph of the metastatic brain tumor showing dedifferentiation of the chondrosarcoma with prominent
cell atypism, pleomorphism, and mitotic activity. Hematoxylin and eosin stain, ×200.

Fig. 3 Axial T1-weighted magnetic resonance image after
gadolinium infusion performed 1 month following tumor
removal showing total resection of the right frontal tumor (arrowhead) and significant growth of the untreated metastatic
foci (arrows).

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S. Tsutsumi et al.
mass, which were all less than 1.5 cm in diameter. The irradiated tumors showed a continuous regression without
relapse in the following 9 months (Fig. 4). The patient has
been doing well for more than 10 months after irradiation
with asymptomatic but gradually enlarging lesions in the
lung and spleen refractory to serial courses of cisplatinbased chemotherapy.

Discussion

Fig. 4 Serial postcontrast T1-weighted magnetic resonance
images before (upper row), 3 month after (middle row), and 9
months after gamma knife radiosurgery (lower row) demonstrating significant tumor regression and even disappearance
of some tumors.

hemiparesis due to the previous cerebral infarction. Cranial MR imaging revealed multiple contrast-enhanced lesions in the cerebral parenchyma (Fig. 1). No enhanced lesion was found within the supra- and infratentorial old infarcts. He underwent surgical extirpation of a superficially located tumor in the right frontal lobe. The grayish
tumor was well-circumscribed, elastic soft, and bled little.
The histological diagnosis was WHO grade III chondrosarcoma based on the prominent cell atypism and mitotic activity (Fig. 2). Cerebral MR imaging performed 1
month following tumor resection revealed significant
growth of the untreated parenchymal lesions (Fig. 3).
Then the patient underwent GKS (marginal dose 22.6 Gy,
maximum dose 45.2 Gy) for the multiple foci, 14 in total,
distributed in the bilateral frontoparietal and occipital
lobes, and except for the totally removed right frontal

In the present case, metastatic foci in the brain parenchyma were controlled well by GKS without relapse or worsening of the daily activities of the patient for more than
10 months. Although GKS is a treatment option for selected cases of primary chondrosarcoma arising in the skull
base, GKS for metastatic lesions has not previously been
documented.7) Generally, chondrosarcoma is considered
to be less sensitive to conventional radiation therapy, as
well to any type of chemotherapy, like other mesenchymal
sarcomas. In the present case, irradiated chondrosarcomas, especially if less than 1 cm in diameter, showed significant regression or even disappearance, without consequent relapse, in a short follow-up period. Considering the
usual dismal prognosis of high-grade chondrosarcoma,
GKS may be a useful option for controlling intracranial
metastases, while simultaneously maintaining the affected
patients' quality of life. More patients and longer followup periods may determine the role and effectiveness of
GKS against parenchymal metastases from chondrosarcoma.
The currently used grading system for chondrosarcomas is defined for convenience in the clinical setting, but
the malignant potential is difficult to evaluate based only
on the histological appearance.14) With recent advances in
genetic and molecular diagnostics, chondrosarcomas are
to be considered as part of a heterogeneous group of
tumors including the diverse family of cartilaginous neoplasms. Developmental therapeutics based on these
modalities may improve the classification algorithm and
appropriate chemotherapeutic regimen for individual
chondrosarcomas.3) GKS should be considered as an option with developing chemotherapy to comprise treatment
strategies for chondrosarcoma. The present case suggests
that GKS may be effective for controlling brain metastases
from chondrosarcoma, especially if the lesions are small.

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Address reprint requests to: Satoshi Tsutsumi, M.D., Department
of Neurological Surgery, Juntendo University Urayasu
Hospital, 2–1–1 Tomioka, Urayasu, Chiba 279–0021, Japan.
e-mail: shotaro＠juntendo-urayasu.jp