Chordomas involving multiple neuraxial bones.
ABSTRACT We present a patient with multifocal symptomatic osseous chordomas having unusual growth patterns with review of the pertinent literature. The patient was 62-year-old male and had multiple osseous chordomas located in sacral, thoracic, and paraclival jugular foramen areas. There was no metastasis in other organs. All affected sites were osseous. The multicentric chordomas are extremely rare. This case could be considered as a chordoma involving multiple neuraxial bones. But, the possibility of multicentricity could also be thought. In such cases radical resection should be performed for each lesion at the initial diagnosis. If complete surgical resections are infeasible or impossible, preoperative or postoperative radiation therapy should be planned for the highest possibility of successful treatment.
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ABSTRACT: Chordomas are malignant tumors arising from notochordal remnants. They are the most frequent tumors of the spine after plasmacytomas. Only 6% of chordomas are localized to the cervical level. In young patients, chordomas are rare and unpredictable. Despite this, the treatment of choice remains the total resection, as much as possible, followed by proton beam radiation. This case was managed using a precarotid and retrocarotid approach at the same time. The tumor was completely resected with the edges free from disease. The cervical spine was stabilized with an anterior plating C2-C4. Eighteen months after surgery the patient is still free from illness. Multilevel oblique corpectomies are an available and safe option for the treatment of upper cervical chordomas.World journal of clinical cases. 03/2014; 2(3):57-61.
Chordomas Involving Multiple Neuraxial Bones
Jae Joon Lim, M.D.,1Sang Hyun Kim, M.D.,1Ki Hong Cho, M.D.,1Do Heum Yoon M.D., Ph.D.,2Se Hoon Kim M.D.3
Department of Neurosurgery,1Ajou University School of Medicine, Suwon, Korea,
Department of Neurosurgery,2Yonsei University College of Medicine, Seoul, Korea,
Department of Pathology,3Yonsei University College of Medicine, Seoul, Korea
J Korean Neurosurg Soc 45 : 35-38, 2009
Chordomas are tumors of the axial skeleton, arising from
primitive notochord remnants, and constitute 2% to 4% of
all primary bone tumors3,4,7). They are slow-growing, locally
invasive, rare tumors of the spine. Multicentric chordomas
are especially rare. To our knowledge, only one case of
multicentric chordomas has been reported1). We present a
patient with multifocal symptomatic osseous chordomas
having unusual growth patterns with review of the pertinent
literatures. The possibility of multicentricity is also discussed.
A 62-year-old male patient had a palpable mass on the
sacrococcygeal area. The protruded part of the parpable
mass on the skin was surgically removed at the private
hospital and it was pathologically diagnosed as a chordoma.
We could not get the data about the pathology and image
study. Postoperatively, the patient was given a course of
radiation therapy, with a total dose of 6400 cGy. The
patient made a full recovery without any symptoms
following the radiation therapy. In January 2004, 20
months later, the patient developed persistent headaches
and left-side tongue atrophy. Brain magnetic resonance
imaging (MRI) showed a tumorous lesion in left side of the
clivus, extending to the left jugular foramen. T1WI images
showed the tumor to be hypointense. Following intravenous
administration of gadolinium, moderate to marked
heterogeneous enhancement was observed. Subsequent
T2WI images showed the tumor to appear hyperintense
(Fig. 1). It was diagnosed as a glomus jugulare tumor and
surgery was performed with a Gamma Knife without
pathologic confirmation. We designed conformal 50%
isodose line to cover 99% of the tumor. The prescription
dose was 26 Gy and the mean margin isodose was 13 Gy.
Tongue atrophy persisted and the patient began to
experience radiating leg pain in the right dermatome,
progressively worsening one year later. The initial X-rays of
the lumbosacral spine showed an osteolytic change in the
right sacroiliac area. MRI showed multiple tumorous
lesions in the thoracic and lumbosacral areas. The patient
was then evaluated with abdominopelvic computed
tomography (CT), chest CT, whole body bone scan, and
positron emission tomography (18-FGD) whole body scan
for possibility of metastasis. There was no metastatic
evidence in other regions. The lumbosacral tumor involved
the iliac bones on both sides, forming a soft mass which
We present a patient with multifocal symptomatic osseous chordomas having unusual growth patterns with review of the pertinent literature.
The patient was 62-year-old male and had multiple osseous chordomas located in sacral, thoracic, and paraclival jugular foramen areas. There
was no metastasis in other organs. All affected sites were osseous. The multicentric chordomas are extremely rare. This case could be
considered as a chordoma involving multiple neuraxial bones. But, the possibility of multicentricity could also be thought. In such cases radical
resection should be performed for each lesion at the initial diagnosis. If complete surgical resections are infeasible or impossible, preoperative or
postoperative radiation therapy should be planned for the highest possibility of successful treatment.
KEY WORDS : Chordoma˙ Multiple˙ Thoracic˙ Sacral˙ Paraclival˙ Jugular foramen.
• Received : July 18, 2008 •Accepted : December 29, 2008
• Address for reprints : Sang Hyun Kim M.D.
Department of Neurosurgery, Ajou University School of Medicine,
Suwon 443-721, Korea
Tel : +82-31-219-5230/5232, Fax : +82-31-219-5238
E-mail : email@example.com
Copyright ©2009 The Korean Neurosurgical Society
Print ISSN 2005-3711 On-line ISSN 1598-7876
was pinching a spinal nerve root (Fig. 2). The thoracic
tumor involved the inferior articular process and lamina of
T3 vertebra on the right side with a soft mass in the
paraspinal area and extended to an adjacent proximal part
of the 3rd rib (Fig. 3). We performed surgery for the sacral
and thoracic tumors to confirm pathology. The thoracic
lesion was totally removed and the soft mass of the sacral
lesion pinching the spinal nerve root was also removed.
The masses were grossly lobulated, grayish, and soft with a
gelatinous consistency. Microscopic
examination showed physaliphorous
cells with myxoid stroma. The
immunohistochemical staining for
cytokeratin (DAKO, AE1/AE3,
1/300) and epithelial membrane
antigen (DAKO, E29, 1/100) showed
positive reaction. However, S-100
protein (DAKO, polyclonal, 1/1000)
and carcinoembryonic antigen
(DAKO, II-7, 1/50) showed negative
reaction. Both lesions were pathologically confirmed as
chordomas (Fig. 4). Immediately following surgery, the
radiating leg pain disappeared and the patient had fully
recovered without any evidence of local recurrence. The
patient had been doing well until post-operative 2 years
Chordomas are rare bone tumors that occur in the midline
of the axial skeleton. They account for only 1% to 4% of
primary bone tumors3,7). More than 50% of these lesions are
located in the sacrococcygeal region, 35% are located in the
clival and cervical area, and the remainders are spread
throughout the vertebral column, with a distribution of
61.1% in the lumbar, 11% in the thoracic and 27.8% in the
This case involved three discrete chordomas located in the
clival, thoracic, and sacral areas respectively. However, the
clival tumor was not pathologically confirmed. Because the
clival lesion was identified prior to the sacral and thoracic
lesions, it was thought to be a glomus jugulare tumor. In
light of the subsequent pathologic confirmations, the clival
lesion was much more likely to be a chordoma rather than a
glomus jugulare tumor.
The radiographic differential diagnoses include metastatic
disease, and primary or secondary spine tumor, such as
multiple myeloma, chondrosarcoma, neurogenic tumor,
tumor-like condition such as Paget’s disease. Chordomas
behave as malignant entities because of their local invasive-
ness, high recurrence rate, and potential for metastasis.
Regions of metastasis include the lymph nodes, lung, liver,
brain, or bone, and the reported incidence of metastasis
varies from 5% to 43%. To detect the metastasis, whole
body bone scan, chest CT, abdominopelvic CT, PET scan
should be done. In this case, the patient did not show any
evidence of metastasis from these stuides.
Chordomas usually show a radiating infiltrative pattern
involving epidural space and prevertebral and lateral
J Korean Neurosurg Soc 45 | January 2009
Fig. 3. Chordoma in the thoracic area involving the inferior articular
process and lamina of T3 vertebra on the right side, with a soft mass in
the paraspinal area extending to an adjacent proximal part of the 3rd rib
Fig. 1. Chordoma in the left jugular foramen, extending to the left side of the clivus.
Fig. 2. Chordoma in the sacral area involving iliac bones on both
sides, forming a soft mass pinching the spinal nerve root.
paravertebral regions. They can cause dural compression,
meningeal sheath invasion, and cerebrospinal fluid spreading.
Furthermore, metastasis along the neuraxis is extremely rare.
Chordomas sometimes occur with multicentric origins,
but it is extremely rare for them to occur in different sites
along the neuraxis, especially in posterior elements of spine.
Vertebral body is usually affected. The disks are usually
spared and the posterior elements are less involved. There are
no specific criteria differentiating multicentric from
metastatic tumors. In this case, the patient had tumors in
both sacral and thoracic regions at presentation. All affected
sites were osseous. Chordoma had been pathologically
confirmed in both regions. There was no metastatic evidence
that was confirmed by imaging studies. If metastatic spread
of chordomas along the neuraxis occurs, there should be
widely disseminated disease. Therefore, each lesion could be
The lesion in the left side of clivus was thought to be
chordoma after surgeries for sacral and thoracic lesions. Even
though the possibility of chondrosarcoma could not be
excluded, there was no extensive bony erosion and soft mass
formation in clival lesion. Furthermore, clival lesion showed
bright signal with lobulation in T2WI and isointensity in
T1WI. These findings are consistent with chordoma.
Because follow-up MR had no interval change in size and
the patient had no progression of symptom associated with
the clival lesion, we did not try to get tissue confirmation.
Surgical “en bloc” resection with wide margins is the
treatment of choice for chordomas the first time they are
diagnosed. Chordomas have a well known tendency to recur
locally. The recurrence rate seems to be related to incomplete
resection. There is no consensus regarding the efficacy of
chemotherapy and radiotherapy to treat chordomas.
Although chordomas are relatively radioresistant, adjuvant
radiotherapy has been used for surgically inaccessible lesions,
contaminated surgical margins, when incomplete surgical
excision of the tumor occurs, and to improve the quality of
life and relieve pain in patients with wide spread
recurrences8,9). In our case, further evaluation of the clival
lesion to clarify surgical resectability may be needed, and
adjuvant radiation therapy may be needed in the sacral and
thoracic areas. The sacral lesion was not completely resected
and radiation therapy seems to be more successful in
controlling microscopic diseases.
Chordomas are slow-growing tumors that tend to recur
locally and metastasize. Chordomas rarely occur multi-
centrically. We could not prove multicentricity in this case.
This case could be regarded as a chordoma involving
multiple neuraxial bones. But, the possibility of multi-
centricity could also be thought. In such cases, radical
resection should be performed for each lesion at the initial
diagnosis. If complete surgical resections are infeasible or
impossible, preoperative or postoperative radiation therapy
should be planned for the highest possibility of successful
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treated at a single institution. Ann Surg Oncol 10 : 291-296, 2003
Fig. 4. Specimens had been obtained from sacral lesion (A) and thoracic lesion (B). Physaliphorous cells are seen in the tissue with small, dark,
round to ovoid nuclei and abundant multivacuolated cytoplasm in both lesions. The immunohistochemical staining for Cytokeratin and EMA show
positive reaction. However, S-100 protein and CEA show negative reaction. These findings are consistent with chordoma.
Chordomas Involving Multiple Neuraxial Bones | JJ Lim , et al.
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J Korean Neurosurg Soc 45 | January 2009