Chordomas of cervical spine: surgical management strategies and

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ARTIGO ORIGINAL / ORIGINAL ARTICLE
Chordomas of cervical spine: surgical management
strategies and outcome
Cordomas da coluna cervical: método cirúrgico e resultados
Cordomas de la columna cervical: estrategias
de manejo quirúrgico y resultados
Amit Kohli1
Ram Chaddha1
Sharookh P. Vatchha1
ABSTRACT
RESUMO
RESUMEN
Objectives: chordomas of cervical spine
are rare tumors but are the primary
malignant tumors of the spine. As
Chordomas has poor sensitivity to
radiotherapy and chemotherapy it
requires surgical resection. The purpose
of this study was to determine the suitable
methods for removal of the tumors
completely and outcome over time in
patients undergoing complete en bloc
excision. Methods: a retrospective study
of twelve patients from October 2003 to
August 2007 between the ages of 45-75
years who presented with gradual onset
of neck pain and upper extremity
weakness. All patients were clinically
evaluated and preoperatively underwent
Digital X-Rays, CT scans and contrast
enhanced MR imaging. All patients
underwent tumors resection and spinal
instrumentation. All cases were followed
up clinically and radiographically for
determination of their status. Results:
eleven patients were available for followup. All patients underwent an anterior
Objetivos: os tumores da coluna cervical
são raros, mas são tumores malignos primários da coluna. Como os cordomas
apresentam baixa sensibilidade à radioterapia e à quimioterapia requerem cirurgia para sua ressecção. O objetivo
deste estudo é determinar os métodos
adequados para a remoção total dos
tumores e conhecer os resultados para
o paciente no período posterior à sua
retirada. Métodos: foi realizado um
estudo retrospectivo de 20 pacientes,
de Outubro de 2003 a Agosto de 2007,
com idade entre 45 e 75 anos, os quais
apresentavam uma gradual dor no
pescoço e uma importante fraqueza nas
extremidades superiores. Todos os
pacientes foram avaliados clinicamente
e no período pré-operatório por meio
de radiografia digital, tomografia
computadorizada e por imagem de
ressonância magnética com contraste.
Todos os pacientes foram submetidos
à ressecção dos tumores e instrumentação da coluna vertebral. Todos os
Objetivos: los tumores de la columna
cervical son raros, siendo tumores
primarios malignos de la columna.
Como los cordomas presentan baja
sensibilidad a la radioterapia y a la
quimioterapia requieren cirugía para su
resección. El objetivo de este estudio
es determinar los métodos adecuados
para la remoción total del tumores y
conocer los resultados de los pacientes
en el período posterior a su retirada.
Métodos: fue realizado un estudio
retrospectivo de 20 pacientes, de
Octubre de 2003 a Agosto de 2007, con
edad entre 45 y 75 años, que presentaron
un dolor gradual en el cuello y una
debilidad importante en las extremidades
superiores. Todos los pacientes fueron
evaluados clínicamente y en el preoperatorio fue realizada radiografía digital,
tomografía computarizada y resonancia
magnética de contraste. Todos los pacientes fueron sometidos a la resección
del tumores y a la instrumentación de
la columna vertebral. Todos los casos
Study carried out at Lilavati Hospital and Research Centre, Bandra (west), Mumbai, India.
1
Orthopaedics from the Department of Orthopedics Lilavati Hospital and Research Centre, Bandra (west), Mumbai, India.
Recebido: 20/01/2008 Aprovado: 20/02/2008
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stabilization procedure combined with
interbody fusion (seven with iliac crest
and five with Interbody cages) whereas
one required an occipitocervical fusion.
At the time of this submission, there were
two signs of recurrence. Discussion: the
uncontrolled growth of chordoma is
commonly the cause of death. En bloc
excision of the lesion, sometimes
combined with radiation therapy as an
adjuvant, obtained the best results.
Conclusion: chordoma occur in most
cases directly from the vertebral body. En
bloc excision of these tumors even if
marginal seems to be the most effective
treatment combined with megavoltage
radiation which can be administered as an
adjuvant.
casos foram acompanhados clínica e
radiograficamente para determinar sua
evolução. Resultados: 11 pacientes estavam disponíveis para o acompanhamento. Todos os pacientes submeteramse ao procedimento de estabilização anterior da coluna com fusão interssomática, sete deles com enxerto do osso
ilíaco, cinco com “cages” interssomáticos, enquanto que um paciente
necessitou de fusão occipto-cervical.
No grupo de pacientes estudados
observaram-se duas recidivas do
tumores. Conclusão: a falta de controle
do crescimento do cordoma da coluna
cervical é uma comum causa de óbito.
A conduta de retirada do bloco de lesão,
às vezes combinada com a radiação e a
terapia obtém bons resultados. O cordoma ocorre em muitos casos diretamente no corpo vertebral. A remoção
do bloco dos tumores parece ser o mais
efetivo tratamento combinado com
irradiação de megavoltagem que é
administrada como coadjuvante ao
tratamento cirúrgico.
fueron acompañados clínica y radiográficamente para determinar su evolución.
Resultados: 11 pacientes estaban disponibles para acompañamiento. Todos
los pacientes se sometieron al procedimiento de estabilización anterior de la
columna con fusión intersomática, siete
de ellos con injerto de hueso iliaco, cinco
con “cages” intersomáticos, mientras
que un paciente necesitó de fusión
occípito-cervical. En el grupo de pacientes estudiados se observaron dos
recidivas de tumores. Conclusión: la
falta de control de crecimiento de la
cordoma de la columna cervical es una
causa común de óbito. La conducta de
retirada del bloque de la lesión, algunas
veces combinada con la radiación y la
terapia ocasiona buenos resultados. La
cordoma ocurre en muchos casos
directamente en el cuerpo vertebral. La
remoción del bloque del tumor parece
ser el tratamiento más efectivo combinado con la irradiación de megavoltage
que es administrada como coadyuvante
al tratamiento quirúrgico.
KEYWORDS: Chordoma/surgery;
Orthopedic procedures/
methods; Spinal neoplasms/
surgery
DESCRITORES: Cordomas/cirurgia;
Procedimentos ortopédicos/
métodos; Neoplasias da
coluna vertebral/cirurgia
DESCRIPTOREs: Cordoma/cirurgía;
Procedimientos Ortopédicos/
métodos; Neoplasias de la
columna vertebral/cirurgía
INTRODUCTION
Chordoma is a low grade malignant tumors accounting for
about 1% to 4% of all malignant bone tumors. It originates
from the remnants of the notochord. It is predominantly
found in the clivus (50%), followed by the sacral spine (15%).
Only rarely is this tumors seen in cervical spine accounting
for 6% of all chordoma1. Cervical spine Chordomas present
significant surgical challenges because of the important
anatomical structures present in this area. The tumors grows
slowly by infiltrating cancellous bone, is seen typically in
adults and the elderly. At the time of initial presentation, the
cervical chordoma usually shows infiltration into the
paravertebral and epidural compartments. It has a marked
tendency toward recurrence following intralesional excision
or biopsy. Metastasis is usually seen in the brain, skin, lungs,
bone or internal organs. Rate of metastasis varies from 0%
to 5%2. Survival seems to be more affected by the local
spread rather than by metastasis. Magnetic resonance
imaging and computed tomography have made the
diagnosis of the size of the tumors faster and easier1, they
also play an important role in improving the prognosis of
chordoma by discovering small tumors, which can be
submitted to en bloc resection4-5.
In this study we present twelve cases with isolated
cervical spine Chordomas. All patients at the time of
presentation had evidence of tumors extension. In this study
we determine the suitable methods for removal of the tumors
completely and outcome over time in patients undergoing
complete en bloc excision.
METHODS
We retrospectively analyzed twelve patients with cervical
chordoma from October 2003 to August 2007 at the Lilavati
Hospital and Research Centre, Mumbai. All data was
obtained from hospital records, including preoperative and
sequential postoperative clinical findings, radiological
details and pictures and details of the status of the patient
till the last follow-up. Neurological deficit was assessed by
Frankel grading.
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Chordomas of cervical spine: surgical management strategies and outcome
Out of the twelve patients studied eight were males and
four were females. Mean age at diagnosis was 60 years.
Follow-up duration ranged from 12 to 36 months. The slow
and gradual onset of pain was the most consistent
complaint; all patients presented with neck pain and upper
extremity symptoms. Upper extremity symptoms ranged from
hand tingling and numbness to overt weakness. One patient
presented with difficulty in swallowing.
All patients preoperatively underwent standard X-rays,
CT scans to assess the degree of bone destruction and
cervical spine instability and contrast enhanced MRI to
evaluate bone and soft tissue involvement, and nuclear bone
scan to check for metastasis, we used MR angiography to
evaluate the patency of vertebral artery in addition to a
complete preoperative workup (Figure 1-2).
Based on the preoperative images we staged all tumors by
using the oncological staging system devised by Enneking et
al. and the extent of the lesion was described according to the
Weinstein- Borian- Biagini (WBB) system. According to the
Enneking staging system, the lesions were classified as 1A (4
cases) and 1B (8 cases)3. The tumors arose mostly central in
the body and invaded the whole vertebrae. Chordomas appear
late on standard X-Rays, the radiographic pattern is mostly
radiolucent with scanty ossifications and huge masses in the
surrounding soft tissues. The discs were spared. MRI and CT
scan proved useful in detecting lesions which were not seen
on radiographs, the MRI signal is hypo dense in T1 weighted
and hyper intense in T2 weighted images. In six cases complete
vertebral collapse occurred. We planned our surgical approach
based on a variety of factors including tumors location, extent,
area of cord compression and degree of instability. In all of the
twelve surgical interventions, two were done by combined
anterior and posterior approach; these had pure anterior, middle
and posterior column involvement. Ten were done by anterior
approach; these had anterior and middle column involvement.
The treatment performed on twelve cases included
› En bloc resections in four cases, the margins were
contaminated at some point and for this reason radiation
therapy was performed as an adjuvant in these cases.
› En bloc resection of the vertebral body (vertebrectomy)
could be performed in five cases (Figure 3).
› Intralesional extra capsular excision with adjuvant
radiation therapy was performed in three cases.
Specimens obtained during resection were sent for
histopatholgical analysis in all cases (Figure 4). Spinal
instrumentation was performed when surgery caused
instability or for reconstruction of spine after tumors removal.
All patients in this series underwent spinal stabilization.
Combined anterior and posterior reconstruction was performed
in two cases; the vertebral body was replaced by autogenous
iliac bone graft in seven cases and by interbody cages in five
cases along with anterior plating (Figure 5).
Post-operative care included immobilization of the operated
spine with a SOMI brace or a hard cervical collar (Philadelphia)
for three months post surgery. The duration of hospital stay
following surgery ranged from five to 14 days. A contrast
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enhanced MR imaging study was obtained at 6 weeks in all
patients to evaluate the status of the tumors. Follow-up evaluation
was on outpatient basis at 6 weeks post tumors excision along
with a fresh digital X-ray, further follow-ups were at 3, 6, 9 and 12
months along with fresh X-Rays for assessment of spinal
alignment and integrity of the instrumentation and to check for
fusion. Successful fusion required the presence of bridging
trabeculae across the fused levels. A contrast
enhanced MR imaging study was obtained in all patients
at 3, 6 and 12 months post excision to check for early tumors
recurrence. Once fusion was established, the patients were
weaned off the brace to a soft cervical collar.
B
A
Figure 1
58 years lady: MRI saggital (A) and axial (B) cuts showing C6
chordoma with cord compression
Figure 2
Patient underwent C6
body excision +
decompression with
interbody iliac crest
bone graft and
stabilization
Figure 3
Microscopic picture of same
patient showing large
polyhedral cells with
eccentric nuclei and large
mucoid foci with a chondroid
appearance
Figure 4
60 year old lady with
chordoma of C2 vertebrae
Figure 5
Patient underwent C2 excision with a two
stage antero-posterior occipito-cervical fusion
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RESULTS
All patients were analyzed using the Frankel grading.
The evolution of the chordomas is known for eleven
patients. All patients were submitted to clinical and
imaging studies during their follow-ups to determine the
relationship between treatment and outcome. In all
patients in this study the tumors were resected using a
standard anterior cervical approach for anterior and
middle column tumours and posterior approach for
involvement of posterior elements. Of the eleven patients
available at follow-up their were two cases of local
recurrence. The mean follow-up duration has been 24
months in this study. Three patients were treated by
intralesional extra capsular excision with adjuvant
radiation therapy, at one year there was one case of local
recurrence which required chemotherapy, this patient is
being treated and one patient of this group was lost to
follow-up. En bloc excision was performed in five cases,
there was no recurrence at 48 months in this group of
patients. Of the four patients who underwent En bloc
resections with contaminated margins and adjuvant
radiation therapy there was one patient (25%) with local
recurrence at 24 months follow-up which required
excision along with radiation therapy.
Chordomas are a frequent cause of cord compression
because of the slow growth which expands toward the
epidural space compressing the dura. In all cases a
significant improvement in the neurological symptoms
arose following decompression and excision of the
tumors. Seven patients received postoperative radiation
therapy of these two patients with local recurrence also
underwent chemotherapy.
DISCUSSION
During the period of this study (2003 to 2007), twelve
chordomas of the cervical spine were treated at Lilavati
hospital. Chordomas of cervical spine always arise within
the vertebral body, they constitute 1 to 4% of malignant
bone tumors6. After plasmacytomas they are the most
frequent primary malignant tumors in the spine, the
cervical region constitutes 6 to 7% of cases. Chordomas
are locally aggressive and have a tendency to recur.
Metastases are noted in approximately 30% of cases7-8.
As it is slow growing tumors it is detected late and often
occupies most of the vertebrae at the time of
presentation, chordomas are often destructive. Late
diagnosis makes it difficult to provide appropriate
treatment. According to the oncological staging
proposed by Enneking, chordomas are classified as
stage 1A/1B lesions 3 . Aggressive resection is the
treatment of choice.
The main problem in the treatment of chordoma is
local recurrence. The uncontrolled growth of these
tumors is commonly the cause of death. The treatment
of chordomas in the cervical spine consists of radical
excision and stabilization, followed by adjuvant
radiotherapy and chemotherapy9. Proton beam radiation
is the modality that has been used extensively for the
treatment of chordomas 10-11.
In this study five patients who underwent En bloc
excisions of the tumors had no recurrence at 48 months.
The results of this study thus confirms most of the
conclusions of the literature and stresses the point that
En bloc resection with tumors free margin seems to be
the procedure of choice to allow a disease free interval.
It has been reported that the margin free, en bloc tumors
resection is the treatment of choice for Chordomas8,12.
This requires the tumors be confined within the vertebral
body, as it is slow growing tumors it is detected late
and often occupies the epidural space after penetrating
the vertebral body at the time of presentation, removing
such large tumors in the cervical spine in an en bloc
manner when the lesion extends well beyond the spine
would be associated with significant morbidity. The main
disadvantage in resecting cervical spine chordoma
result from the involvement of the vertebral artery and
the duramater 13. In this study we were able to achieve
tumors eradication in three such cases with intralesional
excision and adjuvant radiation therapy, one out of three
patients from this group has had a disease free interval
after a follow-up of 36 months, this being a reasonable
method of choice when en bloc resection is not feasible
in tumors with intradural and soft tissue involvement.
In 4 cases the margins were contaminated and in these
cases adjuvant radiation therapy was given obtaining a
disease free interval for 36 months in three out of four
patients.
CONCLUSION
Chordomas in cervical spine are rare tumors. They arise
directly from the vertebral body and tend to expand
the whole body. At presentation, the majority of
patients had tumors beyond the vertebral body, this
tumors spread beyond the vertebral body makes it
difficult to perform an en bloc resection of the body as
the margins would never be disease free, considering
the risk benefit ratio as the surgical risk presumably
exceeds the benefit. In this study the patients with
cervical chordoma presented with nonspecific
symptoms of neck pain with radicular and myelopathic
changes. The result of this study indicates that en
bloc resection of chordoma in some areas seems to be
the most effective treatment in local control of this
disease in combination with stabilization and
reconstruction of the spine. In cases when en bloc
excision is not feasible that is in cases with
involvement of vital neurological structures a complete
excision that is piecemeal removal of the tumors is a
viable technique combined with megavoltage radiation
therapy or proton beam radiation.
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Correspondence
Dr. Amit Kohli
802-Green Blaze CHSL,
Near IDBI Bank
Juhu-Versova link road,
Andheri (West), Mumbai-400053
INDIA
Tel.: + 91-9820504676
E-mail: amitkohli99@yahoo.com
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