case report cordoma
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CASE REPORT
Patient Identity
Name : Tn. DB
Age : 43 years old
Sex : Male
RM : 551616
Date of admission : June 12th 2012
History Taking
Chief Complains:
Low Back Pain
History of Disease:
Suffered since + 10 months ago but became severe in last 1 month before admitted into
Hospital. Initialy he felt pain at the buttock which spread to the soles of the feet, pain felt such as
stabbing sensation. The pain is intermitten, not affected by the activity. Pain is also the same in
both time of day or night. 1 month ago pain more severe. Patient is currently only able to walk
about 10 meters.
There is bladder dysfunction and constipation since 2 months ago.
There is sexual dysfunction since 4 months ago.
History of chronic cough (-), ATD comsuption (-), fever (-), sweat (-), tremor (-),weight loss (-),
bloody stool (-).
History tumor disease and family history (-).
History of trauma (-), diabetic (-) and hypertension (-).
General Status
Moderate/well nourished/conscious
Vital Sign
BP : 120/80 mmHg HR : 88 bpm, regular
RR : 16 tpm Temp. : 36.5°C
Local Status:
Vertebra Region :
I: deformity (-), gibbus (-)
P: lump (+) size 2x2cm, irregular surface, soft consistency, immobile, ill defined, tenderness
(+), lymph enlargement (-).
Clinical Appereance
Motoric Examination : within in normal limit
Sensory Examination : within in normal limit
Physiologic Reflex:
R L
Biceps (+) (+)
Triceps (+) (+)
Achilles (+) (+)
Patellar (+) (+)
Phatologic Reflex:
R L
Babinski (-) (-)
Chadock (-) (-)
Openheim (-) (-)
Laboratory Findings
WBC 6.000 /uL Na 139
RBC 4.920.000 /Ul K 4.0
HGB 12.7 g/dl Cl 103
HCT 38.7% HBsAg(rapid) Negative
PLT 329.000/uL CT 7’00”
Ureum 19 mg/dl BT 3’00”
Creatinin 0,9 mg/dl PT 11,7 Kontrol 11,2
GDS 117 mg/dl APTT 25,6 kontrol 23,6
SGOT 18 U/L CEA 8,48
SGPT 22 U/L PSA 0,676
Lumbosacral X-Ray AP/LAT (June 14th 2012)
MRI lumbosacral (June 7th 2012)
Histopatology :
Microscopic : preparations of tumor tissue showed the sheets or elongated cords of clear cells
with multiple intracytoplasmic vacuoles, so-called physaliphorous (“soap bubble”) cells, some
cell show eosinophilic cytoplasmic, there is tumor cells within mucin bluish mass, focal necrose
and there is little bleeding with little bone trabekel them.
Conclusion: Suitable for chordoma
Resume :
A man 43 years old with chief complain low back pain suffered since 10 months ago and
more severe at this 1 month last. There have bladder dysfunction and constipation since 2 month
ago and sexual dysfunction since 4 months ago.
From physical examination found lump at sacral region with size 2x2cm, irregular
surface, soft consistency, immobile, ill defined, and tenderness. From the thoracolumbal X-Ray
found litic lesion at sacral region. From lumbosacral MRI found T1 Isointense mass and T2
hiperintense heterogeneous at level CV S2- coccygeus which destruction bone and push rectum
susp. Chordoma. From histopathology biopsy found the preparation suitable for Chordoma.
Diagnosis: Sacral Chordoma
Treatment : Analgesic
Wide en-bloc excision
CHORDOMA
BACKGROUND
Chordomas are relatively rare, slow-growing, primary malignant bone tumors. They are
thought to arise from notochordal remnants and thus they occur along the midline from the skull
base to the sacrum. Because of their indolent and low-grade nature, chordomas are typically
diagnosed at a late stage and therefore, often cause significant damage through local bone
destruction and compromise of neurologic structures. The goal of treatment is to achieve surgical
en bloc excision with tumor-free margins to maximize local tumor control and overall survival.
Radiation therapy is often used postoperatively when tumor-free margins cannot be achieved.
Prognosis in terms of both overall survival and prevention of local recurrence is highly
dependent on the adequacy of initial surgical margins.
EPIDEMIOLOGI
Chordomas comprise 1% to 4% of all primary bone tumors. The age adjusted incidence
rate in the general population is 0.8 per 1,000,000 people. These tumors affect men nearly twice
as frequently as women, and they are most commonly diagnosed in middle-aged persons.
Although chordoma can occur in the pediatric population, particularly at the skull base, this is
rare and accounts for <5% of all chordomas. The median age at diagnosis is 58.5 years, and the
incidence increases with age. Chordomas occur only one fourth as frequently in blacks as in
whites. Chordomas are found in the midline of the neuraxis, where they arise from intraosseous
notochordal remnants within spinal segments from the clivus to the coccyx. The anatomic
distribution has been commonly reported to be approximately 50% sacrococcygeal and 35%
sphenooccipital, with15% occurring in the mobile spine, although this distribution varies by case
series.3 In the largest published series to date (400 cases), McMaster et al2 reported that
chordomas appeared in sacral, sphenooccipital, and spinal locations with approximately equal
frequency. Boriani et al4 observed that chordomas that affect the mobile spine involve the
lumbar spine most frequently (57% to 66%), followed by the cervical spine (24% to 29%) and
the thoracic spine (10% to 13.5%). Chordomas are the most common primary bone tumors found
in the mobile spine and the sacrum.
PRESENTATION
Although the presentation of these lesions varies by location, pain is reported to be the
most common presenting symptom regardless of location, in particular, pain with a gradual and
insidious onset. Chordomas often encroach on the spinal canal, and they may cause compression
of the spinal cord, cauda equina, or nerve roots. This is reflected in a wide range of neurologic
symptoms, including weakness, sensory deficits, bowel and bladder incontinence, and sexual
dysfunction. Regional extension dictates symptomatology, including symptoms not directly
attributable to spinal cord or nerve root compromise. Chordomas involving the cervical region
may obstruct the airway, give rise to a retropharyngeal mass, or cause dysphagia, dysphonia, or
Horner syndrome. In the sacral region, presacral extension of chordomas can lead to rectal
dysfunction, including obstipation, constipation, tenesmus, and hemorrhoids, as well as gluteal
masses or masses that are palpable on rectal examination. Because of their slow growth rate and
the often nonspecific nature of their symptoms, chordomas often evade diagnosis until late in the
disease course. The mean duration of symptoms in reported series ranges from 4 to 40 months.
ADVANCED IMAGING
Chordoma classically appears as an osteolytic lesion centered in the midline and in
association with a large soft-tissue mass. Osteosclerotic areas or areas of mixed osteolytic and
osteosclerotic bone destruction may be seen on CT scan. Amorphous intratumoral calcification
can be detected on CT imaging in 30% to 90% of cases. In >50% of cases, a higherattenuation
fibrous pseudocapsule can be seen surrounding the lowerattenuation soft-tissue mass. Compared
with muscle, chordomas range from isointense to hypointense on T1-weighted MRI scans and
are hyperintense on T2-weighted images. Chordomas are well-defined extramedullary masses
that may be seen to compress and sometimes to encase adjacent neurovascular structures. These
tumors commonly invade the intervertebral disk space as they extend between adjacent vertebral
bodies. Chordomas may appear heterogeneous on T2-weighted MRI scans; they have internal
hypointense foci on T1-weighted images because of intralesional calcification, cystic changes,
and hemorrhage. They display prominent contrast enhancement on both CT and MRI scans.
HISTOLOGY
Chordomas consist of lobulated tumor cell nests separated by fibrous septae, often within
an overlying pseudocapsule. Sheets or elongated cords of clear cells with multiple
intracytoplasmic vacuoles, so-called physaliphorous (“soap bubble”) cells, are a pathognomonic
feature of these tumors. The nuclei are small, round, and darkly staining, and they display a mild
to moderate amount of nuclear pleomorphism, with few mitotic figures. Mucin is abundant both
intracellularly and extracellularly in the surrounding myxoid stroma. Necrotic areas are
infrequently seen within chordomas; areas of calcification, hemorrhage, and resultant
hemosiderin deposition are more common. The mucinous stroma may contain prominent
sarcomatous elements, whether fibrous, chondroid, or osteoid, in dedifferentiated chordomas.
These dedifferentiated chordomas are comparatively aggressive, tend to exhibit high-
grade behavior, and carry a poor prognosis. In the well-described variant, chondroid chordoma,
areas of bland-appearing hyaline cartilage make up a substantial component of the specimen.
Benign notochordal cell tumors, also known as notochordal rests, are benign intraosseous lesions
that often are mistaken for chordomas. The anatomic distribution of notochordal rests is the same
as that of chordomas, and it has been suggested that these lesions may be precursors of
chordomas. Benign notochordal rests are made up of sheets of vacuolated cells mixed with less
vacuolated cells. It is important to note that benign notochordal rests lack the surrounding
myxoid stroma characteristic of chordomas and have no mitotic figures or necrotic areas.
DIFFERENTIAL DIAGNOSIS
Although chordomas are the most frequently occurring primary malignantbone tumor in
both the sacrum and the mobile spine, metastatic lesions and multiple myeloma make up the
overwhelming majority of sacral and spinal neoplasms.9 Chordomas may be confused with
plasmacytoma because of their lytic appearance, but chordomas demonstrate positive
scintigraphy. Osteomyelitis and lymphoma are also difficult to distinguish radiographically, but
they can be distinguished from one another because of their distinct clinical courses. Benign
notochordal rests do not display the bony destruction, cortical disruption, or associated soft-
tissue masses seen in chordomas. Clinically, they are indolent and usually asymptomatic.
Chordomas can be distinguished from chondrosarcoma and metastatic tumors on radiographic
evaluation because chordomas lack the associated soft-tissue mass.
Other primary sacral tumors include benignlesions (eg, giant cell tumor, aneurismal bone cyst,
osteoid osteoma, osteoblastoma, hemangioma, nerve sheath tumor) and malignant lesions (eg,
Ewing sarcoma, primitive neuroectodermal tumor, osteosarcoma, Paget’s sarcoma, multiple
myeloma, plasmacytoma). The differential diagnosis for primary vertebral lesions includes all of
these entities as well as teratoma and dermoid. Myxopapillary ependymomas, which are lesions
that arise from the filum terminale, are also occasionally mistaken for chordomas.
Immunohistochemical analysis is one method of distinguishing chordomas from other lesions
that are histologically similar, including choroid meningioma, chondroma, chondrosarcoma,
melanoma, and metastatic adenocarcinoma. Most chordomas display S-100 immunoreactivity,
thus distinguishing them from metastatic adenocarcinoma and meningioma, as well as epithelial
membrane antigen immunoreactivity, thus distinguishing them from chondroma,
chondrosarcoma, and melanoma. Positive cytokeratin CAM 5.2 immunoreactivity is a highly
sensitive but nonspecific means of detecting chordoma.
MANAGEMENT
Surgery remains the mainstay of management of chordomas. However, adjuvant
therapies are currently under investigation. Given the lowgrade nature of these lesions, wide en
bloc excision is mandatory for curative treatment. The importance of obtaining wide tumor-free
margins when possible cannot be underestimated. Numerous studies demonstrate a direct
correlation between the extent of surgical resection and the length of recurrence-free survival.
Sacral chordoma resection involves amputation of a portion of the distal sacrum or removal of
the entire sacrum. A portion of the adjacent bony pelvis may also be removed to achieve an
adequate margin. These procedures often involve the intentional sacrifice of one or more sacral
nerve roots to achieve wide resection of the lesion. This may result in motor, sensory, sphincter,
or sexual dysfunction. The resections can be classified based on the location of the highest
segment removed or according to the highest level of nerve root sacrificed. We herein define
sacral amputations as low (sacrifice of at least one S4 nerve root or any level below), middle
(sacrifice of at least one S3 nerve root), or high (sacrifice of at least one S2 nerve root). Total
sacrectomy is performed when both S1 nerve roots must be sacrificed.
In general, ipsilateral resection of sacral nerve roots leads to ipsilateral motor and sensory
deficits corresponding to the levels sacrificed; however, bowel and bladder function are usually
entirely preserved. Lowsacral amputations commonly result in complete preservation of
sphincter function, although perineal numbness and sexual dysfunction are common. Midsacral
amputations result in a variable degree of functional loss. Most patients are left with saddle
anesthesia and reduced sphincter control but retain intact motor function. Preservation of at least
one S3 root will result in normal bowel and bladder function in most patients. Limited functional
urinary and fecal continence may be preserved when at least one S2 nerve root is spared,
although most patients will have abnormal sphincter function. High sacral amputation and total
sacrectomy with resection of the S1 nerve root frequently results in postoperative motor deficits,
particularly in ankle plantar flexion.
This can impair the patient’s ability to ambulate without external support, even if only
temporarily. Patients undergoing high sacral amputation or total sacrectomy usually experience
complete loss of sphincter control as well as saddle anesthesia and sexual dysfunction. Surgical
management of sacral chordomas is challenging because of the complex regional anatomy, the
often advanced stage of tumor growth, and the proximity to and encroachment on surrounding
tissues. Accordingly, it is appropriate to bring together a multidisciplinary surgical team whose
members may include specialists in surgical oncology, neurosurgery, orthopaedic surgery,
vascular surgery, and plastic surgery. Adequate exposure of the lesions often requires a staged
operation in which standard anterior, posterior, perineal, and lateral approaches are used in
combination. Following tumor excision, advanced techniques in instrumentation (eg, iliac
screws, transiliac bars) may be required to prevent spinopelvic instability, particularly for
patients who require high amputation or total sacrectomy. In addition, soft-tissue reconstruction
with rotational gluteal flaps or transpelvic vertical rectus abdominis myocutaneous flaps is
recommended to promote wound healing and obliterate dead space.
For vertebral body chordomas, en bloc resection with tumor-free margins remains the
goal of surgical treatment. Numerous studies have demonstrated that intralesional excision leads
to a high rate of local recurrence and negatively affects overall survival. To avoid this, en bloc
spondylectomy, or removal of the entire vertebral body in one block, is performed (Figure 7).
This procedure often requires a combination of posterior and anterior approaches, including
thoracoabdominal and retroperitoneal abdominal as well as transpleural thoracotomy). En bloc
removal of alL posterior elements of the vertebra is performed, followed by en bloc resection of
the anterior portion. Spinal reconstruction is necessary. Excellent results have been obtained with
these surgical techniques. Wide en bloc resection is not always possible, either because of the
size or extent of the tumor or because such resection would lead to excessive morbidity. Even
though the margins are intralesional in these cases, an effort is made to perform extracapsular
excision of the chordoma, which involves removal of the specimen without penetration of its
surrounding pseudocapsule.
Often these patients then receive adjuvant radiation therapy to provide local control over
any residual disease.Chordomas in the upper cervical vertebrae require special consideration.
Like sacral chordomas, these are in a region with complex anatomy and many sensitive
structures. These tumors may extend into the retropharyngeal space or may spread epidurally,
causing spinal cord compression. Multidisciplinary teams may be involved, including ear, nose,
and throat specialists and plastic surgeons. The anterior phase of these procedures often consists
of transglossal or transmandibular approaches (as opposed to transoral approaches). These
approaches provide adequate visualization of the tumor pseudocapsule and allow for
extracapsular excision.
Radiation therapy can be used as an adjuvant treatment for chordomas with incomplete
resection or positive margins; however, the efficacy of such treatment is unproved. Their
proximity to sensitive neurologic tissues make chordomas difficult to treat with standard
radiation therapy. These tumors are relatively radiation-resistant and thus are thought to require
doses of ≥60 to 70 Gy, which may surpass the doses safely tolerated by the spinal cord.35
Moreover, metal hardware associated with spinal reconstructive surgery may produce artifacts
that interfere with accurate targeting of the tumor volume during radiation therapy. Conventional
photon-beam radiation therapy is used as an adjuvant treatment in patients undergoing subtotal
excision. However, reports vary as to whether additional survival benefit is derived.36
Conventional treatments with doses of 40 to 60 Gy have produced 5-year local control rates of
10% to 40%.35 It is difficult to interpret and apply results given the rarity of the disease as well
as the variability in surgical procedure, completeness of excision, and the timing and method of
delivery of radiotherapy. However, radiation treatments are improving with recent advances in
photon-beam therapy, including the use of intensity-modulated radiation therapy and stereotactic
radiosurgery. Improved accuracy of tumor targeting can be achieved with both of these
techniques, allowing for an increased tumor dose with reduced collateral damage to surrounding
tissues.
The use of radiosensitizing agents to enhance response to photon-beam therapy has been
reported in a small number of patients. Hadron therapy, which makes use of protons or charged
particles such as carbon ions, helium, and neon, is another promising treatment modalityfor
chordomas. Because of the ballistic properties of these particles, hadron dose deposition is
limited to a sharply defined Bragg peak, which provides a steep gradient between the target dose
and that delivered to the surrounding tissues. Thus, hadron therapy permits delivery of highdose
radiation to the target tissue that, in principle, could surpass even the most sophisticated photon
radiation delivery techniques while minimizing damage to nearby sensitive structures.
Additionally, certain hadron particle beams may offer superior tumor kill properties. Hadron
therapy may be used in combination with conventional photon beam therapy, or it may be given
alone. Proton-beam therapy appears to offer an improvement in chordoma treatment, with
reported local control rates of 50% to 60% at 5 years. However, there is no level I or II evidence
to support this. Most of these studies are smaller case series, and they often combine data related
to patients with chondrosarcoma and those with chordoma. More data are needed to evaluate the
true short- and long-term efficacy of hadron therapy and to better determine the scenarios
inwhich it will be useful as an adjunct treatment for chordoma.
Chordomas have proved to be highly resistant to chemotherapy. This modality may have
some impact on the rare, high-grade dedifferentiated chordoma. However, the development of
newer molecularly targeted agents has led to renewed interest in the use of chemotherapy. One
such agent, imatinib, an inhibitor of platelet-derived growth factor receptor-β (PDGFR-β), was
used to treat chordomas in 18 patients.43 Many of these patients demonstrated symptomatic
improvement and a tumor response evidenced by a reduction in contrast enhancement, with
effects lasting for 1 year. These results are promising, especially in light of the recent report of a
series of 31 chordomas, all of which displayed overexpression and activation of PDGFR-β.44
Clinical trials are ongoing, and other drugs are under investigation for chordoma, including
antiangiogenic agents and epidermal growth factor receptor inhibitors.
PROGNOSIS
Current studies suggest that the prognosis for patients with chordoma has improved
dramatically with the adoption of aggressive surgical treatments. Older studies reported 5-year
survival rates of 50% to 68% and 10-year survival rates of 28% to 40%,2,4 whereas newer case
series report 5-year survival rates of 73% to 86% and 10-year survival rates of 49% to 71%.
Although chordomas are considered to be slow-growing neoplasms, they do have the propensity
to metastasize. Metastases have been found in up to 5% of patients at the time of diagnosis and
in up to 65% at autopsy; the most common sites of metastasis are the lungs, soft tissues, bone,
skin, pancreas, heart, and brain. Most surgeons agree, however, that local recurrence is the most
important determinant of long-term survival and that local control is the key to successful
treatment. Bergh et al3 report a 21-fold increase in risk of tumor-related death in those with
recurrent local disease. Several studies have confirmed that the recurrence rate is greatly
increased for patients with intralesional excision compared with those who had adequate
margins. Local control is difficult to achieve without en bloc excision and tumor-free margins
regardless of the use of adjuvant radiotherapy. Thus, the current standard of care for chordomas
remains aggressive en bloc surgical resection whenever possible to achieve optimal local control
and survival. With the high rate of recurrence and the resulting poor prognosis, extended follow-
up and surveillance is necessary for chordoma patients. At our institution, patients undergo CT
scan of the resection bed immediately postoperatively, and MRI scanning is done within 48
hours. After release from the hospital, surveillance MRI scans are obtained every 3 months in the
first year following resection, every 6 months in the second year, and annually thereafter.
SUMMARY
Chordomas can appear at any location along the spine and often show advanced growth
at the time of diagnosis. Such tumors are minimally responsive to radiation and chemotherapy;
thus, surgical resection remains the mainstay of treatment. Local control and overall survival
have been linked with the ability to perform radical resection. However, given the propensity for
such lesions to become intimately associated with neural, vascular, and visceral structures,
specifically at the skull base and sacrum, surgical morbidity may be substantial. For this reason,
treatment of patients with chordomas should involve a multidisciplinary team consisting of
doctors in the fields of surgical oncology, radiation oncology, neurosurgery, orthopaedic surgery,
general surgery, and plastic surgery, as needed, to provide the best chance for an optimal
outcome.
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