Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_6029_Библиотеки_им_академика_М_И_Перельмана.pdf
268
19 Tu mor s
effective, radical tumor removal respecting all the previously mentioned UCS anatomical restrictions is the
only reasonable option [20, 112].
19.1.4.5 Ewing Sarcoma (ES)
ES is a malignant, aggressive, poorly differentiated
tumor that arises in the bone and soft tissues. ES is the
second most common primary malignant bone tumor in
children [129]. The spine is affected in about 3.5% of
patients and UCS involvement is extremely rare. ES can
invade the intervertebral disk space and multilevel spread
is not exceptional. Metastatic spread is common.
Diagnosis
Clinical symptoms can begin with neurological deficits related to spinal cord compression, and can appear
early because of rapid tumor growth.
Radiological diagnosis is based on detection of
osteolytic lesions that do not respect the vertebral borders with invasion of intervertebral disks and often
leading to vertebral collapse and deformity.
19.1.4.6 Osteogenic Sarcoma (OS)
OS is the most common type of malignant bone cancer,
accounting for 35% of primary bone malignancies. It
is rare in the spine (3.5% of all OS), most often
detected in sacrum and described rarely in the UCS
[108]. Shives and colleagues found 30 cases in the
spine, only 4 of which were cervical [111]. Some
cases of osteosarcoma of the spine are secondary, arising from Paget disease or after radiation therapy [10].
The course of the disease is very rapid involving the
whole vertebra and metastasizing in the majority of
cases. Clinical and radiological appearance is similar
to other sarcomas. The fast osteolytic process can rapidly lead to neurological compromise and spine deformity. The mortality is generally very high. Sciubba
et al. [110] in their recent review analyzed six studies,
and concluded that modern neoadjuvant chemotherapy
(given before the surgery) can substantially improve
the local control as well as the long-term survival.
According to their conclusions, radical resection is also
effective in terms of local control and survival rate.
19.1.4.7 Solitary Plasmocytoma
Treatment Strategy
Traditional treatment for ES in long bones has been
radical surgery (usually meaning amputation) in conjunction with chemotherapy and radiation. ES is known
to be sensitive to chemotherapy. In the past, effective
chemotherapy has been associated with an increase in
the 5-year survival from 5% to 10%. Nowadays, modern chemotherapy regimen used prior to surgery (neoadjuvant chemotherapy) helped to increase the 5-year
survival to 65%–70% [7, 90]. Despite that, a true radi-
cal resection is not possible in the region of UCS thus
surgical tumor removal should be as radical as possible. According to the recent Sciubba et al. [110] systematic review, it can be concluded that neoadjuvant
modern chemotherapy offers significant improvement
in local tumor control and also long-term survival.
Therefore, it follows that chemotherapy should precede surgical procedure. En bloc resection provides
better local control, however, it does not increase the
overall survival rate. Adjuvant radiotherapy is recommended after incomplete tumor resection, which in the
UCS means almost always.
There is still an ongoing debate whether plasmocytoma can exist as solitary lesion [85] as it is principally
a systemic malignant neoplasm of bone marrow originating from plasmacellular differentiation of
B-lymphocytes. Either solitary or multiple tumors can
be found in the entire spine and not uncommonly in the
cervical region including the UCS [54, 68, 99, 120].
The clinical picture is often non-specific similar to the
other tumors with slow growth. CT and MRI will show a
circumscribed lucent lesion visible mostly within the C2
body (Figs. 19.25 and 19.26). MRI is also a better tool
than bone scintigraphy for exclusion of multiple spinal
occurrences. The diagnosis is based on laboratory investigations confirming the presence of paraproteins in
blood serum and histological analysis of the bone marrow smear. Also, a CT-guided or free-hand trocar biopsy
(for C2 vertebra that means transoral) can provide tissue
for histology.
Although combined chemo and radiotherapy are the
treatment of choice of this neoplasm there can be some
specific situations requiring more aggressive approach.
This is true in the case of vertebral collapse and deformity
causing cord compression. In the case of axis involvement, transoral decompression and AA stabilization may
19.1 Extradural UCS Tumors
Fig. 19.25 Solitary plasmocytoma of C2 partially filled by transoral needle bone cement vertebroplasty. (a) Radiolucent area under
the C2 lateral mass. (b) Partial bone cement filling supporting the lateral mass
269
be the right choice [99, 120]. However, wide-margin
resection is never indicated. When the odontoid process
is encompassed, or the lateral C2 mass pillars are substantially weakened, fortification with needle bone
cement vertebroplasty is a reasonable approach. This can
be performed either transorally (Fig. 19.25) or, and in our
experience better, via the minimally invasive high anterolateral approach and vertebral puncture in odontoid
screw-like fashion (Fig. 19.26).
19.1.5 Secondary Bone Tumors
Metastatic disease is probably the most important
problem for spine surgeons dealing with oncologic
presentation in the spine. The problem is so broad and
complex that cannot be shortly described in one chapter of a book focused on different topics and therefore,
only a brief summary will be presented.
The frequency of spine metastatic involvement is
very high, occurring in about 70% of all patients suffering from cancer. The cervical spine is the least common site for metastatic spread with a reported incidence
of between 8 and 20% of all spinal metastases [23,
100]. The average age range of patients diagnosed with
cervical spine metastases is 58–61 years, without gender predominance [5].
If the UCS is involved, often the C2 body and/or
arch are affected [97]. More rarely, C1 lateral masses
or occipital condyles are affected. Different papers
describe varying frequencies of appearance of many
tumor types that occur in the cervical spine without
special attention to CVJ region [23, 37, 116].
According to our experience, the UCS most commonly features metastases of pulmonary (Fig. 19.27),
renal (Fig. 19.28), thyroid gland, and breast carcinomas. We have seen metastases of melanoma
(Fig. 19.29) and gynecologic tumors (Fig. 19.30). To
date, we have not seen prostate or gastrointestinal
tumor metastases to the UCS, but they are certainly
possible.
19.1.5.1 Diagnosis
Clinical symptoms may range from local and referred
pain, mechanical pain of pathological fracture or instability to neurologic manifestations of nerve root and spinal
cord compression [51]. Pain is the predominant symptom
in most patients and is present in 90% of cases. This is
particularly true in the UCS where pain can be provoked
by any direction of head movement. Neurologic dysfunction is estimated to occur in 5–10% of patients with metastatic spine disease. Spinal cord compression with
symptoms and signs of myelopathy is more common in
the subaxial cervical area as opposed to the atlantoaxial
region secondary to the differential size of the spinal
canal at these levels [94]. Constitutional symptoms,
weight loss, and anorexia may also be present. History of
malignancy is always very suspicious and warrants further workup. As for the other tumor types, CT and MRI
are essential for diagnosis and evaluation of surgical
270
19 Tu mor s
Fig. 19.26 Solitary C2 plasmocytoma treated with bone cement
needle vertebroplasty via high anterolateral mini-invasive
approach. (a) Radiolucent areas in C2 body on coronal plane CT
reconstruction. (b) MRI showing the intensity change of C2
body and odontoid process. (c) Lateral plain film depicting bone
cement filling of C2. (d) AP view documenting uniform cement
spread
19.1 Extradural UCS Tumors
271
Fig. 19.27 Metastasis of pulmonary carcinoma destroying the
C2 body treated with palliative occipitocervical fusion. The
patient survived 7.5 months. (a) Preoperative CT 3D reconstruc-
tion. (b) MRI in sagittal plane. (c) Occipitocervical fusion skipping the C2 without any bone grafts
272
19 Tu mor s
Fig. 19.28 Metastasis of renal carcinoma to C3 treated with anterior corpectomy and cage and plate fixation. (a) Preoperative sagit-
tal MRI. (b) Plain lateral film after the surgery
feasibility. In questionable cases, biopsy can help. Whole
body workup has to be performed to exclude other tumor
primary sites in a solitary UCS mass.
patients are appropriate for surgical intervention [122,
123, 125]. However, no single classification is specifi-
cally designed for the UCS and most are utilized to
estimate patient survival time.
19.1.5.2 Classification, Grading, and Scoring
19.1.5.3 Therapeutic Strategy
In an attempt to provide prognosis, to guide appropriate
treatment for each patient, and also to allow for information interchange evaluating outcome, classification
systems have been described. Harrington proposed a
five-level classification scheme [58]. The Kostuik classification system attempts to identify which lesions will
cause mechanical instability and are suitable for surgical intervention [73]. Raycroft and colleagues have
proposed a specific classification system applied to the
management of cervical metastatic tumors [102].
Tomita et al. and Tokuhashi et al. have suggested scoring systems that may assist in differentiating which
Different conservative treatment methods are available
in cervical spinal metastases, including radiotherapy,
hormonal therapy, chemotherapy, and high-dose steroid therapy.
The indications for surgical intervention in upper
cervical metastatic tumors include evidence of gross
instability or neurologic compromise caused by malalignment or direct tumor compression. Also, solitary
appearance of metastasis of radically treated primary
tumor, which is feasible for surgical removal can represent an indication.
19.1 Extradural UCS Tumors
273
Fig. 19.29 Generalized melanoma in a very young man (27 years)
with metastasis in right occipital condyle simultaneously with
tumor destruction of C4 body treated with a combined approach.
This unfortunate patient survived only 6 weeks after surgery.
19.1.5.4 Our Preference
For surgeons deciding whether or not to operate, the
UCS represents a unique area of the spine. As we
know from the thoracolumbar spine, simple laminectomy performed to decompress the neural structures is
useful for pain relief and maintenance of ambulation.
In the UCS, however, the progressing myelopathyrelated deficit will kill the patient while fully conscious. This is not acceptable for us in the majority of
cases. On the other hand, if one cannot offer an ambulatory survival, the surgery ends up being only a technical exercise adding more stress to the unfortunate
patient.
About 80% of metastatic tumors are complex,
which means that they are spreading extra-compartmentally. In those cases, it is illusory to think that any
chance of radical resection is feasible. In those with
intraosseous involvement only or purely epidural
spread, radical resection can certainly be achieved.
Surgical decision making is also imperative in the
cases of solitary tumor appearance of unknown origin
where diagnosis and treatment may be necessary.
In summary, we propose that the most important
factor in surgical decision process is the estimation of
Retrospectively this indication was more than questionable. (a)
Coronal plane reconstruction. (b) Sagittal MRI view showing
critical spinal cord imperil. (c) Lateral plain film depicting anterior
cage and plate C3-5 fusion and posterior occipitocervical fixation
patient’s survival time with good quality of life by the
multidisciplinary oncologic team. Good quality of life,
in our terms, means ambulatory and painless:
In case of expected survival less than 6 weeks, any •
surgical intervention makes no sense, in our
opinion.
If the survival is estimated to be more than 6 weeks •
but less than 6 months, we prefer to decompress the
cord and as simply as possible stabilize the spine
(wires etc.).
If the expected survival is longer than 6 months but •
less than 1 year, then decompression is followed by
spine stabilization (mostly, posterior modular)
without bone grafting.
In cases of radically removable tumors and in those •
with life expectancy longer than 1 year, we attempt
to achieve radical resection (if possible) and reconstruct the spine in the same manner as in non-oncologic patients.
Certainly, this approach cannot be used in absolute
terms but it is sensible to consider each individual
patient and their specific situation. General medical
status must be considered and attention paid to patient’s
wishes and expectations.
274
19 Tu mor s
Fig. 19.30 Gynecological tumor (myxoid leiomyosarcoma)
metastasis to the posterior C2 elements was radically marginally
resected and posterior occipitocervical fusion supplemented by
autologous bone grafts allowed survival 1.5 years after the
surgery. (a) MRI showing the posterior tumor expansion. (b)
Peroperative picture showing capsulated huge tumor. (c)
Peroperative picture of OC fusion. (d) Laterogram of the fused
cervical spine
19.2 Intradural Tumors (Extramedullary, Intramedullary)
275
19.1.6 Tumors of Spine Surrounding
Connective Tissue
Tumors of all the tissue types surrounding the bony
spine can occasionally be seen. Lipomas, chondromas,
angiomas, and extra spinal neurinomas on the benign
side but also sarcomas of muscles and synovial membrane theoretically also exist.
A case of synovial sarcoma affecting the C2-3 joint
is presented here (Fig. 19.31)
19.2 Intradural Tumors
(Extramedullary, Intramedullary)
While this is not the primary focus of this book, intradural tumors of the UCS and CVJ area need to be
briefly mentioned. The aim is not to comprehensively
cover the principles of microsurgical removal but to
inform the readers that these pathologies can present in
the UCS region and that sometimes the approach used
for their removal can damage the statodynamic system
Fig. 19.31 Synovial sarcoma of C2/3 joint removed by marginal resection via high anterolateral approach. (a) Axial MRI. (b)
Frontal plane MRI. (c) Sagittal CT reconstruction. (d) Coronal MRI after tumor removal
276
19 Tu mor s
of the spine and require reconstruction. Rarely, they
can also mimic the above-mentioned pathologies and
therefore, it is good to know their pathoanatomic
appearance.
Meningiomas, followed by nerve sheath tumors, are
the most common primary spinal canal neoplasms
[109]. In fact, meningiomas comprise 38–46% of foramen magnum tumors [55, 56]. Anterior meningiomas
are, by definition, those attached to the foramen magnum on both sides of the midline (Fig. 19.32), lateral
are defined as between the midline and the dentate
ligament, and posterior tumors (Fig. 19.33) are attached
posterior to the dentate ligament [55, 56]. Based on
this definition, nerve-sheath tumors are always lateral
(Fig. 19.34), although may exhibit posterior or anterior
extension [56]. Tumors of the ventral foramen magnum represent formidable surgical lesions, as they may
encase the vertebral, basilar, or their perforating arteries, cranial nerves and be densely adherent to the brainstem. Bony structures of the CVJ may also be invaded
Fig. 19.32 Anterolaterally located CVJ meningeoma. (a) Preoperative sagittal MRI. (b) MRI performed after tumor resection (note
CSF pseudocyst)
Fig. 19.33 Retromedullary located CVJ meningeoma. (a) Sagittal MRI. (b) peroperative picture showing the released tumor before
total extirpation
19.2 Intradural Tumors (Extramedullary, Intramedullary)
277
Fig. 19.34 Dumbbell neurinoma of C2 root. (a) Preoperative sagittal MRI. (b) Tumor extend on coronal MRI. (c) Axial MRI show-
ing the relationship of the tumor to spinal cord. (d) Postoperative MRI showing total tumor removal
[107]. Radical tumor removal can thus be complicated
by these tumor properties, or can be associated with a
relatively high rate of complications. However, with
careful preoperative planning, fine microsurgical techniques, proper use of skull-base approaches, intraoperative neurophysiological monitoring, and close
postoperative management, the rate of complications
can be decreased and patient survival prolonged.
Contrary to meningiomas, commonly encountered
at the CVJ, this region is an unusual location for
intramedullary tumors and presents specific surgical
challenges. Only 43 patients with intramedullary
tumors in this location were seen during a 10-year
period in a highly specialized tertiary center [130]. The
most common histological subtype is ependymoma
and astrocytoma; other tumors are rare [130]. The goal