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P. Truckenmueller et al.
60.9 What Should Patient andFamily Know?
Whereas most of the neurological decits are transient, postoperative disability
might prolong in-hospital stay and delay return to normal life. Especially older age
is associated with a higher rate of permanent decits.
Further Readings
Duong LM, et al. Descriptive epidemiology of malignant and nonmalignant primary spi-
nal cord, spinal meninges, and cauda equina tumors, United States, 2004–2007. Cancer.
2012;118(17):4220–7.
Wostrack M, etal. Spinal ependymoma in adults: a multicenter investigation of surgical outcome
and progression-free survival. J Neurosurg Spine. 2018;28(6):654–62.
Plasmacytoma
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61
İsmailDaldal , AliekberYapar ,
andAlpaslanŞenköylü
61.1 Definition
There is a wide range of clinical highlights, from monoclonal gammopathy of
obscure importance to multiple myeloma (MM) to plasma cell leukemia. Most of
the plasma cell tumors are analyzed as MM and will in general inuence a more
established grown-up population. In any case, a minority (<5%) of patients with
plasma cell malignancies present with either a solitary bone mass called solitary
bone plasmacytoma (SBP) or less ordinarily a soft tissue mass of monoclonal
plasma cells called solitary extramedullary plasmacytoma (SEP).
SBP is a plasma cell problem described by a localized collection of neoplastic
monoclonal plasma cells in the bone. SBP has a male/female ratio of 2:1, with an
average age of 55 years. Solitary type essentially involves axial skeleton, particularly the vertebrae.
Supplementary Information The online version contains supplementary material available at
(https://doi.org/10.1007/978- 3- 030- 80356- 8_61).
İ. Daldal
Department of Orthopaedics and Traumatology, Ankara Lokman Hekim Akay Hospital,
Ankara, Turkey
e-mail: daldal_ismail@hotmail.com
A. Yapar
Department of Orthopaedics and Traumatology, Ankara Oncology Education and Training
Hospital, Ankara, Turkey
e-mail: aliekberyapar@hotmail.com
A. Şenköylü (*)
Department of Orthopaedics and Traumatology, Gazi University Faculty of Medicine,
Ankara, Turkey
© The Author(s), under exclusive license to Springer Nature
Switzerland AG 2022
A. Şenköylü, F. Canavese (eds.), Essentials of Spıne Surgery,
https://doi.org/10.1007/978-3-030-80356-8_61
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Malignant bone tumors of the spine are extremely uncommon. SBP is the most
common separate tumor inside this gathering, representing around 30% of the
aggregate. These tumors place in the spinal bony structures twice as frequently as
other bones.
İ. Daldal et al.
61.2 Natural History
SBP has a high incidence of progression to MM, and on the magnetic resonance
imaging (MRI) assessment, at any rate, 25% of patients with an evident solitary
mass have proof of disorder somewhere else. Interestingly, SEP is almost in every
case genuinely restricted and has a high healing rate with local treatment options.
Most of the patients with obvious SBP progress to myeloma, with a middle opportunity to the progression of 2 to 4 years. The middle by and large endurance in various studies uctuates from 7.5 to 12 years.
If a MM patient has features including low levels of uninvolved immunoglobulins, axial disease, older age, lesion size >5 cm, and persistence of the M protein
after treatment, it will result in disease progression. The presence of M protein has
been accounted for in 24% to 72% of patients in various studies. The recurrence
presumably relies upon the degree of affectability of the tests utilized.
61.3 Physical Examination
The most common manifestation is the pain at rest (Chap. 41); however, it can likewise present spinal cord or root compression ndings (Video 61.4). Inclusion of the
base of the skull can give cranial nerve compromise (paralysis).
61.4 Imaging
Just as the proper blood and urine tests, imaging of the spine is required and best
accomplished by MRI and computed tomography (CT) scan. A mini-brain appearance has also been described in MRI (Fig.61.1). It is seen as curvilinear low-signalintensity areas within the lesion, giving an appearance of sulci in the brain [1].
Indeed, this appearance is a trademark to such an extent that it might block the
requirement for a symptomatic biopsy. The biopsy is generally conceivable percutaneously, guided either by uoroscopy or CT (Video 61.8). Positron emission
tomography (PET) has as of late been assessed in the organizing of patients with
myeloma and plasmacytoma. PET, similar to MRI, seems to be valuable in identifying occult disease in patients with SBP.
There are no detailed rules to characterize contribution on MRI scans with regard
to evident SBP.Be that as it may, the MRI appearances in MM have been very much
portrayed. The presence of at least one foci of anomalous sign force, for example,
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Fig. 61.1 A case with
“mini-brain” appearance in
axial MRI (L2 vertebrae)
375
low on T1-weighted imaging and high on T2-weighted or STIR (short TI inversion
recovery) images, which upgrade after the organization of paramagnetic differentiation without realized late pressure cracks, other essential danger, or average attributes of generous or harmful essential bone tumors, is viewed as proof of removed
association in patients with obvious SBP.
61.5 Differential Diagnosis
The accompanying measures are suggested:
• Single region of bone destruction because of plasma cells.
• Histologically <5% plasma cells in bone marrow suction.
• Totally normal outcomes on skeletal overview including radiology of long bones.
• No hypercalcemia or renal debilitation due to plasma cell dyscrasia.
• Missing or low serum or urinary degree of monoclonal immunoglobulin (level of
>20g/l) dubious of MM.
• No extra lesion on MRI of the spine.
SBP is for the most part diagnosed by biopsy or ne-needle aspiration.
Percutaneously guided biopsy of the spine is typically conceivable either by uoroscopy or CT (Videos 61.7 and 61.8). As these tumors are uncommon, pathology
survey by a pathologist with an exceptional interest in either bone tumors or lymphoproliferative issues is rmly suggested.
61.6 Treatment Options
Although the mainstay treatment for MM is systemic chemotherapy, radiotherapy
often has an important supportive role, offering very effective symptom relief for
plasmacytomas in bone or soft tissues. Radical radiotherapy is the preferred
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İ. Daldal et al.
treatment for SBP.As with other rare tumors, the evidence base for treatment consists largely of retrospective studies of a small number of patients. Progression to
MM is common, despite the high local control rates of 83% to 96% achieved with
medium- dose radiotherapy. Based on the results of retrospective case studies, a dose
of 40Gy in 20 fractions is recommended for SBP.For SBP larger than 5cm in size,
a higher dose up to 50Gy in 25 fractions should be considered [2]. An alternative
strategy for tumors larger than 5cm is chemotherapy followed by radiotherapy. This
is theoretically attractive, but there is little published evidence to support it.
Radiotherapy remains the treatment of choice of the primary pathology, and surgery is contraindicated in the absence of structural instability or neurological compromise. However, early diagnosis and referral for a neurosurgeon/orthopedic
surgeon opinion is advised in most cases with spinal involvement. Due to the development of modern spinal xation systems over the last decade, surgical treatment is
now a viable and successful option for patients who develop pain caused by structural compromise within the vertebra, vertebral instability, neurological compromise, or a combination of these.
Close contact between hematologist, radiotherapist, and neurosurgeon/orthopedic surgeon is along these lines essential in planning ideal treatment options for
patients.
61.7 Expected Outcomes
As a radiotherapy and/or chemotherapy-sensitive neoplasm, the prognosis of plasmacytoma with conservative treatment is satisfactory in general.
61.8 Potential Complications
Spinal instability and neurological compromise are extremely rare.
61.9 What Should Patient andFamily Know?
SBP is a rare disease in the spectrum of plasma cell dyscrasias. Solitary lesions have
a high potential to transform into MM.
Further Readings
1. Ferreira-Filho LA, Pedroso JL, Sato EA, et al. Teaching neuroimages: "mini brain" sign: a
radiologic marker for vertebral solitary plasmacytoma. Neurology. 2014;82(23):e210–1.
2. Soutar R, Lucraft H, Jackson G, et al. Guidelines on the diagnosis and management of soli-
tary plasmacytoma of bone and solitary extramedullary plasmacytoma. Br J Haematol.
2004;124:717–26.
Chordoma
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62
MehmetAliDeveci andS.AykınŞimşek
62.1 Definition
Chordoma is an aggressive, locally invasive tumor representing 1% to 4% of bone
tumors; the prognosis is generally poor. Although it is classied as a low-grade
tumor, its clinical course resembles malignant tumors because of its high recurrence
rate and inltrative growth pattern [1]. It was rst described by Virchow in 1857, but
Ribbert used the term “chordoma” for the rst time in 1890 [2]. Its incidence is
<0.1/100.000, and it is mostly seen in the fth decade of life; the tumor is hardly
ever seen in individuals younger than 30 to 40 years of age, and it is more common
among men; about 5% of all chordomas develop during adolescence [1].
Chordomas originate from the remnants of the notochord. Undifferentiated notochord remnants in the form of microscopic foci can be found within the vertebral
bodies at the cranial and caudal ends of the embryo. Chordomas can rarely develop
from normal notochord remnants such as the nucleus pulposus. Malignant transformations from these residues occur during the third to fourth decade in sphenooccipital lesions and during the fth to sixth decades in sacrococcygeal lesions. The
most important evidence proving the notochord origin of the tumor is the 6q27
duplication detected in the transcription factor-T gene (brachyury) of familial chordomas. Brachyury is found in the normal, undifferentiated embryonic notochord of
the axial skeleton [3].
Supplementary Information The online version contains supplementary material available at
(https://doi.org/10.1007/978-3-030-80356-8_62).
M. A. Deveci (*) · S. A. Şimşek
Department of Orthopedics and Traumatology, School of Medicine, Koç University,
Istanbul, Turkey
e-mail: mdeveci@ku.edu.tr; asimsek@ku.edu.tr
© The Author(s), under exclusive license to Springer Nature
Switzerland AG 2022
A. Şenköylü, F. Canavese (eds.), Essentials of Spine Surgery,
https://doi.org/10.1007/978-3-030-80356-8_62
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M. A. Deveci and S. A. Şimşek
62.2 Natural History
The prognosis is better in patients aged <40 years; the overall survival rate is 6 years
on average, and the 5-year survival is 70%, while 10-year survival is 40%. Local
recurrence is extremely frequent.
62.3 Physical Examination
Chordomas are mostly midline lesions, and 32% of them are located in the skull
base, 32% in the mobile vertebrae, and 29% in the sacrum [2]. Since chordomas are
slow-growing, locally aggressive, and invasive tumors, the disease frequently concerns bone and neural structures. For this reason, effective treatment is very difcult
because of the large lesions at the time of diagnosis, the involvement of neural
structures, compression of the surrounding soft tissues, inability to obtain wide margins during surgery, and resistance to radiotherapy.
Since chordomas are insidious and slow-growing tumors, they tend to remain
asymptomatic until the later stages of the disease.
Depending on their size and involvement of the sella, they may cause endocrinopathies, rarely epistaxis, and intracranial bleeding. Cervical chordomas can cause
airway obstruction and dysphagia and appear as oropharyngeal masses.
Involvement in the mobile spine presents symptoms according to deep-seated
pain (Chap. 41) and the level of the involved nerve. Constipation and sphincter
paralysis, bladder dysfunction, gait disturbances, and hypoesthesia can be seen with
the growth of the mass. Neurological decits are more common in mobile vertebral
involvement than in sacrococcygeal involvement (Video 62.4).
Sacrococcygeal chordomas often involve S4–S5, and the masses usually enlarge
into the pelvis. The invasion of the pelvic structures is limited by the presacral fascia. Rarely, it may present as masses that grow into the postsacral area and are palpated externally.
62.4 Imaging
Midline-located chordomas appear radiographically as destructive bone lesions,
and typically the destruction starts from the midline. While chordomas spread to the
adjacent spine, they may locally invade the intervertebral discs. Lateral radiographs
are more useful in sacrococcygeal chordoma (Fig.62.1).
Computed tomography (CT) is more useful in showing lesions located within the
midline spine. It may show lesions containing scattered calcications and extending
into the bone with soft tissue involvement (Fig.62.2).
Magnetic resonance imaging (MRI) depicts the tumor as iso- or hypointense in
T1-weighted images and as hyperintense in T2-weighted images; soft tissue
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Fig. 62.1 Sacral chordoma. Radiography of the anteroposterior pelvis and lateral lumbosacral
vertebra
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Fig. 62.2 Sacral chordoma. CT ndings show destruction of sacrum with soft tissue lesion
component heavily retains gadolinium. MRI gives a better idea about the soft tissue
involvement (Fig.62.3).
Chordomas show normal or decreased uptake than other bone tumors with the
Tc99m bone scintigraphy.
The PET/CT with 18-FDG shows an increased systemic uptake values (SUV)
highlighting the aggressive nature of the tumor (Fig.62.4).
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M. A. Deveci and S. A. Şimşek
Fig. 62.3 Sacral chordoma. Wide presacral soft tissue involvement of the tumor in T1 sagittal, T2
fat-suppressed sagittal, and T1 axial sections
Fig. 62.4 PET/CT images of sacral chordoma
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62.5 Differential Diagnosis
The histological diagnosis can be done with a needle biopsy under CT scan guidance (trans-pedicular) in about 80% of cases (Video 62.8); open biopsy is exceptional, and it should be performed if two successive needle biopsies are not
conclusive.
Pathology. Chordomas show various degrees of histological atypia, and the relationship between these histopathological ndings and tumor behavior is the subject
of many studies. There are three separate histological subgroups: classical (conventional), chondroid, and dedifferentiated.
The classic chondroma is a soft, gray-white, lobulated tumor (Fig.62.5). Cell
groups settled in a myxoid stroma are separated from each other by brous septa.
Cells are round, have regular nuclei, and show little nuclear atypia. They are
characterized by prominent vacuole cytoplasm. These vacuoles give the cell a
physaliphorous (bubbly) appearance (Fig.62.6). On the other hand, chondroid
chordomas contain cell structures belonging to both classical chordoma and
Fig. 62.5 Macroscopic views after chordoma resection; interior surface shows lobulated, gelatinous nature of the tumor
Fig. 62.6 Chordomas appear as lobulated cells separated by brous septa within the mucinous
matrix. The vacuole structure in the cytoplasm of the cells is remarkable
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