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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_6009_Библиотеки_им_академика_М_И_Перельмана.pdf
Plasmacytoma
KEY FACTS
TERMINOLOGY
• Solitary monoclonal plasma cell tumor of bone or soft
tissue
• Diagnosis of solitary bone plasmacytoma (SBP) requires
○ Solitary lesion, biopsy showing plasma cells
○ Negative skeletal survey, negative MR spine, pelvis,
proximal femora/humeri
○ Negative clonal cells in marrow aspirate
○ No anemia, hypercalcemia, or renal involvement
suggesting systemic myeloma
IMAGING
• Axial skeleton > extremities
Neoplasms, Cysts, and Other Masses
○ Thoracic vertebral body most common site
• Radiographs/CT
○ Lytic, multicystic-appearing lesion ± vertical dense
striations
○ Pathologic compression fracture common
(Left) Sagittal graphic shows
collapse of the thoracic
vertebral body due to tumor
infiltration. There is
retropulsion of the anterior
and posterior margins.
Posterior retropulsion and
tumor mass may result in cord
compression. (Right) Sagittal
T2WI MR demonstrates a
heterogeneously hyperintense
thoracic vertebral body lesion
with hypointense ventral
epidural ſt and paravertebral
soft tissue components
producing cord compression.
• T1 hypointense, T2/STIR hyperintense marrow with lowsignal, curvilinear areas
○ Posterior elements involved in most cases
○ ± associated soft tissue mass (paraspinous or epidural
with draped curtain sign)
PATHOLOGY
• SBP may reflect early (stage I) multiple myeloma
• SBPs considered clinical stage I Durie-Salmon lesions
CLINICAL ISSUES
• Most common symptom = pain due to bone destruction
• Epidural extension may cause compression of cord or nerve
root
• Mean age = 55 years (younger than age of patients with
multiple myeloma)
DIAGNOSTIC CHECKLIST
• Must exclude 2nd unanticipated lesion (33% of cases)
280
(Left) Sagittal bone CT exhibits
the characteristic appearance
of thickened cortical struts ſt,
a result of stress phenomenon
from the lytic process forcing
remaining bone to increase
thickness as a compensatory
response to weakening bone.
(Right) Coronal STIR MR of the
thoracic spine demonstrates
hyperintense lesions involving
3 contiguous vertebral bodies
ſt with an associated soft
tissue mass extending in the
right paravertebral region .
Multiple Myeloma
KEY FACTS
Neoplasms, Cysts, and Other Masses
TERMINOLOGY
• Multifocal malignant proliferation of monoclonal plasma
cells within bone marrow
IMAGING
• Skeletal survey is initial diagnostic imaging evaluation
○ Diffuse osteopenia and multiple lytic lesions
• NECT (bone algorithm)
○ Multifocal lytic lesions
○ Vertebral destruction and fractures
• MR patterns
○ Normal
○ Focal marrow involvement
○ Diffuse marrow involvement
○ Variegated pattern (micronodular, salt and pepper
appearance)
• Compression fractures with variable central canal
narrowing
• FDG PET
○ Identifies active multiple myeloma; useful in monitoring
treatment response
• FSE T2 with fat saturation, STIR, or T1WI C+ with fat
suppression increase lesion conspicuity
TOP DIFFERENTIAL DIAGNOSES
• Metastases
• Leukemia/lymphoma
• Osteoporosis
• Hyperplastic marrow
CLINICAL ISSUES
• Bone pain: 75%
• Marrow failure: Anemia, infection
• Renal insufficiency/failure
• M protein (monoclonal immunoglobulin): Blood ± urine
• Hypercalcemia
• Treatment: Supportive care, local radiation, chemotherapy,
transplants
(Left) Sagittal STIR MR
illustrates innumerable
hyperintense foci in the
cervical and thoracic vertebral
bodies ſt and spinous
processes st. Advanced
imaging is recommended in
those with normal radiographs
and monoclonal gammopathy
or a solitary plasmacytoma.
(Right) Sagittal T1WI MR
demonstrates heterogeneous
marrow signal due to
countless hypointense lesions
in the cervical and thoracic
vertebral bodies st and the
spinous processes . There is
a benign vertebral
hemangioma at T8 ſt.
(Left) Sagittal T2WI MR (left)
shows marrow heterogeneity
with a hypointense L3
vertebral body lesion st,
barely detectable on lateral
MR (right) . Radiographs
allow identification of only
those lesions with advanced
destruction affecting at
minimum 30% of trabecular
bone. (Right) Sagittal CT
shows diffuse osteopenia with
multiple vertebral body lytic
lesions ſt. Pathologic
compression fractures are
seen in multiple midthoracic
vertebral bodies. Compression
fractures can cause variable
central canal narrowing.
281
Neuroblastic Tumor
KEY FACTS
TERMINOLOGY
• Neuroblastoma (NB), ganglioneuroblastoma (GNB), and
ganglioneuroma (GN) are tumors of varying maturity
derived from primordial neural crest cells that form
sympathetic nervous system
IMAGING
• Abdominal (adrenal, paraspinal ganglia) > thoracic > pelvic >
cervical
• Radiographs
○ Widened paraspinal soft tissues ± scoliosis
○ ± stippled abdominal or mediastinal calcifications
• CT
Neoplasms, Cysts, and Other Masses
○ Widened neural foramina & intercostal spaces, pedicle
erosion, adjacent rib splaying (GN, GNB) or destruction
(NB)
• MR for diagnosis, presurgical planning
○ T1WI: Hypo-/isointense paraspinal mass
○ T2WI: Hypo-/hyperintense paraspinal mass
(Left) Coronal graphic depicts
a vascular paraspinal
neuroblastoma (NB)
originating on the right with
spread through the contiguous
neural foramina across the
midline to the left (stage 3).
(Right) Sagittal T1 C+ MR
illustrate a large mass
involving the entire lumbar
epidural space with marked
compression of the thecal sac
and compression of the conus
medullaris ſt. This NB mass
enhances avidly and
homogeneously. The mass
extends from the
retroperitoneum through
multiple lumbar foramina st.
○ ± epidural extension through neural foramina
○ Variable enhancement ± internal hemorrhage, necrosis
• MIBG for NB staging, posttreatment surveillance
TOP DIFFERENTIAL DIAGNOSES
• Ewing sarcoma
• Vertebral metastasis
• Lymphoma
CLINICAL ISSUES
• Abdominal mass/pain, bone pain, fatigue, weight loss,
blanching subcutaneous nodules
• Paraparesis/paraplegia (cord compression)
• NB 5-year survival ~ 83% for infants, 55% for children 1-5
years, and 40% for children > 5 years
DIAGNOSTIC CHECKLIST
• Critical to determine whether tumor extends into spinal
canal or neural foramina
282
(Left) Axial STIR MR displays a
paraspinal NB transgressing
across the neural foramen into
epidural space ſt & displacing
the thecal sac medially. Report
whether the tumor extends
into the spinal canal or neural
foramina, as epidural
extension complicates surgical
management. (Right) Sagittal
T1 C+ FS MR shows an avidly
enhancing posterosuperior
mediastinal mass st. This
ganglioneuroblastoma directly
contacts adjacent vertebral
bodies & ribs without
abnormal marrow
enhancement to imply
invasion.
Schwannoma
KEY FACTS
Neoplasms, Cysts, and Other Masses
TERMINOLOGY
• Neurinoma, neurilemmoma (outdated terms)
• Neoplasm of nerve sheath (Schwann cells)
IMAGING
• 70-75% intradural extramedullary
○ Most common intradural extramedullary mass
• 15% completely extradural
• 15% transforaminal, dumbbell masses
• Bone remodeling due to large intraspinal or intraforaminal
tumor common
• Cystic change common
• Calcifications, hemorrhage are rare
• Uniform, heterogeneous, or peripheral enhancement
patterns
TOP DIFFERENTIAL DIAGNOSES
• Neurofibroma (NF)
• Perineural root sleeve cyst
• Myxopapillary ependymoma
• Meningioma
• Leptomeningeal carcinomatosis
• Neuroblastic tumor
PATHOLOGY
• WHO grade I
• NF2: Loss of tumor suppressor (merlin) on chromosome 22
• Sporadic schwannoma more common than NF2
○ Inactivating mutations of merlin gene in ~ 60%
CLINICAL ISSUES
• Pain, weakness, paresthesias most common clinical findings
• Typically solitary unless part of inherited tumor syndrome
DIAGNOSTIC CHECKLIST
• Schwannoma most likely when solitary enhancing
dumbbell-shaped spinal lesion present
(Left) Axial graphic portrays a
right-sided dumbbell-shaped
spinal nerve root schwannoma
enlarging the neural foramen
and compressing the spinal
cord. Both intra- and
extradural components
(dumbbell) are present. (Right)
Axial CECT shows a hypodense
transforaminal mass ſt
enlarging the right neural
foramen. The intraspinal
component effaces the thecal
sac st and causes canal
stenosis.
(Left) Axial NECT
demonstrates a large soft
tissue mass ſt enlarging the
right L1-2 neural foramen.
There is conspicuous
scalloping of the vertebral
body . The full intraspinal
component is not seen. (Right)
Axial T2WI MR in the same
patient reveals heterogeneous
mass ſt signal intensity.
Foraminal enlargement and
vertebral body scalloping are
again seen . The extradural
component within the spinal
canal is causing significant
canal stenosis .
283
Meningioma
KEY FACTS
IMAGING
• Intradural extramedullary mass
• Thoracic (80%) > cervical (16%) > lumbar (4%)
• Typically round or ovoid (en globe)
○ en plaque variety pancaked or flat along dura
• Broad dural attachment (more often ventral or
ventrolateral)
• Strong homogeneous enhancement
• Calcification in 1-5%
• No bony remodeling or hyperostosis in spine
TOP DIFFERENTIAL DIAGNOSES
Neoplasms, Cysts, and Other Masses
• Schwannoma
• Ependymoma, myxopapillary
• Lymphoma
• Intradural metastases
PATHOLOGY
• Arise from arachnoid cap cells
(Left) Sagittal T2 MR shows a
ventral, dural-based mass at
C5-6 level ſt compressing and
displacing the cervical cord
posteriorly . An intradural
extramedullary mass displaces
the cord with concomitant
widening of the CSF spaces
adjacent to the mass st. This
T2 image shows CSF flow
dephasing with signal loss
adjacent to the mass .
(Right) Sagittal T1W C+ MR
shows a large intradural
extramedullary meningioma
with prominent enhancement.
Note the broad ventral dural
attachment ſt and severe
cord compression .
• > 95% WHO grade I
• Most solitary, sporadic
○ Almost all have 22q12 abnormalities
• Syndromes
○ Multiple lesions with neurofibromatosis type 2
– Intradural extramedullary meningiomas and
schwannomas
– Intramedullary ependymomas
○ Familial clear cell meningioma syndrome
○ Multiple meningiomatosis
CLINICAL ISSUES
• Peak incidence: 5th-6th decades
• > 80% female
• 2nd most common intradural extramedullary tumor
• Sensory and motor deficits (84%)
• Gait disturbances (83%)
• Local pain (47%)
284
(Left) Sagittal reconstructed
bone CT shows a sizable,
partially calcified meningioma
ſt arising on the ventral
margin of the canal at the
level of C2. The mass results in
significant canal stenosis and
cord compression. (Right) Axial
T1 C+ MR shows a large
cervical meningioma with a
ventral, dural-based
attachment at the C5-6 level
ſt severely compressing and
displacing the cervical cord
posteriorly and to the right
.
Solitary Fibrous Tumor/Hemangiopericytoma
KEY FACTS
Neoplasms, Cysts, and Other Masses
TERMINOLOGY
• Hemangiopericytoma (HPC)
• Solitary fibrous tumor (SFT)
IMAGING
• HPC may occur in any compartment of spine except
intramedullary
• Circumscribed, multilobulated morphology
• Vivid enhancement characteristically seen
TOP DIFFERENTIAL DIAGNOSES
• Meningioma
• Schwannoma
• Chordoma and other primary bone malignancies
• Aggressive hemangioma
• Vascular metastases
• Angiosarcoma
PATHOLOGY
• Hypervascular neoplasm; currently considered
mesenchymal tumor of unknown etiology
• Hemangiopericytoma considered part of SFT spectrum
("cellular" or "malignant" SFT)
• Majority of HPC likely classified within cellular end of SFT
spectrum
CLINICAL ISSUES
• Natural history
○ Progressive growth
○ Local recurrence
○ Metastases
• Primary treatment is surgical resection
• Radiation therapy as adjuvant to surgery, primary
treatment for unresectable tumor
○ Limited benefit of chemotherapy
• Continued radiographic surveillance for late recurrence
(Left) Axial T1WI C+ MR shows
a lobulated, avidly enhancing
paraspinous mass with
internal flow voids due to
hypervascularity. There is
transforaminal extension with
an intradural component st.
Bony invasion of the lamina
ſt is also seen. (Right) Axial
NECT shows a dorsal soft
tissue mass ſt destroying the
lamina and extending into the
dorsal epidural space .
(Left) Sagittal T1WI C+ MR
shows an avidly enhancing
mass invading the dorsal
elements and adjacent soft
tissues at 3 levels of the
thoracic spine. An intraspinal
component st is not clearly in
the epidural or intradural
extramedullary
compartments. (Right) Axial
T1WI C+ MR shows an avidly
enhancing osseous metastasis
from an intracranial
hemangiopericytoma to the
C3 vertebral body.
285
Neurofibroma
KEY FACTS
TERMINOLOGY
• Neoplasm containing Schwann cells, fibroblasts, myxoid
material, and peripheral nerve fibers
IMAGING
• Locations
○ Extradural/paraspinal; intradural extramedullary
• Variable involvement of spinal root, neural plexus,
peripheral nerve, or end organs
• Size varies from small circumscribed mass to large
plexiform neurofibromatosis (NF) involving multiple body
compartments
• Plexiform neurofibroma pathognomonic for NF1
Neoplasms, Cysts, and Other Masses
• Target sign on T2WI suggestive but not pathognomonic for
NF
• FDG avidity suggests malignant degeneration
TOP DIFFERENTIAL DIAGNOSES
• Schwannoma
(Left) Axial graphic portrays
bilateral lobulated plexiform
neurofibromas in
neurofibromatosis type 1
(NF1). There is erosion of the
left pedicle by the tumor.
(Right) Coronal STIR shows
extensive, bulky plexiform
neurofibromas st involving
bilateral cervical nerve roots.
This appearance is virtually
pathognomonic for NF1.
• Spinal meningioma
• Perineural root sleeve cyst
• Chronic interstitial demyelinating polyneuropathy
• Malignant nerve sheath tumors
PATHOLOGY
• Neoplastic Schwann cells + fibroblasts
• Tumor, nerve fascicles intermixed (presence of axons
characteristic of NF)
• 90% of neurofibromas are sporadic
CLINICAL ISSUES
• Malignant transformation of plexiform NF to malignant
peripheral nerve sheath tumor (MPNST) in 10% of NF1
patients
DIAGNOSTIC CHECKLIST
• Rapidly growing NF or atypical pain concerning for
malignant transformation to MPNST
• Solitary spinal lesion more likely schwannoma than NF
286
(Left) Axial T1WI C+ MR shows
an oblong enhancing mass
extending through the left C45 neural foramen.
Longstanding remodeling has
enlarged the neural foramen
ſt. The intraspinal component
of the mass causes canal
stenosis and cord compression
st. (Right) Axial T2WI MR in
the same patient shows
central areas of T2
hypointense signal st with a
peripheral rim of
hyperintensity. This target sign
is not specific but is seen more
commonly with neurofibroma
than with schwannoma.
Malignant Nerve Sheath Tumors
KEY FACTS
Neoplasms, Cysts, and Other Masses
TERMINOLOGY
• Malignant peripheral nerve sheath tumor (MPNST)
• Soft tissue sarcoma that arises from or differentiates
toward cells of peripheral nerve sheath
IMAGING
• CT and MR: Large, infiltrative, often hemorrhagic, soft
tissue mass
○ Arise spontaneously or from malignant degeneration
within preexisting neurofibroma
○ Heterogeneous areas correspond to hemorrhage,
calcifications, and necrosis
○ Bony erosion, destruction
○ Marked enhancement
○ Indistinct margins
• FDG PET diagnoses neurofibromatosis type 1 (NF1)associated MPNST with sensitivity of 89% and specificity of
95%
○ CT significantly less accurate than FDG PET for
characterizing tumors as malignant or benign
TOP DIFFERENTIAL DIAGNOSES
• Benign peripheral nerve sheath tumor, other soft tissue
sarcoma, hematoma
PATHOLOGY
• Incidence in general population: 0.001%
• 50-60% associated with NF1
CLINICAL ISSUES
• Enlarging soft tissue mass
• Local or radicular pain, sensory disturbance
• Paraparesis
• Sporadic MPNST presents in 4th decade (mean age: 39.7
years)
• If associated with NF1: 26-42 years (mean age: 28.7)
• Local recurrence: 26-65%; metastases: 20-65%
• Worse prognosis with NF1
(Left) Malignant peripheral
nerve sheath tumors (MPNSTs)
often arise from a major nerve
trunk, such as this sciatic nerve
tumor forming a fusiform,
lobulated, intraneural mass
ſt. These tumors can extend
along a nerve to form satellite
nodules . (Right) Coronal T1
C+ FS MR shows a large
heterogeneously enhancing
mass in the right pelvis
extending through a sacral
foramen into the spinal canal
st and through the sciatic
notch into the buttocks.
(Left) Axial T1 C+ MR shows
an enhancing dumbbellshaped mass involving the
cervical spine. There is
infiltration of the
paravertebral soft tissues .
Tumor invades the cord
parenchyma within the
spinal canal. (Right) Axial T1
C+ MR in a patient with
neurofibromatosis type 1
shows neurofibromas in the
left neural foramen ſt and
within the spinal canal st. The
enhancing tumor in the right
sacrospinalis muscle has
slightly indistinct margins and
was pathologically proven to
be a MPNST.
287
Metastases, CSF Disseminated
KEY FACTS
TERMINOLOGY
• Spread of malignant tumor through subarachnoid spaces of
brain and spinal cord
IMAGING
• Smooth or nodular enhancement along cord, cauda equina
• Located at any point along CSF pathway
• 4 basic patterns
○ Solitary focal mass at bottom of thecal sac or along cord
surface
○ Diffuse, thin, sheet-like coating of cord/roots
(carcinomatous meningitis)
Neoplasms, Cysts, and Other Masses
○ Rope-like thickening of cauda equina
○ Multifocal discrete nodules along cord/roots
TOP DIFFERENTIAL DIAGNOSES
• Multifocal primary tumor
• Pyogenic meningitis
• Granulomatous meningitis
(Left) Sagittal T1WI C+ MR
shows a mixed nodular and
smooth st leptomeningeal
tumor along the distal cord
and conus medullaris due to
drop metastases from
glioblastoma multiforme.
(Right) Sagittal T2WI MR
shows multiple nodular low
signal intensity rounded drop
metastases st involving roots
of the cauda equina, spreading
from an intracranial
glioblastoma multiforme.
• Chemical meningitis
• Recent lumbar puncture
• Congenital hypertrophic polyradiculoneuropathies
• Thick nerve roots/cauda equina
PATHOLOGY
• Hematogenous dissemination from solid tumors (1-5%)
• Drop metastases from patients with primary CNS tumor (12%)
• Leukemia, lymphoma (5-15%)
• False-negative CSF cytology in up to 40%
CLINICAL ISSUES
• Typically seen in advanced cancer cases
• Prevalence increasing as cancer patients are living longer
• Median patient survival of 3-6 months with treatment, 4-6
weeks without
DIAGNOSTIC CHECKLIST
• MR more sensitive than CSF cytology
288
(Left) Sagittal T1WI C+ MR
shows bulky nodular drop
metastases from a pineal
germinoma along the conus
and cauda equina. (Right)
Sagittal T1WI C+ MR shows
CSF metastasis from small cell
lung carcinoma with a bulky
enhancing dural mass
compressing the
cervicomedullary junction
and linear enhancing
metastases along the dorsal
pial surface of the cord from
C7 caudally . An additional
enhancing focus is seen in the
region of the gracile tubercle
st.
Paraganglioma
KEY FACTS
Neoplasms, Cysts, and Other Masses
TERMINOLOGY
• Synonyms: Glomus tumor, extraadrenal
pheochromocytoma, chemodectoma
IMAGING
• Hypervascular, intensely enhancing intradural
extramedullary mass
○ Prominent flow voids due to enlarged draining veins
○ ± cystic areas, hemosiderin from prior hemorrhage
• Large tumors may show osseous remodeling
• Malignant paraganglioma may → osteolytic spinal
metastases
• Usually positive on I-123 or I-131 MIBG scan
TOP DIFFERENTIAL DIAGNOSES
• Myxopapillary ependymoma
○ Usually indistinguishable from paraganglioma on
imaging studies
• Schwannoma
○ Rarely associated with dilated vessels
• Meningioma
• Metastasis
• Hemangioblastoma of filum terminale
PATHOLOGY
• WHO grade I
• Slow-growing, generally benign behavior
CLINICAL ISSUES
• Presents with back pain/radiculopathy (often chronic)
• Prognosis varies with tumor location but generally
excellent
DIAGNOSTIC CHECKLIST
• MR imaging features nonspecific
• Ependymoma and schwannoma much more common than
paraganglioma
(Left) Sagittal T1WI C+ MR of
the lumbar spine
demonstrates a large,
homogeneously enhancing
intradural mass ſt filling the
lumbar canal from T12
through L4. (Right) Axial T1WI
C+ MR of the same patient
shows posterior vertebral
body scalloping ſt,
neuroforaminal enlargement
, and thinning of the left
pedicle st secondary to
longstanding osseous
remodeling. Recognition of
these osseous changes
confirms the slow-growing
nature of the tumor.
(Left) Sagittal T2WI MR
reveals a heterogeneous mass
within the caudal thecal sac
demonstrating a cystic area
superiorly and a prominent
flow void ſt indicating
hypervascularity. A small
amount of hemorrhage layers
dependently st in the caudal
tip of the thecal sac. (Right)
Sagittal T1WI C+ MR in the
same patient confirms diffuse,
mildly heterogeneous
enhancement throughout the
tumor. Mild scalloping of the
posterior L5 vertebral body is
also noted, indicating chronic
remodeling ſt.
289