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Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_6009_Библиотеки_им_академика_М_И_Перельмана.pdf
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Osteoblastoma
KEY FACTS
TERMINOLOGY
• Benign tumor that forms osteoid
• Differentiated grossly from osteoid osteoma by larger size (> 1.5 cm)
IMAGING
• 40% of osteoblastomas occur in spine
• Well-circumscribed, expansile lesion of neural arch ○ Frequent extension into vertebral body ○ Narrow zone of transition, sclerotic rim
• Periosteal inflammatory response of adjacent ribs, pleural thickening, effusion
• Peritumoral edema (flare phenomenon)
Neoplasms, Cysts, and Other Masses
○ On MR, edema enhances, obscures tumor margins, and
mimics malignancy
TOP DIFFERENTIAL DIAGNOSES
• Osteoid osteoma
• Aneurysmal bone cyst
(Left) Axial graphic shows expansile, highly vascular osteoblastoma (OB) ſt arising in the right lamina and impinging on the exiting nerve root. (Right) Axial bone CT in the same patient shows an expansile mass ſt containing thin, irregular bone trabeculae ﬇ that is characteristic of OB. Although cortical breakthrough is present, the zone of transition to adjacent bone is narrow and sclerotic.
• Metastasis
• Osteogenic sarcoma
• Chordoma
• Infection
PATHOLOGY
• Tumor prostaglandins release causes extensive peritumoral edema
CLINICAL ISSUES
• 90% in 2nd-3rd decades of life
• Dull, localized pain
• Painful scoliosis
• Neurologic symptoms due to compression of cord, nerve roots
DIAGNOSTIC CHECKLIST
• May be occult on radiographs; consider MR in young patients with painful scoliosis
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(Left) Axial T2WI MR shows an osteoblastoma ﬇of L5 with characteristic heterogeneous signal intensity. The bony matrix is low signal. There is edema in the adjacent vertebral body. (Right) Sagittal bone CT shows expansile lower thoracic OB
extending from the lamina
into the spinal canal. The lesion is sharply circumscribed and bilobed, involving 2 adjacent levels.
Aneurysmal Bone Cyst
KEY FACTS
Neoplasms, Cysts, and Other Masses
IMAGING
• 10-30% of aneurysmal bone cysts (ABCs) occur in spine and sacrum
• Centered in neural arch, extends into vertebral body
• Balloon-like expansile remodeling of bone ○ Thinned, "eggshell" cortex ○ Focal cortical destruction common
• Absent pedicle sign: Expansion of pedicle results in loss of pedicle contour on AP radiographs
• Contains multiple round cysts with fluid-fluid levels ○ Caused by hemorrhage, blood product sedimentation
• Blood-filled cysts separated by septa of varying thickness ○ Periphery and septa enhance ○ Solid ABC variant enhances diffusely
• Calcified tumor matrix absent
• Narrow, nonsclerotic zone of transition with adjacent bone
• CT best to differentiate from telangiectatic osteogenic sarcoma
○ Narrow zone of transition in ABC ○ Absence of infiltration into surrounding soft tissues
• MR shows epidural extent, cord compromise
TOP DIFFERENTIAL DIAGNOSES
• Osteoblastoma
• Telangiectatic osteogenic sarcoma
• Metastases
• Giant cell tumor
• Tarlov cyst
PATHOLOGY
• Now considered true neoplasm since cytogenetic abnormalities found in > 50%
• Translocation between chromosomes 16 and 17
CLINICAL ISSUES
• Young patient with back pain of insidious onset
(Left) Axial graphic shows an aneurysmal bone cyst with an expansile, multicystic mass in the posterior vertebral body and pedicle extending into the epidural space. Fluid-fluid levels are characteristic. (Right) Anteroposterior radiograph shows an aneurysmal bone cyst of T12 with an absent pedicle sign ﬇. The superior and inferior extent of tumor is shown by bone destruction ﬈, which involved the vertebral body as well as the neural arch.
(Left) Axial T2WI MR shows an aneurysmal bone cyst of T12 with multiple fluid-fluid levels due to layering blood products. The zone of transition to normal bone is narrow and sclerotic. There is cortical breakthrough as well as extension into the spinal canal compressing the spinal cord. (Right) Axial NECT shows an extensive aneurysmal bone cyst involving the entire C2 body with body ſt and posterior element ﬇ expansion. The bony expansion is the typical soap bubble type with intact cortical margins.
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Giant Cell Tumor
KEY FACTS
TERMINOLOGY
• Giant cell tumor (GCT)
• Locally aggressive neoplasm composed of osteoclast-like giant cells
IMAGING
• 3% of all GCTs occur in spine
• 4% of all GCTs occur in sacrum
• Lytic, expansile lesion of vertebral body or sacrum
• Narrow zone of transition, margin not sclerotic
• Matrix absent but may have residual bone trabeculae
• May have cortical breakthrough
Neoplasms, Cysts, and Other Masses
• Heterogeneous contrast enhancement
• Nonenhancing areas of necrosis often present
• May be associated with aneurysmal bone cyst component
TOP DIFFERENTIAL DIAGNOSES
• Metastasis
• Myeloma
(Left) AP radiograph shows a giant cell tumor (GCT) st of the sacrum. Lytic lesions in this region are difficult to see on radiographs and may be mistaken for bowel gas. Arcuate lines are a useful landmark. In this case, the S1 ﬇ & S2 ſt arcuate lines are visible on the normal right side but destroyed on the left. (Right) Axial NECT shows a nearly complete cortical rim around a large sacral mass. There is a small region of cortical breakthrough ſt & extension of tumor from the sacrum into right iliac wing across the sacroiliac joint ﬇.
• Chordoma
• Osteogenic sarcoma
CLINICAL ISSUES
• Back pain of insidious onset, greatest at night
• Locally aggressive: 12-50% recurrence rate
• Curettage alone associated with high rate of recurrence ○ Curettage defect filled with methylmethacrylate or bone
graft
○ Denosumab: Human monoclonal antibody to RANKL
– In one study, 86% of patients had tumor response
DIAGNOSTIC CHECKLIST
• Most likely causes of solitary sacral mass in adult patient are GCTs, chordoma, and plasmacytoma
• Malignant GCTs rare, difficult to distinguish from typical GCT ○ Zone of transition usually less well defined
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(Left) Lateral radiograph shows a lytic lesion ſt of L1. The lesion is sharply demarcated with a nonsclerotic margin. There is probable cortical breakthrough inferiorly ﬇. This constellation of findings is consistent with GCT and other intermediate aggressivity lesions, such as plasmacytoma, chordoma (rare at this site), or some metastases. (Right) Axial CECT in the same patient shows a heterogeneously enhancing mass with cortical breakthrough st.
Osteochondroma
KEY FACTS
Neoplasms, Cysts, and Other Masses
TERMINOLOGY
• Osteochondroma (OC)
• Osteocartilaginous exostosis, exostosis
• Cartilage-capped osseous growth contiguous with parent bone
IMAGING
• Sessile or pedunculated osseous cauliflower-like lesion
• Continuity of bony cortex and medullary space between lesion and underlying bone
• May see chondroid calcifications in cartilage cap
• Center follows normal marrow signal on MR
• Cartilage cap of similar intensity to cord on T1WI, hyperintense on T2WI
TOP DIFFERENTIAL DIAGNOSES
• Chondrosarcoma
• Osteoblastoma
• Aneurysmal bone cyst
• Tumoral calcinosis
• Enthesopathy
PATHOLOGY
• Idiopathic, trauma, perichondrial ring deficiency
• Radiation-induced OC
• Syndromic: Hereditary multiple exostoses (HME)
• Vertebral OC rare; 1-5% of sporadic OC, 1-9% OC in HME
CLINICAL ISSUES
• Often asymptomatic; incidental diagnosis on radiography
• Palpable mass
• Mechanical impingement of joint, muscle
• Cord compression, radiculopathy unusual
• Peak age = 10-30 years
DIAGNOSTIC CHECKLIST
• Multiplicity → consider HME
• Cartilage cap > 1.5 cm in adults raises concern for malignant transformation (chondrosarcoma)
(Left) Axial graphic of the cervical spine demonstrates a typical osteochondroma (exostosis) ſt protruding into the spinal canal causing canal stenosis and cord compression. (Right) Axial NECT shows a large osseous excrescence projecting ventrally from the right lateral mass of C2 ſt. There is ossification of the cartilaginous cap ﬇.
(Left) Axial bone CT shows a pedunculated osseous lesion ſt extending into the canal from the right pedicle with cortical and medullary continuity. There is resulting canal stenosis. The right pedicle and superior facet are irregularly enlarged ﬇ due to the sessile component of the osteochondroma. (Right) Axial T2WI FS MR in the same patient shows the medullary continuity of the osseous mass with the right pedicle ſt and a hyperintense cartilaginous cap st. The spinal cord ﬇ is displaced and compressed.
273
Chondrosarcoma
KEY FACTS
TERMINOLOGY
• Malignant tumor of connective tissue characterized by formation of cartilage matrix by tumor cells
IMAGING
• Lytic mass with chondroid matrix ○ Higher grade lesions tend to have larger areas without
calcifications
• Lobular morphology often seen
• Enhancing periphery and internal septa
• May penetrate cortex with epidural or paravertebral extension
• Thoracic most commonly involved spine segment
Neoplasms, Cysts, and Other Masses
○ Both posterior elements and body (45%) ○ Posterior elements (40%)
• Variable size, typically large at presentation
TOP DIFFERENTIAL DIAGNOSES
• Metastases
(Left) Sagittal graphic of the lumbosacral junction shows a large soft tissue mass centered within the sacrum. This tumor produces bone destruction with presacral and epidural extension. (Right) Axial T1WI C+ MR shows a large vertebral mass with paraspinal st and epidural ﬇ extension. There is irregular peripheral enhancement with some faintly enhancing central septations ſt.
• Plasmacytoma
• Lymphoma
• Osteosarcoma
• Malignant fibrous histiocytoma
PATHOLOGY
• Lobulated tumor composed of translucent hyaline nodules (resemble normal cartilage)
• May be primary or secondary
CLINICAL ISSUES
• Palpable mass
• Localized pain, typically long duration of symptoms
• Neurologic symptoms in 45% of vertebral chondrosarcoma ○ Weakness, paresthesias, paralysis
• Age at presentation: 20-90 years; peak: 40-60 years
DIAGNOSTIC CHECKLIST
• Enlarging or painful enchondroma or osteochondroma suspicious for sarcomatous transformation
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(Left) Axial unenhanced bone CT shows a large mass arising from the neural arch, containing ringed and stippled calcifications st, the typical appearance of a chondroid tumor matrix. (Right) Left paravertebral sagittal T2WI in the same patient shows a large, lobulated mass st and a predominately hyperintense signal with thin, hypointense signal around the periphery of the lobules due to calcification. This is the typical MR appearance of a chondroid neoplasm.
Osteosarcoma
KEY FACTS
Neoplasms, Cysts, and Other Masses
TERMINOLOGY
• Sarcoma containing osteoid matrix produced directly by malignant cells
IMAGING
• Permeative or moth-eaten appearance
• Cortical breakthrough
• Bone sclerosis due to production of immature bone
• Wide zone of transition
• Discontinuous periosteal reaction, usually multilaminar
• Soft tissue mass [fluid-fluid levels seen in telangiectatic osteogenic sarcoma (OGS)]
TOP DIFFERENTIAL DIAGNOSES
• Sclerotic metastasis
• Osteoblastoma
• Aneurysmal bone cyst
• Chordoma
• Osteomyelitis
• Ewing sarcoma
• Chondrosarcoma
• Lymphoma
• Malignant giant cell tumor
PATHOLOGY
• 4% of all primary OGS occurs in spine and sacrum
• Majority of OGS are of unknown etiology = primary OGS
• Association with retinoblastoma (Rb gene mutation)
CLINICAL ISSUES
• Insidious onset of back pain, greatest at night
• Neurologic symptoms including radicular pain, weakness
• 3% of 10-year survivors of all OGS develop 2nd malignancy
DIAGNOSTIC CHECKLIST
• CT scan best method for evaluation of tumor matrix, zone of transition
• All telangiectatic OGS are lytic on radiographs and CT but not all lytic OGS are telangiectatic
(Left) Axial graphic shows secondary osteosarcoma arising in a Pagetic vertebral body, destroying the cortex and invading adjacent soft tissues. The soft tissue mass in osteosarcoma usually contains ossification. (Right) Axial CECT shows a very aggressive­appearing soft tissue mass ſt that both produces a bony matrix and has areas of bone destruction st. There is a large epidural soft tissue component with cord compression ﬇.
(Left) Axial T2 FS MR shows a large soft tissue mass ſt, which has a relatively low T2 signal that is consistent with a highly cellular tumor. The mass has epidural extension with the cord displaced to the left ﬇. The right vertebral artery is encased by tumor st. (Right) Postcontrast T1W MR shows diffuse enhancement of the mass with prevertebral ſt and paravertebral extension as well as extensive epidural extension ﬇ and cord compression.
275
Chordoma
KEY FACTS
TERMINOLOGY
• Malignant tumor arising from notochord remnants
IMAGING
• Sacrococcygeal > sphenooccipital > mobile spine
• Osseous destruction with disproportionately large soft tissue mass
• Circumscribed, scalloped, or sclerotic bony margins
• Amorphous intratumoral calcifications
• Hyperintense to disc on T2WI with multiple septa
• May extend into disc space, involve 2 or more adjacent vertebrae
Neoplasms, Cysts, and Other Masses
TOP DIFFERENTIAL DIAGNOSES
• Chondrosarcoma
• Giant cell tumor
• Metastases
• Plasmacytoma
(Left) Sagittal graphic of the cervical spine shows an extradural soft tissue mass with the epicenter in the posterior aspect of the C2 body causing bone destruction and epidural extension with cord compression. (Right) Sagittal T2WI MR shows a hyperintense mass involving the C3 body with extensive epidural extension and cord compression.
PATHOLOGY
• Lobulated, soft, grayish gelatinous (myxoid) mass
• Areas of calcification and hemorrhage
CLINICAL ISSUES
• Skull base ○ Diplopia (CNV2 palsy, most common) ○ Headaches ○ Facial pain
• Mobile spine: Cord compression, radiculopathy (50%)
• Sacral: Altered sacrogluteal sensation
• Symptoms tend to be longstanding (4-24 months)
• Recurrence common
• 5-year survival: 50-68%; 10-year survival: 28-40%
DIAGNOSTIC CHECKLIST
• High signal intensity mass on T2WI with septations, little enhancement is chordoma or chondrosarcoma
276
(Left) Sagittal T2WI show a large, well-defined hyperintense soft tissue mass engulfing the distal sacrum and coccyx with extension into the presacral space and dorsal soft tissues st. (Right) Sagittal T2WI FS MR shows a posterior mediastinal mass with involvement of 4 adjacent thoracic vertebral bodies. The mass is hyperintense with fine septations st. There is a pathologic compression fracture of the top vertebra with ventral epidural tumor
. Note the scalloped
anterior margins ſt.
Ewing Sarcoma
KEY FACTS
Neoplasms, Cysts, and Other Masses
TERMINOLOGY
• Ewing sarcoma family of tumors
• Aggressive childhood cancer, which includes Ewing sarcoma, Askin tumor, and peripheral primitive neuroectodermal tumor
IMAGING
• Spine: 5% of all Ewing tumors ○ Sacrum most common spinal site ○ Spreads along peripheral nerves
• May originate in epidural or paraspinous soft tissues
• Permeative/moth-eaten bone destruction ○ 5% sclerotic (represents host reaction, not tumor matrix) ○ Areas of central necrosis common ○ "Percolates" through tiny perforations in cortex
• Lower signal intensity than disc or muscle on T1WI ○ May be isointense to red marrow on T2WI ○ Intermediate to high signal intensity on STIR
• MR best shows involvement of adjacent bones and soft tissues, which can be underestimated on CT scan ○ Heterogeneous enhancement with gadolinium
• CT useful to confirm absence of tumor matrix, distinguish from osteogenic sarcoma
TOP DIFFERENTIAL DIAGNOSES
• Primitive neuroectodermal tumor
• Langerhans cell histiocytosis
• Osteosarcoma
• Metastatic neuroblastoma
• Osteomyelitis
CLINICAL ISSUES
• 90% of all Ewing sarcoma patients present before 20 years of age
• Fever, leukocytosis, elevated ESR (simulating osteomyelitis)
• Spine and sacral lesions often present in older patients than peripheral Ewing sarcoma
(Left) Sagittal graphic shows a vertebral body replaced by Ewing sarcoma resulting in mild collapse. Tumor extends into adjacent soft tissues through small perforations in the bone cortex. (Right) Axial CECT shows a large paraspinal soft tissue mass of Ewing sarcoma engulfing the left psoas muscle ſt with extension into the dorsal musculature ﬇, destruction of left side of lumbar body ﬉, and epidural extension st.
(Left) Axial T2WI MR shows Ewing sarcoma involving the paraspinal muscles ſt and extending into the epidural space st, compressing and displacing the thecal sac to the right. Infiltration of the vertebral body is poorly defined on this sequence. (Right) Axial T1WI C+ MR shows diffuse heterogeneous enhancement of the tumor involving the left side of lumbar body ﬇, epidural space ſt, and paravertebral and dorsal muscles.
277
Lymphoma
KEY FACTS
TERMINOLOGY
• Lymphoreticular neoplasms with myriad of specific diseases and cellular differentiation
IMAGING
• Multiple types with variable imaging manifestations
• Epidural lymphoma: Thoracic > lumbar > cervical ○ Enhancing epidural mass ± vertebral involvement
• Osseous lymphoma: Long bones > spine ○ Bone destruction (ivory vertebra, rare), vertebra plana
• Lymphomatous leptomeningitis ○ Smooth/nodular pial enhancement
Neoplasms, Cysts, and Other Masses
• Intramedullary lymphoma: Cervical > thoracic > lumbar ○ Poorly defined, enhancing mass
• Secondary > primary involvement
• Extradural > intradural > intramedullary
• FDG PET useful for staging, monitoring treatment response, predicting treatment outcomes, and risk stratifying lymphoma patients
(Left) Sagittal T2WI MR demonstrates a discrete hypointense mass in the posterior epidural space with a cap of epidural fat st. The spinal cord is displaced anteriorly ſt. Lymphoma is the most common malignancy of the epidural space. (Right) Sagittal T2WI MR (left) shows an amorphous hypointense tumor mass insinuating along the cauda equina ſt. Sagittal T1WI C+ MR (right) reveals enhancing lymphoma within the leptomeninges surrounding the distal conus and involving the nerve roots diffusely ﬇.
PATHOLOGY
• Non-Hodgkin lymphoma (NHL) > > Hodgkin disease (HD); 80-90% are B cell ○ CNS lymphoma > 85% NHL (B cell > > > T cell)
• CNS lymphoma may be primary or secondary (hematogenous or direct geographic extension)
CLINICAL ISSUES
• Most common presenting symptom = back pain
• Intramedullary = myelopathy (weakness, numbness)
• Cord compression occurs in up to 5-10% of systemic lymphomas
• Generally poor prognosis for CNS lymphoma
• Markedly sensitive to chemotherapy/XRT
• Depressed humoral and cell-mediated immunity leads to opportunistic infections
• Treatment: XRT ± chemotherapy (markedly sensitive to chemotherapy/XRT); ± surgery
278
(Left) Coronal PET (left) shows FDG uptake in the left cervical lymph node mass ſt that was clinically evident. PET/CT fused image (right) shows abnormal activity at T11 ﬇. This is an example of osseous metastases from cervical Hodgkin lymphoma primary tumor. (Right) Sagittal CT (left) demonstrates a pathologic compression fracture of a midthoracic vertebral body ſt. Sagittal T1WI C+ MR (right) confirms abnormal marrow enhancement st and a ventral paraspinal mass ﬇.
Leukemia
KEY FACTS
Neoplasms, Cysts, and Other Masses
TERMINOLOGY
• Acute or chronic myeloid or lymphoid white blood cell neoplasia with spinal involvement as component of systemic disease
IMAGING
• Radiographs/CT ○ Diffuse osteopenia with multiple vertebral fractures ±
lytic spine lesions
○ Variable enhancing isodense soft tissue mass with
adjacent bone destruction
• MR ○ T1WI: Hypointense marrow and focal tumor masses ○ T2WI: Hyperintense marrow ± focal vertebral mass, cord
signal abnormality
○ T1WI C+: Abnormal enhancement of marrow, focal
lesion, or leptomeninges
TOP DIFFERENTIAL DIAGNOSES
• Metastases
• Lymphoma
• Ewing sarcoma
• Langerhans cell histiocytosis
CLINICAL ISSUES
• Localized or diffuse bone pain
• Symptomatic patients present with fever, ↑ ESR, hepatosplenomegaly, lymphadenopathy, joint effusions, petechial and retinal hemorrhage, anemia, frequent infections
DIAGNOSTIC CHECKLIST
• Marrow infiltration in child with osteoporosis raises suspicion for leukemia
• Consider leukemia in patient with unexplained compression fractures
(Left) Sagittal T1WI MR (left) and T2WI MR (right) of the cervical spine demonstrate diffuse abnormal hypointense signal intensity within the vertebra. Signal intensity is lower than the adjacent disc spaces. (Right) Sagittal T1WI MR (left) and T2WI MR (right) of the lumbar spine exhibit diffuse abnormal hypointense signal intensity within the vertebra as compared to the adjacent disc spaces. The signal abnormality may be a combination of tumor marrow infiltration and hyperplastic marrow secondary to anemia.
(Left) Coronal planar bone scan of the pelvis reveals abnormal increased uptake within the right sacral ala ſt in this case of focal leukemic metastasis to the axial skeleton. Bone scan may underestimate disease extent, especially in absence of significant cortical destruction. (Right) Axial T1WI MR in the same patient clarifies the right sacral alar metastatic lesion ſt. There is no definite cortical breakthrough.
279