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Spine 01ade6cf-e6ab-4b60-b19f-4132e55c4b3a 25 01/18/23 Craniovertebral Junction Soft Tissue Abnormality Spine, Differential Diagnosis, Craniovertebral Junction, Anatomically Based Differentials, Craniovertebral Junction Soft Tissue Abnormality Craniovertebral Junction Soft Tissue Abnormality | STATdx Craniovertebral Junction Soft Tissue Abnormality DDX true
Spine
Differential Diagnosis
Craniovertebral Junction
Anatomically Based Differentials
Craniovertebral Junction Soft Tissue Abnormality

title: "Craniovertebral Junction Soft Tissue Abnormality" docid: "3a8dca6c-3452-4cbb-ab83-5e9610d3f0f0" authors:

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ESSENTIAL INFORMATION

  • Key Differential Diagnosis Issues

    • Do intralesion calcifications represent arc-whorl intralesional calcifications (chondrosarcoma) or fragmented destroyed bone (chordoma, metastasis)?
    • Does patient have known primary neoplasm (metastasis), myeloma (plasmacytoma), or nasopharyngeal mass (nasopharyngeal carcinoma)?

  • Helpful Clues for Common Diagnoses

    • Rheumatoid Arthritis - Thickened and inflamed synovium called pannus - Never involves spine without involvement of hands &/or feet - Odontoid erosions, ligamentous laxity - C1-C2 instability in 33% of all rheumatoid arthritis (RA) patients - Neutral, flexion, and extension lateral radiographs performed for evaluation - High correlation to neurologic symptoms with distance 9 mm or more between C1-C2
    • Retroodontoid Pseudotumor - Increased soft tissue dorsal to odontoid secondary to C1-C2 osteoarthritis - Low-signal mass on T1 and T2 (fibrotic) - May cause cervicomedullary junction compression - Usually seen with altered biomechanics of lower cervical spine → surgical/congenital fusion - Mimics appearance of RA - Multiple other levels of degenerative disc disease
    • Calcium Pyrophosphate Dihydrate Deposition Disease - Pseudogout: Acute, painful episode due to calcium pyrophosphate dihydrate deposition disease (CPPD) - Crowned dens syndrome: CPPD of atlantooccipital joint causing periodic acute cervicooccipital pain with fever, neck stiffness, and laboratory inflammatory signs - Calcifications commonly in transverse and alar ligaments, posterior longitudinal ligament, ligamentum flavum
    • Osteomyelitis, C1-C2 - Infection starts as septic arthritis of C1-C2 - Risk factors include diabetes, drug abuse, endocarditis, immunocompromise - Soft tissue mass and bone destruction at C1-C2 level - Staphylococcus aureus most common organism in USA - Mycobacterium tuberculosis most common worldwide - MR shows low T1 signal mass centered at C1-C2 with variable involvement of odontoid and lateral masses at C2 - May show enlarged atlantodental interval - Epidural mass with thecal sac/cord compression - Grisel syndrome: Inflammatory, nontraumatic subluxation of C1-C2 following peripharyngeal infection
    • Extramedullary Tumor - Metastases - Multiple lesions, bone destruction, systemic primary - Lymphoma - Large pharyngeal mucosal space mass with associated cervical adenopathy > 50% of time - Non-Hodgkin lymphoma (NHL) 5x as common as Hodgkin disease in head & neck - Nasopharyngeal carcinoma - Mass centered in lateral pharyngeal recess of nasopharyngeal with deep extension and cervical adenopathy - Nodal metastases present in 90% of cases at presentation - Multiplanar images show invasion of clivus, sphenoid bone and sinus, C1 and C2 bodies - Neurofibromatosis type 1 - Plexiform neurofibroma → diffuse enlargement of major nerve trunks/branches → bulky rope-like ("bag of worms") nerve expansion with adjacent tissue distortion - Look for kyphoscoliosis ± multiple nerve root tumors, plexiform neurofibroma, dural ectasia/lateral meningocele - Schwannoma - Hypoglossal or upper cervical roots as site of origin - Hypoglossal neuropathy results in tongue denervation - Dumbbell with uniform enhancement - Larger lesions may show central cystic formation - Paraganglioma - Multiple black dots ("pepper") in tumor substance indicating high-velocity flow voids from feeding arterial branches - Jugular foramen (JF) or vagal varieties may present with upper cervical/skull base level mass - Chordoma - Mass is hyperintense to discs on T2WI with multiple septa - Destructive, lytic lesion - May extend into disc, involve 2 or more adjacent vertebrae - Chondrosarcoma - Lytic mass ± chondroid matrix, cortical disruption, and extension into soft tissues - Chondroid matrix mineralization of rings and arcs (characteristic) - Meningioma - Foramen magnum, JF, upper cervical dura locations - Carotid space → connection to JF above with JF margins showing permeative-sclerotic or hyperostotic changes on bone CT - Absence of high-velocity flow voids on T1 MR - T1 C+ MR shows enhancing JF mass

  • Helpful Clues for Less Common Diagnoses

    • Aneurysm/Vertebral Dissection - Multiple etiologies → dissection, posttraumatic, atherosclerotic, iatrogenic, congenital
    • Synovial Cyst - Round, central T2-hyperintense mass with low-signal margin - Associated with dorsal C1-C2 articulation or degenerated facets

  • Helpful Clues for Rare Diagnoses

    • Neurenteric Cyst - Intraspinal cyst + vertebral abnormalities (persistent canal of Kovalevsky, segmentation and fusion anomalies)

References

Selected References

  1. Wang HB et al: Cervical myelopathy due to idiopathic retro-odontoid pseudotumor. World Neurosurg.160:e256-60, 2022
  2. Bi WL et al: Skull base tumors: neuropathology and clinical implications. Neurosurgery. 90(3):243-61, 2021
  3. Parperis K et al: Management of calcium pyrophosphate crystal deposition disease: a systematic review. Semin Arthritis Rheum. 51(1):84-94, 2021
  4. Pascart T et al: Treatment of nongout joint deposition diseases: an update. Arthritis. 2014:375202, 2014
  5. Chang EY et al: Frequency of atlantoaxial calcium pyrophosphate dihydrate deposition at CT. Radiology. 269(2):519-24, 2013
  6. Tojo S et al: Factors influencing on retro-odontoid soft-tissue thickness: analysis by magnetic resonance imaging. Spine (Phila Pa 1976). 38(5):401-6, 2013
  7. Di Maio S et al: Current comprehensive management of cranial base chordomas: 10-year meta-analysis of observational studies. J Neurosurg. 115(6):1094-5, 2011
  8. Smith JS et al: Basilar invagination. Neurosurgery. 66(3 Suppl):39-47, 2010

Images

Selected Images

Sagittal T1WI MR shows rheumatoid arthritis (RA) involving C1-C2 articulation with dens erosion and extensive pannus formation . There is mild compression of medulla by the pannus and obscuration of fat planes. Rheumatoid Arthritis Sagittal T1WI MR shows rheumatoid arthritis (RA) involving C1-C2 articulation with dens erosion and extensive pannus formation . There is mild compression of medulla by the pannus and obscuration of fat planes.

Sagittal T1WI MR shows rheumatoid arthritis (RA) involving C1-C2 articulation with dens erosion and extensive pannus formation . There is mild compression of medulla by the pannus and obscuration of fat planes. Rheumatoid Arthritis Sagittal T1WI MR shows rheumatoid arthritis (RA) involving C1-C2 articulation with dens erosion and extensive pannus formation . There is mild compression of medulla by the pannus and obscuration of fat planes.

Sagittal T1WI MR shows C1-C2 degenerative pseudopannus in a patient with diffuse idiopathic skeletal hyperostosis. The odontoid is not eroded, but the atlantodental interval is increased. Cord compression occurs between the degenerative pannus and posterior C1. Retroodontoid Pseudotumor Sagittal T1WI MR shows C1-C2 degenerative pseudopannus in a patient with diffuse idiopathic skeletal hyperostosis. The odontoid is not eroded, but the atlantodental interval is increased. Cord compression occurs between the degenerative pannus and posterior C1.

CTA study shows multiple punctate foci of calcification within a large soft tissue mass posterior to the odontoid process that causes cord compression . No gross erosive changes are present within the odontoid process, as would be present in RA. Calcium Pyrophosphate Dihydrate Deposition Disease CTA study shows multiple punctate foci of calcification within a large soft tissue mass posterior to the odontoid process that causes cord compression . No gross erosive changes are present within the odontoid process, as would be present in RA.

Sagittal T1WI MR shows a large mass with low T2 signal posterior to the odontoid process with severe cord compression . Calcium Pyrophosphate Dihydrate Deposition Disease Sagittal T1WI MR shows a large mass with low T2 signal posterior to the odontoid process with severe cord compression .

Sagittal T2WI MR shows a large prevertebral abscess spanning C1 to C4 and extension posteriorly involving the interspinous region . These findings are typical for tuberculosis (TB). Osteomyelitis, C1-C2 Sagittal T2WI MR shows a large prevertebral abscess spanning C1 to C4 and extension posteriorly involving the interspinous region . These findings are typical for tuberculosis (TB).

Axial T1 C+ FS MR shows metastatic lung cancer with extensive extracapsular nodal spread. The mass has ill-defined borders with invasion of the longus capitis muscle and invasion to the pharyngeal mucosal space . Metastases Axial T1 C+ FS MR shows metastatic lung cancer with extensive extracapsular nodal spread. The mass has ill-defined borders with invasion of the longus capitis muscle and invasion to the pharyngeal mucosal space .

Axial CTA shows the classic appearance of thyroid metastasis with a thin, expansile, bony margin with the predominately lytic lesion within the left facet/lamina of C3 . Metastases Axial CTA shows the classic appearance of thyroid metastasis with a thin, expansile, bony margin with the predominately lytic lesion within the left facet/lamina of C3 .

Axial CECT shows a homogeneous mass in the retropharyngeal space, displacing the parapharyngeal fat anterolaterally and encircling the right internal carotid artery . Lymphoma Axial CECT shows a homogeneous mass in the retropharyngeal space, displacing the parapharyngeal fat anterolaterally and encircling the right internal carotid artery .

Sagittal T1 C+ MR shows a variant MR case of an unusually large skull base plasmacytoma engulfing the clivus and extending into the nasopharynx and abutting C1-C2. Plasmacytoma Sagittal T1 C+ MR shows a variant MR case of an unusually large skull base plasmacytoma engulfing the clivus and extending into the nasopharynx and abutting C1-C2.

Sagittal T1WI MR shows a typical case of an aggressive nasopharyngeal squamous cell carcinoma with invasion of the skull base by direct extension . Nasopharyngeal Carcinoma Sagittal T1WI MR shows a typical case of an aggressive nasopharyngeal squamous cell carcinoma with invasion of the skull base by direct extension .

Axial T1WI C+ MR shows multiple large neurofibromas within dorsal soft tissues and paravertebral regions. Symmetrical, large intradural lesions compress the cervical cord at the C2 level. Neurofibromatosis Type 1 Axial T1WI C+ MR shows multiple large neurofibromas within dorsal soft tissues and paravertebral regions. Symmetrical, large intradural lesions compress the cervical cord at the C2 level.

Sagittal T1 C+ MR demonstrates an isointense, expansile mass arising from the clivus . Notice the posterior indentation, or "thumbing," of the pons. Chordoma Sagittal T1 C+ MR demonstrates an isointense, expansile mass arising from the clivus . Notice the posterior indentation, or "thumbing," of the pons.

Sagittal T1WI C+ MR shows a large, heterogeneous-signal mass in the cervical epidural space involving the dorsal aspect of C2-C3 junction and extending laterally with diffuse enhancement. Chordoma Sagittal T1WI C+ MR shows a large, heterogeneous-signal mass in the cervical epidural space involving the dorsal aspect of C2-C3 junction and extending laterally with diffuse enhancement.

Coronal T1WI MR shows a typical MR case of petrooccipital fissure skull base chondrosarcoma . Chondrosarcoma Coronal T1WI MR shows a typical MR case of petrooccipital fissure skull base chondrosarcoma .

Sagittal T1 C+ MR shows a well-defined, homogeneously enhancing mass with a broad dural margin at the foramen magnum, typical for meningioma. There is compression of the medulla. Meningioma Sagittal T1 C+ MR shows a well-defined, homogeneously enhancing mass with a broad dural margin at the foramen magnum, typical for meningioma. There is compression of the medulla.

Axial CTA shows the CT features of a pseudoaneurysm of the internal carotid artery located below the skull base. Aneurysm/Vertebral Dissection Axial CTA shows the CT features of a pseudoaneurysm of the internal carotid artery located below the skull base.

Additional Images

Axial T1 C+ FS MR shows the appearance of multiple neurofibromas in a child with neurofibromatosis type 1 with bilateral, symmetric masses involving neural foramen in the carotid sheath and posterior cervical space, consistent with neurofibromas. Neurofibromatosis Type 1 Axial T1 C+ FS MR shows the appearance of multiple neurofibromas in a child with neurofibromatosis type 1 with bilateral, symmetric masses involving neural foramen in the carotid sheath and posterior cervical space, consistent with neurofibromas.

Axial T1 C+ FS MR shows the typical imaging appearance of a glomus tumor (vagale paraganglioma): A soft tissue mass below the skull base with small, focal signal voids indicating the presence of high-flow vessels. Paraganglioma Axial T1 C+ FS MR shows the typical imaging appearance of a glomus tumor (vagale paraganglioma): A soft tissue mass below the skull base with small, focal signal voids indicating the presence of high-flow vessels.

Axial CECT shows a variant case of carotid space meningioma from recurrent intracranial disease with an oval-shaped mass in the right carotid space, medial to the styloid process . Meningioma Axial CECT shows a variant case of carotid space meningioma from recurrent intracranial disease with an oval-shaped mass in the right carotid space, medial to the styloid process .

Sagittal T2WI MR shows a Chiari 1 malformation with inferiorly positioned, peg-shaped tonsils with small, associated cervical syrinx . Chiari 1 Malformation Sagittal T2WI MR shows a Chiari 1 malformation with inferiorly positioned, peg-shaped tonsils with small, associated cervical syrinx .

Sagittal T2WI MR shows a typical case of mild Chiari 2 malformation with a small posterior fossa and vermian ectopia to the C2/C3 level . The 4th ventricle is small and elongated. Chiari 2 Malformation Sagittal T2WI MR shows a typical case of mild Chiari 2 malformation with a small posterior fossa and vermian ectopia to the C2/C3 level . The 4th ventricle is small and elongated.

Sagittal T2WI MR shows a variant Chiari 2 with syringobulbia. There is a characteristic small posterior fossa and vermian ectopia . There is focal cervicomedullary syrinx . Chiari 2 Malformation Sagittal T2WI MR shows a variant Chiari 2 with syringobulbia. There is a characteristic small posterior fossa and vermian ectopia . There is focal cervicomedullary syrinx .

Sagittal T2WI MR demonstrates an extensive T2-hyperintense mass expanding the medulla and cervical cord from astrocytoma . The tumor causes a septated-appearing neoplastic syrinx in the more caudal cord . Glioma, Brainstem Sagittal T2WI MR demonstrates an extensive T2-hyperintense mass expanding the medulla and cervical cord from astrocytoma . The tumor causes a septated-appearing neoplastic syrinx in the more caudal cord .

Sagittal T1WI MR shows well-defined, complex cyst at the cervicomedullary junction due to hemangioblastoma. Hemangioblastoma, Spinal Cord Sagittal T1WI MR shows well-defined, complex cyst at the cervicomedullary junction due to hemangioblastoma.

Axial T1 C+ MR shows a well-defined, homogeneously enhancing mass with a broad dural margin at the foramen magnum, typical for meningioma. The left distal vertebral artery is adjacent to the tumor . Meningioma Axial T1 C+ MR shows a well-defined, homogeneously enhancing mass with a broad dural margin at the foramen magnum, typical for meningioma. The left distal vertebral artery is adjacent to the tumor .

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