1. eEdE-174
How to facilitate differentiation of various bumps and lumps in the pediatric head and neck by MRI and Dynamic Contrast Enhanced MRA
Aylin Tekes, Madhan Bosemani, Luke Higgins, Thierry A.G.M. Huisman
Section of Pediatric Neuroradiology, Division of Pediatric Radiology, Russell H Morgan Department of Radiology and Radiological Science,
The Johns Hopkins University School of Medicine, Baltimore, MD
ASNR 53rd Annual Meeting, Chicago, April 25-30, 2015
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2. Disclosure We have nothing to disclose
No relevant financial relations interfering with our presentation
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3. Learning objectives
Classification of various lumps and bumps in the head and neck in children
MRI and Dynamic contrast enhanced MRA for head and neck imaging in children
Diagnosis and differential diagnosis based on
MR Imaging characteristics
Signal characteristics and enhancement patterns on MRI
Hemodynamic behavior on MRA (especially vascular anomalies)
Location
Patient age, clinical presentation
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4. Common Lumps and Bumps in the Head and Neck in Children
Congenital lesions: teratoma, branchial cleft/pouch cysts, lipoma.…
Vascular anomalies: hemangiomas, venous malformation, lymphatic malformation, AVM…
Neoplastic lesions : lymphoma, rhabdomyosarcoma, ...
Infectious/inflammatory lesions: abscess, fibromatosis colli
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5. MRI Various contrasts (T1, T2, T2*) Contrast enhancement Not bedside
Dynamic cMRA
MRA/MRV
DWI/DTI/PWI,SWI
High resolution for deep locations
Less dependent on technologist’s skills
Not bedside
Time consuming
Susceptible to motion
Expensive
Not readily available
Less “convenient” to child/parents
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6. Dynamic Contrast Enhanced MRA (TWIST)
Combination of parallel imaging and k-space undersampling
The high resolution components encoded in the k-space periphery are relatively stable over time while the low-frequency k-space center carries the significant contrast changes during bolus passage.
High frequency information (periphery of k-space)
Low frequency information (center of k-space)
TWIST: Time-resolved imaging WIth Stochastic Trajectories
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7. Dynamic Contrast Enhanced MRA (Ablavar)
Blood Pool Contrast agents such as Gadofosveset trisodium (Ablavar) are especially useful for imaging of vascular anomalies
higher intravascular concentration of contrast over a longer time period
better signal (SNR) and contrast to noise ratios (CNR) for imaging
can be administered at an approximately 3-fold lower dose than diffusible contrast agents while achieving an SNR gain
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8. Let’s sort out the Lumps and Bumps, Ready?
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9. Common Lumps and Bumps in the Head and Neck in Children
Congenital lesions: teratoma, branchial cleft/pouch cysts…
Vascular anomalies: hemangiomas, venous malformation, lymphatic malformation, AVM…
Neoplastic lesions : lymphoma, rhabdomyosarcoma, ...
Infectious/inflammatory lesions: abscess, fibromatosis colli
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10. Case 1 Large neck mass identified on ultrasonography T2 T2 T2 T1
38 GW fetus with large, solid and cystic mass in the right face and neck. Note the internal focus of T2 dark, T1 bright calcification (arrows). Polyhydramnios.
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11. Case 1
DWI
SWI
MRA
ADC CT
Post-natal imaging: SWI shows high vascularity of the solid component as confirmed on the MRA (feeders off of ECA)
CT shows large calcification/bony formation in the solid component, corresponding to the large focus of signal drop on SWI
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12. Teratoma Most common congenital tumor of the neck
Cystic or mixed solid and cystic mass
Large infiltrative mass
Frequently contains calcification
May cause significant airway and feeding problems
Histologic immaturity doesn’t necessarily reflect an adverse outcome unlike those seen in adults
DD: Lymphatic malformation, branchial apparatus cysts…
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13. Case 2 Cystic neck mass identified on ultrasonography
34 GW, cystic well defined unilocular lateral neck mass
Closely associated with the carotid sheath
Continuous with mediastinal thymus
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14. 3rd Branchial apparatus cyst Cervical Thymic Cyst
Cystic remnant of thymopharyngeal duct
Location: Anywhere along the tract of the thymopharyngeal duct from the pyriform sinus to the anterior mediastinum
50% continuous with the mediastinum
Left > Right
Closely associated with carotid sheath
Rare
DD: 2nd and 1st branchial apparatus cysts, lymphatic malformation
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15. Common Lumps and Bumps in the Head and Neck in Children
Congenital lesions: teratoma, branchial cleft/pouch cysts…
Vascular anomalies: hemangiomas, venous malformation, lymphatic malformation, AVM…
Neoplastic lesions : lymphoma, rhabdomyosarcoma, ...
Infectious/inflammatory lesions: abscess, fibromatosis colli
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16. ISSVA Classification of Vascular Anomalies ISSVA: International Society for the Study of Vascular Anomalies)
Vascular Tumors
Vascular Malformations
Benign vascular tumors
Infantile Hemangiomas
Congenital Hemangiomas (CH)
Rapidly Involuting CH
Non-involuting CH
Partially involuting CH
Locally aggressive vascular tumors
Kaposiform Hemangioendothelioma
Etc.
Malignant Vascular tumors
Angiosarcoma
Simple Vascular Malformations
Venous malformations
Lymphatic malformations
Arteriovenous malformations
Capillary malformations
Combined Vascular Malformations
Arteriovenous malformations/fistulas
Capillary-venous
Capillary-arteriovenous
Lymphaticovenous malformation
Simplified/Modified from ISSVA 2014
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17. Misnomers in Vascular Anomalies
Misleading term
Appropriate term
Strawberry hemangioma
Capillary hemangioma
Cavernous hemangioma
Port wine stain
Lymphangioma
Infantile hemangioma Venous malformation Capillary malformation Lymphatic malformation
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18. Problem solving in VAs: Incorporating clinical exam, symptoms and imaging
S.E. Mitchell’s Flow Chart
Tekes A, ..//.., Mitchell SE. Vascular Anomalies: Classification and terminology. Mauro, et al: Image-Guided Interventions, 2/e
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19. Problem solving in VAs: Incorporating clinical exam, symptoms and imaging
S.E. Mitchell’s Flow Chart
Yes No
Tekes A, ..//.., Mitchell SE. Vascular Anomalies: Classification and terminology. Mauro, et al: Image-Guided Interventions, 2/e
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20. Case 3 TWIST with Ablavar
6 mo presenting with an enlarging mass. Mass was not present at the time of birth T2 T1
TWIST with Ablavar
Arterial
Early Venous
Late Venous
T1 +C
Subtraction
Early arterial enhancement of a solid mass with feeders taking off of ECA. Note the venous drainage.
Well defined, solid, avidly enhancing mass No peripheral edema
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21. Infantile Hemangioma The most common tumor of infancy
Typically recognized in the first few weeks of life
Not present at the time of birth!
3 phases: Proliferation, Involution, Involuted
Stains positive for Glut-1
Spontaneous regression in majority of the cases!
If not, medical treatment with propranolol
Rarely surgery
Large/segmental IH in the head and neck raises suspicion for PHACES
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22. PHACES Posterior fossa malformations Hemangiomas (Segmental, >5 cm)
Arterial Anomalies
Coarctation of the aorta
Eye abnormalities
Sternal cleft, Supraumbilical raphe T2
T1+C
MRA
Extensive IHs surrounding the upper airway in addition to the skin/subcutaneous fat involvement in the right temporal/auricular region. Note aplasia of the right ICA.
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23. Case 5 Newborn, prenatally diagnosed with a facial mass
T1 +C
Solid T2 bright, enhancing mass, infiltrating the skin with somewhat irregular inner contours
newborn
2 mo
10 mo
Progressive reduction in size over time along with normalization of the skin color
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24. Rapidly Involuting Congenital Hemangioma
Very rare
They are present at birth (can be diagnosed prenatally)
Similar imaging features with infantile hemangioma
Differentiation between rapidly involuting, non-involuting and partially involuting congenital hemangiomas are usually done retrospectively
Clinical follow-up is critical!
Does not stain positive for Glut-1
Does not respond to treatment with propranolol
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25. Case 6 TWIST with Ablavar
12 mo presenting with right facial asymmetry which was not present at the time of birth T2 T1
TWIST with Ablavar
Arterial
Early venous Late venous
T1 +C
Subtraction
No enhancement during arterial phase
Progressive enhancement during venous phase
T2 bright mass infiltrating the right masticator space
Chunky phleboliths
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26. Venous Malformation Most common vascular malformation
Most commonly in the head and neck
Although present at birth, they may not be visible later in life
Sporadic
No arterial feeder
Histopathology reveals thrombi and phleboliths
Treatment with embolization
Enlarging size during crying
Compressible mass
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27. Venous Malformation Small well defined VM
T2 -FS
T2+FS
Small well defined VM
Large, trans-spatial infiltrative VM
Small, well-circumscribed to large transspatial
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28. Case 7 TWIST with Ablavar
3 yo presents with an enlarging left lower neck mass which was present at birth
TWIST with Ablavar T2 T1
T1 +C
Subtraction
Large, cystic mass in the posterior cervical triangle
with fluid-fluid levels Only peripheral cyst wall and septa enhance No internal enhancement within the cysts
No arterial or venous enhancement
Note the displacement of the external jugular vein due to mass effect
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29. Lymphatic Malformation
Macrocystic, microcystic or mixed
Head neck is the most common location
Posterior cervical triangle
Avascular lesions: septa and wall may enhance
Internal cyst contents do not enhance!
Fluid-fluid levels can be seen although not specific
Venous malformations can show fluid-fluid levels
Treatment with sclerotherapy
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30. Lymphatic Malformation
39 GW, large T2 bright macro and microcystic lymphatic malformation infiltrating the face and lower neck. Note the extension into the mediastinum.
2nd week of life, similar findings with the prenatal third trimester imaging.
Enlargement and increased T2 signal of
the lips and tongue appear more prominent,
Which could either represent microcystic lymphatic malformation and/or lymphedema.
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31. Case 8 TWIST with Ablavar
12 yo presenting with pulsatile mass in the right face
TWIST with Ablavar T2 T1
T1 +C
Subtraction
Early arterial enhancement with feeders from ECA. Note the nidus formed by the tangle of vessels
T2 dark, serpiginous flow voids infiltrating subcutaneous fat of the right cheek. Note the increased T2 signal in the surrounding soft tissues and enhancement
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32. Arteriovenous Malformation
Unpredictable growth pattern, lying dormant for long periods or undergoing phases of explosive growth spontaneously or secondary to trauma, surgery, or hormonal influences
Local high flow effects: adjacent mass effect, soft tissue destruction or erosion
Arterial feeders and venous drainage
Treatment with embolization
Difficult to treat: Pick up new feeders after treatment
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33. Key MRI and DCE-MRA features of Vascular Anomalies
In Summary….
Key MRI and DCE-MRA features of Vascular Anomalies
Starts in the arterial phase
continues in the venous phase
Venous only!
No enhancement in the
arterial phase
No enhancement
Neither arterial nor venous
IH: Infantile Hemangioma; VM: Venous Malformation
LM: Lymphatic Malformation; AVM: Arteriovenous Malformation
DCE-MRA: Dynamic Contrast enhanced MRA
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34. Common Lumps and Bumps in the Head and Neck in Children
Congenital lesions: teratoma, branchial cleft/pouch cysts…
Vascular anomalies: hemangiomas, venous malformation, lymphatic malformation, AVM…
Neoplastic lesions : lymphoma, rhabdomyosarcoma, ...
Infectious/inflammatory lesions: abscess, fibromatosis colli…
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35. Case 9 13 yo presenting with firm, non tender right lower neck mass
Failed multiple percutaneous sclerotherapies for treatment of lymphatic malformation
T2 hypointense, peripherally enhancing conglomerate of small solid masses in the right lower neck
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36. Hodgkins Lymphoma
The most common head and neck malignancy: Hodgkin Lymphoma
Typically unilateral, firm, non-tender mass
Contiguous lymph node involvement
40% have neck presentation have
Mediastinal involvement
Histological hallmark: Reed-Sternberg cells
Etiology: ?? Ebstein-Barr virus infection
Treatment: Chemotheraphy +/- radiation
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37. Rhabdomyosarcoma Case 10
9 yo girl presents with snoring and sleep apnea for 2 months
CISS
CISS
T1+C
Large T2 bright solid mass centered in the nasopharynx extending into bilateral nasal cavities
Obliteration of the nasopharyngeal airway Note increased T2 signal and enhancement in the anteriorly displaced soft palate consistent with infiltration
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38. Rhabdomyosarcoma
Most common soft tissue sarcoma and 2nd most common pediatric head and neck tumor after lymphoma
Location: masticator space and orbits in the 1st decade, paranasal sinuses in the teenagers
Sporadic
Mutations in p53 tumor suppressor gene and NF-1 gene have been described
Histologic types: embryonal and alveolar
Embryonal in younger children, alveolar in teenagers
Treatment: Radiation, chemotherapy, surgery
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39. Common Lumps and Bumps in the Head and Neck in Children
Congenital lesions: teratoma, branchial cleft/pouch cysts…
Vascular anomalies: hemangiomas, venous malformation, lymphatic malformation, AVM…
Neoplastic lesions : lymphoma, rhabdomyosarcoma, ...
Infectious/inflammatory lesions: abscess, fibromatosis colli…
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40. Case 11 T2 T1
T1 +C
T1 +C
Heterogenous/hyperechoic fusiform neck mass with increased vascularity
Fusiform enlargement of the right
sternocleidomastoid muscle. Note mild increased T2 signal and contrast enhancement
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41. Fibromatosis Coli
Non neoplastic enlargement of the sternocleidomastoid muscle in early infancy
Mid to lower third of the SCM is involved
Fusiform, firm, non-tender enlargement
Right>Left
Variable echogenicity/signal intensity
Enhancement (+)
Pathology: fibrocollagenous infiltration
?precipitated by in-utero head positioning, trauma
Treatment: physical theraphy
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42. Conclusion
Pediatric head and neck masses require special attention, since the differential diagnosis are quite different compared to the adult counterparts
Radiation free high resolution MR imaging is critical to diagnosis and assessment of head neck masses in children
Anatomical location, MRI signal characteristics and contrast enhancement pattern are essential for correct diagnosis
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