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.

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

eEdE-174

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Disclosure

• We have nothing to disclose • No relevant financial relations interfering with

our presentation

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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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• 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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Common Lumps and Bumps in the Head and Neck in Children

MRI

– Various contrasts (T1, T2, T2*)– Contrast enhancement– 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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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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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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Let’s sort out the Lumps and Bumps,Ready?

• 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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Common Lumps and Bumps in the Head and Neck in Children

T2

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Case 1

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.

T1T2

T2

Large neck mass identified on ultrasonography

DWI SWI

ADC CT

MRA

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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

Case 1

Teratoma

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• 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…

34 GW, cystic well defined unilocular lateral neck massClosely associated with the carotid sheathContinuous with mediastinal thymus

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Case 2

Cystic neck mass identified on ultrasonography

3rd Branchial apparatus cystCervical 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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• 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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Common Lumps and Bumps in the Head and Neck in Children

Vascular Tumors

• 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– Etc.

Vascular Malformations

• 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

ISSVA Classification of Vascular AnomaliesISSVA: International Society for the Study of Vascular Anomalies)

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Misnomers in Vascular Anomalies

Misleading term

• Strawberry hemangioma• Capillary hemangioma• Cavernous hemangioma

• Port wine stain• Lymphangioma

Appropriate term

Infantile hemangioma

Infantile hemangioma

Venous malformation

Capillary malformation

Lymphatic malformation

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Problem solving in VAs: Incorporating clinical exam, symptoms and imaging

Tekes A, ..//.., Mitchell SE. Vascular Anomalies: Classification and terminology. Mauro, et al: Image-Guided Interventions, 2/e©ATB

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 ©ATB

Problem solving in VAs: Incorporating clinical exam, symptoms and imaging

S.E. Mitchell’s Flow Chart

T2 T1

T1 +C Subtraction

TWIST with Ablavar

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Well defined, solid, avidly enhancing mass No peripheral edema

Arterial Early Venous Late Venous

Early arterial enhancement of a solid mass with feeders taking off of ECA. Note the venous drainage.

6 mo presenting with an enlarging mass. Mass was not present at the time of birth

Case 3

• 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

Infantile Hemangioma

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T2 T1+C MRA

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PHACES

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.

– Posterior fossa malformations

– Hemangiomas (Segmental, >5 cm)

– Arterial Anomalies

– Coarctation of the aorta

– Eye abnormalities

– Sternal cleft, Supraumbilical raphe

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Newborn, prenatally diagnosed with a facial mass

T2 T1 +CT1

newborn

Case 5

10 mo2 mo

Solid T2 bright, enhancing mass, infiltrating the skin with somewhat irregular inner contours

Progressive reduction in size over time along with normalization of the skin color

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©ATB

T2 T1

T1 +C Subtraction

Arterial Early venous Late venous

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TWIST with Ablavar

T2 bright mass infiltrating the right masticator spaceChunky phleboliths

No enhancement during arterial phaseProgressive enhancement during venous phase

Case 612 mo presenting with right facial asymmetry which was not present at the time of birth

Venous Malformation

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• 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 cryingCompressible mass

Small well defined VM

Venous Malformation

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T2+FST2 -FS

Large, trans-spatial infiltrative VM

Small, well-circumscribed to large transspatial

T2 T1

T1 +C Subtraction

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TWIST with Ablavar

Case 7

Large, cystic mass in the posterior cervical trianglewith fluid-fluid levelsOnly peripheral cyst wall and septa enhanceNo internal enhancement within the cysts

No arterial or venous enhancementNote the displacement of the external jugular vein due to mass effect

3 yo presents with an enlarging left lower neck mass which was present at birth

Lymphatic Malformation

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• 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

Lymphatic Malformation

39 GW, large T2 bright macro and microcystic lymphatic malformation infiltrating the face and lower neck. Note the extension into the mediastinum.

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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.

T2 T1

T1 +C Subtraction

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Case 8

TWIST with Ablavar

T2 dark, serpiginous flow voids infiltrating subcutaneous fat of the right cheek. Note the increased T2 signal in the surrounding soft tissues and enhancement

12 yo presenting with pulsatile mass in the right face

Early arterial enhancement with feeders from ECA. Note the nidus formed by the tangle of vessels

Arteriovenous Malformation

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• 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

Starts in the arterial phase continues in the venous phase

Venous only!No enhancement in the arterial phase

No enhancementNeither arterial nor venous

Key MRI and DCE-MRA features of Vascular Anomalies

IH: Infantile Hemangioma; VM: Venous MalformationLM: Lymphatic Malformation; AVM: Arteriovenous MalformationDCE-MRA: Dynamic Contrast enhanced MRA

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In Summary….

• 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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Common Lumps and Bumps in the Head and Neck in Children

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Case 9

13 yo presenting with firm, non tender right lower neck massFailed multiple percutaneous sclerotherapies for treatment of lymphatic malformation

T2 hypointense, peripherally enhancing conglomerate of small solid masses in the right lower neck

Hodgkins Lymphoma

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• 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

Rhabdomyosarcoma

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CISS

T1+C

CISS

9 yo girl presents with snoring and sleep apnea for 2 months

Large T2 bright solid mass centered in the nasopharynx extending into bilateral nasal cavities

Obliteration of the nasopharyngeal airwayNote increased T2 signal and enhancement in the anteriorly displaced soft palate consistent with infiltration

Case 10

Rhabdomyosarcoma

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• 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

• 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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Common Lumps and Bumps in the Head and Neck in Children

T2 T1

T1 +C T1 +C

Case 11

Heterogenous/hyperechoic fusiform neck mass with increased vascularity

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Fusiform enlargement of the right sternocleidomastoid muscle. Note mild increased T2 signal and contrast enhancement

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©ATB

• 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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Conclusion