Case Studies in Infrahyoid Neck Nicholas A. Koontz, M.D. Neuroradiology Fellow, University of Utah

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Objectives Review Infrahyoid Neck Anatomy Cases, Cases, Cases Deep Spaces Nodal Stations Cases, Cases, Cases Tackle challenging cases Develop an appropriate differential diagnosis Identify useful discriminators Multiple choice questions

Anatomy

Anatomic Spaces of Infrahyoid Neck Visceral Space Carotid Space Retropharyngeal Space Perivertebral Space Posterior Cervical Space

Infrahyoid Lymph Node Stations

Cases

Case 1 65 year-old woman with neck pain, palpable lump

Red Arrow = Enlarged, heterogeneous attenuation/enhancing thyroid mass with internal calcs Yellow Arrow = Indistinct & infiltrating margins, effacement of IJV Green Arrow = Cystic level III met

Red Arrow = Intrathyroidal calcs

Red Arrow = Punctate calc associated with necrotic level III node Orange Arrows = Additional solid adenopathy

Differential Diagnosis Differentiated Thyroid Ca Medullary Thyroid Ca Anaplastic Thyroid Ca Thyroid NHL Multinodular Goiter

Most Likely Diagnosis Differentiated Thyroid Ca (DTCa) Age & Sex Ill-defined Infiltrating, invasive Mixed solid/cystic Intra-thyroidal Calcs Intra-nodal Adenopathy Some solid Some cystic Punctate calcs

http://www.polleverywhere.com/multiple_choice_polls/1rtqKTxSvODxUER

Question 1 Which of the following is a TRUE statement? A. Follicular is the most common subtype of DTCa B. Hematogenous spread is more commonly associated with Papillary carcinoma C. The peak incidence of DTCa is seen in women in the third or fourth decade D. Rising free T4 is a clinical marker for disease recurrence

Question 1 Which of the following is a TRUE statement? A. Follicular is the most common subtype of DTCa B. Hematogenous spread is more commonly associated with Papillary carcinoma C. The peak incidence of DTCa is seen in women in the third or fourth decade D. Rising free T4 is a clinical marker for disease recurrence Papillary = 80%, Follicular = 10% Papillary  nodal, follicular  hematogenous 3x more common in women, peak incidence in 20s to 30s Thyroglobulin = clinical marker of recurrence

DTCa Companion Cases

1) 75 year-old-woman, neck lump Yellow Arrow = Heterogeneous thyroid mass Red Arrows = Punctate calcs within thyroid mass and enlarged superior mediastinal node

2) 48 year-old-woman, enlarging mass Red Arrow = Heterogeneous thyroid mass with punctate & stippled calcs Green Arrows = Heterogeneously enhancing/cystic adenopathy

3) Nodal Manifestations of DTCa Green Arrow = Cystic, necrotic adenopathy Red Arrow = Punctate calc in LN Blue Arrow = High T1 SI node (thyroglobulin) Yellow Arrow = Low T1 SI node

4) 30 year-old-woman, adenoma Yellow Arrow = Circumscribed, heterogeneous attenuation/enhancment thyroid mass Red Arrow = Effaced subglottic & tracheal airway Blue Arrow = Prominent solid jugulodigastric node, maintained reniform shape. No calcs or necrosis. Magnified Cor CECT of LN

Case 2 55 year-old-woman with right neck mass, cough

Yellow Arrows = Heterogeneous attenuation LNs

Differential Diagnosis H&N SCCa Metastatic Nodes Systemic Nodal Metastases Thyroid Ca Metastatic Nodes HL or NHL Nodes Granulomatous Lymph Nodes Reactive Adenopathy

Most Likely Diagnosis Systemic Nodal Mets Central low-density/necrosis Infrahyoid (level IV) location H&N primary SCCa more commonly levels II & III Non-calcified Sarcoid, DTCa often Ca++ Central low-density/necrosis HL, NHL, & reactive nodes usually solid, but can be low-density

Use Everything at Your Disposal “I’ll tell you right now – that ain’t normal.” -- Rick Wiggins

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Question 2 Which of the following is MOST suggestive of systemic nodal metastases in the neck? A. Enlarged suprahyoid (level I or II) node B. Enlarged left supraclavicular lymph node C. Centrally necrotic lymph node D. Calcification within an enlarged cervical node

Question 2 Which of the following is MOST suggestive of systemic nodal metastases in the neck? A. Enlarged suprahyoid (level I or II) node B. Enlarged left supraclavicular lymph node C. Centrally necrotic lymph node D. Calcification within an enlarged cervical node Systemic nodal mets infrahyoid > suprahyoid “Signal” node. Central necrosis nonspecific, can be from H&N SCCa, systemic mets, suppurative nodes, etc. Calcs may be seen in DTCa, adenoCa mets.

Companion Case

25-year-old man with neck mass Yellow Arrows = Heterogeneous low attenuation supraclavicular LNs and axial adenopathy

HL with “Signal” Node AKA Virchow node Isolated left supraclavicular adenopathy  look to the chest & abdomen for primary Most HL patients present with neck nodes Concurrent mediastinal nodes common Rarely extranodal H&N disease M > F Peak incidence in mid-20s

http://www.polleverywhere.com/multiple_choice_polls/ckaTijYFCR2gpAT

Question 3 Which of the following is a TRUE statement? A. HL is more common than NHL B. Extranodal disease favors HL over NHL C. Imaging can reliably differentiate NHL from HL D. HL has an earlier peak incidence than NHL

Question 3 Which of the following is a TRUE statement? A. HL is more common than NHL B. Extranodal disease favors HL over NHL C. Imaging can reliably differentiate NHL from HL D. HL has an earlier peak incidence than NHL NHL is more common than HL (HL = 14% of lymphoma) HL is RARELY extranodal (<1%), NHL extranodal in 30% Imaging is good for staging disease, but poor at differentiating HL from NHL HL median age = 27, NHL median age = 53

Case 4 55-year-old woman with known thyroid nodules, reportedly benign – surveillance US

Longitudinal Transverse Power Doppler

Prior biopsy reported benign

Yellow Arrows = Low-density, poorly enhancing carotid space mass

Differential Diagnosis Congenital lesion Lymphatic malformation Venolymphatic malformation Venous malformation 3rd Branchial cleft cyst Neurofibroma Schwannoma Malignant Lymph node Carotid artery Pseudoaneurysm

Most Likely Diagnosis Congenital lesion Why not a NST? Lymphatic malformation Benign, circumscribed No flow on US Demonstrably separate from IJV and CCA Venolymphatic malformation Possible, but would have essentially no venous component Why not a NST?

Carotid Space Nerve Sheath Tumor Image c/o Lauren Ladd, M.D. Pros Cons Location Size Morphology Low Density Echogenicity Lack of vascularity

CS Nerve Sheath Tumor Comparison   CS Schwannoma CS Neurofibroma Shape Fusiform Ovoid or fusiform (***unless plexiform) Margins Circumscribed Circumscribed (***unless plexiform) Size 2 - 8 cm 2 - 5 cm M:F Male predominance Female Predominance NECT Isodense to muscle Hypodense CECT Uniform enhancement, rare low density Poorly enhancing T1WI -C Variable, no flow voids Isointense to muscle T1WI +C Marked uniform enhancement Homogeneous or patchy enhancement T2WI Hyperintense to muscle, +/- intratumoral cysts Very hyperintense, "target sign"

http://www.polleverywhere.com/multiple_choice_polls/JBIvqBFeitzbp8Z

Question 4 Which of the following is a FALSE statement? A. Most lymphatic malformations are diagnosed before age 2 B. Lymphatic malformations can be acquired C. Lymphatic malformations have no malignant potential D. Microcystic lymphatic malformations are less likely to recur than macrocystic malformations

Question 4 Which of the following is a FALSE statement? A. Most lymphatic malformations are diagnosed before age 2 B. Lymphatic malformations can be acquired C. Lymphatic malformations have no malignant potential D. Microcystic lymphatic malformations are less likely to recur than macrocystic malformations 90% diagnosed by age 2 In adults lymphatic malformations can be acquired  think trauma. No malignant potential. LM can be micro or macro cystic – microcystic are more likely to recur than macrocystic.

Case 5 25-year-old man with enlarging neck mass, recent URI

Yellow Arrow = Thick walled, peripherally enhancing midline infrahyoid lesion just below hyoid Red Arrow = Additional thick walled, peripherally enhancing midline lesion FOM

Yellow Arrow = same peripherally enhancing lesion in Cor Cyan Arrow = corticated osseous defect in hyoid bone

Yellow Arrow = TGDC Red Arrow = FOM abscess

Same patient, 3 days prior Yellow Arrow = Same TGDC, smaller, less enhancement Red Arrow = Blush of enhancement, no abscess

Differential Diagnosis Thyroglossal Duct Cyst Lymphatic Malformation Mixed Laryngocele Necrotic Lymph Node Abscess Thyroid Ca Lymphatic malformation can look identical  history is your friend

Most Likely Diagnosis Infected Thyroglossal Duct Cyst with associated FOM Abscess Classic history Midline/paramidline infrahyoid Wall enhancement  infected Round or ovoid Cyst No calcs or solid component

Thyroglossal Duct Cyst Key Points Cystic remnant of TGD Lesion of the young Location 20-25% = Suprahyoid 50% = Hyoid 25% = Infrahyoid Infrahyoid typically embedded in strap muscles  “claw” sign Wall enhancement if infected < 1% will develop Thyroid Ca

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Question 5 Which of the following is a TRUE statement? A. Thyroglossal duct cyst is the most common congenital neck mass B. Thyroglossal duct cysts are always midline structures C. The most common malignancy to develop in a thyroglossal duct cyst is medullary thyroid Ca D. Treatment of thyroglossal duct cyst is typically needle aspiration

Question 5 Which of the following is a TRUE statement? A. Thyroglossal duct cyst is the most common congenital neck mass B. Thyroglossal duct cysts are always midline structures C. The most common malignancy to develop in a thyroglossal duct cyst is medullary thyroid Ca D. Treatment of thyroglossal duct cyst is typically needle aspiration True. Off-midline below the hyoid Well-differentiated thyroid Ca, usually papillary Sistrunk procedure (cyst, tract, and branch excision requiring removal of the central hyoid bone)

TGD Cyst Companion Cases

1) 50-year-old man with neck mass TGD Cyst. High density = heme, protein.

TGD Cyst. Suprahyoid/BOT. 2) Young girl, dysphagia TGD Cyst. Suprahyoid/BOT.

TGD Cyst Thyroid Ca. Enhancing nodule. Coarse calc. Nodal Met. 3) Enlarging neck mass TGD Cyst Thyroid Ca. Enhancing nodule. Coarse calc. Nodal Met.

4) Ectopic Thyroid

Case 6 31-year-old woman with difficult intubation during elective surgery

Yellow Arrows = Circumscribed, heterogeneous submucosal mass originating in subglottic larynx. No deep invasion.

“I’ll tell you right now – that ain’t normal.” -- Rick Wiggins Green Arrow = Low density mass at skull bass, carotid space Red Arrows = Similar density L infraclavicular masses

Ax T1WI +C FS Ax T1WI +C FS Cor T1WI +C FS Ax T2WI FS Ax T2WI FS Yellow Arrows = Subglottic larynx mass Red Arrows = Infraclavic masses Green Arrows = CS mass Cor T1WI +C FS Ax T2WI FS Ax T2WI FS

Differential Diagnosis NF1 NF2 Schwannomatosis Laryngeal SCCa with Mets Chondrosarcoma with Mets Intrinsic vs Extrinsic causes

Ax T2WI FS Ax T1WI +C FS Normal IACs

Most Likely Diagnosis Schwannomatosis No matrix calcification Morphology & Margins SCCa  infiltrative/invasive Distribution CS + Brachial plexus  NST NORMAL IACs NF2 less likely Age NF1 = 1st decade NF2 = 2nd decade Schwannomatosis = 3-4th decades No matrix calcification MR signal  NST Intrinsic vs Extrinsic causes

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Question 6 Which of the following is a TRUE statement? A. Schwannomas grow centrally within an involved nerve B. Schwannomatosis patients demonstrate a normal life expectancy C. Schwannomas arise from pericytes in the nerve sheath D. Schwannomatosis is more common than NF1

Question 6 Which of the following is a TRUE statement? A. Schwannomas grow centrally within an involved nerve B. Schwannomatosis patients demonstrate a normal life expectancy C. Schwannomas arise from pericytes in the nerve sheath D. Schwannomatosis is more common than NF1 Eccentric to affected nerve. Normal life expectancy, unlike NF2 Arive from Schwann cells, not pericytes NF1 > NF2 ~ Schwannomatosis

Schwannomatosis Key Points Multiple nonintradermal schwannomas WITHOUT vestibular nerve involvement Separate disease entity from NF2 Different gene mutation SMARCB1 vs. NF2 Later onset 4th decade vs. 2nd decade Normal life expectancy (unlike NF2) Pain >> neurologic deficits (unlike NF2) Similar incidence to NF2 (~ 1/40,000)

Infrahyoid Neck Conclusion Several deep spaces & nodal stations Wide variety of pathology Look for useful discriminators: Age History Deep space of origin

Thanks nick.koontz@hsc.utah.edu