The many faces of extranodal lymphoma
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1 The many faces of extranodal lymphoma Frank Pameijer Departments of Radiology and Radiation Oncology University Medical Center Utrecht
2 Special thanks to Ilona M Schmalfuss, MD University of Florida Gainesville, USA For supplying material used in this lecture
3 The many faces.. Let s look at 5 patients with pathology confirmed extranodal head&neck lymphoma
4 Middle-aged male Referred otalgia right side T1 STIR
5 Elderly lady Bilateral orbital swelling, visual disturbance
6 50 year old male Nasal stuffiness
7 Middle-aged female Bilateral parotid swelling, dry eyes
8 Female patient Enlarging thyroid mass
9 Purpose To present the spectrum of imaging features of extra-nodal H&N lymphoma Contrast this with other diseases in comparable locations
10 Extranodal lymphoma H&N region: 2nd most frequent site (after GI tract) DD from other malignancies difficult because of overlapping clinical presentations and imaging manifestations => Diagnosis should be considered in any extranodal H&N mass
11 Extranodal (H&N) Lymphoma General imaging features CT: Isodense to muscle, homogeneous enhancement MR: T1: isointense to muscle, T2: variable, +Gd: homogeneous enhancement, ADC: restriction ++ Pet-CT: Usually high metabolic activity (depends on cell type and lymphoma grade)
12 Let s go back to the cases More systematically
13 Extranodal H&N Lymphoma Lymphatic Extralymphatic
14 Extranodal H&N Lymphoma Lymphatic Waldeyer s ring Extralymphatic
15 Extranodal H&N Lymphoma Lymphatic Waldeyer s ring Extralymphatic Orbit Upper respiratory tract (sinonasal) Salivary glands Thyroid
16 Extranodal H&N Lymphoma 80% additional nodal involvement Enhanced CT / MR (usually) non-necrotic nodes SCC
17 Waldeyer s ring Anatomical term describing a circle of lymphoid tissue in the naso- and oropharynx Nasopharynx
18 Waldeyer s ring Anatomical term describing a circle of lymphoid tissue in the naso- and oropharynx Nasopharynx Tonsillar fossa
19 Waldeyer s ring Anatomical term describing a circle of lymphoid tissue in the naso- and oropharynx Nasopharynx Tonsillar fossa Tongue Base
20 Large nasopharyngeal mass No destruction / infiltration DWI: restriction ++ Associated adenopathy b=1000 Nasopharyngeal lymphoma
21 Middle-aged man Referred otalgia right side T1 STIR Tonsillar fossa lymphoma
22 Tonsillar fossa lymphoma T2 (STIR) Pre RT Post RT
23 Companion case Female, 64 year; swelling right neck Tonsillar fossa SCC with ipsilateral lymph node metastasis
24 Lymphoma SCC
25 Patient with swallowing complaints Exofytic mass base of tongue (BOT), no ventral infiltration Ipsilateral non-necrotic adenopathy Proven BOT lymphoma
26 Companion case Exofytic BOT mass with left-sided ventral infiltration Bilateral non-homogeneous adenopathy BOT SCC with bilateral lymph node metastases
27 Lymphoma SCC
28 Waldeyer s ring DD: between lymphoma & SCC Primary tumor Usually impossible Lymph nodes Lymphoma usually non-necrotic SCC usually necrotic!
29 Extranodal H&N Lymphoma Lymphatic Waldeyer s ring Extralymphatic Orbit Upper respiratory tract (sinonasal) Salivary glands Thyroid
30 Orbital lymphoma Extraocular Intraocular Exceedingly rare!
31 Extraocular lymphoma Conjunctival involvement (25%) Without conjunctival involvement (75%)
32 Conjunctival involvement Salmon red patch swollen conjunctiva
33 Extraocular lymphoma Conjunctival involvement T1 T1 + Gd
34 Extraocular lymphoma Without conjunctival involvement (75%) Any structure may be affected In decreasing order of frequency DD Lacrimal gland conal compartment (i.e. extraocular muscles) intraconal compartment (retrobulbar Dx) Idiopathic inflammatory pseudotumor Graves s eye disease Other orbital malignancies
35 Extraocular lymphoma Clinical presentation Palpable (usually painless) mass Exophthalmos Ptosis Diplopia and/or abnormal eye movement Usually preserved vision
36 Swelling left eye T1 T1Gd DWI b=1000 DD: Lacrimal gland malignancy PET: multifocal Dx! Lacrimal gland lymphoma
37 Elderly lady Bilateral orbital swelling, visual disturbance Bilateral lacrimal gland lymphoma
38 Conal involvement Infiltrating mass of right inferior rectus (proven lymphoma) Medially extending beyond muscle boundary DD: Inflammatory pseudotumor Graves s eye disease
39 Companion case Enlargement of inferior & lateral rectus muscles Respecting muscle boundaries Proven Graves s eye disease
40 Lymphoma Graves s
41 Intraconal (retrobulbar) involvement T1 T1FS Gd Smooth intraconal mass, homogeneous enhancement Melting around optic nerve Proven extraorbital lymphoma
42 CT Loss of vision left eye
43 Loss of vision left eye CT T1 T1+Gd
44 Loss of vision left eye CT T1 T1+Gd DWI b=1000 Smooth intraocular mass left eye with intraconal extension. MR DWI: restriction DD: Inflammatory pseudotumor Proven intraocular lymphoma (very rare!)
45 Extranodal H&N Lymphoma Lymphatic Waldeyer s ring Extralymphatic Orbit Upper respiratory tract (sinonasal) Salivary glands Thyroid
46 Sinonasal lymphoma Two distinct subgroups DD B-cell lymphomas: less aggressive NK-T-cell lymphomas: destructive! (indistinguishable from SCC) (other) Malignancies; SCC! Infection Sinusitis, invasive fungal rhinosinusitis Granulomatous diseases Polyangiitis, sarcoid
47 Adult male: rhinosinusitis, B-symptoms Non aggressive mucosal thickening DD: sinusitis Biopsy: B-cell lymphoma
48 Companion case Female patient: rhinosinusitis Non aggressive mucosal thickening Nasoseptal / palatal defect Biopsy: granulomatosis with polyangiitis (Wegener)
49 Lymphoma Wegener s
50 50 year old male Nasal stuffiness
51 50 year old male nasal stuffiness Biopsy: lymphoma (NK-T-cell) Carcinoma look-alike!!
52 Extranodal H&N Lymphoma Lymphatic Waldeyer s ring Extralymphatic Orbit Upper respiratory tract (sinonasal) Salivary glands (80% parotid gland) Thyroid
53 Parotid lymphoma Primary Parotid parenchyma Rare MALT lymphoma Mucosa Associated Lymphoid Tissue Increased incidence in Sjögren s syndrome! Secondary * Within intraparotid lymph nodes Systemic Dx * : i.e. nodal disease!
54 Female patient Bilateral parotid swelling, dry eyes Sjögren s disease & (MALT) lymphoma
55 Companion case Female patient: Sjögren s disease MALT lymphoma lymph nodes
56 Companion case: Female 85 years Progressive swelling right cheek Transspatial mass (Waldeyer s / masticator / parotid space) - Proven lymphoma
57 Same patient Post chemotherapy (3x CHOP) Complete radiological & metabolic response
58 Secondary Parotid Lymphoma Bilateral disease! Multiple intra- and peri-parotid nodes Associated Level I nodes R. Hermans
59 Companion case Male patient; bilateral parotid&neck swelling Multiple intraparotid lesions & neck adenopathy DD: Lymphoepithelial cysts in HIV Infection!
60 Extranodal H&N Lymphoma Lymphatic Waldeyer s ring Extralymphatic Orbit Upper respiratory tract (sinonasal) Salivary glands Thyroid
61 Primary thyroid gland lymphoma F > M, typically (peak years) 80% associated with chronic lymphocytic thyroiditis (Hashimoto s) DD: Other thyroid malignancies, esp. anaplastic thyroid carcinoma Presentation: enlarging mass +/- lymphadenopathy Imaging: CT/MR: infiltrating pattern with invasion of surrounding structures (trachea and esophagus) Pet-CT: usually hypermetabolic
62 Female patient Enlarging thyroid mass Markedly enlarged thyroid gland; left > right Extension into left tracheoesophageal groove Marked FDG avidity Proven thyroid lymphoma
63 Elderly patient Rapidly enlarging thyroid mass Diffuse enlarged thyroid, associated lymphadenopathy Infiltration of right TE-groove & trachea Proven thyroid lymphoma R. Hermans
64 Summary: extranodal lymphoma Indeed: it has many faces Lymphatic Waldeyer s ring Extralymphatic Orbit Upper respiratory tract (sinonasal) Salivary glands Thyroid
65 Key points Lymphoma should be considered in any extranodal head and neck mass
66 Key points Waldeyer s ring is the most common site of extranodal head and neck lymphoma Nasofarynx Tonsil Base of tongue
67 Key points Associated lymph nodes in extranodal lymphoma (usually) are non-necrotic
68 Key points Tissue sampling is (almost) always neccessary to establish the diagnosis J.A. Castelijns
69 The many faces of extranodal lymphoma Frank Pameijer
70 Question? Can perineural spread occur with lymphoma? Answer:
71 Question? Can perineural spread occur with lymphoma? Answer: YES
72 Extensive PNS along V2, V3 in lymphoma
73 Secondary bone involvement R. Hermans Permeative
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