The many faces of extranodal lymphoma Frank Pameijer Departments of Radiology and Radiation Oncology University Medical Center Utrecht
Special thanks to Ilona M Schmalfuss, MD University of Florida Gainesville, USA For supplying material used in this lecture
The many faces.. Let s look at 5 patients with pathology confirmed extranodal head&neck lymphoma
Middle-aged male Referred otalgia right side T1 STIR
Elderly lady Bilateral orbital swelling, visual disturbance
50 year old male Nasal stuffiness
Middle-aged female Bilateral parotid swelling, dry eyes
Female patient Enlarging thyroid mass
Purpose To present the spectrum of imaging features of extra-nodal H&N lymphoma Contrast this with other diseases in comparable locations
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
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)
Let s go back to the cases More systematically
Extranodal H&N Lymphoma Lymphatic Extralymphatic
Extranodal H&N Lymphoma Lymphatic Waldeyer s ring Extralymphatic
Extranodal H&N Lymphoma Lymphatic Waldeyer s ring Extralymphatic Orbit Upper respiratory tract (sinonasal) Salivary glands Thyroid
Extranodal H&N Lymphoma 80% additional nodal involvement Enhanced CT / MR (usually) non-necrotic nodes SCC
Waldeyer s ring Anatomical term describing a circle of lymphoid tissue in the naso- and oropharynx Nasopharynx
Waldeyer s ring Anatomical term describing a circle of lymphoid tissue in the naso- and oropharynx Nasopharynx Tonsillar fossa
Waldeyer s ring Anatomical term describing a circle of lymphoid tissue in the naso- and oropharynx Nasopharynx Tonsillar fossa Tongue Base
Large nasopharyngeal mass No destruction / infiltration DWI: restriction ++ Associated adenopathy b=1000 Nasopharyngeal lymphoma
Middle-aged man Referred otalgia right side T1 STIR Tonsillar fossa lymphoma
Tonsillar fossa lymphoma T2 (STIR) Pre RT Post RT
Companion case Female, 64 year; swelling right neck Tonsillar fossa SCC with ipsilateral lymph node metastasis
Lymphoma SCC
Patient with swallowing complaints Exofytic mass base of tongue (BOT), no ventral infiltration Ipsilateral non-necrotic adenopathy Proven BOT lymphoma
Companion case Exofytic BOT mass with left-sided ventral infiltration Bilateral non-homogeneous adenopathy BOT SCC with bilateral lymph node metastases
Lymphoma SCC
Waldeyer s ring DD: between lymphoma & SCC Primary tumor Usually impossible Lymph nodes Lymphoma usually non-necrotic SCC usually necrotic!
Extranodal H&N Lymphoma Lymphatic Waldeyer s ring Extralymphatic Orbit Upper respiratory tract (sinonasal) Salivary glands Thyroid
Orbital lymphoma Extraocular Intraocular Exceedingly rare!
Extraocular lymphoma Conjunctival involvement (25%) Without conjunctival involvement (75%)
Conjunctival involvement Salmon red patch swollen conjunctiva
Extraocular lymphoma Conjunctival involvement T1 T1 + Gd
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
Extraocular lymphoma Clinical presentation Palpable (usually painless) mass Exophthalmos Ptosis Diplopia and/or abnormal eye movement Usually preserved vision
Swelling left eye T1 T1Gd DWI b=1000 DD: Lacrimal gland malignancy PET: multifocal Dx! Lacrimal gland lymphoma
Elderly lady Bilateral orbital swelling, visual disturbance Bilateral lacrimal gland lymphoma
Conal involvement Infiltrating mass of right inferior rectus (proven lymphoma) Medially extending beyond muscle boundary DD: Inflammatory pseudotumor Graves s eye disease
Companion case Enlargement of inferior & lateral rectus muscles Respecting muscle boundaries Proven Graves s eye disease
Lymphoma Graves s
Intraconal (retrobulbar) involvement T1 T1FS Gd Smooth intraconal mass, homogeneous enhancement Melting around optic nerve Proven extraorbital lymphoma
CT Loss of vision left eye
Loss of vision left eye CT T1 T1+Gd
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!)
Extranodal H&N Lymphoma Lymphatic Waldeyer s ring Extralymphatic Orbit Upper respiratory tract (sinonasal) Salivary glands Thyroid
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
Adult male: rhinosinusitis, B-symptoms Non aggressive mucosal thickening DD: sinusitis Biopsy: B-cell lymphoma
Companion case Female patient: rhinosinusitis Non aggressive mucosal thickening Nasoseptal / palatal defect Biopsy: granulomatosis with polyangiitis (Wegener)
Lymphoma Wegener s
50 year old male Nasal stuffiness
50 year old male nasal stuffiness Biopsy: lymphoma (NK-T-cell) Carcinoma look-alike!!
Extranodal H&N Lymphoma Lymphatic Waldeyer s ring Extralymphatic Orbit Upper respiratory tract (sinonasal) Salivary glands (80% parotid gland) Thyroid
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!
Female patient Bilateral parotid swelling, dry eyes Sjögren s disease & (MALT) lymphoma
Companion case Female patient: Sjögren s disease MALT lymphoma lymph nodes
Companion case: Female 85 years Progressive swelling right cheek Transspatial mass (Waldeyer s / masticator / parotid space) - Proven lymphoma
Same patient Post chemotherapy (3x CHOP) Complete radiological & metabolic response
Secondary Parotid Lymphoma Bilateral disease! Multiple intra- and peri-parotid nodes Associated Level I nodes R. Hermans
Companion case Male patient; bilateral parotid&neck swelling Multiple intraparotid lesions & neck adenopathy DD: Lymphoepithelial cysts in HIV Infection!
Extranodal H&N Lymphoma Lymphatic Waldeyer s ring Extralymphatic Orbit Upper respiratory tract (sinonasal) Salivary glands Thyroid
Primary thyroid gland lymphoma F > M, typically (peak 70-80 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
Female patient Enlarging thyroid mass Markedly enlarged thyroid gland; left > right Extension into left tracheoesophageal groove Marked FDG avidity Proven thyroid lymphoma
Elderly patient Rapidly enlarging thyroid mass Diffuse enlarged thyroid, associated lymphadenopathy Infiltration of right TE-groove & trachea Proven thyroid lymphoma R. Hermans
Summary: extranodal lymphoma Indeed: it has many faces Lymphatic Waldeyer s ring Extralymphatic Orbit Upper respiratory tract (sinonasal) Salivary glands Thyroid
Key points Lymphoma should be considered in any extranodal head and neck mass
Key points Waldeyer s ring is the most common site of extranodal head and neck lymphoma Nasofarynx Tonsil Base of tongue
Key points Associated lymph nodes in extranodal lymphoma (usually) are non-necrotic
Key points Tissue sampling is (almost) always neccessary to establish the diagnosis J.A. Castelijns
The many faces of extranodal lymphoma Frank Pameijer
Question? Can perineural spread occur with lymphoma? Answer:
Question? Can perineural spread occur with lymphoma? Answer: YES
Extensive PNS along V2, V3 in lymphoma
Secondary bone involvement R. Hermans Permeative