Orbital Tumor and Tumorlike. Thiparom Sananmuang, MD. Neuroradiologist Ramathibodi hospital, Mahidol University

Transcription

1 Orbital Tumor and Tumorlike lesion Thiparom Sananmuang, MD. Neuroradiologist Ramathibodi hospital, Mahidol University

2 Outline Principle of orbital imaging Case-based approach tumor & tumor-like lesion Take home message

3 Principle of orbital Imaging Compartment-based approach is essential CT scan & MRI play key role in work-up Don t forget clinical! Age : Adult VS Ped Symptom and underlying disease

4 Orbital compartment Globe Preseptal Postseptal Conal Intraconal + optic nerve complex Extraconal + Lacrimal gl. Bone and Sinus

5 medial lateral Axial T1FS+Gd

6 Superior Superior oblique Lacrimal gland Lateral Medial Inferior Coronal T1FS+Gd

7 Optic nerve have BBB Enhanced normal size Normal optic nerve Acute optic neuritis

8 Case 1 4Y girl left eye blurring 4 M

9 Possible : Optic N. complex disease Optic nerve complex tumor Optic neuritis Optic neuropathy

10 T2W T1FS+Gd Enhancing enlarged optic nerve : Optic nerve glioma

11 Optic nerve glioma Nearly all Juvenile pilocytic astrocytoma (WHO gr I) Who? : Children, associate with NF 1 Symptom : Slow progressive decreased visual acuity Imaging: CT : Fusiform enlarged optic nerve, posterior extension along optic tract MRI : Enhanced, hypert2, **Watch for intracranial extension

12 Optic nerve Glioma VS Meningioma Enlarged optic nerve Wrap around optic nerve Tram-Track

13 Case 2 42 F, Painless proptosis

14 42 F, Painless proptosis T1W T2W T1FS+Gd

15 DDX Adult well-circumscribed orbital mass Cavernous Malformation Hemangiopericytoma Schwannoma Key! Congenital vascular malform M/C benign orbital mass in adult Well-circumscribed oval mass Lateral intraconal Iso-T1/Hyper-T2 Progressive filling in enhancement Key! Rare tumor originate from pericyte Well-circumscribed oval mass Extraconal adj PNS Iso-T1/ Iso-T2 Vivid arterial enhance & early wash-out Key! Benign prolif of Schwann cell (CN 3-5) Well-circumscribed tubular/fusiform mass Heterogeneous Hyper-T2 & Enhance Abuts optic aperture

16 T1FS+Gd Patho: Cavernous Malformation

17 Case 3 24 M, Painless proptosis right eye 1 Y Prior CT found lacrimal mass

18 NCCT + C Dermoid cyst

19 Dermoid M/C congenital orbital lesion Arise from epithelial sequestration (zygomaticofrontal and frontoethmoidal sutures) Symptoms : Slow growing mass or Mimic acute inflammation (ruptured) Imaging Extraconal & Well circumscribed CT : Cystic or Fat density MRI : Hyper-T1/T2, Fat suppressed +/- Fat-fluid level or Calcification

20 Epidermoid DDX Extraconal cystic lesion Subperiosteal abscess Encephalocele Key! Ectodermal rest Heterogeneous cystic Restricted diffusion +/-HyperT1 : high protein Key! Paranasal sinus inflammation Fat reticulation Key! Track leading to cranial fossa Korean J Radiol 2007;8:

21 Case 4 58 F, Painless lacrimal mass

22 58 F, painless proptosis T1W T2W Benign mixed tumor T1FS+Gd

23 Lacrimal mass Epithelial Benign mixed tumours (BMT)* Adenoid cystic carcinoma (ACC)$ Carcinoma ex-pleomorphic adenoma Adenocarcinoma Mucoepidermoid carcinoma Squamous cell CA * M/C benign $ M/C malignant Non-epithelial Lymphoproliferative Inflammatory (Dacryocystitis) Non-carcinomatous metastases

24 Benign mixed tumour (BMT) M/C benign epithelial tumor of lacrimal gl. Who?: 4 th -5 th decade of life Symptom: Painless, slow-growing mass Imaging : CT: Well-circumscribed lacrimal mass, cystic degeneration, calcifications MRI: Heterogeneous signal T2 & enhancement

25 Malignant ACC VS BMT ACC Painful (perineural spread) Painless BMT Bone erosion More calcification Bone remodeling: Smooth concave * Imaging of early ACC may be identical to BMT

26 72 F, Painful proptosis : Adenoid cystic carcinoma T2W T1FS+Gd T1FS+Gd

27 Case 5 35 M, Right eye proptosis 1 M

28 T1FS+Gd

29 DDX Conal lesion Thyroid opthalmopathy M/C Malignancy : lymphoma, leukemia, metastases Infectious myositis Vascular congestion : DAVF at cavernous sinus

30 Imaging characteristic : TAO Fusiform enlargement Orderly involvement I m Slow Spare myotendinous junction Fat proliferation Bilateral > Unilateral May precede the onset of abnormal thyroid function

31 Idiopathic orbital inflammatory disease

32 Conal lesion alert: Not TAO!!! Disproportionate fat reticulation Unusual order EOM involvement Involvement of Myotendinous junction Other associated findings: Abnormal vessel, other organ involvement

33 Case 6 67 M, Painless proptosis both eyes 1 Y

34 T2W T1FS+Gd

35 DDX: Multicompartment lesions (Adult) Venolymphatic malformation Lymphoproliferative disorder Metastasis Infection

36 Venolymphatic malformation Metastasis Lymphoproliferative disorder Key! Vascular malformation with lymphoid & venous element Ped > Adult Cystic Fluid-fluid level +/- Phelobolith Key! Various imaging features Hx of underlying CA Key! Diffuse, Homogeneous enhance soft tissue Mold structure VariableT2 Hyperdense in NCCT, +/- restricted diffusion Other organ involvement (lacrimal upto 40%) Bilateral lesion 24%

37 NCCT DWI ADC Patho: B-Cell lymphoma

38 Lymphoproliferative disorder M/C in older Adult (>60 yrs) Lymphoid hyperplasia Idiopathic orbital inflammation Reactive lymphoid hyperplasia IgG4 Atypical lymphoid hyperplasia Lymphoma NHL (MALT) is M/C

39 Benign lymphoproliferative VS Lymphoma Benign lymphoproliferative Painful More common Iso-T2/ Hypo-T2* Higher ADC Lymphoma Painless Hyper-T2 Lower ADC * IgG4 due to fibrosis

40 IgG4 : Myositis IgG4 : Tolosa hunt Lymphoma : Tram-Track IgG4 mimic infection

41 IgG4 : Lacrimal Lymphoma : Lacrimal Clues! : Bilateral & Other organ involvement!!

42 Case 7 68 M, visual field defect

43 T1FS T2FS Subtract T1FS+Gd

44 Occular mass Melanocytic tumor (Adult) Retinoblastoma (Ped) Choroidal hemangioma Metastasis

45 Adult HyperintenseT1 occular lesion Uveal Melanoma Choroidal hemangioma Key! M/C intraocular malignancy in adult (6 th decade) Moderate enhance HyperintenseT1 (20% amelanoic mimic metas) HypointenseT2 Collar button shape Key! Strong & Early enhance Iso/HyperintesenT1 HyperintenseT2

46 Our case T1FS T2FS Subtract T1FS+Gd Patho: NSCLC metastasis

47 Orbital metastasis Breast, Prostate, Melanoma, Lung Various imaging features CA breast: Scirrhous met Carcinoid tumor: Nodular EOM mass CA Prostate: Bone met

48 Case 8 25 F, Intermittent proptosis right eye

49 Intermittent Proptosis Orbital varix (90%) Highly vascular neoplasms with liable congestion (eg. Angioma, lymphangioma) Recurrent orbital hemorrhage Venous congestion during menstruation or intense muscular effort Recurrent emphysema of orbit

50 What is orbital Varix? Congenital weakness of postcapillary venous wall dilatation of the valveless orbital veins Symptoms: Stress proptosis, Spontaneous intraorbital hemorrhage Imaging: Normal/ mild enlarged vein distend during increased venous pressure, phlebolith

51 Resting Valsava NCCT: Resting Irregular soft tissue Extraconal space Phleboliths Contrast CT: Val Increased size Enhance match venous structure (SOV) Orbital venous Varix

52 Case 9 7 Y boy, Rapid Proptosis right eye

53 T1W T2W T1FS+Gd DWI Patho: Rhabdomyosarcoma

54 DDX Pediatric orbital mass (Non-Globe) Venolymphatic malformation Optic nerve glioma Rhabdomyosarcoma Infantile hemangioma Plexiform neurofibroma Metastasis (Neuroblastoma)

55 Rhabdomyosarcoma (RMS) Metastasis Neuroblastoma Key! M/C extraocular tumor in Ped (6-8 yrs) Well-circumscribed Extraconal/Conal+Extraconal Homogeneous Marked enhance Restricted diffusion Key! Skull metastasis with Sun-ray periosteal reaction

56 Infantile Hemangioma Orbital plexiform neurofibroma (OPNF) Key! M/C vasculogenic tumor in infancy Strawberry-like skin mass Grow and then involute with time (start at 1 yr of age) Infiltrative & Extraconal lesion Thin septa or intralesion/perilesion flow void Key! Target sign on T2W Orbital and periorbital infiltrative soft tissue masses Diagnostic for NF1

57 57

58 Take home message Compartmental base approach is essential Don t forget clinical: Age, Symptoms, Underlying Look for other organ involvement

59 What we saw today Intraconal Conal Extraconal Occular Multicompartment ONG Dermoid HyperT1 met Lymphoma Look for other causes!!! Myotenidinous jc Disp fat reticulation Abn vascular Unusual order EOM Cavernous malforn Lacrimal tumor **Lymphoproliferative disorder & Metastasis can have many features

60 Special thanks to my colleagues and fellows at Ramathibodi for their support!!!

61 References Tina D. Tailor, MD Divakar Gupta, MD Roberta W et.al, Orbital Neoplasms in Adults: Clinical, Radiologic, and Pathologic Review. RadioGraphics 2013; 33: Bela S. Purohit, Maria Isabel Vargas, Angeliki Ailianou et al. Orbital tumours and tumour-like lesions: exploring the armamentarium of multiparametric imaging. Insights Imaging (2016) 7:43 68 Ellen M. Chung, James G. Smirniotopoulos, MD, Charles S. Specht, MD, Pediatric Orbit Tumors and Tumorlike Lesions: Nonosseous Lesions of the Extraocular Orbit, RadioGraphics 2007; 27: