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CT and MRI imaging of intra orbital extra bulbar tumors for deciding clinical approach Ando K, Ishikura R, Kawanaka Y, Katsuura T, Wakata Y, and Hirota S. Dept. of Radiology, Hyogo College of Medicine Noshinomiya, Hyogo, Japan ☑ The author has no conflict of interest to disclose with respect to this presentation. Introduction Various kinds of tumors occur in intra orbital extra bulbar region. Even each tumor is relatively rare among ophthalmic disease, it is important to know imaging characteristics of it, because imaging diagnosis takes important role in deciding clinical approach. 1,2,3) Normal Orbital Anatomy Extraconal Globe Optic nerve and globe Intraconal Lacrimal gland Optic nerve Extra ocular muscles Extra ocular muscles Intra orbital extra bulbar tumors in Japan Primary benign tumors n=323 % Primary malignant tumors n=52 Idiopathic orbital inflammatory disease (IOI) Malignant lymphoma (ML) 8 Adenoid cystic carcinoma 2 Adenocarcinoma 1 25 Refractory (Non neoplastic) Lymphoproliferative disease (RLD) 6 Dermoid cyst 5 Pleomorphic adenoma 5 Venous malformation (cavernous hemangioma) 4 Meningioma 3 Lymphangioma 5 Optic glioma 8 Infantile hemanigoma (capillary hemangioma) 7 % sebaceous gland carcinoma >1 Rhabdomyosarcoma >1 馬詰和比古ら 眼科51:31-35,2009東京医科大学 Schwannoma 2% in US Secondary tumors: Tumors from paranasal sinus, Metastatic tumors, Malignant lymphomas, Wegener's granulomatosis. Orbital tumors and their locations ohotsuka K. et al. Jpn J Ophthalmol. 2005 Jan-Feb;49(1):49-55. 橋本雅人 日本眼科紀要 51 2 2000.02 札幌医科大学 intraconal 50%/extraconal 50% (lacrimal gland 37%) Location Pathology % Intraconal 51% 25% Extraconal 12% Venous malformation (cavernous hemangioma) Optic glioma Meningioma IOI/RLD ML Lacrimal gland 37% ML Pleomorphic adenoma 40% 24% 14% 14% 22% 20% Clinical approaches for intra orbital extra bulbar tumors 1. 2. Basically there are four types of clinical approaches for intra orbital extra bulbar tumors: Observation with imaging follow-ups Total removal of tumor 3. 4. (1 or 2 depends on whether tumor is asymptomatic or symptomatic.) Biopsy of tumor to decide therapies Others Tumor location and surgical approach To visualize tumor location in orbital cavity is also important to decide the way of surgical intervention: Trans cranial approach Lateral approach Trans cranial approach For tumors in Medial to optic nerve, Upper half of orbital cavity Invasive To approach optic nerve or medial to optic nerve: medial to LPS and SRM To approach lateral to optic nerve: lateral to LPS and SRM (Levator palpebrae superioris (LPS) muscle and superior rectus muscle (SRM)) 新井一 脳神経外科33:7-16,2005 東京医科大学 Lateral approach Fort tumors in Lateral to optic nerve, Under half of orbital cavity Less invasive 新井一 脳神経外科33:7-16,2005 東京医科大学 Purpose To show the key findings to determine the clinical approaches is one of the essential roles for imaging. 1-3) In this pictorial essay, we classify intra orbital extra bulbar tumors along with their clinical approaches and clarify CT and MR imaging characteristics of them. Approach/Methods: We retrospectively reviewed 54 cases with intra orbital extra bulbar tumors experienced in our hospital from 20072014. Our cases (pathologically or clinically proven) Benign Malignant Intraconal and/or extraconal Venous malformation (cavernous hemangioma) 4 Infantile hemanigoma (capillary hemangioma) 1 Schwannoma/Neurofibraoma 3 Lymphangioma 1 Malignant lymphoma 11 Acute lymphoblastic leukemia 1 Granulocytic sarcoma 1 Intraconal Optic glioma 3 Optic nerve sheath meningioma 1 Extraconal Dermoid/Epidermoid 6 Adenoid cystic carcinoma Pleomorphic adenoma 4 1 IgG4 related disease 4 Metastatic tumor 0 Refractory lymphoproliferaitve disease (RLD) 1 Solitary fibrous tumor 1 Unknown: 11 cases 1. 2. 3. And classified them into three groups along with the clinical approaches: Observation with imaging follow-ups or total removal of tumor Biopsy of tumor to decide therapies Others. Typical CT and MR imaging findings of them were investigated Findings/Discussion Clinical Approach 1: Observation with imaging follow-ups or total removal of tumors Most of the benign tumors were in this group: Venous malformation, infantile hemangioma, lymphangioma, schwannoma, neurofibroma, epidermoid, dermoid, pleomorphic adenoma. Clinical Approach 1: Tumor localization and imaging findings Tumors located in intraconal or extraconalextra lacrimal gland location except for pleomorphic adenoma, which affected lacrimal gland. They were well circumscribed round or oval mass on CT or MRI except for lymphangioma, infantile hemangioma and neurofibroma. On T2-weighted images, they showed homogenous or mixed hyper intensity. Clinical Approach 1: Observation with imaging follow-ups or total removal of tumors Location Benign Malignant Both in intra and extraconal Venous malformation Malignant lymphoma (cavernous hemangioma) Infantile hemanigoma (capillary hemangioma) Schwannoma/Neurofibraoma Lymphangioma Mainly in intraconal Optic glioma Optic nerve sheath meningioma Mainly in extraconal (Lacrimal gland: LG) Dermoid/Epidermoid Pleomorphic adenoma (LG) IgG4 related disease (LG) Adenoid cystic carcinoma (LG) Metastatic tumor Venous malformation (cavernous hemangioma) One of venous malformation Most common orbital tumor (often incidental) Middle age/ woman T1WI Sometimes enlarge with pregrancy Common in intraconal and lateral T2WI Both intra/extra orbital Gd-enhanced Smooth/lobulated well defined mass 53y.o. Male Hyper dense on CT Hypo to hyper intense on T1WI, hyper intense on T2WI of MRI Gradually well enhanced by contrast media 4) Infantile hemanigoma (capillary hemangioma) (benign hemangioendothelioma) Common in neonate Enlarge until 1-year after birth, decrease in size during 1-5 years after birth. Steroid therapy if there is visual impairment From intra orbital to subcutaneous to intra orbital, sometimes intra cranial Hypo intense on T1WI, hyper intense with septum on T2WI of MRI Well enhanced by contrast media T2WI T1WI Gd-enhanced 4 months girl Case from Wendy R. et al. Radiographics 28: 185-204,2008 CT Schwannoma Only 2% of orbital tumor Middle aged female From Ⅲ、Ⅳ、Ⅴ、Ⅵ、Ⅶ cranial nerve (sensory nerve) Common in intraconal, medial upper T2WI Both intra/extra orbital Well defined ovoid or fusiform tumor Two component: Antoni A/B inhomogeneous hypo dense on CT Target sign on imaging Hyper intense (Antoni A) and hypo intense (Antoni B) on T2WI of MRI. Mixed enhancement on contrast media Strongly enhanced: Antoni B Faintly enhanced: Antoni A T1WI Gd-enhanced 42y.o. female Neurofibroma T2WI Plexiform neurofibroma Neurofibrosis type 1 pediatric to adolescence age extend to frontal and supra orbital Localized neurofibroma 30s-40s pseudo capsule、layer structure (central and peripheral) Low dense on CT Hypo intense on T1WI, hyper intense with central hypo intense on T2WI of MRI. Central enhancement with contrast media. T1WI Gd-enhanced Lymphangioma Neonate-Pediatric age Lymphatic malformation Mono or multi locular mass Infiltrrates orbita to subctaneous, intra cranial heterogenous hypo to hyepr intense both on T1WI and T2WI of MRI Varinous contrast enhancement T1WI Gd-enhanced 笠井健一郎等 眼窩疾患 1580-1590眼科画像診断最近の進歩 眼科 2010年9月臨時増刊号 Epidermoid/Dermoid Epidermoid 3 y.o. girl Well defined cystic mass Often with bone erosion Epidermoid: Low dense on CT, hypo intense on T1WI, hyper intense on T2WI, hyper intense on DWI, may have peripheral enhancement Dermoid: With fat and calcification T1WI T2WI 藤田晃史 頭頚部の画像診断 P158-159 78 y.o. female Pleomorphic adenoma Lacrimal gland Female>male 10% with malignant change Often with incomplete resection Often with bone erosion Inhomogeneous lobulated mass both on CT, and MRI Inhomogeneous contrast enhancement Differential diagnosis: Adenoid cystic carcinoma CT T2WI T1WI Gd-enhanced Clinical Approach 1. Observation with imaging follow-ups or total removal of tumors Benign tumors Well-circumscribed round or oval mass Homogenous or mixed hyper intensity on T2WI Venous malformation Neurinoma Pleomorphic adenoma Neurofibroma Lymphangiom Caution Malignant change of pleomorphic adenoma Adenoid cystic carcinoma Well defined round or ovoid mass with smooth surface Calcification, Cystic change Bone distraction Heterogeneous inside Rapid growth with pain may be a marker. Clinical approach 2. Biopsy of tumor and following treatment decided by pathologic findings Lymphoproliferative disorders (LPDs) and other malignant tumors were in this group: malignant lymphoma, reactive lymphoid hyperplasia, leukemia, IgG4 related disease, adenoid cystic carcinoma and metastatic tumors. Clinical approach 2: Tumor localization and imaging findings They were both in intra- and extra-conal-intralacrimal gland location, often diffusely involved orbital cavity including extraocular muscles and bilateral lacrimal glands. They show homogenous hypo intense on T2weighted images, and hyper intense on DWI, except for adenoid cystic carcinoma, which showed mixed hyper intense on T2-weighted images. Metastatic tumors were often with bony destruction. Clinical approach 2. Biopsy of tumor and following treatment decided by pathologic findings Location Benign Malignant Both in intraconal and extraconal Venous malformation Malignant lymphoma (cavernous hemangioma) Infantile hemanigoma (capillary hemangioma) Schwannoma/neurofibraoma Lymphangioma Mainly in intraconal Optic glioma Optic nerve sheath meningioma Mainly in extraconal (Lacrimal gland: LG) Dermoid/epidermoid Pleomorphic adenoma (LG) IgG4 related disease (LG) Adenoid cystic carcinoma (LG) Metastatic tumor Malignant lymphoma T2WI 10-20% of orbital tumor Most common malignant tumor Mostly B-cell non Hodgkin lymphoma Eye lid, conjunctiva, lacrimal gland, extraocular muscles, intraconal. Bilateral in 25% 50s to 70s Well defined mass to diffusely infiltrative Hyper dense on CT Homogenous on MRI: hyper intense on T1WI, hypo intense on T2WI, hyper intense on DWI, homogenously enhanced with contrast media No bone destruction Difficult to differentiate malignant lymphoma from IOI, RLD, Malt lymphoma, and IgG4 related disease. Mafee MF:Orbit and visual pathways;Eyes and orbit.In:Head and Neck Imaging,Third Edition, Som PM, Curtin HD. Mosby-year Book,Inc 1996,p1059-1128. Various manifestations of Malignant lymphomas CT Gd-enhanced CT T2WI Malignant lymphoma in bilateral lacrimal gland Bilateral lacrimal gland Hypo intense on T2WI Homogenous enhancement T2WI STIR 66 y.o. female T1WI Gd-enhanced ALL involved bilateral lacrimal gland Bilateral lacrimal gland Hypo intense on T2WI Homogenous enhancement CT T2WI T1WI Gd-enhanced 11 y.o. girl Granulocytic sarcoma 2 y.o. boy Hypo intense on T2WI Homogenous enhancement T1WI T2WI CT Gd-enhanced IgG4 related disease (IgG4-RD) Mikulicz disease or IgG4 related chronic sclerosing dacryoadenitis 40s -60s Serum IgG4↑ Part of systemic IgG4-related disease Chronic sclerosing sialadenitis, autoimmune pancreatitis, retro peritoneal fibrosis Bilateral lacrimal gland Involvement of infraorbital nerve (←) Homogenous on CT, MRI Hypo intense on T2WI Homogenous contrast enhancement T2WI 53 y.o. female Idiopathic orbital inflammatory disease (IOI) ① So-called inflammatory pseudo tumor One of the manifestation of IgG4 related disease Idiopathic orbital inflammatory disease ② Idiopathic orbital inflammatory myositis Diffusely infiltrative Sudden onset flare, pain, swelling Swelling of extra ocular muscle Acute(steroid-responsive)/chronic T2WI Differential diagnosis:thyroid ophthalomology, Malt lymphoma, RLD, metastatic tumor Homogenous mass with diffuse infilteration or swelling of extra ocular mass on MRI T2WI Lymphoproliferative diseases LPDs Difficult to differentiate, But always homogenous on MRI ① Refractory lymphoproliferative disease (RLD) ② MALT lymphoma ③ IgG4 related disease ←1 ← 2 ↓3 ↓3 Adenoid cystic carcinoma Lacrimal gland Mimics pleomorphic adenoma But with rapid growth Infiltrates along nerve →pain Well defined round or ovoid mass with smooth surface Calcification, Cystic change Bone destraction Mixed density on CT Mixed intensity on MRI Moderate enhancement CT CT CE-CT 68 y.o. male Clinical Approach 2. Biopsy of tumor to decide therapies Lymphoproliferative disorders and other malignant tumors Diffuse T2WI & DWI hypo-intense lesion involving extraocular muscles and/or bilateral lacrimal glands Exception: Adenoid cystic carcinoma (localized) Pleomorphic adenoma Malignant lymphoma in bilateral lacrimal gland IgG4 related disease Adenoid cystic carcinoma Clinical approach 3. Others Different clinical approaches were chosen for optic glioma and optic nerve sheath meningioma Because these might cause optical impairment either by tumor progression or by therapeutic intervention. Both are in intraconal location, and extend along optic nerve. Clinical approach 3. Others Location Benign Malignant Both in intraconal and extraconal Venous malformation (cavernous hemangioma) Infantile hemanigoma (capillary hemangioma) Neurinoma/neurofibraom a Lymphangioma Malignant lymphoma Mainly in intraconal Optic glioma Optic nerve sheath meningioma Mainly in extraconal (Lacrimal gland: LG) Dermoid/epidermoid Adenoid cystic carcinoma Pleomorphic adenoma (LG) (LG) IgG4 related disease (LG) Optic glioma 4-8% of orbital tumor, 2/3 of optic nerve tumor Mostly pilocytic astrocytoma WHO grade I Visual impairment 90% in pediatric age especially <10 y.o. 10-38% of optic glioma with NF-1 15-40% of NF-1 with optic glioma Fusiform enlargement of optic nerve Hypo intense on T1WI, hyper intense on T2WI of MRI Various enhancement Cystic (without NF-1), bilateral and extend to optic tract (with NF-1) Optic glioma (without NF-1) 49 y.o. female 術後残存腫瘍 T2WI T2WI DWI T1WI Optic glioma (with NF-1) 12 y.o. female T2WI T1WI Optic nerve sheath meningioma 3% of orbital tumor 30s-50s female From optic nerve sheath Sometimes with NF-2 9 y.0. boy without NF-2 T2WI Thickening of optic nerve Calcification on CT (30%) Tram track sign on contrast enhanced study Gd-enhanced T1WI Clinical approach 3. Others Optic glioma and optic nerve sheath meningioma Intraconal location, and extending along optic nerve Pleomorphic adenoma Optic glioma Optic nerve sheath menigioma Orbital tumors and their clinical approaches 1, 2, 3 Location Benign Malignant Both in intraconal and extraconal Venous malformation (cavernous hemangioma) Infantile hemanigoma (capillary hemangioma) Neurinoma/neurofibraom a Lymphangioma Malignant lymphoma Mainly in intraconal Optic glioma Optic nerve sheath meningioma Mainly in extraconal (Lacrimal gland: LG) Dermoid/epidermoid Adenoid cystic carcinoma Pleomorphic adenoma (LG) Metastatic tumor (LG) IgG4 related disease (LG) Clinical Approach 1. Observation with imaging follow-ups or total removal of tumors Benign tumors Often well-circumscribed round or oval mass Exceptions: Neurofibroma, Infantile hemanigoma, lymphoangioma Homogenous or mixed hyper intensity on T2WI Venous malformation Pleomorphic adenoma Schwannoma Neurofibroma Lymphangiom Caution Malignant change of pleomorphic adenoma Adenoid cystic carcinoma Well defined round or ovoid mass with smooth surface Calcification, Cystic change Bone distraction hetrogenous inside Rapid growth with pain may be a marker. Clinical Approach 2. Biopsy of tumor to decide therapies Lymphoproliferative disorders and other malignant tumors Diffuse T2WI & DWI hypo-intense lesion involving extraocular muscles and/or bilateral lacrimal glands Exception: Adenoid cystic carcinoma (localized) Pleomorphic adenoma Malignant lymphoma in bilateral lacrimal gland IgG4 related disease Adenoid cystic carcinoma Clinical approach 3. Others Optic glioma and optic nerve sheath meningioma Intraconal location, and extending along optic nerve Pleomorphic adenoma Optic glioma Optic nerve sheath menigioma Summary/Conclusion CT and MRI imaging bring key findings to decide clinical approach for intra orbital extra bulbar tumors. Well circumscribed intraconal or extraconal tumors showing hyper intense on T2weighted on MRI need for observation with imaging follow-ups if they are asymptomatic or total removal of tumors if they are symptomatic. Often they are benign tumors except for adenoid cystic carcinomas which need biopsy. Tumors with diffuse extension in orbital cavity, especially with involvement of extraocular muscles and/or both lacrimal gland showing hypo intense on T2-weighted images and hyper intense on DWI need for biopsy to decide following therapies. Often they are LPDs and IgG4-RD. Tumors extend along optic nerve need different clinical approaches. They are optic gliomas and optic nerve sheath meningiomas. References 1. 2. 3. 4. Hassler W, et al. Orbital Tumors: Diagnosis and surgical treatment. Dtsch Arztebl 2007;104:A496-501 Markowski J, et al. Primary orbital tumors: a review of 122 cases during a 23-year period: a histo-clinical study in material from the ENT Department of the Medical University of Silesia. Med Sci Monit. 2014; 20:988-94. Rao AA1, et al. A clinical update and radiologic review of pediatric orbital and ocular tumors. J Oncol. 2013;2013:975908. doi: 10.1155/2013/975908. Atsuo Tanaka, et.al. Differentiation of Cavernous Hemangioma from Schwannoma of the Orbit:A dynamic MRI study. AJR:183.1799-1804,December 2004 Thank you for your attention.