Central Annals of Otolaryngology and Rhinology

Review Article *Corresponding author

Jorge Rodrigues, Department of Otolaryngology, Senhora da Oliveira Hospital, Rua da Lagoa nº53,4905- Differential Diagnosis of Clival 396 Barroselas, Viana do Castelo, Portugal, Tel: 00351- 964167236; Email: Lesions – Literature Review Submitted: 21 October 2017 Accepted: 27 November 2017 of the Clinical and Radiological Published: 30 November 2017 ISSN: 2379-948X Copyright Features © 2017 Rodrigues et al. Jorge Filipe Rodrigues1,2*, Francisco Moreira da Silva1, João OPEN ACCESS

Mangussi-Gomes3,4, Felipe Marconato3, Camila Soares Dassi3, Keywords Leonardo Balsalobre3,4, and Aldo Stamm3 • Clivus • Clival lesions 1Department of Otorhinolaryngology, Senhora da Oliveira Hospital, Portugal • Chordoma 2Department of Anatomy, University of Porto, Portugal • Chondrosarcoma 3Department of Otorhinolaryngology, Complexo Hospitalar Edmundo Vasconcelos, • Metastasis Brazil 4Department of Otolaryngology - Head and Neck Surgery, Federal University of São Paulo, Brazil

Abstract Background and objectives: Solitary lesions of the clivus are uncommon and represent a diagnostic and therapeutic challenge. There is a wide range of entities, with several behaviours, that can affect the central region of skull base. Imaging studies constitute an important tool in differentiating between malign, benign and non-tumoral lesions. The aim of this study is to provide an overview of the clinical and radiological characteristics of clival lesions. Subjects and methods: A literature review was conducted, with special attention to clinical and imaging features of the different diseases that can affect the clivus. Clinical examples of some cases are also given. Results: Chordoma is the most frequent lesion in this location, although they are difficult to distinguish from chondrosarcomas or metastases on CT scans and MR images. Benign neoplasms can also be life-threatening and clinically undistinguishable from malignancies. Non-tumoral lesions can mimic neoplasms and lead to unnecessary aggressive treatments. Conclusion: Clival disorders comprise a large range of entities with different characteristics and behaviors. Imaging study allows an evaluation of the tumor extent, a reliable distinction between benign and malignant processes and helps in the suspicious diagnosis. Despite imaging is an important supporting element, a final diagnosis often cannot be found without histologic biopsy.

INTRODUCTION

are different studies describing neoplastic processes – like chordoma, plasmacytoma, multiple myeloma, pituitary adenoma, The skull base forms the floor of the cranial cavity and chondrosarcoma, meningioma and metastatic tumors – or separates the brain from facial structures. This complex region is tumor-like conditions affecting the clival region – such as fibrous composed by five bones and can be subdivided into three regions: dysplasia. Most of these studies, however, are limited to single the anterior, middle, and posterior cranial fossae. Tumors that case reports or case series. Revision papers on this matter are arise in the skull base are usually classified based on this division. scarce. The posterior cranial fossa is composed by the occipital bone, Although most of the lesions show a benign behaviour, clival with contributions from the sphenoid and temporal bones. The diseases can be potentially devastating and life-threatening [3,4]. basilar part of the occipital bone and the posterior portion of These tumours usually present as incidental imaging findings. the sphenoid body form the clivus, which constitutes the most Patients may complain of headache or cranial neuropathies, anterior region of the posterior fossa. The clivus occupies a especially diplopia due to abducens nerve palsy. Then, other central position in the skull base, establishing close relationships cranial nerves or the brainstem can be affected by compression with the brainstem and other noble structures [1,2]. and invasion. Lesions that arise from or within the clivus are rare and Given the poor clinical presentation, imaging plays a pivotal represented by a wide range of different diseases. There role in achieving a correct diagnosis. High resolution computed Cite this article: Rodrigues JF, da Silva FM, Mangussi-Gomes J, Marconato F, Dassi CS, et al. (2017) Differential Diagnosis of Clival Lesions – Literature Review of the Clinical and Radiological Features. Ann Otolaryngol Rhinol 4(9): 1200. Rodrigues et al. (2017) Email: Central tomography (CT) and magnetic resonance imaging (MRI) are helpful to differentiate between non-tumoral and tumoral and vascular structures. Lateral to the clivus is a groove for the conditions and in searching for malignant characteristics. They the skull base, the clivus has close relationships with nervous also evaluate the extension of the lesions and the invasion of adjacent structures. CT is more sensitive in the assessment betweeninferior petrosalthe clivus sinus, and pons. the foramenThe abducent magnum nerve and arises the from internal the of trabecular and cortical bone destruction and MRI allows carotid arteries. Superiorly, basilar artery and its branches run visualization of bone marrow and soft tissue involvement [5]. piercing the dura covering the clivus. Continuing upward in brainstem between the pons and medulla and passes forward, Scintigraphy permits a functional approach in the research of other bone lesions. The Fluorodeoxyglucose - Positron Emission the Dorello’s canal, it crosses the superior edge of the petrous Tomography/Computed Tomography (FDG-PET/CT) scanning temporal bone, enters and crosses the cavernous sinus [1, 2, 7]. technique may be helpful in the evaluation of metabolic activity notochord in the cephalic region of the fetus has begun to of the tumor and in the investigation of other involved structures. The clivus develops by endochondral ossification. The begin in the future basioocciput and spread superiorly to unite A correct differential diagnosis of a clival lesion is important withdevelop cartilaginous by the third centres week of in gestation. the region Chondrofication of the basiosphenoid. centres to define the most effective therapeutic or surgical approach. For By the sixth week of fetal life, the basal portion of the sphenoid a definitive diagnosis, biopsy or resection of the lesion followed bone contains one or two ossification centers. At birth, the clivus by histopathologic analyses are usually necessary. However, this consists of partially ossified components of the basioocciput and task can sometimes be difficult. That is why an accurate diagnosis The synchondroses between the basilar and the lateral parts pathologists in a real multidisciplinary team work [6]. sphenoid body, separated by the spheno-ocipital synchondrosis. needs a close cooperation between surgeons, radiologists, and This study aims to review the main differential diagnosis of the lesions that typically arise from the clivus and describe the of the occipital component usually close very early, at between imaging characteristics that can be helpful to perform a correct one and four years. However, the spheno-ocipital synchondrosis diagnosis. closes between 18 to 25 years. This synchondrosis, which is one of the last sutures in the body to fuse, has an important role in the - Hyperintensity on T2-weighted images and strong postnatallyNormal Imaging growth of of the the skull Clivus base [1,2]. contrast enhancement with gadolinium suggest chordoma and chondrosarcoma. Imaging study is determinant in detection and diagnosis of clival lesions. High resolution CT-scan and MRI are complementary techniques necessary to clarify and characterize chondrosarcoma is negative for cytokeratin and EMA. the lesion. The clivus is best analysed in the sagittal and axial - Chordoma reacts with cytokeratin and EMA, whereas - Chordomas commonly enhance markedly with the dorsum sellae and the apex pointed to the foramen magnum. view. In a sagittal view, clivus seems a triangle with the base in with contrast addition. A normal clivus appears on the CT-scan as a central portion gadolinium, although EP does not show any enhancement DISCUSSION & CONCLUSION signalof cancelllous intensity bone depends with on marrow the nature bone of elements, the marrow bounded in the Anatomy and Embryology by peripheral compact cortical bone. On the normal MRI, the the predominance of the hematopoietic cells. With advancing and it meaning “slope or declivity”. The clivus is a sloping bony cancellous bone. Young patients reveal a low T1 signal due to structureThe term that clivus forms was the firstlyanterior used portion by von of Sommerringthe posterior in cranial 1791 age, occurs conversion of red to yellow bone marrow. With the base and is bounded posteriorly by the margin of the foramen clivusfatty transformation relative to the of pons the bone should marrow, be considered the T1 signal suspicious becomes in fossa, in a median position. It occupies a central part in the skull brighter in adults. Thus, hypointense T1-weighted images of the with the sphenoid sinus. The lateral margins are formed by the magnum. The anterior margin is not well defined, since it blends notadults. show On a T2-weightedconsiderable enhancement sequences, clivus in healthy is usually patients isointense [5]. of the temporal bones. The exocranial surface of the clivus is relative to the pons. Commonly, contrast with gadolinium does petro-occipital fissures, separating it from the petrous portion Differential Diagnosis giving rise to the pharyngeal tubercle and faces the roof of the Clival lesions comprise tumoral and non-tumoral pathologies. nasopharynx.roughened by the The attachments intracranial of surface the fibrous is smooth raphe of and the facespharynx, the Malignant neoplasms are more common than benign tumours in portion of the body and then the dorsum sellae of the sphenoid can also be life threatening by compression of noble structures. pons and medulla [1,2]. The clivus is composed by the posterior Chordomasthis location. are Despite the most the benign frequent behaviour in this regionof some and entities, represent they about 40% of all cases. Non-chordomatous lesions are rare bone, also called basisphenoid, and the basilar portion of the thirdoccipital is formed bone, by denominated the basisphenoid basiocciput. bone and This it is bony at the structure level of thecan sphenoidbe divided sinus. into Theupper, middle middle, and and lower lower thirds thirds. are Theformed upper by and much diversified [8]. The main differential diagnoses are the basiocciput and are divided by a line connecting the caudal presentedPituitary in Adenomas the Table (1). Pituitary adenomas are the most common cause of sellar and ends of the petroclival fissures. Given its central position in Ann Otolaryngol Rhinol 4(9): 1200 (2017) 2/8 Rodrigues et al. (2017) Email: Central

Table 1: Differential diagnosis. Malign Neoplasms Benign Neoplasms Non-tumoral lesions all cases demonstrate calcifications. CT-scan images may also Echordosis Chordoma Pituitary adenoma MRIbe helpful is generally in determining isointense if the to slightlymeningioma hypointense invades relative the bone, to Physaliphora theshowing brain. an Meningiomas intense hyperostotic vary from bonyhypointense response. to hyperintense T1-weighted Petroclival Chondrossarcoma Fibrous dysplasia meningioma Metastasis Osteoblastoma Dermoid cyst on T2-weighed images. Approximately 50% are isointense, 40% Multiple myeloma Condroblastoma Epidermoid cyst beinghyperintense the most and sensitive 10% hypointense method forrelative detecting to the meningiomas. gray matter. Plasmocytoma Arachnoid cyst AngiographyThey reveal a with strongly vertebral and homogeneous and carotid studies contrast is usually enhancement, needed Lymphoma Neurenteric cyst to elucidate the blood supply to the tumor and their relation with Osteosarcoma Arachnoid herniation theFibrous major Dysplasiaarterial branches [15, 16]. FD is a developmental disorder characterized by the parasellar mass, comprising about 10 to 15% of all intracranial tumors. Despite histologically benign, they have the capacity to progressive replacement of mature bone by fibrous tissue, caused invade adjacent structures, such as: sphenoidal sinus, cavernous benign disease of unknown etiology results in the formation of by an abnormal proliferation and maturation of fibroblasts. This pituitarysinus, skull adenomas base or nasopharynx. are extremely The clivus unusual invasion and ariseis rare from and basisphenoid is the most commonly affected portion [9]. Ectopic two decades of life and it may be present in the monostotic form residual cells along the migration tract of the anterior portion woven and structurally weak bone. FD is more frequent in the first involving multiple bones. The polyostotic form can also occur as a to the sellaturcica. Ectopic locations include the suprasellar (70%), affecting a single bone, or in the polyostotic form (30%), of the pituitary gland, during passage from Rathke’s pouch part of McCune-Albright syndrome [17,18]. Craniofacial skeleton region, sphenoid sinus, cavernous sinus and clivus. In these ectopic clival pituitary adenomas are exceedingly rare. Pituitary is involved in 30% of patients with monostotic disease and in cases pituitary gland is commonly normal. However, purely 50% of patients with the polyostotic variant, affecting sphenoid, frontal, ethmoid, temporal and maxilla bones. FD rarely affects adenomas classically present with bitemporal hemianopsia, the clivus and when it occurs, does not seem to produce any however ectopic adenomas usually do not, unless they involve functional limitation or aesthetic problem. However, clivus the optic chiasm. Headache is a common complain, but it is other lesions. Invasive and ectopic pituitary adenomas can involvement may present headache, neck pain, craniocervical unspecific and cannot distinguish a pituitary adenoma from manifest as a clival neoplasm in the imaging study and lead to instability or hypoglossal nerve palsy [17,18]. high-resolution CT scan is the tool of choice. The characteristic Diagnosis of FD is based in the imaging findings and the misdiagnosis [10-12]. CT-scan typically reveals an enhancing hypointensesoft-tissue mass mass that that shows enhances bone after destruction. administration On T1-weighted of contrast appearance of the disease is ground-glass opacity, ballooning of MRI, pituitary adenomas demonstrate an isointense to slightly the affected bone and thinning of the cortex. However, FD may present several patterns: ground glass, sclerotic, cystic or mixed (Figure 1). On T2-weighted images, they range from isointense signalpattern. intensity The MRI on appearance T2-weighted is images.less specific Contrast than enhancement CT. FD usually is to hyperintense, unless they contain intratumoral hemorrhage or shows low signal intensity on T1-weighted images and variable cysticPetroclival changes Meningioma [13]. Meningiomas are benign and slow-growing tumors that cystintermediate. formation. This Clival variation isolated is determined lesions with by low the ordisease intermediate activity, originate from the arachnoidal cells. The majority of them occur overall cellularity, extent of bone trabeculae, mineralization and intracranially and they account for about 20% of all primary intracranial tumors in adults. Meningiomas are more frequent signal intensity on T2-weighted MR imaging are rare. However, in women and their incidence increases with age. Intracranially when FD presents high intensity on T2-weighted images, differentialCysts and diagnosisHerniations is much more complex [19, 20]. Cysts are other important differential diagnoses in clival canmeningiomas be affected. can Meningiomas be classified at accordingthe center placeof the ofclivus origin proper and arecellular very type. uncommon. The clivus Petroclival is covered meningiomas with dura mater, arise in therefore the upper it and arachnoid cysts. lesions. They can be divided in epidermoid, dermoid, neuroenteric Epidermoid cysts are congenital lesions which result from the trigeminal nerve. They have a close relationship with the inclusion of ectodermal elements during neural tube closure. brainstemtwo thirds and of thelarge clivus tumours at thecan petroclivaldisplace it or junction, encase the medial basilar to They have a thin capsule of squamous epithelium and they can artery. The symptoms depend on the size of the tumor and they usually develop as a result of compression of surrounding with desquamated epithelial keratin and cholesterol crystals. structures. These patients commonly present with insidious be smooth or lobulated. Internal components are often filled

Epidermoid cysts are usually less sharply defined than arachnoid onset of headache, seizures, unilateral hearing loss, facial sensory intensity tends to increase with the lipid content. The lesions are a hyperdense enhancing lesion and approximately a quarter of cysts and more heterogeneous. On MRI, the T1-weighted signal disturbances or trigeminal neuralgia [14,15]. The CT-scan reveals

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Figure 1

Coronal and sagittal T1-weighted images revealing a giant pituitary adenoma. Table 2: Imagological characteristics of different lesions. Contrast Type of Histological Diagnosis CT T1-MRI T2-MRI Enhancement Enhancement Chordoma Osteolytic Hypo/Isointense Hyperintense High Heterogeneous Chondrosarcoma Osteolytic Hypointense Hyperintense High Heterogeneous Metastasis Osteolytic/ Osteoblastic Hypointense Hypointense High Heterogeneous Lymphoma Osteolytic/ Osteoblastic Hypointense Hyperintense High Homogeneous Pasmocytoma Osteolytic Hypointense Hyperintense High Heterogeneous Multiple Myeloma Osteolytic Hypointense Hyperintense High Heterogeneous Osteossarcoma Osteolytic Isointense Hyperintense High Homogeneous Pituitary Adenoma Osteolytic Hypo/Isointense Variable Variable Heterogeneous Osteoblastic Petroclival Meningioma Hypo/Isointense Variable High Homogeneous

Condroblastoma Osteolytic Hypo/Isointense Variable Variable Variable Osteoblastoma Osteolytic Hypointense Hyperintense High Heterogeneous EchordosisPhysalyphora Focal Sclerosis Hypointense Hyperintense No No Osteoblastic Fibrous Dysplasia Hypointense Variable Moderate Heterogeneous Several Paterns Cyst Osteolytic Variable Variable Variable Variable

weighted images. Epidermoid cysts present markedly restricted usually isointense relative to cerebrospinal fluid (CSF) on T2- the margins of the lesion are well defined and sharp. They are unilocular, homogeneous, non enhancing and noncalcified and diffusion,Dermoid different cysts from are inclusion arachnoid cysts cysts composed [21]. of ectodermal they present CSF density. On MRI, the signal is usually isoinense on T1andIntracranial T2-weighted hypertension images can [24]. originate arachnoid herniations attenuation values are suggestive of the diagnosis of dermoid or lead to spontaneous CSF leaks. Theses nontraumatic osseous elements such as hair, fluid, skin glands and rich in fat. Low defects occur most frequently within the ethmoid and sphenoid cyst on CT. They also are hyperintense on T1-weighted images wall of the sphenoid sinus allowing for small arachnoid pits to Neurenteric cysts are endoderm derived lesions with ovoid/ and hypointense on T2-weighted images, similar to fat [22]. herniatesinus. In into the the clivus, sinus the [24]. defects usually penetrate the posterior lobulated aspect. Neurenteric cysts of the clivus are unusual Chordoma lytic lesion with intact cortex. The signal intensity depends of thecauses protein for clivalcontent. masses. They Onare CT-scan,isointense they to slightly usually hyperintense appear as a Chordomas are slow-growing malignant tumors that arise from notocord remnants along the length of the neuraxis. These enhancement. Neurenteric cysts may show mild restriction on compared with CSF on T1 W images, without contrast appearsneoplasms to havebe responsible an incidence for aboutabout 0.0840% perof all 100,000 chordomas and theyand diffusion-weightedArachnoid cysts images are [23]. avascular lesions containing CSF. are more frequent in fourth and fifth decades of life. Skull base

the clivus is commonly involved location [25, 26]. Chordomas Arachnoid cysts at the clivus are extremely rare. On the CT-scan, are locally aggressive and tend to recur after surgical resection, Ann Otolaryngol Rhinol 4(9): 1200 (2017) 4/8 Rodrigues et al. (2017) Email: Central however they rarely metastasize to distant sites. Patients with primary and metastatic cancer. FDG-PET/CT has an important secondary to abducent nerve palsy. Progressive compression and roleHowever, in identifying imaging analone unknown does not primary allow or differentiation progression ofbetween a prior localClival invasion chordoma of the usually lesion present may lead with to other headache lower andcranial diplopia, nerve Lymphoma, Plasmacytoma and Multiple Myeloma mass arising from the intraosseous part of the clivus and causing tumor [33,34]. extensivedeficits [7, lytic26]. CT-scan bone destruction typically shows and an showing expansive intratumoral soft-tissue less than 5% of all primary bone tumors. Femur and pelvis are heterogeneous enhancement following contrast administration. Primary bone lymphoma is a rare malignancy, accounting for calcifications. Surrounding sclerosis is not common. There is be involved. CT-scan reveals a lytic or blastic-sclerotic pattern withthe most cortical common breakthrough affected locations, and periosteal although reaction. the clivus MRI can reveals also At MRI, chordomas are isointense to slightly hypointense on T1- 2).weighted Contrast images addiction and hyperintense reveals a strong on T2-weighted enhancement images, in bothwith images. These areas generally appear bright on T2-weighted areas of decreased signal intensity due to calcifications (Figure areas of low signal intensity within the marrow on T1-weighted techniques, distinguish it from Ecchordosisphysaliphora (EP) sequences, as well as peri-tumoraloedema and reactive marrow [26-28].Chondrosarcoma changesPlasmacytoma [35]. and multiple myeloma are clonal neoplasms of terminally differentiated plasma cells. Plasmacytoma is a Chondrosarcomas are malignant mesenchymal tumors localized collection of plasma tumour cells and usually presents composed of cartilage-producing cells. They can be developed as a solitary lytic lesion. Multiple myeloma is a multifocal and destructive tumour involving the axial skeleton with features of or can be associated with other osseous diseases. These tumors de novo, resulted of a malignant transformation of a chondroma are more frequent in men in the fourth decade of life. Intracranial pain and serum monoclonal protein levels. The most commonly osteolytic lesions, pathological fractures, hypercalcaemia, bone from chondrocytes within cartilaginous remnants in petroclival andchondrosarcomas spheno-occipital are synchondroses. more common They in the represent skull base, about arising 6% of affected bones are the vertebrae, ribs, skull and pelvis. In the appearskull base, as a plasmacytomaspunched-out lytic can lesion affect without clivus as sclerotic a solitary rims lesion and of challenging diagnosis [36]. On CT-scan, these neoplasms localskull base aggressiveness. neoplasms and Skull they constitute base chondrosarcomas a challenge, given usually their location. Despite they are overall slow growing, they manifest generally show a focal hypointense area and T2-weighted present with insidious onset headache and cranial nerve palsies imagesan expansive and gadolinium-enhanced soft-tissue mass. On images MRI, present T1-weighted a hiperintense images focal lesion relative to the hypointense marrow background. such as hoarseness, dysphagia, diplopia, facial dysesthesia or Scintigraphy has a limited interest because mainly detect osteoblastic response of the skeleton and plasma cells tumours ahearing sharp zone loss [29,30].of transition CT findings to normal include tissue. enhancing CT-scan is soft-tissueuseful for mass with a calcified matrix, bone erosion and destruction and

activeare osteolytic lesion and lesions, allows underestimating the detection of the other extent involved of the places disease in detecting subtle calcifications in the matrix when the diagnosis is [36]. The FDG-PET/CT shows focal uptake in the metabolically in doubt. On MRI, lesions are hypointense on T1-weighted images multiple myeloma. FDG-PET/CT also reveals different patterns commonlyand hyperintense exhibit on heterogeneous T2-weighted images, internal relative areas ofto decreasedthe brain (Figure 3). The contrast enhancement is very strong. Neoplasms of Fluorodeoxyglucose uptake, according the activity of the tumour. Plasmacytomas show a mild-intense signal, helping in Metastasis signal, corresponding to internal calcifications [31]. theOsteosarcoma differential diagnoses of solitary clivus neoplasms [37]. Metastatic lesions to skull base are infrequent. Clivus Osteosarcomas are the most common primary bone malignancies. They are highly aggressive lesions that comprise head and neck cancer. Hematogenous spread of cancer cells metastases commonly arise from prostate, lung, breast and osteoid-producing spindle cells. Osteosarcomas usually occur in

Fibrous dysplasia or prior radiation. Predominant locations are communicatesto the clivus can superiorly occur via with two the routes: basilar a)plexus through at the the clivus; Batson b) the second decade of life and may be associated with Paget disease, plexus into the internal vertebral venous plexus system, which the metaphysis of long bones but skull base can also be affected. CT-scan reveals a destructive mass involving the clivus with sinus and communicates with the inferior petrosal sinus and basilaror via the plexus. inferior Cranial ophthalmic neuropathies vein, which are drainthe main into theexpression cavernous of skull base metastases and abducens palsy can occur in the clivus appearancemultiple ossifications and T2-weighted and slight images contrast are enhancement. slightly hyperintense On MRI, T1-weighted images show an isointense mass with expansive CT-scan of the clivus metastases reveals areas of lytic invasion [31,32]. [38].Chondroblastoma Chondroblastomas are benign primary bone tumors that bone marrow destruction, by material except of prostatic decreased cancer, signal whichintensity. is usuallyOn T2- originate from chondroblasts. They typically develop in the osteoblastic. T1-weighted MRI shows replacement of normal the addition of contrast material usually reveals enhancement. also be affected. Chondroblastomas usually present in the second weighted images, metastases are generally hyperintense and epiphyses of long bones, however skull base including clivus can

Ann Otolaryngol Rhinol 4(9): 1200 (2017) 5/8 Rodrigues et al. (2017) Email: Central the clivus the patient can refers diplopia and headache. CT-scan decade of life, with a male predominance. When they implicate destruction, different from chordomas. EP is well-circumscribed afterand homogeneouslygadolinium addition. hypointense This lack onof enhancement T1-weighted is images also useful and reveals an osteolytic lesion with areas of punctate calcifications tohyperintense distinguish onit from T2-weighted chordomas images, and other without malignant enhancement tumours and sclerotic margins. On the MRI, they usually have low-to intermediate Intensity on T1 and low to-high intensity on T2 with variableOsteoblastoma contrast enhancement following gadolinium [39]. [42,DIFERENTIAL 43]. DIAGNOSIS Osteoblastomas are uncommon primary bone neoplasms Clivus is a sloping structure that forms the anterior portion with slow growing and benign behaviour. Clivus involvement

disordersof the posterior typically cranial affect fossa, the clivus. in the Clival center lesions of the are skull rare base. and is uncommon, but it can occur. CT-scan presents a well- Due to its strategic location and its embryologic origin, some hopyintensecircumscribed and osteolytic T2-weighted and multiloculated images are hyperintense mass, mottled with by contrastcalcifications enhancement and with [40].an intact cortex. T1-weighted images are they represent a wide range of pathologies, including non- tumoral lesions, benign and malign neoplasms. Chordomas Ecchordosis Physaliphora account for about 40% of all entities, although it is often difficult Ecchordosisphysaliphora (EP) is a tumor-like congenital to distinguish them from non-chordomatous lesions [2, 8]. Clinical symptoms and signs of clival disease are nonspecific or absent. Headache and cranial neuropathies, especially abducent sacrococcygealmalformation which region. arises Although from ectopic these remnantsnotochordal usually remnants, have nerve palsy, are the most common complains. Furthermore, located along the midline craniospinal axis, from the clivus to the theseImaging lesions study often constitutes present as a an useful incidental tool in differentialimaging finding diagnosis. [3]. the dorsal wall of the clivus. Intracranial EP is typically found CT-scan is the best exam for evaluating cortical bone and for inan the intraosseous prepontine location, cistern and they attached occasionally to the perforatedorsal wall through of the demonstrating the detailed anatomy of the cortical margins of clivus by a small pedicle. EP is habitually asymptomatic and

andthe clivus. subsequent However, trabecular CT has lessbone sensitivity loss has in occurred. evaluating CT-scan bone found incidentally, however tumor expansion and compression marrow space involvement until significant marrow infiltration of surrounding structures were reported in some cases [41,42]. is important in primary bone lesions, distinguishing bone On CT-scan, these lesions reveal focal sclerosis without bone tumors from fibrous dysplasia or other bone disease. One the

Figure 2 Intra-operative images of a clivalchordoma on neuronavigation system.

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Figure 3 Coronal and axial T2-weighted images showing an extensive Petroclival condrosarcoma.

CONCLUSION signal abnormalities in the skull base and in discriminating soft other hand, MRI is more sensitive in assessing bone marrow Clival disorders comprise a large range of entities with relative to the pons should be considered suspicious. On T2- different characteristics and behaviors. The knowledge of these tissue. In adults, hypointense T1-weighted images of the clivus pathologies is crucial to establish a correct workup and achieved pons [5]. Hyperintensity on T2-weighted images and strong the right diagnosis. Imaging study allows an evaluation of the contrastweighted enhancement sequences, clivus with is gadolinium usually isointense suggest chordomarelative to and the processes and helps in the suspicious diagnosis. Despite imaging chondrosarcoma (Table 2). MRI also gives additional information tumor extent, a reliable distinction between benign and malignant about dural infiltration and the intracerebral origin of the is an important supporting element, a final diagnosis often tumor [5, 31]. Despite imaging study, a final diagnosis cannot be radiologistcannot be found and pathologist without histologic to achieved biopsy. the Thus,correct it diagnosis.is necessary a achieved without histologic analyses because, sometimes, lesions multidisciplinary team with close cooperation between surgeon, remains a challenge and needs a close cooperation between REFERENCES are radiologically difficult to specify. Thus, an accurate diagnosis work. surgeon, radiologist, and pathologist in a multidisciplinary team 1. Kbksel T, Crockard HA. Clivus – through the eyes of the transoral 2. surgeon. No Shinkei Geka. 1998; 26:169-175. Chordomas and chondrosarcomas are difficult to distinguish Standring S. Head and neck: intracranial region. In: Standring S, ed. because both of them present hypointense signal on T1-weighted Gray’s Anatomy: The Anatomical Basis of Clinical Practice.40th ed. isimages, based onhyperintense the immunohistochemical signal on T2-weighted study. Chordoma images and reacts contrast with London, England: Churchill Livingstone/Elsevier. 2008: 429. enhancement with gadolinium [26, 29]. Thus, definitive diagnosis 3. Borges A. Skull base tumours: Part II. Central skull base tumours and chondrosarcoma is negative for cytokeratin and EMA [44]. Clival intrinsic tumours of the bony skull base.Eur J Radiol. 2008; 66: 348- 4. metastasescytokeratin also and are epithelial an important membrane differential antigen diagnosis. (EMA), They whereas can 362. occur in the context of a known history of a distant malignancy Hofmann E, Prescher A. The clivus: anatomy, normal variants and or can be the presenting symptom. Imaging alone does not 5. imaging pathology. Clin Neuroradiol. 2012; 22: 123-139. allow differentiation between primary and metastatic cancer. Laine FJ, Nadel L, Braun IF. CT and MR imaging of the central skull FDG-PET/CT may help in identifying an unknown primary or base. Part 1: Techniques, embryologic development, and anatomy. 6. Radiographics. 1990; 10: 591-602. on plasma cells neoplasms because it reveals different patterns AB. Diagnostic Imaging Dilemma of a Clival Lesion and Its Clinical Yuh S, Woulfe J, Corsten MJ, Carrau RL, Prevedello DM, Kassam ofprogression Fluorodeoxyglucose of a prior tumor uptake [33,34]. and allows FDG-PET/CT the detection is also of usefulother Management Implications. J Neurol involved places in multiple myeloma. Plasmacytomas show a Surg. 2014; 75: 177–182. to clival and posterior fossa chordomas. Oper Tech Otolaryngol Head 7. Stamm AC, Balsalobre L, Hermann D. Endonasal endoscopic approach histologic and immunohistochemistry similarity reinforce the mild-intense uptake, different from other clival lesions [37]. The Neck Surg. 2011; 22: 274–280. Incidence and survival patterns of cranial chordoma in the United althoughpossibility EP that does chordomas not show may any arise enhancement from EP. On with MRI contrast study, 8. States.Chambers KJ, Lin DT, Meier J, Remenschneider A, Herr M, Gray ST. chordomas commonly enhance markedly with gadolinium, Laryngoscope. 2014; 124: 1097–1102. Current comprehensive be considered in the differential diagnosis. Given their very slow addition [42,43]. Non-tumoral lesions as FD or cysts also need to 9. Di Maio S, Temkin N, Ramanathan D, Sekhar LN. management of cranial base chordomas: 10-year meta-analysis of growth potential, asymptomatic FD of the clivus and cysts can be observational studies. J Neurosurg. 2011; 115: 1094–1105. usually treated with clinical observation [36]. 10. Mangussi-Gomes J, Beer-Furlan A, Balsalobre L, Vellutini E, Stamm

Ann Otolaryngol Rhinol 4(9): 1200 (2017) 7/8 Rodrigues et al. (2017) Email: Central

A. Endoscopic Endonasal Management of Skull Base Chordomas.

28. Lobato RD, Gonzalez P, Alday R, Ramos A, Lagares A, Alen JF, et al. Surgical Technique, Nuances, and Pitfalls. Otolaryngol Clin N Am. Meningiomas of the posterior fossa. Surgical experience in 80 cases. 2016; 49: 167–82. Neurocirurgia. 2004; 15: 525-542. Comprehensive review of intracranial chordoma. Radiographics. characteristic symptoms of petroclivalmeningiomas. Neurol Med Chir. 11. Erdem E, Angtuaco EC, Van Hemert R, Park JS, Al-Mefty O. 29. Kawse T, Shiobara R, Ohira T, Toya S. Developmental patterns and

2003; 23: 995–1009. 1996; 36:1-6. 12. Meyers SP1, Hirsch WL Jr, Curtin HD, Barnes L, Sekhar LN, Sen C. 30. Kim KS, Rogers LF, Goldblatt D. CT features of hyperostosing Chordomas of the skull base: MR features. AJNR Am J Neuroradiol. meningioma en plaque. AJR Am J Roentgenol. 1987; 149: 1017-1023. 1992; 13: 1627–1636. 31. Liu J, Ahmadpour A, Bewley AF, Lechpammer M, Bobinski M, Shahlaie 13. Korten AG, ter Berg HJ, Spincemaille GH, van der Laan RT, Van de Wel K. Chondroblastoma of the Clivus: Case Report and Review. J Neurol AM. Intracranial chondrosarcoma: review of the literature and report Surg Rep. 2015; 76: 258-264. of 15 cases. J Neurol Neurosurg Psychiatry. 1998; 65: 88–92. Chondrosarcoma of the petrous apex. Dilemmas in diagnosis and 32. Kroon HM, Schurmans J. Osteoblastoma: clinical and radiologic 14. Lau DP, Wharton SB, Antoun NM, Bottrill ID, Moffat DA. findings in 98 new cases. Radiology. 1990; 175: 783-790. 33. Rodriguez L, Colina J, Lopez J, Molina O, Cardozo J. Intradural prepontine treatment. J Laryngol Otol. 1997; 111: 368–371. growth: giant ecchordosisphysaliphora or extraosseouschordoma? 15. Ginsberg LE. Neoplastic diseases affecting the central skull base: CT Neuropathology. 1999; 19: 336–340. and MR imaging. AJR Am J Roentgenol. 1992; 159: 581-589. 34. Mehnert F, Beschorner R, Küker W, Hahn U, Nägele T. Retroclival 16. Pallini R, Sabatino G, Doglietto F, Lauretti L, Fernandez E, Maira G. ecchordosisphysaliphora: MR imaging and review of the literature. Clivus metastases: report of seven patients and literature review. Acta Am J Neuroradiol. 2004; 25:1851–1855. Neurochir. 2009; 151: 291–296. 35. Toda H, Kondo A, Iwaski K. Neuroradiological characteristics of 17. Gangadhar K, Santhosh D. Radiopathological evaluation of primary ecchordosisphysaliphora: case report and review of the literature. J malignant skull tumors: a review. Clin Neurol Neurosurg. 2012; 114: Neurosurg. 1998; 89: 830–834. 833–839. 36. Adada B, Al-Mefty O. Fibrous dysplasia of the clivus. Neurosurgery. 18. Fogelman I, Cook G, Israel O, Van der Wall H. Positron emission 2003; 52: 318–322. tomography and bone metastases. SeminNucl Med. 2005; 35: 135– 37. Levy ML, Chen TC, Weiss MH. Monostotic fibrous dysplasia of the 142. clivus. Case report. J Neurosurg. 1991; 75: 800-803. 19. Krishnan A, Shirkhoda A, Tehranzadeh J, Armin AR, Irwin R, Les K. 38. Atalar M, Ozum U. Monostotic fibrous dysplasia of the clivus: imaging Primary bone lymphoma: radiographic-MR imaging correlation. findings. Turk Neurosurg. 2010; 20: 77-81. 20. Radiographics. 2003; 23: 1371-1383. 39. Sirvanci M, Karaman K, Onat L, Duran C, Ulusoy OL. Monostotic fibrous Angtuaco E, Fassas A. Multiple Myeloma: clinical review and diagnostic dysplasia of the clivus: MRI and CT findings. Neuroradiology. 2002; imaging. Radiology. 2004; 231:11-23. 40. 44: 847–850. 21. Cistaro A, Durando S, Pazè F, Limberti A, Cogoni M, Junemann C, et al. atypical MR imaging features of intracranial epidermoid tumors. AJR in The Metabolic Assessment of Skeletal Lesions. J Radiol Case Rep. Kallmes DF, Provenzale JM, Cloft HJ, McClendon RE. Typical and Expansive Masses Arising From TheClivus: The Role of FDG-PET/CT Am J Roentgenol. 1997; 169: 883-887. 22. 2009; 3: 33-40. 41. Orakcioglu B, Halatsch ME, Fortunati M, Unterberg A, Yonekawa Y. Park YK, Yang MH, Choi WS, Lim YJ. Well-differentiated, low-grade Intracranial dermoid cysts: variations of radiological and clinical osteosarcoma of the clivus. Skeletal Radiol. 1995; 24: 386-388. 42. features. Acta Neurochir. 2008; 150:1227-1234. 23. Terada T, Kovacs K, Stefaneanu L, Horvath E. Incidence, pathology, and pathologic correlation in a neurenteric cyst of the clivus. AJNR Am J Kapoor V, Johnson DR, Fukui MB, Rothfus WE, Jho HD. Neuroradiologic- recurrence of pituitary adenomas: study of 647 unselected surgical 24. cases. Endocrine Pathology. 1995; 6: 301–310. . Neuroradiol. 2002; 23: 476–479. De Witte O, Massager N, Salmon I, Meyer S, Dooms G, Brotchi J. Ectopic skull base defects with spontaneous CSF rhinorrhea and arachnoid 43. Schuknecht B, Simmen D, Briner HR, Holzman D. Nontraumatic 25. prolactinoma in the clivus. Acta Chir Belg. 1998; 98: 10–13 . Anand VK, Osborne M, Harkey HL. Infiltrative clival pituitary adenoma herniation: imaging findings and correlation with endoscopic sinus 26. of ectopic origin. Otolaryngol Head Neck Surg. 1993; 108: 178–183 44. surgery in 27 patients. Am J Neurorad. 2008; 29: 542-549. Pituitary adenoma as an unsuspected clival tumor. Am J Surg Pathol. Wong K, Raisanen J, Taylor SL, McDermott MW, Wilson CB, Gutin PH. studyRosenberg of 200 AE, cases Nielsen with GP, emphasis Keel SB, Renard on its distinctionLG, Fitzek MM, from Munzenrider chordoma. JE, et al. Chondrosarcoma of the base of the skull: a clinicopathologic 1995; 19: 900-903. to predict complete excision of parasellar pituitary macroadenoma on Am J Surg Pathol. 1999; 23: 1370–1378. 27. Connor SE, Wilson F, Hogarth K. Magnetic resonance imaging criteria

postoperative imaging. J Neurol Surg B Skull Base. 2014; 75: 41-46.

Cite this article Rodrigues JF, da Silva FM, Mangussi-Gomes J, Marconato F, Dassi CS, et al. (2017) Differential Diagnosis of Clival Lesions – Literature Review of the Clinical and Radiological Features. Ann Otolaryngol Rhinol 4(9): 1200.

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