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Clival Chordoma in an Indian Female Patient: A Case Report |
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Akshat Sanjay Shukla, Sanjay Rajendraprasad Shukla, Feral Ravi Daruwala 1. Consultant Radiologist, Department of Radiology, Shukla Diagnostic Center, Balaghat, Madhya Pradesh, India. 2. Consultant Radiologist, Department of Radiology, Shukla Diagnostic Center, Balaghat, Madhya Pradesh, India. 3. Medical Writer, Department of Clinical Research, Shukla Diagnostic Center, Balaghat, Madhya Pradesh, India. |
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Correspondence Address : Dr. Akshat Sanjay Shukla, Consultatnt Radiologist, Department of Radiology, Shukla Diagnostic Center, Balaghat-480001, Madhya Pradesh, India. E-mail: akshat.shukla006@gmail.com |
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| ABSTRACT |  | ||||||||||||||||||||||||||||||||||||
: Chordomas are rare tumours that originate anywhere along the craniospinal axis and present various diagnostic hurdles. High recurrence rate even after treatment and tendency to involve other regions within the craniospinal axis make this condition more devastating. Hence, early and timely recognition is of utmost importance in order to choose an appropriate therapeutic modality as well as to achieve better recovery and long-term survival. This case report discusses a case of 47-year-old woman who presented with a history of gradually worsening headaches and nasal discharge. The findings obtained from Contrast-Enhanced Computed Tomography (CECT) of the brain were indicative of clival chordoma with associated mass-effects and loco-regional spread. To the best of our knowledge, only a few cases of clival chordoma are reported from the Indian subcontinent. | |||||||||||||||||||||||||||||||||||||
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| Keywords : Clivus, Contrast-enhanced computed tomography, Tumours | |||||||||||||||||||||||||||||||||||||
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DOI and Others :
10.7860/IJARS/2021/49795:2714
Date of Submission: Apr 06, 2021 Date of Peer Review: May 07, 2021 Date of Acceptance: May 26, 2021 Date of Publishing: Oct 01, 2021 AUTHOR DECLARATION: • Financial or Other Competing Interests: None • Was informed consent obtained from the subjects involved in the study? Yes • For any images presented appropriate consent has been obtained from the subjects. Yes PLAGIARISM CHECKING METHODS: • Plagiarism X-checker: Apr 07, 2021 • Manual Googling: May |
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| Case Report |
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A 47-year-old Indian woman, presented to the Department of Radiology with a history of gradually worsening headaches since eight months, and a history of nasal discharge since five months that raised with forced sneezing and coughing. The headache was moderate to severe with dull aching pain and spontaneous fluctuation in intensity. The pain was non-positional and partially relieved by paracetamol. Prior systemic medical history was non-contributory. She was disoriented and febrile when arrived at the clinic. For visual field testing and ophthalmologic examination, she was referred to an ophthalmologist. All ocular findings were normal. A few days later the patient underwent CECT of the brain using an ultra-fast multislice system. The CECT findings of the brain revealed a single ill-defined lesion with predominantly large soft tissue component causing lysis of the clivus and erosion of the skull base, measuring at least 4.5×4.5×6.5 cm in maximal orthogonal planes (Table/Fig 1)a,b,c. The postcontrast sequence showed avid enhancement. The lesion showed a patchy rim of calcification along the margins of the soft tissue. Overall, the soft tissue component of the lesion was disproportionately large to the degree of bony destruction. The soft tissue extended contiguously into the sphenoid sinus and along the medial aspect of both infratemporal fossae. Posteriorly, the soft tissue was seen indenting on pons without invasion. Posteroinferiorly, the soft tissue extended along the nasopharynx and just about the soft palate. There was epidural extension of mass lesion in the middle cranial fossa causing inward buckling on right basal temporal lobe showing intracranial extension (Table/Fig 1)d. The optic chiasma and the pituitary gland appeared likely to be displaced superiorly by the mass-effect from the lesion (Table/Fig 1)e,f. There was remarkable vascular recruitment from the surrounding brain parenchyma. The basilar artery was seen along the posterior margin of the lesion and did not show circumferential encasement. There was a reactive mucosal thickening/opacification of the ethmoid air cells. Rest of the cerebral hemisphere was normal. Imaging features suggested the diagnosis of clival chordoma with associated mass-effects and loco-regional spread. The patients were referred to higher centre for further medical assistance. | |||||||||||||||||||||||||||||||||||||
| Discussion |
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Chordomas are rare slow-growing malignant bony tumours that are remnants of the notochord that stem from the bone and can advance anywhere along the craniospinal axis. They represent just over 1% of intracranial tumours (1), and their prevalence is 0.08 per 100,000 individuals per year (2). Histologically, chordoma are mainly classified into three types: a) chondroid; b) dedifferentiated; or c) classical (3). Chordomas arise in the sacral (29.2%), cranial (32%) and spinal (32.8%) regions (4). Clival chordomas comprise about half of the cranial chordomas and these tumours are most frequently evident in women and younger patients (5). In 1857, chordomas were first characterised microscopically by Virchow (6). The manifestations of clival chordoma are mainly based upon the site of the tumour and the adjacent structures. The most typical manifestations of clival chordoma are headache, visual changes and cranial nerve palsies (7), whereas rare presentations include Cerebrospinal Fluid (CSF) rhinorrhoea and epistaxis (8). Not only therapeutic challenges, but also diagnostic challenges are associated with chordomas. Diagnostic challenge is chiefly attributed to the rarity of the condition and tendency to involve any location within the craniospinal axis. As far as we know, in the literature, hardly a few cases have been reported on clival chordomas from the Indian subcontinent. Demographics and clinical manifestations of the present and earlier reported cases are compared in (Table/Fig 2) (9),(10),(11),(12),(13),(14),(15). Differential diagnosis can be made using multiple imaging modalities. On plain radiographs, local bony erosion and scattered calcifications are observed. CT findings showed reduced attenuation and hyperdense regions. It may also exhibit the erosion of neighbouring structures and an inner calcified matrix. Brain MRI showed typical characteristics of bony expansion together with hypointensity on T1-weighted images and hyperintensity on T2-weighted images. Heterogeneous enhancement pattern on T1-weighted images following administration of gadolinium may also be noted. Perioperative biopsy using multiple analytical techniques is useful to confirm the diagnosis. For all skull base tumours, CT scan and MRI are the gold standards for diagnosis. As compared to MRI, CT scan is better in illustrating tumoural calcification and associated bone destruction. As chordoma originates along the craniospinal axis, spine imaging is required to rule out lesions elsewhere, however it was not done in the present case. Surgical intervention is the recommended treatment modality having a goal to achieve a gross total resection of the tumour. Conventionally, clival chordomas can be managed by transcranial,transsphenoidal, transoropharyngeal and maxillary osteotomy approaches (16). At present, endoscopic surgery has been an emerging avenue for managing clival chordomas. Recently, Imatinib, a tyrosine kinase inhibitor, was endorsed as a monotherapy for chronic myelogenous leukaemia. Imatinib has been investigated in chordomas as its use has been associated with not only tumour liquefaction but also decreased density of chordomas expressing the Platelet-Derived Growth Factor Receptor-Beta (PDGFRß) (17). Also, radiotherapy is the mainstay in treating chordomas. In the literature, multiple modalities have been reported varying from proton-beam radiotherapy to recent ones such as fractionated photon radiotherapy, carbon-ion radiotherapy and stereotactic radiosurgery. | |||||||||||||||||||||||||||||||||||||
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