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CSF Rhinorrhea: a Rare Clinical Presentation of Choroid Plexus Papilloma

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CSF Rhinorrhea: a Rare Clinical Presentation of Choroid Plexus Papilloma Case Report CSF Rhinorrhea: A Rare Clinical Presentation of Choroid Plexus Papilloma Layth Mula-Hussain 1,* , Julia Malone 1, Marlise P. dos Santos 2, John Sinclair 3 and Shawn Malone 1 1 Radiation Oncology Division, The Ottawa Hospital—University of Ottawa, Ottawa, ON K1H 8L6, Canada; [email protected] (J.M.); [email protected] (S.M.) 2 Department of Medical Imaging, The Ottawa Hospital—University of Ottawa, and Ottawa Hospital Research Institute, Ottawa, ON K1H 8L6, Canada; [email protected] 3 Neuro-Surgery Division, The Ottawa Hospital—University of Ottawa, Ottawa, ON K1Y 4E9, Canada; [email protected] * Correspondence: [email protected] Received: 6 November 2020; Accepted: 27 January 2021; Published: 31 January 2021 Abstract: Choroid plexus papilloma (CPP) is a rare brain tumour occurring mostly in infants and children. Most CPPs are intraventricular and present with symptoms and signs of increased intracranial pressure (ICP). This case report describes a middle-aged female who presented with spontaneous cerebrospinal fluid (CSF) rhinorrhea from a tumour located in the cerebellopontine angle (CPA). She underwent craniotomy with subtotal tumour resection and remained progression and rhinorrhea-free for several years. Upon clinical progression, the patient was treated with Cyberknife stereotactic radiosurgery. The patient clinically improved and demonstrated a favourable radiologic response to radiosurgery. Keywords: choroid plexus tumour; cerebellopontine angle; CSF rhinorrhea; stereotactic radiosurgery 1. Introduction Choroid plexus tumour (CPT) is a rare epithelial intracranial extra-axial brain tumour that usually grows inside ventricles and is connected to the choroid plexus or near the natural openings of the ventricles. The annual incidence is 0.3 cases per million [1], and they account for about 0.5% of all intracranial neoplasms. CPT can be classified as choroid plexus papilloma (CPP, WHO grade I) or choroid plexus carcinoma (CPC, WHO grade III), with a ratio of 5:1, respectively. Approximately 80% of CPCs occur in children [2]. The most common location of CPTs in adults is the fourth ventricle, followed by the cerebellopontine angle (CPA) [3,4]. Clinically, CPT tends to cause hydrocephalus, and patients present with symptoms of raised intracranial pressure. The treatment of choice is gross total resection (GTR) [5], when clinically feasible. Neurosurgeons must assess the pros and cons of GTR versus the potential morbidity of complete excision. Historically, radiotherapy has been used in cases where surgery is not possible or ended up with subtotal resection, after local recurrence, or in high-grade CPC [6]. Adjuvant chemotherapy has been used in CPC; however, its benefit is not clear. The five-year survival rates are reported to be as high as 100% in CPP following complete resection, with low local recurrence rates. CPC grows more rapidly and has a less favourable outcome, with a 5-year survival rate of 26–40% [2]. Improvements in surgical techniques and post-operative care have improved survival [7]. Curr. Oncol. 2021, 28, 750–756; doi:10.3390/curroncol28010073 www.mdpi.com/journal/curroncol Curr. Oncol. 2021, 28 751 Curr. Oncol. 2021, 28, 2 2.2. CaseCase StudyStudy AA 43-year-old 43-year-old woman woman presented presented in in January January 20072007 withwith cerebrospinalcerebrospinal fluid fluid (CSF) (CSF) rhinorrhea rhinorrhea from from thethe left left nostrilnostril onon bendingbending forward, headache,headache, and later later on on with with ataxia. ataxia. There There was was no no prior prior history history of ofskull skull trauma trauma or orsurgery surgery and and no nosignificant significant past past medical medical hist history.ory. After After the theconfirmation confirmation of a ofCSF a CSF leak leakwith with a β-2 a tβransferrin-2 transferrin test, a test, CT scan a CT of scan the ofparanasal the paranasal sinuses sinuses on December on December 2007 showed 2007 showed the enlarge- the enlargementment of the right of the foramina right foramina rotundum rotundum and ovale and ( ovaleFigure (Figure 1 A, B1).A,B). A B C D FigureFigure 1. 1.Coronal Coronal plane, plane unenhanced, unenhanced CT CT scan scan of theof the paranasal paranasal sinuses sinuses obtained obtained with with bone bone window window and inand prone in prone position. position. (A–D ,( Dec.A – D 2007)., Dec. ( A2007).): prominent (A): prominent right (arrow) right and(arrow normal) and left normal (triangle) left foramen(triangle) rotundum;foramen rotundum; (B): prominent (B): prominent right (arrow) right and (arrow normal) and left normal foramen left ovale foramen (triangle); ovale ((Ctriangle): prominent); (C): prom- right sphenoidalinent right arachnoidsphenoidal pits arachnoid (dashed pits circle); (dashed (D): circle focal); defect (D): focal of the defect left planum of the left sphenoidale planum sphenoidale (chevron) and(chevron adjacent) and opacification adjacent opacification of the left ethmoid of the left sinus ethmoid and upper sinus nasal and cavityupper (CSFnasal leak,cavity triangles). (CSF leak, tri- angles). An MRI of the brain on May 2008 (Figure2A,B) revealed an enhancing, T1-isointense and T2-hyperintenseAn MRI of extra-axialthe brain o CPAn May mass 2008 lesion (Figure measuring 2 A, B) r 24evealed24 an30 enhancing, mm, associated T1-isointense with prominent and T2- × × flow-voidshyperintense and extra causing-axial mass-e CPAff ectmass on thelesion lower measuring pons and 24 the × medulla24 × 30 oblongata.mm, associated The mass with encroached prominent onflow the-voids cisternal and segments causing ofmass the-effect right 7thon andthe 8thlower canal pons nerves and complex.the medulla Inferiorly, oblongata. the mass The extendedmass en- upcroached to the level on the of cisternal the foramen segments magnum. of the Our right retroactive 7th and 8th reassessment canal nerves of complex. the CT scan Inferiorly, of the paranasal the mass sinusesextended identified up to the multiple level of unilaterally the foramen prominent magnum. aberrant Our retroactive arachnoid reassessment granulations of (arachnoid the CT scan pits) of the of theparanasal right sphenoid sinuses identified bone and multiple the focal unilaterally disruption prominent of the left planumaberrant sphenoidale, arachnoid granulations contiguous (arach- to an abnormalnoid pits) opacification of the right sphenoid of the medial bone left and ethmoid the focal sinus disruption and upper of the nasal left cavity,planum in sphenoidale keeping the, sourcecontig- ofuous a CSF to an leak abnormal with high opacification intracranial of pressure the medial features left ethmoid (Figure sinus1C,D). and The upper retrospective nasal cavity, review in keeping of the abovethe source mentioned of a CSF MRI leak showed with ahigh very intracranial mild compensated pressure communicating features (Figure hydrocephalus 1 C, D). The retrospective (Figure2B). reviewIn September of the above 2008, mentioned the patient MRI underwent showed a very a right mild posterior compensated fossa craniotomy communicating with hydrocepha- facial nerve monitoring.lus (Figure 2 AB). frozen section of the tumour revealed a CPP. The tumour was extremely vascular, soft, friable,In September and adherent 2008, the to somepatient cranial underwent nerves. a Aright subtotal posterior excision fossa of craniotomy the tumour with was fac performedial nerve inmonitoring. an attempt A tofrozen preserve section cranial of the nerve tumour function. revealed Final a CPP. pathology The tumour revealed was extremely a WHO vascular, grade 1 CPP. soft, Histologically,friable, and adherent the tumour to some tissue cranial had nerves. a papillary A subtotal structure excision with aof fibrovascular the tumour was backbone performed and in was an coveredattempt by to cuboidalpreserve tocranial low columnar nerve function. cells. Postoperatively, Final pathology her revealed headache a WHO and CSF grade rhinorrhea 1 CPP. Histolog- stopped, butically, she the developed tumournew tissue vocal had cord a papillary palsy and structure partial with right a hearing fibrovascular loss and backbone her ataxia and continued. was covered MRI atby eightcuboidal weeks to postoperativelylow columnar cells. revealed Postoperatively, encephalomalacia her headache and an 11 and9 CSF mm rhinorrhea residual enhancing stopped, tumourbut she × developed new vocal cord palsy and partial right hearing loss and her ataxia continued. MRI at eight weeks postoperatively revealed encephalomalacia and an 11 × 9 mm residual enhancing tumour in the right internal auditory canal extending into the right CPA without mass effect and resolution of Curr. Oncol. 2021, 28 752 Curr. Oncol. 2021, 28, 3 in the right internal auditory canal extending into the right CPA without mass effect and resolution of the hydrocephalus (Figure 22C,D). C, D). She She was was kept kept on on annual annual clinical clinical and and radiologic radiologic follow-up follow-up forfor overover four years, till December 2012, where her annual follow up was discontinued as she was feeling better with stable radiological residual disease.disease. Figure 2. PrePre-operative-operative ( ((AA, axial) axial,, and and B, ( Bcoronal)) coronal) and and 2 months 2 months post post-operative-operative (C, axial ((C) axial,, and D and, coro- (D) coronal)nal) post- post-gadoliniumgadolinium T1-W T1-W MRI MRIsequences. sequences. The CPA The CPAtumour tumour measured measured 24 × 24 24 × 3024 mm30 and mm 11 and × 9 × × 11mm, 9respectively. mm, respectively.
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