VOL. 20, NO. 1, 2021 Case Report

VOL. 20, NO. 1, 2021 Case Report

CLINICAL PAIN 39 https://doi.org/10.35827/cp.2021.20.1.39

접수일 : 2020 년 10 월 24 일 , 게재승인일 : 2020 년 11 월 9 일 책임저자 : 류병주 , 서울 동대문구 망우로 82

뿸 02500, 삼육서울병원 재활의학과 Tel: 02-2210-3133, Fax: 02-2210-3133 E-mail: [email protected]

방사성 증상이 초기 증상으로 나타난 비정형 맥락막 신경총 유두종

삼육서울병원 재활의학과

, 고려대학교 의과대학 신경외과학교실

이철재¹ㆍ김준형¹ㆍ김윤희¹ㆍ이장보²ㆍ류병주¹

An Atypical Choroid Plexus Papilloma Initially Manifestating as Radicular Symptoms

Cheol-Jae Lee, M.D.

, Jun-Hyung Kim, M.D.

, Yunhee Kim, M.D.

, Jang-Bo Lee, M.D., Ph.D.

and Byungju Ryu, M.D.

1

Department of Physical Medicine and Rehabilitation, Sahmyook Medical Center,

2

Department of Neurosurgery, Korea University College of Medicine, Seoul, Korea

A 29-year-old woman had 1-month history of back pain radiating into lower extremities, motor weakness, and sensory abnormal- ities in both lower extremities. Contrast-enhanced spinal magnetic resonance imaging (MRI) revealed a homogeneously enhanc- ing mass at the T12∼L1 and several intradural enhancing nodular lesions at L2∼S1. Tumor resection surgery was performed and following histological examination showed that the tumor satisfied the diagnostic criteria for atypical choroid plexus papilloma (CPP). To find primary tumor sites, contrast-enhanced brain MRI, whole spine MRI, and PET-CT were carried out and additional lesions were detected at the fourth ventricle, right cerebellum, and upper thoracic spinal cord. This is a very rare case of metastatic atypical CPP that involves brain, upper thoracic spinal cord, and cauda equina with initial manifestation of radicular symptoms without clinical signs of primary brain lesion. (Clinical Pain 2021;20:39-42)

Key Words: Choroid plexus papilloma, Back pain, Spinal cord injury

INTRODUCTION

Choroid plexus papilloma (CPP) is a rare primary neuro- ectodermal tumor of the choroid plexus accounting for 0.3∼

0.8% of all intracranial tumors. Drop metastasis of CPP in- to the spinal subarachnoid space causing spinal cord injury is rarely observed [1-3].

We report a very rare case of spinal drop metastasis of atypical CPP with initial manifestation of radicular symp- toms due to cauda equina involvement and invading multi- ple sites. This is, to the best of our knowledge, the first report that CPP involves brain, upper thoracic spinal cord, and cauda equina.

Informed consent was obtained from the patient for pub- lication of patient’s data.

CASE REPORT

A 29-year-old woman was admitted to our rehabilitation department and represented 1-month history of severe back pain radiating to lower extremities with numerical rating scale (NRS) of 9. She also complained of weakness when bending her hips. The patient did not show any other neu- rologic symptoms including nystagmus, dysmetria, ataxia, nausea, vomiting and dizziness.

Physical examinations showed motor weakness in bi- lateral lower extremities with fair grade of the medical re- search council scale in bilateral hip flexors and good grade in bilateral knee extensors in manual muscle testing. On sensory examination, there was hypoesthesia on the left S1 dermatome including buttock, back of thigh, and lower leg.

Her deep tendon reflexes were normoactive and no patho- logic reflex was observed in both lower limbs. Also, there were no abnormal findings in the cranial nerve examination and cerebellar function test.

On lumbar radiographs, intervertebral disc space narrow- ing at L4∼5 was noted.

Contrast-enhanced spinal magnetic resonance imaging

(MRI) was performed immediately on the same day be-

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Fig. 2. Contrast-enhanced T1-weigh- ted MRI (A-E) and PET CT scan image (F) after CPP diagnosis. (A) Homogenously enhancing tumor in the 4th ventricle (arrow) is seen in sagittal view. (B) Tumor in the posterior aspect of right cerebellar hemisphere (arrow) is seen in axial image. (C) Homogenously enhanced multiple tumors in the 4th ventricle (arrow), right cerebellar hemisphere (arrowhead), and upper thoracic spinal cord at T3∼5 (star) are seen in sagittal view. (D, E) Axial view of homogenously enhancing tumors (arrows) at T4 (D) and around L2 (E). (F) Hot uptake at T3∼5 (arrow) is detected in sagittal image.

Fig. 1. Sagittal T1-weighted MRI with contrast. (A) Preoperative MRI shows a homogeneously enhancing intradural tumor at the T12∼L1 level (arrow) and multiple nodular lesions at the L2∼

S1 level (arrowheads). (B) Residual tumors are seen in post- operative MRI (arrowheads).

cause of her extreme pain and progressive motor weakness of her hip flexors and knee extensors. MRI revealed rela- tively well-defined elongated mass lesion with iso-signal intensity on T1-weighted image (WI) and high signal in- tensity on T2WI at T12∼L1 and several intradural enhanc- ing nodular lesions at L2∼S1 (Fig. 1-A) which suggested suspicious multiple tumorous lesions rather than cauda equina thickening.

For the treatment of the tumor, laminectomy at T12∼L1 and tumor resection surgery were performed at the neuro- surgery department. After surgery, back pain radiating to

lower extremities was dramatically improved, from NRS 9 to NRS 2. The muscle strength of the right lower extremity was fully recovered while that of the left one worsened to poor grade in left hip flexor and trace grade in left knee extensor. Hypoesthesia below L1 dermatome was identified in bilateral lower extremities. In addition, the urinary symptom of the inability to completely empty the bladder was developed, but it was well controlled with oral medications.

Postsurgical contrast-enhanced spinal MRI showed de- compressed central canal at T12∼L1 and a part of remain- ing tumor after surgery (Fig. 1-B and Fig. 2-E).

Immunohistochemical analysis revealed that the cyto- plasm of the neoplastic cells was negative for cytokeratin, and negative for vimentin. The luminal surfaces of the cy- toplasm were negative for epithelial membrane antigen.

The results of testing for the glial fibrillary acidic protein and anti-cytokeratin (CAM5.2) were positive. The Ki 67 proliferation index was 20∼30%; that means the tumor is relatively proliferative than benign lesion (median K-67 rate of CPP: 1.3% (0∼11.9%), atypical CPP: 9.1% (0.5∼

35.6%) choroid plexus carcinoma: 20.3% (7.8∼42.5%)) [4]. On the basis of these findings, a diagnosis of atypical CPP was made.

Considering the unusual location of the tumor and sus-

pecting cerebrospinal fluid (CSF) dissemination from a pri-

이철재 외 4인: 방사성 증상이 초기 증상으로 나타난 비정형 맥락막 신경총 유두종

CLINICAL PAIN 41 mary CPP, contrast-enhanced brain and whole spine MRI

and PET-CT scan were performed. Unexpectedly, not only was the primary tumor found in the 4th ventricle (Fig. 2-A) but also metastatic tumor in the right cerebellar hemisphere was found (Fig. 2-B). Moreover, spinal cord tumor at T3∼5 was also observed (Fig. 2-C, D, and F). Afterwards, the patient was transferred to a neurosurgery department for treatment of newly discovered lesions. Surgical resection was performed on the thoracic lesion and atypical CPP was diagnosed on pathological examination, same as the lesions of cauda equina. Then radiotherapy was initiated on the brain lesions.

DISCUSSION

This is the first case of CPP with initial manifestation of radicular symptoms without signs of a primary brain tu- mor that involves multiple sites including brain, upper thoracic spinal cord, and cauda equina.

CPP is a rare tumor of the central nervous system, most commonly in the lateral ventricle in children and in the fourth ventricle in adults. The most common symptoms and signs of CPP in the fourth ventricle are those of hydro- cephalus, such as headache, diplopia and ataxia [5].

In very rare cases of CPP, intracranial tumors can meta- stasize into the spinal subarachnoid space through the flow of CSF, known as drop metastases. Even in the case of drop metastases, the first presenting symptoms and signs are known to be related to the primary brain tumor other than spinal cord injury or cauda equina syndrome [6]. In our case, there were no symptoms and signs of hydrocephalus. The patient complained of only one-month history of severe back pain radiating into lower extremities, the weakness of lower extremity muscles, and hypoesthesia on the left S1 dermatome that mimics lumbosacral radiculopathy. It is probably because the tumor in the 4th ventricle was not large enough to cause stenosis of the aqueduct of sylvius, central canal, lateral aperture, or me- dian aperture. If the tumor had been large enough to cause an occlusion in one or more of these, it would have likely caused hydrocephalus and associated symptoms.

Immunohistochemical analysis confirmed that the tumor was atypical CPP. The WHO classification of choroid plexus tumor is divided into three categories. Type I called CPP (58.2%) is benign and type III called choroid plexus

carcinoma (34.4%) is malignant. Type II called atypical CPP is an additional entity with intermediate character- istics, which is primarily distinguished from the CPP by raised mitotic activities [7,8]. Most of the treatment of CPP is surgical resection (91.4%) and rarely radiotherapy (6.4%) or chemotherapy (6.4%). On the other hand, in cho- roid plexus carcinoma, surgery (50%) and chemotherapy (45.8%) are performed at a similar rate [4]. The overall and event-free survival rates for atypical CPP are intermediate between those for CPP and choroid plexus carcinoma. The 5-year overall survival and event-free survival rates for atypical CPP are 89% and 83%, respectively [7].

Dissemination of CPP has been seldom observed along the entire neuraxis involving the supratentorial, infratentorial compartments, and the spine [6,9,10]. Nevertheless, our case is the first report in South Korea that CPP was discovered in the 4th ventricle, cerebellum, upper spinal cord, and cau- da equina that encompass the entire nervous system.

In our case, motor symptoms were on the upper lumbar root level and sensory symptoms were on the lower lumbo- sacral root level. When the motor symptoms and sensory symptoms do not match as in our case, the spinal cord tu- mor should be fully considered when diagnosing a patient with low back pain. The limitation of our case is that elec- trodiagnostic study was not performed for evaluation of radicular symptoms because emergent MRI was done im- mediately due to her severe pain and progressive motor weakness.

Our case is a first description of CPP that has initial manifestation of radicular symptoms due to cauda equina involvement and invades multiple sites including 4th ven- tricle, right cerebellar hemisphere, and thoracic spinal cord tumor discovered by later work-up. Therefore, we suggest that spinal cord tumor including CPP should be considered as one of the differential diagnosis when radicular symp- toms are present, even if there are no symptoms and signs of hydrocephalus. In addition, if CPP is found in the spinal cord, efforts must be made to find the origin of the tumor that includes brain and whole spine.

CONFLICT OF INTEREST

The authors have no potential conflicts of interest to

disclose.

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