Choroid Plexus Carcinoma in Adults: Two Case Reports

Kim, Tae-Hoon; Park, Mee Rim; Hong, Eun Kyoung; Gwak, Ho Shin

doi:10.14791/btrt.2019.7.e23

Cited by 10 publications

(10 citation statements)

References 15 publications

(23 reference statements)

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“…Metastatic disease developed from 3 months to 8 years after these initial treatments, within one year in three patients (43%) and five years in six patients (86%) after initial treatment. 5,[7][8][9]12) Leptomeningeal spread around the spinal cord was most common in these patients. [7][8][9]11,13) Four of five patients died of progressive disease within 18 months after the salvage treatment.…”

Section: Discussionmentioning

confidence: 91%

“…5,6) Metastatic recurrence in adult CPCs usually develops within 5 years after initial treatments, and patients with metastasis have suffered aggressive clinical course and died of progressive disease within 18 months. 5,[7][8][9][10] We present a rare case of adult CPC in the fourth ventricle that slowly developed progressive metastatic disease at 17 years after initial treatment. The consent for publication was obtained from the patient.…”

Section: Introductionmentioning

confidence: 99%

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Delayed Recurrence of Choroid Plexus Carcinoma in the Sacral Spinal Cord 17 Years after Its Initial Presentation

Nagai

Kanamori

SHIMODA

et al. 2022

NMC Case Report Journal

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Choroid plexus carcinomas (CPCs) are rare malignant tumors of neuro-ectodermal origin, accounting for less than 1% of all intracranial tumors. The recurrence rates of CPCs are very high and typically occur in the short-term following surgery, even after gross total removal. Here we present a rare case of CPC with spinal metastasis, which occurred long after its initial presentation. A 25-year-old woman with a history of increased intracranial pressure underwent resection for a tumor of the fourth ventricle, with a histopathological diagnosis of CPC. After tumor resection, she received 30 Gy of radiation therapy to the craniospinal axis and 20 Gy to the primary site, followed by nimustine hydrochloride chemotherapy. The residual lesion completely responded to these treatments. She suffered sensory loss in the sacral region 13 years later, followed by refractory skin ulcer in the sacral region 17 years after the initial treatments. Magnetic resonance imaging at 17 years after the initial treatments showed tumor in the sacral region, which was enlarged upon follow-up after 18 months, causing incontinence and loss of urinary intention. She underwent tumor resection, with a histological diagnosis of recurrent CPC. She received salvage re-irradiation. This case shows that CPC can spread via the cerebrospinal fluid pathways and cause spinal metastasis, with relatively slow clinical course. The present case suggests that patients with CPCs may need long-term follow-up imaging of the total neural axis to identify late recurrence at both the primary site and spinal metastasis.

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Section: Discussionmentioning

confidence: 91%

Section: Introductionmentioning

confidence: 99%

Delayed Recurrence of Choroid Plexus Carcinoma in the Sacral Spinal Cord 17 Years after Its Initial Presentation

Nagai

Kanamori

SHIMODA

et al. 2022

NMC Case Report Journal

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“…CPCs arise from modified ependymal cells surrounding a core of capillaries and loose connective tissue. CPCs are typically intraventricular, however, extraventricular tumors have been reported [7][8][9]. Ectopic sites are not commonly reported but include the suprasellar region, foramen magnum, and spinal canal in the absence of other intracranial lesions [10].…”

Section: Discussionmentioning

confidence: 99%

“…The most common site for adults is in the fourth ventricle (63%) while in the pediatric group is the lateral ventricle (72%) [10]. Presentation with multiple CPCs is rare, occurring in only 5% of CPC patients [9]. Metastatic lesions have a higher frequency of being supratentorial [10].…”

Section: Discussionmentioning

confidence: 99%

Cerebellopontine Angle Primary Choroid Plexus Carcinoma Present in an Adult: Case Report and Literature Review

Witten

Mendenhall

DeWitt

et al. 2021

Cureus

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Choroid plexus tumors (CPTs) are rare intraventricular neoplasms that primarily occur in children and are rare in adults. Of the CPT subtypes, choroid plexus carcinomas (CPC) are highly aggressive and malignant and of World Health Organization (WHO) Grade III. Dissemination through the cerebrospinal fluid space is the inevitable natural course of the disease. In this case report, we present a 33-year-old female with a past medical history notable for schizophrenia and bipolar disease who suffered from left-sided acute vision loss and hearing loss. Magnetic resonance imaging (MRI) demonstrated multiple enhancing masses found in the left cerebellopontine angle (CPA), right internal auditory canal, the atrium of the left ventricle, and the left foramen of Monroe. After surgical decompression of the CPA tumor, the permanent final pathology was consistent with CPC. To our knowledge, this is the first reported case of a primary CPC occurring within the CPA in an adult. The unique presentation and progression of this rare adult-onset CPC provide insight for the diagnosis and treatment of other rare instances of CPTs.

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“…Approximately 80% of choroid plexus carcinoma (CPC) occur in children. [3] Choroid plexus tumors range from well-differentiated papilloma (WHO Grade I) to very aggressive choroid plexus carcinoma (WHO Grade III) with an intermediate form named atypical choroid plexus papilloma (WHO Grade II). [1] These tumors arise in ventricles, most commonly in lateral and fourth ventricle; lateral ventricle being the commonest site in children and fourth ventricle being in adults.…”

Section: Introductionmentioning

confidence: 99%

Choroid Plexus Carcinoma of Third Ventricle: A Rare Case Report

Awasthi¹,

Kinra²,

Verma³

2021

Ann of Pathol and Lab Med

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Choroid plexus tumors are very rare tumors accounting for 0.5% of all brain tumors. They most commonly occur in childhood and present with features of raised intra-cranial pressure. World Health Organization (WHO) classifies them into 3 broad categories namely papilloma (grade 1), atypical papilloma (grade 2) and carcinoma (grade 3). These tumors are most commonly seen in the supratentorial compartment with lateral ventricle followed by third ventricle, being the most common site. Rarely, they may present in infratentorial region in adults. Dissemination through cerebrospinal fluid space is the inevitable natural course of the disease. Treatment requires total surgical resection with adjuvant chemoradiotherapy being controversial. In this study, we present a case of 7-year-old child who visited to the out-patient department of our center with progressive quadriparesis and altered sensorium and on imaging was found to have a well-defined, lobulated mass lesion with intense post contrast enhancement in posterior part of third ventricle with resultant obstructive hydrocephalus. The patient underwent craniotomy and on immuno-histopathological examination was diagnosed as a case of choroid plexus carcinoma. However, the patient succumbed to his illness a month after his surgery. The aim of this report is to highlight a rare entity, its diagnostic challenge and effect of early management in the form of surgery and chemo-radiotherapy.

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Choroid Plexus Carcinoma in Adults: Two Case Reports

Cited by 10 publications

References 15 publications

Delayed Recurrence of Choroid Plexus Carcinoma in the Sacral Spinal Cord 17 Years after Its Initial Presentation

Delayed Recurrence of Choroid Plexus Carcinoma in the Sacral Spinal Cord 17 Years after Its Initial Presentation

Cerebellopontine Angle Primary Choroid Plexus Carcinoma Present in an Adult: Case Report and Literature Review

Choroid Plexus Carcinoma of Third Ventricle: A Rare Case Report

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