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

Abstract

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.

Recurrent choroid plexus carcinoma treated with CyberKnife radiosurgery: illustrative case

“…Age appears to influence the tumor location, with children typically presenting with tumors in the lateral ventricle and adults commonly showing occurrences in the fourth ventricle. 2 Nevertheless, CPCs tend to spread extensively along the ventricle into the spinal canal; thus, they are frequently associated with leptomeningeal seeding upon initial diagnosis. 4 , 7 The manifestation of CPCs in patients generally includes symptoms of increased intracranial pressure and hydrocephalus, alongside neurological symptoms linked to the mass effect of the lesion.…”

“… 4 , 7 The manifestation of CPCs in patients generally includes symptoms of increased intracranial pressure and hydrocephalus, alongside neurological symptoms linked to the mass effect of the lesion. 2 …”

“…Surgical removal with GTR continues to be the cornerstone of treatment, demonstrating statistically superior OS and PFS in comparison to those with subtotal resection, with respective median durations of 131 versus 25 months and 60 versus 11 months. 5 Local and distant recurrences are frequent events and have also been documented to occur as late as 17 years after the initial surgery, 2 which forms the rationale behind employing adjuvant therapy, in the form of conventional radiotherapy and chemotherapy, which has exhibited the capacity to enhance OS. 5 , 8 , 9 …”

“…In pediatric cases CPTs typically manifest supratentorially (lateral ventricle), whereas in adults they tend to occur infratentorially in the fourth ventricle and cerebellopontine angle. 2 Patients display symptoms of elevated intracranial pressure and hydrocephalus, along with neurological manifestations arising from the lesion’s space-occupying effects. 2 …”

“… 2 Patients display symptoms of elevated intracranial pressure and hydrocephalus, along with neurological manifestations arising from the lesion’s space-occupying effects. 2 …”