Brain Tumor Res Treat.  2019 Apr;7(1):48-52. 10.14791/btrt.2019.7.e23.

Choroid Plexus Carcinoma in Adults: Two Case Reports

• Kim T
• Park MR
• Hong EK
• Gwak HS

• KMID: 2444796
• DOI: http://doi.org/10.14791/btrt.2019.7.e23

Abstract

Choroid plexus tumors are uncommon brain tumors that primarily occur in children. Most of these tumors originate from the intraventricular area, and the most common clinicalpresentation is increased intracranial pressure. Dissemination through the cerebrospinal fluid space is the inevitable natural course of the disease. Here, we present 2 rare cases of adult choroid plexus carcinoma (CPC), each with distinct clinical presentation and progression. The first case was a 40-year-old male who presented with multiple intraventricular masses. After surgical biopsy, radiation and intrathecal chemotherapy failed to elicit any response. The patient progressed with spinal cord dissemination and expired 1 year later. The second case presented with visual disturbance, and brain MRI revealed a large ovoid juxtaventricular mass with peritumoral edema. This 49-year-old female patient underwent craniotomy for what was thought to be a high-grade glioma; however, the mass was connected to the choroid plexus at the operative field. Her pathology specimen was diagnosed as CPC, and adjuvant systemic chemotherapy was administered. She has now been free of recurrence for 10 months. The description of the presentation and progression of these rare adult-onset CPC provides insight for the diagnosis and treatment of other rare instances of choroid plexus tumors.

Keyword

Adult; Cerebrospinal fluid; Choroid plexus carcinoma; Fourth ventricle

MeSH Terms

Adult*
Biopsy
Brain
Brain Neoplasms
Cerebrospinal Fluid
Child
Choroid Plexus Neoplasms
Choroid Plexus*
Choroid*
Craniotomy
Diagnosis
Drug Therapy
Edema
Female
Fourth Ventricle
Glioma
Humans
Intracranial Pressure
Magnetic Resonance Imaging
Male
Middle Aged
Pathology
Recurrence
Spinal Cord

Figure

• 

  Fig. 1 T1-weighted gadolinium enhanced brain MRI revealed enhancing masses (A) on the 4th ventricular floor (arrow), and (B) around the tectum (arrow), (case 1).

• 

  Fig. 2 T1-weighted gadolinium enhanced spine MRI showed multiple linear nodular enhancements of (A) cervicothoracic spinal cord surface, and (B) conus medullares, suggesting leptomeningeal metastases (case 1).

• 

  Fig. 3 Goldmann perimetry of case 2. A: Preoperative visual field test revealed right side homonymous hemianopsia. B: Postoperative 3-month follow-up test showed a largely resolved visual field defect (case 2).

• 

  Fig. 4 Preoperative brain image of the patient (case 2). The mass was located on the trigone of the left lateral ventricle. CT showed an isodense mass with peritumoral edema (A). T2-weighted (B, C) and T1-weighted (D) gadolinium enhanced brain MRI revealed a well-enhanced juxtaventricular parenchymal mass with an internal cystic portion. MR spectroscopy demonstrated an elevated choline peak, suggesting a malignant tumor (E).

• 

  Fig. 5 Postoperative brain image of the patient. A: Immediate postoperative brain CT showed intraventricular hemorrhage. B: T1-weighted gadolinium enhanced images from the postoperative MRI suggested gross total removal of the tumor (case 2).

• 

  Fig. 6 Photomicrographs of surgical specimens (case 2). A: The tumor was well-encapsulated with a distinct fibrous capsule with normal choroid plexus attached (arrow) and showed papillary growth pattern (H&E stain, ×12.5). B: The tumor cells were composed of columnar epithelial cells, and some cells had frank nuclear pleomorphism that resulted in a blurred papillary growth pattern (H&E stain, ×200). C: Immunohistochemical staining for transthyretin was positive focally (×200).

Reference

1. Sun MZ, Oh MC, Ivan ME, et al. Current management of choroid plexus carcinomas. Neurosurg Rev. 2014; 37:179–192.

2. Ozdogan S, Gergin YE, Gergin S, et al. Choroid plexus carcinoma in adults: an extremely rare case. Pan Afr Med J. 2015; 20:302.

3. Bahar M, Hashem H, Tekautz T, et al. Choroid plexus tumors in adult and pediatric populations: the Cleveland Clinic and University Hospitals experience. J Neurooncol. 2017; 132:427–432.

4. Sav A, Scheithauer BW, Mazzola CA, Ketterling SR, Thompson SJ, Reilly MH. Oncocytic choroid plexus carcinoma: case report. Clin Neuropathol. 2010; 29:14–20.

5. Chow E, Reardon DA, Shah AB, et al. Pediatric choroid plexus neoplasms. Int J Radiat Oncol Biol Phys. 1999; 44:249–254.

6. Wrede B, Hasselblatt M, Peters O, et al. Atypical choroid plexus papilloma: clinical experience in the CPT-SIOP-2000 study. J Neurooncol. 2009; 95:383–392.

7. Gwak HS, Lee SH, Park WS, Shin SH, Yoo H, Lee SH. Recent advancements of treatment for leptomeningeal carcinomatosis. J Korean Neurosurg Soc. 2015; 58:1–8.

8. Guo P, Tang W, Li S, et al. Choroid plexus carcinoma in the external ventricle of an adult. J Craniofac Surg. 2015; 26:e664–e666.

9. Lozier AP, Arbaje YM, Scheithauer BW. Supratentorial, extraventricular choroid plexus carcinoma in an adult: case report. Neurosurgery. 2009; 65:E816–E817.

10. Bohara M, Hirabaru M, Fujio S, et al. Choroid plexus tumors: experience of 10 cases with special references to adult cases. Neurol Med Chir (Tokyo). 2015; 55:891–900.

11. Yip CM, Tseng HH, Hsu SS. Choroid plexus carcinoma: a rare tumor in adult. Surg Sci. 2014; 5:146–149.

12. Gopal P, Parker JR, Debski R, Parker JC Jr. Choroid plexus carcinoma. Arch Pathol Lab Med. 2008; 132:1350–1354.

13. Wolff JE, Sajedi M, Coppes MJ, Anderson RA, Egeler RM. Radiation therapy and survival in choroid plexus carcinoma. Lancet. 1999; 353:2126.

14. Cannon DM, Mohindra P, Gondi V, Kruser TJ, Kozak KR. Choroid plexus tumor epidemiology and outcomes: implications for surgical and radiotherapeutic management. J Neurooncol. 2015; 121:151–157.

15. Fabi A, Salesi N, Di Cocco B, et al. Choroid plexus carcinoma in the adult: is there a role for chemotherapy. J Exp Clin Cancer Res. 2005; 24:493–496.