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Year : 2012  |  Volume : 7  |  Issue : 1  |  Page : 71-73

Choroid plexus carcinoma: Case report and review of literature

Department of Neurosurgery, C.S.M. Medical University, Lucknow, India

Date of Web Publication28-Jun-2012

Correspondence Address:
Arvind Mishra
Department of Neurosurgery, C.S.M. Medical University, Lucknow
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1817-1745.97633

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How to cite this article:
Mishra A, Srivastava C, Singh SK, Chandra A, Ojha BK. Choroid plexus carcinoma: Case report and review of literature. J Pediatr Neurosci 2012;7:71-3

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Mishra A, Srivastava C, Singh SK, Chandra A, Ojha BK. Choroid plexus carcinoma: Case report and review of literature. J Pediatr Neurosci [serial online] 2012 [cited 2023 Jun 6];7:71-3. Available from: https://www.pediatricneurosciences.com/text.asp?2012/7/1/71/97633

Dear Sir,

Carcinoma of the choroid plexus is an uncommon intracranial neoplasm with a particularly virulent course. Around 80% of choroid plexus carcinoma (CPC) arise in children, in whom they constitute 15-20% of choroid plexus tumors. [1] Due to their rarity, reports on CPC most often focus on single cases or single-institution experiences with a limited number of patients. [2] Recently, we also encountered a case of left lateral ventricle CPC in a young child who was operated in poor neurological grade.

A 6-year-old male child presented to us with history of gradually progressive holocranial headache for 4 months and mild weakness right side of the body for 3 months. At the time of admission patient was conscious, with Glasgow coma score (GCS) E4V5M6, pupils were bilateral normal size and reactive, bilateral papilledema was present. Right-side hemiparesis (power: MRC grade 4/5) with right upper motor neuron facial paresis was present. Rest of the neurological examination was within normal limits.

Computed tomography (CT) brain was showing a large irregular hyperdense lesion with calcification and perilesional edema in left parietal region with infiltration of left lateral ventricle with hydrocephalus [Figure 1]a. Magnetic resonance imaging (MRI) brain was showing a large ill-defined tumor in left parietal region, hypointense on T1 and hyperintense on T2-weighted image and strong heterogeneous enhancement with gadolinium. The lesion was infiltrating left lateral ventricle and left thalamic region with dilatation of opposite lateral ventricle with midline shift and perilesional edema [Figure 2]. Based on radiology, a differential diagnosis of ependymoma, primitive neuroectodermal tumor, and high-grade glioma was kept.
Figure 1: Preoperative contrast Computed tomography (CT) scan (a) and postoperative contrast CT scan (b)

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Figure 2: Preoperative axial T1-weighted (a), axial T2-weighted (b) and sagittal and coronal gadolinium enhanced (c and d) magnetic resonance imaging images

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Preoperatively child deteriorated following an episode of seizure to GCS E1V1M3. His pupil became bilateral semidilated and sluggish reactive. Immediate ventricular tapping was done. Ventricular pressure was raised. External ventricular drain was put and patient was taken for emergency surgery.

A left parieto-occipital craniotomy was made and gross total excision of the tumor was done. Intraoperatively, brain was tense at dural opening. Tumor was reaching up to the cortical surface. Tumor was soft, moderately vascular, and partly suckable with a gritty consistency at places. Tumor appeared to arise from choroid plexus of lateral ventricle. Postoperative period was uneventful. Histopathology and immunohistochemistry were consistent with diagnosis of CPC [Figure 3]. Postoperative CT scan showed gross total excision of tumor [Figure 1]b. Patient gradually improved to GCS E4V2M5 and transferred to radiotherapy department. Patient died 15 days after completing radiotherapy due to nonsurgical cause.
Figure 3: (a) Hematoxylin and eosin staining showing papillary architecture with columnar cells having high nucleo cytoplasmic ratio with prominent nucleoli, fair numbers of mitosis, and areas of necrosis. (b) focally positive (dot like) glial fibrillary acidic protein. (c) focally positive cytokeratin

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CPCs are rare neoplasms of neuroectodermal origin corresponding to WHO grade III tumor. CPC account for 15-20% of choroid plexus tumors, but 80% of these malignant tumors are found in children. [1] Due to their rarity, reports on CPC most often focus on single cases or single-institution experiences with a limited number of patients. Only seven previous series have analyzed 8 or more patients in the last 30 years. [2]

Clinically, this group of tumors tends to cause hydrocephalus and increased intracranial pressure. Bleggi-Torres et al. reported 15 cases of CPC and pointed out that the main symptoms of this tumor are hydrocephalus (62.5%), intracranial hypertension (25%), and convulsion (12.5%). [2] Neuroradiological features are nonspecific in CPC. Some features may suggest the diagnosis, such as when the tumor invades the parenchyma or presents with metastatic nodules in the third, fourth, or lateral ventricles. But some choroid plexus papillomas also demonstrate adjacent cerebral edema and invasion, whereas some carcinomas do not. All the tumors in the differential diagnosis, including ependymoma, primitive neuroectodermal tumor, astrocytoma, teratoma, and meningioma, can have similar imaging characteristics and modern imaging cannot yet accurately define the pathologic diagnosis. [2],[3]

Surgical resection is considered to be the most effective treatment for CPCs. The extent of surgical resection remains the most important factor in determining long-term survival in patients with CPC, but patients treated only with surgery have had a very poor outcome: disease progresses rapidly and patients often die within 1 year. The early use of radiation therapy may extend the survival. [2],[3] Unfortunately, radiotherapy is not an option in the majority of cases because of the young age of the patients and the size of the field to be irradiated. Chemotherapy contributes to long-term survival, but it cannot prevent recurrence. Current data strongly support the use of combined chemoradiation in patients older than 3 years and chemotherapy alone if the patients are younger, but the total amount of necessary adjuvant treatment and the order in which the modalities are to be used are still controversial. Unfortunately, the incidence of CPC is too low to set up a randomized study assessing radiotherapy or chemotherapy protocols for patients with CPC. [3],[4]

Most of the time, we do not suspect this entity preoperatively and the diagnosis is established after histopathology report. Sometimes, the distinction between choroid plexus papilloma and CPC is not obvious even with histopathology and in these cases immunohistochemistry becomes important in establishing the diagnosis, as happened in our case. [5] Choroid plexus papillomas have histologic features that are very similar to those of the normal choroid plexus. The histologic criteria for malignant tumors of the choroid plexus, that is, CPCs were first developed by Lewis in the 1960s, refined by Russell and Rubinstein two decades later, and most recently modified by the WHO. The established criteria are as follows: (1) obvious invasion of adjacent neural tissue with the infiltrating cells on a stromal base; (2) loss of regular papillary architecture; and (3) evidence of increased mitotic activity, nuclear atypia, and necrosis. [1],[3] CPCs typically stain positive for cytokeratins and display variable expression of vimentin, S100, transthyretin, and glial fibrillary acidic protein (GFAP). Positivity for S100 and transthyretin is typically less than that seen in CPP. CPCs stain positive for GFAP in approximately 20% of tumors. [3],[6] In our case, immunohistochemical staining was positive for GFAP and cytokeratin.

Although this tumor is still associated with a poor prognosis, there has been a slight but significant increase in survival throughout the past decades. Dohrmann and Collias reported a 9-month median survival time in a review of 16 children operated on for CPC. [7],[8] In 1992, Packer et al. reported 11 patients with CPC with a 45% event-free survival rate and a median progression-free time of 48 months. [9] Girish et al. reported median survival of 58 months for CPCs who underwent gross total excision with adjuvant therapy and of 36 months who had a subtotal resection with adjuvant therapy. [3]

In our case, patient died 3 months after operation despite gross total excision of tumor and adjuvant radiotherapy, as the long-term survival not only depends on complete excision but also on preoperative neurological status. Our patient was operated in low GCS, i.e., E1V1M3, although he gradually improved to GCS E4V2M5 but expired due to nonsurgical cause, probably aspiration pneumonitis.

   References Top

1.WHO classification of tumors of central nervous system. 4 th ed. WHO; 2007. p. 82-6.  Back to cited text no. 1
2.Bleggi-Torres LF, Urban LA, Antoniuk A, Carboni P, Ramina R, Gugelmin ES. Choroid plexus carcinoma: Report of 15cases. Arq Neuropsiquiatr 2000;58:505-11.  Back to cited text no. 2
3.Menon G, Nair SN, Baldewa SS, Rao RB, Krishnakumar KP, Gopalakrishnan CV. Choroid plexus tumors: An institutional series of 25 patients. Neurol India 2010;58:429-35.  Back to cited text no. 3
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4.Wrede B, Liu P, Wolff JE. Chemotherapy improves the survival of patients with choroid plexus carcinoma: A meta-analysis of individual cases with choroid plexus tumors. J Neurooncol 2007;85:345-51.  Back to cited text no. 4
5.Singh A, Yermani S, Shruti S. Choroid plexus carcinoma: Report of two cases. Indian J Pathol Microbiol 2009;52:405-7.  Back to cited text no. 5
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6.Gopal P, Parker JR, Debski R, Parker JC Jr. Choroid plexus carcinoma. Arch Pathol Lab Med 2008;132:1350-4.  Back to cited text no. 6
7.Collias JC. Choroid plexus carcinoma. J Neurosurg 1975;43:225-32.  Back to cited text no. 7
8.Berger C, Thiesse P, Lellouch-Tubiana A, Kalifa C, Pierre-Kahn A, Bouffet E. Choroid plexus carcinomas in childhood: Clinical features and prognostic factors. Neurosurgery 1998;42:470-5.  Back to cited text no. 8
9.Packer RJ, Perilongo G, Johnson D, Sutton LN, Vezina G, Zimmermann RA, et al. Choroid plexus carcinoma of childhood. Cancer 1992; 69:580-5.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3]

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