Supratentorial, Extraventricular Choroid Plexus Carcinoma in an Adult

Abstract

CPCa infrequently affects adults. Only rarely does it present as a supratentorial, extraventricular mass. Resection is the mainstay of therapy. Adjuvant radiation and chemotherapy are rational treatment options.

MGMT promoter methylation and temozolomide response in choroid plexus carcinoma

“…Although meta-analysis of CPC demonstrated that etoposide was the most effective drug, the response rate was only less than half of cases [33]. Several reports demonstrated efficacy of temozolomide for CPTs (Table 1) [7,12]. In two cases of recurrent CPP with craniospinal dissemination treated with temozolomide, one case showed radiographically stable disease for 17 months after surgery, whereas in the other case tumor control failed [7].…”

“…Some authors recommend regional radiation [30] whereas others performed craniospinal radiation [11,24]. Information concerning UC, U138MG cell line as unmethylated control; P, patient's tumor sample; MW, molecular weight marker appropriate doses and extent of radiation needed is still lacking [12,18]. For CPC patients, because dissemination and relapses outside the primary site are likely to occur, craniospinal radiation therapy should be considered, especially in adults who have less risk of intellectual sequelae caused by radiation than do children [11].…”

“…There is no standardized chemotherapy protocol for this rare tumor [1,[10][11][12]. Although meta-analysis of CPC demonstrated that etoposide was the most effective drug, the response rate was only less than half of cases [33].…”

“…The outcome of benign choroid plexus papilloma (CPP) is usually excellent following gross total resection, whereas the more aggressive biological behavior of CPC necessitates postoperative adjuvant treatment [1,9]. Chemotherapy has led to remission of choroid plexus carcinoma but does not always improve survival or avert recurrence [1,[10][11][12]. The DNA-alkylating agent temozolomide is effective for malignant gliomas, whereas evidence for its efficacy for CPT remains unclear [7,[12][13][14].…”

“…Chemotherapy has led to remission of choroid plexus carcinoma but does not always improve survival or avert recurrence [1,[10][11][12]. The DNA-alkylating agent temozolomide is effective for malignant gliomas, whereas evidence for its efficacy for CPT remains unclear [7,[12][13][14]. Promoter methylation of the O 6 -methylguanine-DNA methyltransferase (MGMT) DNA-repair gene is a marker for the response to alkylating agents in malignant gliomas, although this has not been probed in CPT [13][14][15].…”