MGMT promoter methylation and temozolomide response in choroid plexus carcinoma

Abstract

Choroid plexus carcinoma (CPC) is a malignant tumor with a strong tendency to spread along the cerebrospinal fluid pathway. There is no standardized chemotherapy protocol for this rare tumor. We report a 38-year-old man with CPC in the lateral ventricle with obstructive hydrocephalus. Because of the poor demarcation between thalamus and fornix, subtotal tumor resection was performed. Postoperative spine magnetic resonance (MR) image revealed whole spinal axis dissemination. After diagnosis of CPC, the patient was treated with whole ventricular and spine radiation concomitant with temozolomide chemotherapy, although the O 6 -methylguanine-DNA methyltransferase (MGMT) promoter was found to be unmethylated. Although MR images revealed transient stable disease during adjuvant therapy, tumor progression was depicted after four cycles of temozolomide therapy. We discuss the ineffectiveness of adjuvant temozolomide therapy for CPC in connection with O 6 -methylguanine-DNA methyltransferase promoter methylation.

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

“…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.…”

“…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.…”

“…This case was also characterized by the absence of diffuse leptomeningeal spread, which was a common feature in the previous cases of spinal metastasis of CPC. 7 - 9 , 11 , 13 ) CPPs and atypical CPPs show various patterns of spinal metastasis, such as diffuse leptomeningeal spread, 15 , 16 ) multiple nodular pattern, 17 - 19 ) and localized pattern. 20 , 21 ) Tumor control was good for the localized spinal metastasis but poor for the diffuse leptomeningeal spread.…”

“…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.…”

Choroid plexus carcinoma with leptomeningeal spread in an adult: a case report and review of the literature

“…Although the best chemotherapy regimen for CPC is still unknown, a report revealed long-term outcomes while applying temozolomide (TMZ) in CPC patient with MGMT methylation, while another report showed no effect of TMZ in the absence of MGMT methylation [ 4 , 5 , 14 ]. In our case, TMZ-based chemotherapy did not proceed in the patient because MGMT methylation was negative in the patient.…”