Improving survival in recurrent medulloblastoma: earlier detection, better treatment or still an impasse?

Abstract

Summary Early detection of relapse has been advocated to improve survival in children with recurrent medulloblastoma. However, the prognostic factors and the longer term outcome of these patients remains unclear. Pattern of recurrences were analysed in three consecutive protocols of the Societe Frangaise d'Oncologie Pediatrique . A uniform surveillance programme including repeated lumbar puncture combined with computerized tomography (CT) or magnetic resonance imaging (MRI) scan was applied for all registered patients. Forty-six out of 116 patients had progressive or recurrent disease. The median time from diagnosis to recurrence was 10.5 months and 76% relapses occurred during the first 2 years. Seventeen patients had asymptomatic relapses that were detected by the surveillance protocol. Forty-one patients were treated at time of progression. Twenty-three responded to salvage therapy and 11 achieved a second complete remission. The median survival time after progression was 5 months (<1-41 months), and only two patients remained alive at time of followup. Length of survival is primarily related to some specific patterns of relapse (time from diagnosis to recurrence, circumstances of relapse, extent of relapse) and to the response to salvage therapy. No evidence of long-term benefit appeared from any form of treatment.

Changes Mimicking New Leptomeningeal Disease After Intensity-Modulated Radiotherapy for Medulloblastoma

“…Recurrent medulloblastoma has a dismal prognosis (18)(19)(20)(21)(22)(23)(24). Therefore, the distinction between radiographic changes related to normal tissue damage by chemoradiotherapy and true disease progression affects the patient's subsequent treatment and prognosis.…”

Phase II study of oxaliplatin in children with recurrent or refractory medulloblastoma, supratentorial primitive neuroectodermal tumors, and atypical teratoid rhabdoid tumors

“…1,40 Several single agents and combination chemotherapy regimens have demonstrated activity in this group of patients. [2][3][4][7][8][9][10][11][12] Among the most active are platinum analogs.…”

“…[1][2][3][4][5][6] Both cisplatin [7][8][9] and carboplatin [10][11][12] have shown significant activity in this group of tumors, with cisplatin remaining the preferred agent by many neuro-oncologists. [13][14][15] However, cisplatin-associated ototoxicity and nephrotoxicity remain of great concern.…”

Promises and challenges of exhausting pediatric neural cancer stem cells

“…Unfortunately, a significant percentage of these tumors will recur. The conventional thinking regarding this phenomenon was that resistant clones were emerging in unstable cancer cells carrying additional mutations that enabled them to survive the insults of specific therapies (26)(27)(28). This concept is supported by the more aggressive behavior of recurrent tumors and their tendency to respond less to therapy.…”