Management of pineal region tumors in a pediatric case series

Abstract

Pineal region tumors commonly present with non-communicating hydrocephalus. These heterogeneous histological entities require different therapeutic regimens. We evaluated our surgical experience concerning procurance of a histological diagnosis, management of hydrocephalus, and choice of antitumoral treatment. We analyzed the efficacy of neuroendoscopic biopsy and endoscopic third ventriculocisternostomy (ETV) in patients with pineal region tumors between 2006 and 2019 in a single-center retrospective cross-sectional study with regard to diagnostic yield, hydrocephalus treatment, as well as impact on further antitumoral management. Out of 28 identified patients, 23 patients presented with untreated hydrocephalus and 25 without histological diagnosis. One patient underwent open biopsy, and 24 received a neuroendoscopic biopsy with concomitant hydrocephalus treatment if necessary. Eighteen primary ETVs, 2 secondary ETVs, and 2 ventriculoperitoneal shunts (VPSs) were performed. Endoscopic biopsy had a diagnostic yield of 95.8% (23/24) and complication rates of 12.5% (transient) and 4.2% (permanent), respectively. ETV for hydrocephalus management was successful in 89.5% (17/19) with a median follow-up of more than 3 years. Following histological diagnosis, 8 patients (28.6%) underwent primary resection of their tumor. Another 9 patients underwent later-stage resection after either adjuvant treatment (n = 5) or for progressive disease during observation (n = 4). Eventually, 20 patients received adjuvant treatment and 7 were observed after primary management. One patient was lost to follow-up. Heterogeneity of pineal region tumor requires histological confirmation. Primary biopsy of pineal lesions should precede surgical resection since less than a third of patients needed primary surgical resection according to the German pediatric brain tumor protocols. Interdisciplinary decision making upfront any treatment is warranted in order to adequately guide treatment.

Keywords: Biopsy; ETV; Neuroendoscopy; Pineal region tumor; Shunt.

Fig. 1

Neuroendoscopic biopsy and third ventriculostomy. a Planning of two ideal trajectories, both through the foramen of Monro to the floor of the third ventricle and the pineal region tumor. The bisecting trajectory is the resulting trajectory into the ventricular system. b Intraoperative view of the right foramen of Monro with the superimposed trajectories to the floor and the pineal region. c Performing of an endoscopic third ventriculostomy. d Intraoperative view of the pineal region tumor (histology: germinoma) with superimposed preplanned target area (augmented reality—SCOPIS Neuro Navigation, Stryker)

Fig. 2

Flow chart of patient treatment regimen. Of the 28 patients with pineal region tumors and confirmed histological diagnosis, only 8 patients (28.6%) underwent a primary resection. Due to the heterogeneity of pineal region tumors, the appropriate treatment should be determined in a multidisciplinary approach

Fig. 3

Representative MR images. a Papillary tumor of the pineal region, treated with ETV and biopsy, microsurgical supracerebellar infratentorial resection, radiation, and chemotherapy (outcome: stable disease). b Disseminated pineoblastoma with involvement of the anterior third ventricular floor, treated with stented ETV and biopsy (right image demonstrating the stent in place though the floor of the third ventricle), radiation, and individualized poly-chemotherapy (outcome: death). c Pineoblastoma, treated with ETV and biopsy, neoadjuvant chemotherapy, and transcallosal microsurgical resection, followed by radiation and chemotherapy (outcome: stable disease). d Mixed malignant germ cell tumor, transferred after primary biopsy (external hospital), treated by transcortical, transventricular microsurgical resection; radiation; and chemotherapy (outcome: complete remission)