Management of pineal region tumors in a pediatric case series

Matthias Schulz,Pablo Hernáiz Driever,Ulrich-Wilhelm Thomale,Arne Grün,David Capper,Anna Tietze,Arend Koch,Melissa Afshar-Bakshloo

doi:10.1007/s10143-020-01323-1

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.

Highlights

Tumors of the pineal region constitute only 2.8–11% of all brain tumors in children and adolescents and yet represent a challenge in terms of diagnosis and treatment [24]
We identified a total of 28 pediatric patients (13 females) with a pineal region tumor that were surgically treated at the pediatric neurosurgical department at Charité-Universitätsmedizin Berlin
At the time of first referral to our department, the histological diagnosis was established in 3 patients (10.7%; one pilocytic astrocytoma (PA), one mixed malignant germ cell tumor, and one choriocarcinoma) via stereotactic, open, and endoscopic biopsy procedures at the referring hospital, respectively

Summary

Introduction

Tumors of the pineal region constitute only 2.8–11% of all brain tumors in children and adolescents and yet represent a challenge in terms of diagnosis and treatment [24]. The combination of a typical tumorous lesion of the pineal region and the laboratory findings of elevated alpha fetoprotein (AFP) and beta human choriogonadotropin (β-HCG) in either serum or cerebrospinal fluid establishes the diagnosis of a secreting germ cell tumor. In this situation, according to the respective guidelines, no further histological confirmation is necessary [5]. Secreting mixed germ cell tumors do represent complex neoplasms which may warrant surgical therapy during the course of treatment

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Conclusion