Curative treatment for central nervous system medulloepithelioma despite residual disease after resection

Abstract

Medulloepithelioma of the central nervous system (CNS) is an uncommon primitive neuroectodermal tumor (PNET) usually occurring in early childhood. It is characterized by highly malignant behavior with a propensity for progression, recurrence, and dissemination despite intensive therapy. Due to its rarity, the optimal management is still unknown. However, gross total resection (GTR) has been considered crucial to achieve cure. In this article, the authors report on 2 cases of CNS medulloepithelioma in which long-term survival (more than 6 years) could be achieved despite evidence of, or suspected postoperative residual disease with an otherwise dismal prognosis.The patients were treated according to different strata of the protocol for primitive neuroectodermal tumors (PNET) of the German-Austrian multicenter trial of the German Society for Pediatric Oncology and Hematology (GPOH) for childhood brain tumors (HIT 2000). Treatment included postoperative hyperfractionated radiotherapy of the craniospinal axis followed by a boost to the tumor site in combination with chemotherapy. A review of the 2 reported and 37 previously published cases confirmed GTR and older age as positive prognostic factors.

Clinicoradiological features and surgical outcomes of primary intracranial medulloepitheliomas: a single-center experience and pooled analysis of individual patient data

“…components versus solid components varied greatly. After administration of gadolinium in 14 prior cases, MEPL usually presented with nonhomogeneous enhancement (n = 8), 5,20,22,24,26,29,32,33 but no enhancement (n = 3) was seen in studies by Hayase et al, 15 Mekni et al, 21 and Syal et al, 34 which is similar to case 5 from our series. The radiological characteristics of MEPL are nonspecific and cannot directly imply the diagnosis of MEPL or distinguish MEPL from other solid-cystic lesions.…”

“…The morphology was reported in only 21 cases (38.2%), among which 6 cases were solid, 8,18,22,26,34 11 cases were solid-cystic, 1,5,7,10,15,18,20,29,32,33 and 4 cases were cystic-solid. 24,28,37 Combined with our cases, it could be speculated that cystic degeneration is a common trait of MEPL; however, the proportion of lesions with cystic FIG. 4.…”

“…Among 10 patients with PFS, 5,10, 15,20-24, 29,38 9 patients received GTR, 8 patients received radiotherapy, and 6 patients received chemotherapy, which emphasizes the importance of GTR combined with adjuvant treatment, with GTR having the most striking impact. 24 Radiation regimens were only detailed in 4 of these 10 cases, including CSI (doses of 23.4, 36.0, and 26.0 Gy) plus local radiation (doses of 32.4, 32.0, and 20.0 Gy) in 3 cases, [22][23][24] and local radiotherapy (20.0 Gy) in 1 case. 20 In addition, CSI was used in another 5 patients 1,12,17,23,24 (dose range 25.2-41.0 Gy), among whom only 1 survived.…”

“…5,15 However, its side effects have been thoroughly discussed by Moftakhar et al, 22 and further investigation is needed to confirm whether AuPBSCT is superior to conventional therapy or if it can be used as an alternative to radiotherapy in pediatric patients. 15,22 Chemotherapy regimens, which were diverse, included intrathecal methotrexate injection, 23,24 4 cycles of thiotepa, 23 and/or traditional regimens comprising vincristine, 7,15,[22][23][24][25][26] etoposide, 15,23-26 cyclophosphamide 7,15,[22][23][24][25][26] / ifosfamide, 25 lomustine, 23,24 and/or carboplatin [23][24][25] /cisplatin. 7,15,[22][23][24][25][26] Overall, the combination of vincristine/cisplatin/cyclophosphamide was the most frequently used.…”

A Clinical Update and Radiologic Review of Pediatric Orbital and Ocular Tumors

“…Neoadjuvant chemotherapy includes combination ifosfamide, carboplatin, and etoposide. Multiple modalities of brachytherapy have been reported, including hyperfractionated radiotherapy of the craniospinal axis followed by a boost to the tumor site [30] and consolidating intrathecal brachytherapy using Yttrium 90 tagged with DOTA0-D-Phe1-Tyr3-octreotide (DOTATOC).…”