Pediatric low-grade glioma treatment

Pediatric low grade glioma treatment

Low-grade gliomas (LGGs) constitute the largest, yet clinically and (molecular-) histologically heterogeneous group of pediatric brain tumors of WHO grades I and II occurring throughout all pediatric age groups and at all central nervous system (CNS) sites. The tumors are characterized by a slow growth rate and may show periods of growth arrest. Around 40% of all LGG patients can be cured by complete neurosurgical resection and are followed by close observation. In case of relapse, the second resection often is possible. Following incomplete resection, observation is recommended, as long as there is no radiologic tumor growth and the patient does not suffer from significant, tumor-related symptoms. This also applies to patients with a diagnosis of LGG on the basis of radiological criteria. By contrast, clinical worsening and/or radiologic progression are an indication of treatment with either chemo- or radiotherapyOverall survival is around 90%, and many patients survive with residual tumor, i. e. they suffer from chronic disease. All patients need comprehensive neuro-oncological care, the principles, and details of which are summarized in the current guidelines. These represent the standard of care for diagnostic work-up (including neuroimaging and neuropathology), and for therapeutic decisions (including the indications to non-surgical treatment) as well as concepts for neurosurgical intervention, chemotherapy, and radiotherapy as well as surveillance and rehabilitation. The current treatment algorithm was compiled by members of the LGG working group of the SIOP-E brain tumor group (SIOP-E-BTG) and is based upon the results of previous European LGG studies and international reports 1).


Within the multidisciplinary tumor board, all decisions concerning biopsy or resection at the time of diagnosis and progression must be carefully made weighing the potential risk of surgery vs. the therapeutic benefit of elucidating the histologic and molecular subtype of the tumor 2).

A treatment algorithm was compiled by members of the LGG working group of the SIOPE brain tumor group (SIOP-E-BTG) and is based upon the results of previous European LGG studies and international reports 3).

Since 2006 the German federal joint committee (G-BA, Gemeinsamer Bundesausschuss) has established that in line with all other pediatric hematological and oncological diseases – newly diagnosed low grade gliomas (LGG) patients must be treated within the current active society of pediatric oncology and hematology (Gesellschaft fuer paediatrische Onkologie und Haematologie, GPOH) trial or registry to ensure high quality standards of care and use of established referral systems 4).


Pediatric low grade gliomas (LGG) that are unresectable often require adjuvant chemotherapy such as carboplatin/vincristine. Small phase II studies have suggested equivalent efficacy of single agent 4-weekly carboplatin. A single-institution retrospective review captured all patients aged 0 to 18 years diagnosed with LGG between 1996 and 2013 and treated with carboplatin monotherapy. The response and survival according to tumor site was compared to published results for multi-agent chemotherapy. Of 268 children diagnosed with LGG diagnosed in this period, 117 received chemotherapy and 104 children received single agent carboplatin as first line chemotherapy. All patients received carboplatin at 560mg/m2 , four-weekly for a median of 12 courses. The mean age at diagnosis was 5.8 years (range 3m-16y) and 32% had neurofibromatosis type 1. With a mean followup of 54 months, 86% of patients achieved stabilisation or better (SD/PR/CR). 3-year progression free survival (PFS) 66% (95% C.I. 57% – 76%), and 5-year PFS was 51% (95% C.I. 41% – 63%). 5-year overall survival was 97%. Multivariate analysis showed poorer PFS for those with chiasmatic/hypothalamic tumors. In this retrospective analysis single agent carboplatin shows comparable efficacy to historical multiagent chemotherapy for the treatment of patients with unresectable LGG. Equivalent outcomes are achieved with less chemotherapy, reduced side effects and fewer hospital visits. Further research is required to establish the place of this simplified regimen in the up-front treatment of unresectable LGG 5).

Measures

Tumor measurement is important in unresectable pediatric low-grade gliomas (pLGGs) to determine either the need for treatment or assess response. Standard methods measure the product of the largest 2 lengths from transverse, anterior-posterior, and cranio-caudal dimensions (SM, cm). This single-institution study evaluated tumor volume measurements (VM, cm) in such pLGGs. Of 50 patients treated with chemotherapy for surgically inaccessible pLGG, 8 met the inclusion criteria of having 2 or more sequential MRI studies of T1-weighted Fast-Spoiled Gradient Recalled acquisition. SM and VM were performed by 2 independent neuroradiologists. Associations of measurement methods with defined therapeutic response criteria and patient clinical status were assessed. The mean tumor size at the first MRI scan was 20 cm and 398 cm according to SM and VM, respectively. VM results did not differ significantly from SM-derived spherical volume calculations (Pearson correlation, P<0.0001) with a high interrater reliability. Both methods were concordant in defining the tumor response according to the current criteria, although radiologic progressive disease was not associated with clinical status (SM: P=0.491, VM: P=0.208). In this limited experience, volumetric analysis of unresectable pLGGs did not seem superior to the standard linear measurements for defining tumor response 6).

References

1)

Gnekow AK, Kandels D, Tilburg CV, Azizi AA, Opocher E, Stokland T, Driever PH, Meeteren AYNS, Thomale UW, Schuhmann MU, Czech T, Goodden JR, Warmuth-Metz M, Bison B, Avula S, Kortmann RD, Timmermann B, Pietsch T, Witt O. SIOP-E-BTG and GPOH Guidelines for Diagnosis and Treatment of Children and Adolescents with Low Grade Glioma. Klin Padiatr. 2019 May;231(3):107-135. doi: 10.1055/a-0889-8256. Epub 2019 May 20. Erratum in: Klin Padiatr. 2019 May;231(3):e2. PubMed PMID: 31108561.
2)

PackerRJ,Pfister S,BouffetEetal.Pediatric low-grade gliomas: implications of the biologic era. Neuro-oncology 2017; 19: 750–761
3) , 4)

Gnekow AK, Kandels D, Tilburg CV, Azizi AA, Opocher E, Stokland T, Driever PH, Meeteren AYNSV, Thomale UW, Schuhmann MU, Czech T, Goodden JR, Warmuth-Metz M, Bison B, Avula S, Kortmann RD, Timmermann B, Pietsch T, Witt O. SIOP-E-BTG and GPOH Guidelines for Diagnosis and Treatment of Children and Adolescents with Low Grade Glioma. Klin Padiatr. 2019 May;231(3):107-135. doi: 10.1055/a-0889-8256. Epub 2019 May 20. PubMed PMID: 31108561.
5)

Dodgshun AJ, Maixner WJ, Heath JA, Sullivan MJ, Hansford JR. Single agent carboplatin for pediatric low-grade glioma: A retrospective analysis shows equivalent efficacy to multi-agent chemotherapy. Int J Cancer. 2015 Aug 1. doi: 10.1002/ijc.29711. [Epub ahead of print] PubMed PMID: 26235348.
6)

Kilday JP, Branson H, Rockel C, Laughlin S, Mabbott D, Bouffet E, Bartels U. Tumor volumetric measurements in surgically inaccessible pediatric low-grade glioma. J Pediatr Hematol Oncol. 2015 Jan;37(1):e31-6. doi: 10.1097/MPH.0000000000000168. PubMed PMID: 25517914.

Scalp cirsoid aneurysm treatment

Scalp cirsoid aneurysm treatment

In a systematic review, a total of 58.5% of cases scalp cirsoid aneurysm were managed with surgical excision only, 21.6% with endovascular embolization only, and 14.5% with a combination of both methods. 1).


The commonest artery involved in the scalp cirsoid aneurysm is the superficial temporal artery, due to its long and twisted course. The different methods of treatment include ‘en bloc’ resection and primary closure of the lesion, and sclerotherapy in which sodium tetradecyl sulfate is injected into the unwanted vessels with carbon dioxide gas, and the vessel is made to undergo sclerosis. The latter is associated with complications such as thromboembolismallergy and skin necrosis. Direct puncture endovascular embolization, using either chemical NBCA, absolute alcohol or mechanical coils, is another effective method widely used for AVM correction and an old method of ligation of feeding arteries, which is associated with the formation of collaterals and recurrence 2).


Heiferman published a case of a patient who underwent transvenous endovascular embolization followed by surgical excision via a bicoronal incision, as shown in a operative video. Care was taken to identify, cauterize, and transect feeding vessels from the superficial temporal, supratrochlear, and supraorbital arteries circumferentially to completely devascularize and resect the galeal nidus from overlying scalp tissue and underlying pericranium. Previously unreported in the literature, transosseous emissary veins partially draining the lesion was noted on angiography and were waxed thoroughly during surgery 3).


Although most SAVMs can be operated by traditional method of excision, use of temporary clipping of feeding arteries (like Superficial temporal artery[STA], External carotid artery[ECA]) enables total excision of giant SAVMs with minimal blood loss for a definitive cure. This novel technique obviates the need for preoperative embolization 4).


Munakomi et al. presented one case where staged embolization, excision, and subsequent grafting was done 5).


Percutaneous injection of sotradecol can be considered as one of the treatment options for arteriovenous fistula of the scalp. Further experience is needed to compare the safety and effectiveness of sotradecol with other agents currently used in the treatment of scalp arteriovenous fistulae 6).


Cirsoid aneurysms of the facial region, an uncommon cause of tinnitus, can be effectively managed by endovascular embolisation. This treatment obviates the need for surgery, which is associated with an increased risk of complications such as scarring, deformity and bleeding 7).


Well-planned surgery of cirsoid aneurysm of the scalp without preoperative interventions could achieve complete excision of the lesion without any residual masses or recurrence and with a low incidence of complications 8)9).

References

1)

Sofela A, Osunronbi T, Hettige S. Scalp Cirsoid Aneurysms: Case Illustration and Systematic Review of Literature. Neurosurgery. 2020 Feb 1;86(2):E98-E107. doi: 10.1093/neuros/nyz303. Erratum in: Neurosurgery. 2019 Dec 1;85(6):861. PubMed PMID: 31384940.
2)

Elkin DC. Cirsoid aneurysm of the scalp with the report of an advanced case. Ann Surg 1924; 80:332-40.
3)

Heiferman DM, Syed HR, Li D, Rothstein BD, Shaibani A, Tomita T. Resection of an Embolized Cirsoid Aneurysm With Intracranial Venous Drainage: 2-Dimensional Operative Video. Oper Neurosurg (Hagerstown). 2019 Mar 1;16(3):E94. doi: 10.1093/ons/opy303. PubMed PMID: 30295882.
4)

Gangadharaswamy SB, Maulyavantham Nagaraj N, Pai BS. Surgical management of scalp arteriovenous malformations using a novel surgical technique-Case series. Int J Surg Case Rep. 2017;37:250-253. doi: 10.1016/j.ijscr.2017.06.057. Epub 2017 Jul 8. PubMed PMID: 28715722; PubMed Central PMCID: PMC5514622.
5)

Munakomi S, Bhattarai B, Cherian I. Conquering the odds: Cirsoid aneurysm with holocranial feeders-staged embolization, excision and grafting. Asian J Neurosurg. 2015 Jul-Sep;10(3):259-61. doi: 10.4103/1793-5482.161167. PubMed PMID: 26396624; PubMed Central PMCID: PMC4553749.
6)

Hendrix LE, Meyer GA, Erickson SJ. Cirsoid aneurysm treatment by percutaneous injection of sodium tetradecyl sulfate. Surg Neurol. 1996 Dec;46(6):557-60; discussion 560-1. PubMed PMID: 8956889.
7)

Kumar A, Ahuja CK, Khandelwal N, Bakshi JB. Cirsoid aneurysm of the right pre-auricular region: an unusual cause of tinnitus managed by endovascular glue embolisation. J Laryngol Otol. 2012 Sep;126(9):923-7. doi: 10.1017/S0022215112001466. Epub 2012 Jul 5. PubMed PMID: 22874530.
8)

El Shazly AA, Saoud KM. Results of surgical excision of cirsoid aneurysm of the scalp without preoperative interventions. Asian J Neurosurg. 2012 Oct;7(4):191-6. doi: 10.4103/1793-5482.106651. PubMed PMID: 23559986; PubMed Central PMCID: PMC3613641.
9)

Chowdhury FH, Haque MR, Kawsar KA, Sarker MH, Momtazul Haque AF. Surgical management of scalp arterio-venous malformation and scalp venous malformation: An experience of eleven cases. Indian J Plast Surg. 2013 Jan;46(1):98-107. doi: 10.4103/0970-0358.113723. PubMed PMID: 23960313; PubMed Central PMCID: PMC3745130.

Third ventricular tumor treatment

Third ventricular tumor treatment

A plethora of surgical strategies have been described to reach deep-seated lesions situated within the third ventricle including the Rosenfeld, or transcallosal anterior interfoniceal approach.

Third ventricle tumors are surgical challenges because of the complex surrounding structures, including the hypothalamus, infundibulum, optic pathways, limbic system, and nearby vasculature 1).

These tumors cause obstructive hydrocephalus and thus necessitate a CSF diversion procedure such as an endoscopic third ventriculostomy (ETV), often coupled with an endoscopic biopsy (EBX). Lesions located posterior to the massa intermedia pose a technical challenge, as the use of a rigid endoscope for performing both an ETV and EBX is limited.

Roth and Constantini, recommend using a combined rigid-flexible endoscope for endoscopic third ventriculostomy and biopsy to approach posterior third ventricular tumors (behind the massa intermedia). This technique overcomes the limitations of using a rigid endoscope by reaching 2 distant regions 2).

The first choice treatment option for third ventricle lesions with dilated ventricles was endoscopic management 3). Among microsurgical approaches, the expanded transcallosal transforaminal approach was a more recently practiced and safe method of accessing the anterior and middle third ventricle. With this approach, the risk of damage to most of the vital structures, such as the fornix or the thalamus was avoided 4). The location of the junction of the anterior septal and internal cerebral vein is essential. Preoperative magnetic resonance (MR) venography can identify the junction. Some areas remain inaccessible, such as the anterosuperior and posterosuperior regions of the third ventricle 5).

The expanded transcallosal transforaminal approach remains a safe and relatively secure method of gaining access to the third ventricle 6).


There are three broad categories – anterior, lateral, and posterior routes. The anterior routes include transforaminal, interforniceal, transchoroidal, and subchoroidal. The subtemporal route is the main lateral corridor to the third ventricle and recommended if the tumor is located lateral to the sella turcica or extends into the middle cranial fossa 7). A transtubular access to the third ventricle is also practical. It enables blunt dissection of the corpus callosum which may minimize retraction injuries. Three-dimensional endoscopic visualization, coupled with a transparent plastic retractor, provides absolute and undeviating monitoring of the surgical corridor 8). In the third ventricle’s anterior portion, the endoscopic endonasal approach permits surgical maneuverability. The lamina terminalis and tuber cinereum are thought to be safe entry points for this approach 9). Tumors leading to the blockage of the Sylvian aqueduct can cause obstructive hydrocephalus; this calls for a CSF diversion procedure, endoscopic third ventriculostomy, combined with an endoscopic biopsy. Posterior third ventricular tumors should be approached using a combination of a rigid-flexible endoscope 10).


Colloid cyst treatment.

Choroid plexus papilloma treatment.

Craniopharyngioma treatment.


Operative approaches to tumors of the third ventricle, mainly the bifrontal approach through the lamina terminalis, has several advantages. First, the main arteries can be exposed and the operative field is sufficiently wide to render the operative procedure safe. Second, cortical incision or excision is unnecessary. By cutting the lamina terminalis, which is usually thin and expanded as a result of hydrocephalus, even a large tumor can be removed. In addition, lethal complications are avoided, because this approach has less possibility of damage to the lateral wall of the third ventricle. Seventeen cases of tumor in the third ventricle underwent operation via this approach. The operative technique for the bifrontal approach through the lamina terminalis and three representative cases are reported. This approach can be applied not only to tumors, but to arteriovenous malformations or giant aneurysms adjacent to the third ventricle 11).

References

1)

Tomasello F, Cardali S, Angileri FF, Conti A. Transcallosal approach to third ventricle tumors: How I do it. Acta Neurochir. 2013;155:1031–4.
2) , 10)

Roth J, Constantini S. Combined rigid and flexible endoscopy for tumors in the posterior third ventricle. J Neurosurg. 2015 Jun;122(6):1341-6. doi: 10.3171/2014.9.JNS141397. Epub 2015 Mar 27. PubMed PMID: 25816082.
3)

Chibbaro S, Di Rocco F, Makiese O, Reiss A, Poczos P, Mirone G, Servadei F, George B, Crafa P, Polivka M, Romano A. Neuroendoscopic management of posterior third ventricle and pineal region tumors: technique, limitation, and possible complication avoidance. Neurosurg Rev. 2012 Jul;35(3):331-38; discussion 338-40. doi: 10.1007/s10143-011-0370-1. Epub 2012 Jan 19. PubMed PMID: 22258494.
4) , 6)

Patel P, Cohen-Gadol AA, Boop F, Klimo P Jr. Technical strategies for the transcallosal transforaminal approach to third ventricle tumors: expanding the operative corridor. J Neurosurg Pediatr. 2014 Oct;14(4):365-71. doi: 10.3171/2014.6.PEDS1452. Epub 2014 Aug 8. PubMed PMID: 25105512.
5)

Ahmed SI, Javed G, Laghari AA, Bareeqa SB, Aziz K, Khan M, Samar SS, Humera RA, Khan AR, Farooqui MO, Shahbaz A. Third Ventricular Tumors: A Comprehensive Literature Review. Cureus. 2018 Oct 5;10(10):e3417. doi: 10.7759/cureus.3417. Review. PubMed PMID: 30542631; PubMed Central PMCID: PMC6284874.
7)

Cikla U, Swanson KI, Tumturk A, Keser N, Uluc K, Cohen-Gadol A, Baskaya MK. Microsurgical resection of tumors of the lateral and third ventricles: operative corridors for difficult-to-reach lesions. J Neurooncol. 2016 Nov;130(2):331-340. Epub 2016 May 27. Review. PubMed PMID: 27235145; PubMed Central PMCID: PMC5090015.
8)

Shoakazemi A, Evins AI, Burrell JC, Stieg PE, Bernardo A. A 3D endoscopic transtubular transcallosal approach to the third ventricle. J Neurosurg. 2015 Mar;122(3):564-73. doi: 10.3171/2014.11.JNS14341. Epub 2015 Jan 2. PubMed PMID: 25555026.
9)

Cavallo LM, Di Somma A, de Notaris M, Prats-Galino A, Aydin S, Catapano G, Solari D, de Divitiis O, Somma T, Cappabianca P. Extended Endoscopic Endonasal Approach to the Third Ventricle: Multimodal Anatomical Study with Surgical Implications. World Neurosurg. 2015 Aug;84(2):267-78. doi: 10.1016/j.wneu.2015.03.007. Epub 2015 Mar 28. PubMed PMID: 25827043.
11)

Suzuki J, Katakura R, Mori T. Interhemispheric approach through the lamina terminalis to tumors of the anterior part of the third ventricle. Surg Neurol. 1984 Aug;22(2):157-63. PubMed PMID: 6740479.

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