Subependymal giant cell astrocytoma surgery

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1], Associate Editor(s)-in-Chief: Ifeoma Odukwe, M.D. [2], Sujit Routray, M.D. [3]

Overview

Surgery is the first line therapy for subependymal giant cell astrocytoma. It is preferably indicated in cases such as tumor growth, acute hydrocephalus, and worsened seizure burden. The tumors that have invaded neighboring structures, those located bilaterally, and growing residual tumors are difficult to treat surgically. Medical therapy is favored in these cases. Some of the complications of surgical resection include transient memory loss, infection, and death. Gamma knife radiosurgery may also be used to treat subependymal giant cell astrocytoma with the risk of causing radiation-induced secondary tumor.

Indications

  • The indications for surgery in subependymal giant cell astrocytoma include:[1][2]

Surgery

  • Surgery is the mainstay of treatment for subependymal giant cell astrocytoma.[1]
  • Treatment has been solely surgical because of a lack of responsiveness to other strategies such as chemotherapy or radiation. The latter may also be associated with an increased risk of secondary malignancies.[3]
  • Generally, small tumors are usually less invasive and resecting them is associated with excellent clinical outcomes with low morbidity and mortality. However, when diagnosed at a later stage, the tumor more often affects and invades neighboring structures (fornix, hypothalamus, basal ganglia, and genu of internal capsule) and is associated with higher surgical morbidity and mortality.[2]
  • The tumors that have invaded neighboring structures, those located bilaterally, and growing residual tumors are difficult to treat with gross total resection. Treatment with everolimus, an mTOR inhibitor may be favored in these cases.[2]
  • Individuals with bilateral subependymal giant cell astrocytomas, tumors bigger than 2 cm, and children younger than 3 years of age are associated with significant surgical risk.[4]
  • After surgical resection, there is a possibility for residual tumors to enlarge although some may remain stable.[5]
  • Patients with tumors less than 2 cm and between 2 and 3 cm have a 0% and 46% chance of surgery-related complications respectively.[4]
  • Bilateral subependymal giant cell astrocytomas are associated with a 67% chance of surgery-related complications.[4]
  • Surgery may be associated with the risk of developing post-surgery subependymal giant cell astrocytoma and a repeat of the surgical procedure.[6]
  • The choice of treatment is still dependent on the experience of the individual physician. Despite the growing evidence on mTORi-induced tumor shrinkage, many centers still strictly advocate surgical treatment, whereas others prefer medical therapy.[3]
  • Tumors that are not completely resected can recur, requiring a repeat of the procedure.[7]
  • A ventriculoperitoneal (VP) shunt can be used to treat acutely symptomatic patients.[8]
  • Complications of surgery in the treatment subependymal giant cell astrocytoma include:[1][4]

Gamma Knife Radiosurgery

  • Gamma knife radiosurgery has been used to treat subependymal giant cell astrocytoma.[1]
  • The gamma knife surgery may significantly decrease the volume (70-80%) of subependymal giant cell astrocytoma at 6 months of treatment.[9]
  • Radiation-induced secondary tumor is a potential side effect. As a result of this, gamma knife radiosurgery is usually reserved for patients with contraindications to surgical and medical therapies as they are not associated with this side effect.[1]
  • Gamma knife radiosurgery is contraindicated in large tumors causing notable hydrocephalus because of its non-immediate tumor reduction effect.[1]

References

  1. 1.0 1.1 1.2 1.3 1.4 1.5 1.6 Campen CJ, Porter BE (2011). "Subependymal Giant Cell Astrocytoma (SEGA) Treatment Update". Curr Treat Options Neurol. 13 (4): 380–5. doi:10.1007/s11940-011-0123-z. PMC 3130084. PMID 21465222.
  2. 2.0 2.1 2.2 Jung TY, Kim YH, Jung S, Baek HJ, Lee KH (2015). "The clinical characteristics of subependymal giant cell astrocytoma: five cases". Brain Tumor Res Treat. 3 (1): 44–7. doi:10.14791/btrt.2015.3.1.44. PMC 4426277. PMID 25977907.
  3. 3.0 3.1 Roth, Jonathan; Roach, E. Steve; Bartels, Ute; Jóźwiak, Sergiusz; Koenig, Mary Kay; Weiner, Howard L.; Franz, David N.; Wang, Henry Z. (2013). "Subependymal Giant Cell Astrocytoma: Diagnosis, Screening, and Treatment. Recommendations From the International Tuberous Sclerosis Complex Consensus Conference 2012". Pediatric Neurology. 49 (6): 439–444. doi:10.1016/j.pediatrneurol.2013.08.017. ISSN 0887-8994.
  4. 4.0 4.1 4.2 4.3 Kotulska K, Borkowska J, Roszkowski M, Mandera M, Daszkiewicz P, Drabik K; et al. (2014). "Surgical treatment of subependymal giant cell astrocytoma in tuberous sclerosis complex patients". Pediatr Neurol. 50 (4): 307–12. doi:10.1016/j.pediatrneurol.2013.12.004. PMID 24507694.
  5. de Ribaupierre S, Dorfmüller G, Bulteau C, Fohlen M, Pinard JM, Chiron C; et al. (2007). "Subependymal giant-cell astrocytomas in pediatric tuberous sclerosis disease: when should we operate?". Neurosurgery. 60 (1): 83–89, discussion 89-90. doi:10.1227/01.NEU.0000249216.19591.5D. PMID 17228255.
  6. Sun P, Kohrman M, Liu J, Guo A, Rogerio J, Krueger D (2012). "Outcomes of resecting subependymal giant cell astrocytoma (SEGA) among patients with SEGA-related tuberous sclerosis complex: a national claims database analysis". Curr Med Res Opin. 28 (4): 657–63. doi:10.1185/03007995.2012.658907. PMID 22375958.
  7. Krueger, Darcy A.; Care, Marguerite M.; Holland, Katherine; Agricola, Karen; Tudor, Cynthia; Mangeshkar, Prajakta; Wilson, Kimberly A.; Byars, Anna; Sahmoud, Tarek; Franz, David Neal (2010). "Everolimus for Subependymal Giant-Cell Astrocytomas in Tuberous Sclerosis". New England Journal of Medicine. 363 (19): 1801–1811. doi:10.1056/NEJMoa1001671. ISSN 0028-4793.
  8. Jansen AC, Belousova E, Benedik MP, Carter T, Cottin V, Curatolo P; et al. (2019). "Newly Diagnosed and Growing Subependymal Giant Cell Astrocytoma in Adults With Tuberous Sclerosis Complex: Results From the International TOSCA Study". Front Neurol. 10: 821. doi:10.3389/fneur.2019.00821. PMC 6688052 Check |pmc= value (help). PMID 31428037.
  9. Ouyang, Taohui; Zhang, Na; Benjamin, Thomas; Wang, Long; Jiao, Jiantong; Zhao, Yiqing; Chen, Jian (2014). "Subependymal giant cell astrocytoma: current concepts, management, and future directions". Child's Nervous System. 30 (4): 561–570. doi:10.1007/s00381-014-2383-x. ISSN 0256-7040.


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