Childhood Cancer and Functional Impacts Across the Care Continuum (2021) / Chapter Skim
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6 Selected Central Nervous System Tumors
6 Selected Central Nervous System Tumors
Pages 275-300
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From page 275... ...
The seven most common CNS tumors that are diagnosed and treated in children are glial, ependymal, pineal, embryonal, CNS germ cell, choroid plexus, and craniopharyngioma tumors. The focus of this chapter is on the diagnosis, prognosis, treatment, and outcomes of these pediatric tumors.
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From page 276... ...
• Grade IV: These are the fastest-growing tumors and generally re quire the most aggressive treatment. Diagnostic Evaluation Diagnostic evaluation of a CNS tumor involves a number of scans, tests, and procedures (PDQ Pediatric Editorial Board, 2020c)
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From page 277... ...
For many types of tumors in other parts of the body, a staging system is used to describe where a tumor is located and whether it has spread. For CNS tumors, however, there is no universally accepted or recommended staging system because, unlike other cancers, tumors that start in the brain or spinal cord rarely spread to distant organs outside of the CNS (PDQ Pediatric Editorial Board, 2020c)
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From page 278... ...
The standard therapies for CNS tumors are surgery, radiation therapy, and chemotherapy (discussed briefly below) (PDQ Pediatric Editorial Board, 2020c)
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From page 279... ...
In sum, treatment of pediatric cancers is complex and requires a multidisciplinary approach. Optimal therapeutic management of children with primary brain or spinal cord tumors requires the coordinated efforts of a team of pediatric specialists who have expertise in the care of patients with these diseases (PDQ Pediatric Editorial Board, 2020c)
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From page 280... ...
Annex Tables 6-1 and 6-2 summarize the diagnostic, prognostic, and treatment information for each type of tumor discussed below. Glial Tumors The terms glioma and astrocytoma, commonly used interchangeably to describe the same type of CNS tumor, denote the most common types of pediatric CNS tumors.
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From page 281... ...
Most impairments caused by HGG are due to the tumor location and treatment interventions. Patients may develop paralysis, seizures, swallowing difficulties, endocrine dysfunction, and complications from surgery and radiation therapy (Ullrich, 2009)
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From page 282... ...
. Although rare, metastatic spinal disease and primary spinal LGG can occur, and for this reason, many practitioners will perform both baseline brain and spine MRI.
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From page 283... ...
. Central Nervous System Germ Cell Tumors Primary CNS germ cell tumors (GCTs)
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From page 284... ...
Patients may also develop paralysis; motor dysfunction; seizures; swallowing difficulties; and complications from surgery, chemotherapy, and radiation therapy. Because many children with CPT are very young at diagnosis, the tumor location, surgical intervention, and potential radiotherapy may lead to long-term neurocognitive dysfunction.
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From page 285... ...
The treatment of embryonal tumors includes attempted surgical resection, fractionated external beam radiation to the entire brain and spine with a focal boost to the tumor bed for approximately 6 weeks, and multidrug chemotherapy for an additional 12–15 months for children older than 3 years. Children younger than 3 years undergo surgical resection followed by chemotherapy for 12 months (Rutkowski et al., 2005)
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From page 286... ...
. Pineocytomas remain in the pineal region and rarely spread, whereas pineal parenchymal tumors spread in 10–15 percent of cases, while pineoblastomas spread throughout the CNS in up to 35 percent of cases (PDQ Pediatric Editorial Board, 2020d)
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From page 287... ...
Multimodal therapy for pineal tumors is of high intensity, typically requiring at least 6 months' recovery after treatment has been completed before the child has recovered sufficiently to resume activities, including full-time attendance at school. Specific neurologic impairments are highly related to the location of the primary tumor in the pineal gland, the severity of hydrocephalus at presentation, and craniospinal radiation.
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From page 288... ...
6-8 Optimal therapeutic management of children with primary brain or spinal cord tumors requires the coordinated efforts of a team of pediatric specialists who have expertise in the care of patients with these diseases. Conclusions 6-1 lassification of pediatric CNS tumors is not static and will C continue to evolve.
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From page 289... ...
, have a worse prognosis relative to nonmetastatic CNS tumors. 6-5 Although the overall survival rate for pediatric CNS tumors is promising, most children experience impairment as a result of the tumor and/or its treatment.
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2019. Chemotherapy followed by radiation therapy in treating younger patients with newly diagnosed localized central nervous system germ cell tumors.
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2001. Combined che motherapy and radiation therapy for CNS germ cell tumors: The Japanese experience.
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2020d. Childhood medulloblastoma and other central nervous system embryonal tumors treatment.
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From page 293... ...
2019. Diffuse intrinsic pontine glioma: From diagnosis to next generation clinical trials.
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From page 294... ...
Brain MRI, spine MRI, surgical resection or biopsy, lumbar puncture Subependymoma Brain MRI and surgical resection or biopsy Pineal tumor Pineocytoma Brain MRI, with or without spine MRI, surgical resection or biopsy Pineal parenchymal Brain MRI, plus spine MRI, surgical resection or biopsy Pineoblastoma Brain MRI, spine MRI, surgical resection or biopsy, lumbar puncture
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From page 295... ...
, but can 2015 occur in supratentorial region Myxopapillary and spine subtype (spine) >90% Localized Most commonly 4th ventricle >90% Localized or Pineal region >85% Clark et al., metastatic 2010 Localized or Pineal region 40–75% Al-Hussaini et metastatic al., 2009 Localized or Pineal region Children <3 Tian et al., metastatic years old, 2018 <20% continued
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From page 296... ...
Non-germinomatous Brain MRI, spine MRI, surgical resection or biopsy, lumbar puncture, tumor markers in blood and CSF Choroid plexus tumor c(5) Brain MRI, with or without spine MRI, with or without lumbar puncture, surgical resection or biopsy Craniopharyngioma c(6)
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From page 297... ...
Classic ependymoma; anaplastic ependymoma; tanycytic ependymoma; myxopapillary ependymoma (located in the spine) ; ependymoma RELA fusion positive; and ependymoma posterior fossa group A and group B. 4.
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From page 298... ...
c Subependymoma Pineal tumor Pineocytoma Pineal parenchymal Pineoblastoma Embryonal tumor * d CNS germ cell tumor Germinoma Non-germinomatous Choroid plexus tumor *
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From page 299... ...
for children >3–5 12–15 months years old, craniospinal radiation with tumor field boost and multidrug chemotherapy; (2) for children <3–5 years old, multidrug chemotherapy and often high-dose chemotherapy with autologous stem cell rescue Surgical resection/biopsy, plus chemotherapy and 6 months radiation Surgical resection/biopsy, plus chemotherapy and 6 months radiation Surgical resection alone for choroid plexus papilloma CPC and aCPP: variable (CPP)
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