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:Pineal tumors
:Pineal tumors
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*Pineal tumors typically cause obstructive hydrocephalus
*Pineal tumors typically cause obstructive hydrocephalus.
*Germ cell tumors in the pineal region, independent of histological subtype, most commonly present with hydrocephalus, Parinaud syndrome (vertical gaze impairment, convergence nystagmus, and light-near pupillary response dissociation) obtundation, pyramidal tract signs, and ataxia.
*Patients with germinomas may present with hydrocephalus and obtundation or Parinaud's Syndrome.
*At the time of diagnosis, patients with nongerminomatous germ cell tumors tend to have larger tumors and more severe neurologic compromise, including a higher incidence of hydrocephalus and visual dysfunction.<ref>Packer, Roger J., Bruce H. Cohen, and Kathleen Cooney. "Intracranial germ cell tumors." The Oncologist 5.4 (2000): 312-320.</ref>
*In approximately 25 to 50 percent of cases symptoms of increased intracranial pressure are present, which include the following:
*In approximately 25 to 50 percent of cases symptoms of increased intracranial pressure are present, which include the following:
**Headache
**Headache
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**Behavioral changes
**Behavioral changes
**Decline in academic performance
**Decline in academic performance
*Neuroopthalmologic abnormalities are present in approximately 50 percent of cases
*Neuroopthalmologic abnormalities are present in approximately 50 percent of cases. Parinaud syndrome (vertical gaze impairment, convergence nystagmus, and light-near pupillary response dissociation)
**Paralysis of upward gaze  
**Paralysis of upward gaze  
**Paralysis of convergence  
**Paralysis of convergence  
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**Visual field deficit (e.g, bitemporal hemianopsia)
**Visual field deficit (e.g, bitemporal hemianopsia)
**In majority of cases patients with suprasellar GCTs have chronic subtle symptoms, and their tumors are diagnosed incidentally on imaging studies performed for unrelated reasons.
**In majority of cases patients with suprasellar GCTs have chronic subtle symptoms, and their tumors are diagnosed incidentally on imaging studies performed for unrelated reasons.
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:Multifocal or bifocal tumors<ref name="pmid1848284">{{cite journal| author=Hoffman HJ, Otsubo H, Hendrick EB, Humphreys RP, Drake JM, Becker LE et al.| title=Intracranial germ-cell tumors in children. | journal=J Neurosurg | year= 1991 | volume= 74 | issue= 4 | pages= 545-51 | pmid=1848284 | doi=10.3171/jns.1991.74.4.0545 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1848284  }} </ref>
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*Patients with multifocal or bifocal tumors may present with both suprasellar and pineal region syndromes.
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Revision as of 23:30, 15 February 2016

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]

Overview

Symptoms of germinoma include headache, vomiting, papilledema, lethargy, somnolence, ataxia, behavioral changes, decline in academic performance, paralysis of upward gaze,,paralysis of convergence, diabetes insipidus, delayed pubertal development, precocious puberty, isolated growth hormone deficiency, hypopituitarism (central hypothyroidism, adrenal insufficiency), decreased visual acuity from chiasmic or optic nerve compression, and visual field deficit (e.g, bitemporal hemianopsia). Presenting symptoms of patients with intracranial GCTs depend upon the location, size of the tumor, and the patient's age.[1][2][3]

History

Symptoms

  • Presenting symptoms of patients with intracranial GCTs depend upon the location, size of the tumor, and the patient's age.
  • Delays in diagnosis of germ cell tumors are common, especially symptoms related to endocrinopathy (diabetes insipidus, delayed vertical growth, etc.) are associated with delays in diagnosis of greater than 12 months, and are associated with higher incidences of disseminated disease.
  • In majority of patients with unrecognized CNS GCTs may have had a long history of symptoms such as enuresis, movement disorders, anorexia, and psychiatric complaints. In such cases diagnosis has been delayed from 7 months to 3 years.

The symptoms of CNS germ cell tumors by age are shown below in a tabular form:[4]

Age of the patient Symptoms of CNS GCT
Prenatal/neonate
  • Congenital teratomas produce polyhydramnios and hydrocephalus
Young infants
  • The teratoma and choriocarcinoma subtypes of nongerminoma germ cell tumor are most common in young infants; these patients may present with the following:
    • Irritability
    • Listlessness
    • Failure to thrive
    • Macrocephaly
    • Bulging fontanelle
Beyond infancy
  • Presentation depends on tumor location

Symptoms of pineal and suprasellar germ cell tumors are shown below in a tabular form:[1][2][3]

TYpe of the tumor Symptoms
Pineal tumors
  • Pineal tumors typically cause obstructive hydrocephalus.
  • Germ cell tumors in the pineal region, independent of histological subtype, most commonly present with hydrocephalus, Parinaud syndrome (vertical gaze impairment, convergence nystagmus, and light-near pupillary response dissociation) obtundation, pyramidal tract signs, and ataxia.
  • Patients with germinomas may present with hydrocephalus and obtundation or Parinaud's Syndrome.
  • At the time of diagnosis, patients with nongerminomatous germ cell tumors tend to have larger tumors and more severe neurologic compromise, including a higher incidence of hydrocephalus and visual dysfunction.[5]
  • In approximately 25 to 50 percent of cases symptoms of increased intracranial pressure are present, which include the following:
    • Headache
    • Vomiting
    • Papilledema
    • Lethargy
    • Somnolence
  • Other symptoms associated with pineal germ cell tumors (GCTs) and obstructive hydrocephalus include the following:
    • Ataxia
    • Behavioral changes
    • Decline in academic performance
  • Neuroopthalmologic abnormalities are present in approximately 50 percent of cases. Parinaud syndrome (vertical gaze impairment, convergence nystagmus, and light-near pupillary response dissociation)
    • Paralysis of upward gaze
    • Paralysis of convergence
  • Endocrinopathies are rarely associated with pineal tumors, although diabetes insipidus is sometimes observed which may indicate occult tumor involvement of the floor of the fourth ventricle and the suprasellar area
Suprasellar tumors
  • In majority of cases suprasellar germ cell tumors GCTs are commonly present with hypothalamic/pituitary dysfunctions which include the following:
    • Diabetes insipidus
    • Delayed pubertal development
    • Precocious puberty
    • Isolated growth hormone deficiency
    • Hypopituitarism (central hypothyroidism, adrenal insufficiency)
  • Suprasellar germ cell tumors GCTs can also cause ophthalmologic abnormalities such as:
    • Decreased visual acuity from chiasmic or optic nerve compression
    • Visual field deficit (e.g, bitemporal hemianopsia)
    • In majority of cases patients with suprasellar GCTs have chronic subtle symptoms, and their tumors are diagnosed incidentally on imaging studies performed for unrelated reasons.
Multifocal or bifocal tumors[6]
  • Patients with multifocal or bifocal tumors may present with both suprasellar and pineal region syndromes.

References

  1. 1.0 1.1 Sethi RV, Marino R, Niemierko A, Tarbell NJ, Yock TI, MacDonald SM (2013). "Delayed diagnosis in children with intracranial germ cell tumors". J Pediatr. 163 (5): 1448–53. doi:10.1016/j.jpeds.2013.06.024. PMID 23896184.
  2. 2.0 2.1 Packer RJ, Cohen BH, Cooney K, Coney K (2000). "Intracranial germ cell tumors". Oncologist. 5 (4): 312–20. PMID 10964999.
  3. 3.0 3.1 Crawford JR, Santi MR, Vezina G, Myseros JS, Keating RF, LaFond DA; et al. (2007). "CNS germ cell tumor (CNSGCT) of childhood: presentation and delayed diagnosis". Neurology. 68 (20): 1668–73. doi:10.1212/01.wnl.0000261908.36803.ac. PMID 17502547.
  4. Goodwin TL, Sainani K, Fisher PG (2009). "Incidence patterns of central nervous system germ cell tumors: a SEER Study". J Pediatr Hematol Oncol. 31 (8): 541–4. doi:10.1097/MPH.0b013e3181983af5. PMID 19636276.
  5. Packer, Roger J., Bruce H. Cohen, and Kathleen Cooney. "Intracranial germ cell tumors." The Oncologist 5.4 (2000): 312-320.
  6. Hoffman HJ, Otsubo H, Hendrick EB, Humphreys RP, Drake JM, Becker LE; et al. (1991). "Intracranial germ-cell tumors in children". J Neurosurg. 74 (4): 545–51. doi:10.3171/jns.1991.74.4.0545. PMID 1848284.


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