Germinoma natural history: Difference between revisions

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{{Germinoma}}
{{Germinoma}}
{{CMG}}{{AE}}{{Simrat}}
{{CMG}}{{AE}}{{Simrat}}
==Overview==
==Overview==
 
If left untreated, more than 50% of patients may continue to suffer from endocrine abnormalities such as [[growth hormone deficiency]], growth retardation, [[hypopituitarism]], and [[hypothyroidism]], and may require lifelong [[hormonal replacement therapy]]. Common complications of germinoma include secondary cancers such as [[acute myeloid leukemia]] and radiation-induced brain neoplasms. Prognosis is generally excellent for germinomas, and the 5-year survival rate of patients with germinoma is approximately 70-90%. However, prognosis in general depends on the histological diagnosis and staging of the extent of disease.
==Natural history==
==Natural History==
* Malignant transformation of primordial germ cells that inappropriately migrated during development (either failing to migrate into or out of an area) are the originators of germinomas. There is no histologic differentiation whereas [[germ cell tumor|nongerminomatous germ cell tumors]] display a variety of differentiation.
*Usually, patients with tumors in the pineal region tend to present with a brief history of symptoms compared with tumors of the basal ganglionic or [[suprasellar]] region, which presents a long history of symptoms including [[Diplopia|double vision]] related to [[tectal]] and [[Cerebral aqueduct|aqueductal]] compression.<ref name="urlChildhood Central Nervous System Germ Cell Tumors Treatment (PDQ®) - PDQ Cancer Information Summaries - NCBI Bookshelf">{{cite web |url=https://www.ncbi.nlm.nih.gov/books/NBK82800/ |title=Childhood Central Nervous System Germ Cell Tumors Treatment (PDQ®) - PDQ Cancer Information Summaries - NCBI Bookshelf |format= |work= |accessdate=}}</ref>
*Approximately 35% of patients with suprasellar tumors will be asymptomatic for more than six months, and in these patients, the time between first symptom and diagnosis may be prolonged. Tumors arising in the suprasellar region often present with overt or subtle hormonal deficiencies and a prolonged prodrome often lasting months to years. A wide majority of patients may present with [[diabetes insipidus]] as the presenting finding; patients can usually compensate for antidiuretic hormone  deficiency for months to years by drinking excessive amounts of fluid. This consequently leads to development of [[hormonal]] and [[Visual impairment|visual changes]] as the [[tumor]] expands dorsally and compresses the [[optic chiasm]]. [[Symptoms]] related to endocrinopathy (delayed vertical growth, [[diabetes insipidus]], etc.) are associated with delays in diagnosis of greater than 12 months, and are associated with higher incidences of disseminated disease.<ref name="pmid25266413">{{cite journal |vauthors=Kilday JP, Laughlin S, Urbach S, Bouffet E, Bartels U |title=Diabetes insipidus in pediatric germinomas of the suprasellar region: characteristic features and significance of the pituitary bright spot |journal=J. Neurooncol. |volume=121 |issue=1 |pages=167–75 |date=January 2015 |pmid=25266413 |doi=10.1007/s11060-014-1619-7 |url=}}</ref><ref name="pmid28436607">{{cite journal |vauthors=Frappaz D, Pedone C, Thiesse P, Szathmari A, Conter CF, Mottolese C, Carrie C |title=Visual complaints in intracranial germinomas |journal=Pediatr Blood Cancer |volume=64 |issue=10 |pages= |date=October 2017 |pmid=28436607 |doi=10.1002/pbc.26543 |url=}}</ref>
*In approximately 22% of cases metastasis has been noted at time of diagnosis.
*The presentation is often delayed with the tumors of the [[thalami]] and [[basal ganglia]], with a larger [[tumor]] at diagnosis.
*Although germ cell tumors may be disseminated at the time of diagnosis, signs and symptoms of [[spinal cord]] or cerebral cortical involvement are uncommon, except for some infrequent cases of germinomas which arise in the basal ganglionic region or [[thalamus]].<ref name="NCI">Germ cell tumors. National Cancer Institute(2015) http://www.cancer.gov/types/brain/hp/child-cns-germ-cell-treatment-pdq#link/_60_toc Accessed on February 16, 2016</ref><ref name="pmid19820898">{{cite journal| author=Afzal S, Wherrett D, Bartels U, Tabori U, Huang A, Stephens D et al.| title=Challenges in management of patients with intracranial germ cell tumor and diabetes insipidus treated with cisplatin and/or ifosfamide based chemotherapy. | journal=J Neurooncol | year= 2010 | volume= 97 | issue= 3 | pages= 393-9 | pmid=19820898 | doi=10.1007/s11060-009-0033-z | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19820898  }} </ref><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><ref>Packer, Roger J., Bruce H. Cohen, and Kathleen Cooney. "Intracranial germ cell tumors." The Oncologist 5.4 (2000): 312-320.</ref><ref name="radio">Germ cell tumors. Radiopedia(2015) http://radiopaedia.org/articles/central-nervous-system-germinoma Accessed on January 25, 2016</ref><ref name="wiki">Germinoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Germinoma Accessed on January 26, 2016</ref><ref name="pmid23896184">{{cite journal| author=Sethi RV, Marino R, Niemierko A, Tarbell NJ, Yock TI, MacDonald SM| title=Delayed diagnosis in children with intracranial germ cell tumors. | journal=J Pediatr | year= 2013 | volume= 163 | issue= 5 | pages= 1448-53 | pmid=23896184 | doi=10.1016/j.jpeds.2013.06.024 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23896184  }} </ref>


==Complications==
==Complications==
*Patients with intracranial tumors located in the basal ganglia perform poorly compared with those who have tumors in the suprasellar and pineal regions; they have lower short-term retention of visual and verbal stimuli and full-scale IQs.
*Patients with intracranial tumors located in the [[basal ganglia]] perform poorly compared with those who have tumors in the [[suprasellar]] and [[pineal]] regions; they have lower short-term retention of visual and verbal stimuli and full-scale IQs.
*Larger irradiation volume and dose effect the following functions of the brain adversely:
*Larger irradiation volume and dose effect the following functions of the brain adversely:<ref name="pmid20425608">{{cite journal |vauthors=Giglio P, Gilbert MR |title=Neurologic complications of cancer and its treatment |journal=Curr Oncol Rep |volume=12 |issue=1 |pages=50–9 |date=January 2010 |pmid=20425608 |pmc=3637950 |doi=10.1007/s11912-009-0071-x |url=}}</ref>
**Intellectual functions
**Intellectual functions
**Concept
**Concept
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**Memory
**Memory
**Decline in neurocognitive function, and performance IQs
**Decline in neurocognitive function, and performance IQs
*Approximately more than 50% of patients may continue to suffer from endocrine abnormalities such as growth hormone deficiency, growth retardation, hypopituitarism, and hypothyroidism, and may require lifelong hormonal replacement therapy
*Approximately more than 50% of patients may continue to suffer from endocrine abnormalities such as [[growth hormone deficiency]], growth retardation, [[hypopituitarism]], and [[hypothyroidism]], and may require lifelong [[hormonal replacement therapy]]<ref name="pmid10206457">{{cite journal |vauthors=Hale GA, Marina NM, Jones-Wallace D, Greenwald CA, Jenkins JJ, Rao BN, Luo X, Hudson MM |title=Late effects of treatment for germ cell tumors during childhood and adolescence |journal=J. Pediatr. Hematol. Oncol. |volume=21 |issue=2 |pages=115–22 |date=1999 |pmid=10206457 |doi= |url=}}</ref><ref name="pmid25295241">{{cite journal |vauthors=Barnes N, Chemaitilly W |title=Endocrinopathies in survivors of childhood neoplasia |journal=Front Pediatr |volume=2 |issue= |pages=101 |date=2014 |pmid=25295241 |pmc=4172013 |doi=10.3389/fped.2014.00101 |url=}}</ref>
*Due to surgical resection of tumor or due surgical biopsies the following complications may occur:
*Due to surgical resection of tumor or due surgical biopsies the following complications may occur:
**Poor performance in psychosocial skills
**Poor performance in psychosocial skills
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*The surgical morbidity associated with pineal-region tumors is approximately 2-5%. Patients may suffer from the following:
*The surgical morbidity associated with pineal-region tumors is approximately 2-5%. Patients may suffer from the following:
**Transient movement abnormalities of eyes
**Transient movement abnormalities of eyes
**Ataxia
**[[Ataxia]]
**Cognitive dysfunction
**Cognitive dysfunction
*The other complications that may present in patients with intracranial germ cell tumors are following:
*The other complications that may present in patients with intracranial germ cell tumors are following:
**Brain atrophy
**[[Brain atrophy]]
**Multifocal encephalomalacia
**[[Multifocal encephalomalacia]]
**Leukoencephalopathy
**[[Leukoencephalopathy]]
**Focal necrosis  
**Focal necrosis  
**Cerebrovascular occlusion  
**Cerebrovascular occlusion  
*The incidence of secondary cancer is approximately 6%, in patients with intracranial tumors. The risk of death due to malignancy is approximately 16%. Radiation therapy and chemotherapy may both promote the development of secondary cancers such as acute myeloid leukemia and radiation-induced brain neoplasms.<ref name="pmid19329867">{{cite journal| author=Sugiyama K, Yamasaki F, Kurisu K, Kenjo M| title=Quality of life of extremely long-time germinoma survivors mainly treated with radiotherapy. | journal=Prog Neurol Surg | year= 2009 | volume= 23 | issue=  | pages= 130-9 | pmid=19329867 | doi=10.1159/000210059 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19329867  }} </ref><ref name="pmid10598695">{{cite journal| author=Sutton LN, Radcliffe J, Goldwein JW, Phillips P, Janss AJ, Packer RJ et al.| title=Quality of life of adult survivors of germinomas treated with craniospinal irradiation. | journal=Neurosurgery | year= 1999 | volume= 45 | issue= 6 | pages= 1292-7; discussion 1297-8 | pmid=10598695 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10598695  }} </ref><ref name="pmid23373627">{{cite journal| author=Jinguji S, Yoshimura J, Nishiyama K, Aoki H, Nagasaki K, Natsumeda M et al.| title=Factors affecting functional outcomes in long-term survivors of intracranial germinomas: a 20-year experience in a single institution. | journal=J Neurosurg Pediatr | year= 2013 | volume= 11 | issue= 4 | pages= 454-63 | pmid=23373627 | doi=10.3171/2012.12.PEDS12336 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23373627  }} </ref><ref name="pmid25422317">{{cite journal| author=Acharya S, DeWees T, Shinohara ET, Perkins SM| title=Long-term outcomes and late effects for childhood and young adulthood intracranial germinomas. | journal=Neuro Oncol | year= 2015 | volume= 17 | issue= 5 | pages= 741-6 | pmid=25422317 | doi=10.1093/neuonc/nou311 | pmc=PMC4482856 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25422317  }} </ref><ref name="pmid24715277">{{cite journal| author=Martens T, Rotermund R, Zu Eulenburg C, Westphal M, Flitsch J| title=Long-term follow-up and quality of life in patients with intracranial germinoma. | journal=Neurosurg Rev | year= 2014 | volume= 37 | issue= 3 | pages= 445-50; discussion 451 | pmid=24715277 | doi=10.1007/s10143-014-0544-8 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24715277  }} </ref><ref name="pmid22420962">{{cite journal| author=Odagiri K, Omura M, Hata M, Aida N, Niwa T, Ogino I et al.| title=Treatment outcomes, growth height, and neuroendocrine functions in patients with intracranial germ cell tumors treated with chemoradiation therapy. | journal=Int J Radiat Oncol Biol Phys | year= 2012 | volume= 84 | issue= 3 | pages= 632-8 | pmid=22420962 | doi=10.1016/j.ijrobp.2011.12.084 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22420962  }} </ref><ref name="pmid25621861">{{cite journal| author=Chaudhry NS, Ahmad FU, Whittington E, Schatz N, Morcos JJ| title=Primary intrinsic chiasmal germinoma. | journal=J Neuroophthalmol | year= 2015 | volume= 35 | issue= 2 | pages= 171-4 | pmid=25621861 | doi=10.1097/WNO.0000000000000201 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25621861 }} </ref>
*The incidence of secondary cancer is approximately 6%, in patients with intracranial tumors. The risk of death due to malignancy is approximately 16%. [[Radiation therapy]] and [[chemotherapy]] may both promote the development of secondary cancers such as [[acute myeloid leukemia]] and radiation-induced brain neoplasms.<ref name="pmid19329867">{{cite journal| author=Sugiyama K, Yamasaki F, Kurisu K, Kenjo M| title=Quality of life of extremely long-time germinoma survivors mainly treated with radiotherapy. | journal=Prog Neurol Surg | year= 2009 | volume= 23 | issue=  | pages= 130-9 | pmid=19329867 | doi=10.1159/000210059 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19329867  }} </ref><ref name="pmid10598695">{{cite journal| author=Sutton LN, Radcliffe J, Goldwein JW, Phillips P, Janss AJ, Packer RJ et al.| title=Quality of life of adult survivors of germinomas treated with craniospinal irradiation. | journal=Neurosurgery | year= 1999 | volume= 45 | issue= 6 | pages= 1292-7; discussion 1297-8 | pmid=10598695 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10598695  }} </ref><ref name="pmid23373627">{{cite journal| author=Jinguji S, Yoshimura J, Nishiyama K, Aoki H, Nagasaki K, Natsumeda M et al.| title=Factors affecting functional outcomes in long-term survivors of intracranial germinomas: a 20-year experience in a single institution. | journal=J Neurosurg Pediatr | year= 2013 | volume= 11 | issue= 4 | pages= 454-63 | pmid=23373627 | doi=10.3171/2012.12.PEDS12336 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23373627  }} </ref><ref name="pmid25422317">{{cite journal| author=Acharya S, DeWees T, Shinohara ET, Perkins SM| title=Long-term outcomes and late effects for childhood and young adulthood intracranial germinomas. | journal=Neuro Oncol | year= 2015 | volume= 17 | issue= 5 | pages= 741-6 | pmid=25422317 | doi=10.1093/neuonc/nou311 | pmc=PMC4482856 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25422317  }} </ref><ref name="pmid24715277">{{cite journal| author=Martens T, Rotermund R, Zu Eulenburg C, Westphal M, Flitsch J| title=Long-term follow-up and quality of life in patients with intracranial germinoma. | journal=Neurosurg Rev | year= 2014 | volume= 37 | issue= 3 | pages= 445-50; discussion 451 | pmid=24715277 | doi=10.1007/s10143-014-0544-8 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24715277  }} </ref><ref name="pmid22420962">{{cite journal| author=Odagiri K, Omura M, Hata M, Aida N, Niwa T, Ogino I et al.| title=Treatment outcomes, growth height, and neuroendocrine functions in patients with intracranial germ cell tumors treated with chemoradiation therapy. | journal=Int J Radiat Oncol Biol Phys | year= 2012 | volume= 84 | issue= 3 | pages= 632-8 | pmid=22420962 | doi=10.1016/j.ijrobp.2011.12.084 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22420962  }} </ref><ref name="pmid14967424">{{cite journal| author=Ogawa K, Shikama N, Toita T, Nakamura K, Uno T, Onishi H et al.| title=Long-term results of radiotherapy for intracranial germinoma: a multi-institutional retrospective review of 126 patients. | journal=Int J Radiat Oncol Biol Phys | year= 2004 | volume= 58 | issue= 3 | pages= 705-13 | pmid=14967424 | doi=10.1016/j.ijrobp.2003.07.001 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14967424  }} </ref><ref name="pmid24101738">{{cite journal| author=Liang SY, Yang TF, Chen YW, Liang ML, Chen HH, Chang KP et al.| title=Neuropsychological functions and quality of life in survived patients with intracranial germ cell tumors after treatment. | journal=Neuro Oncol | year= 2013 | volume= 15 | issue= 11 | pages= 1543-51 | pmid=24101738 | doi=10.1093/neuonc/not127 | pmc=PMC3813422 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24101738 }} </ref>
 
==Prognosis==
==Prognosis==
The following two factors are of prognostic significance:
*Histological diagnosis
**Germinomas were associated with significantly longer survival than nongerminomatous GCT's
*Staging of the extent of disease
**Germ-cell tumors may infiltrate the [[hypothalamus]] (11%), or disseminate to involve the [[third ventricle]] (22%) and [[spinal cord]] (10%).<ref name="pmid2991485">{{cite journal| author=Jennings MT, Gelman R, Hochberg F| title=Intracranial germ-cell tumors: natural history and pathogenesis. | journal=J Neurosurg | year= 1985 | volume= 63 | issue= 2 | pages= 155-67 | pmid=2991485 | doi=10.3171/jns.1985.63.2.0155 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2991485  }} </ref>
*Generally, germinomas are associated with an excellent prognosis. Intracranial germinomas have a reported 90% survival to five years after diagnosis.<ref>{{cite journal |author=Packer RJ, Cohen BH, Cooney K, Coney K |title=Intracranial germ cell tumors |journal=Oncologist |volume=5 |issue=4 |pages=312–20 |year=2000 |pmid=10964999 |doi= |url=http://theoncologist.alphamedpress.org/cgi/content/full/5/4/312#R9}}</ref> The 10-year survival of germinomas is 70%.
*Generally, germinomas are associated with an excellent prognosis. Intracranial germinomas have a reported 90% survival to five years after diagnosis.<ref>{{cite journal |author=Packer RJ, Cohen BH, Cooney K, Coney K |title=Intracranial germ cell tumors |journal=Oncologist |volume=5 |issue=4 |pages=312–20 |year=2000 |pmid=10964999 |doi= |url=http://theoncologist.alphamedpress.org/cgi/content/full/5/4/312#R9}}</ref> The 10-year survival of germinomas is 70%.
Based on the prognosis of the tumor, intracranial germ cell tumors may be classified into either good, intermediate, or poor prognosis. Pure germinomas carry a better prognosis than non germinomatous germ cell tumors (NGGCTs). Secreting germ cell tumors (GCTs) are generally considered to behave more aggressively and carry a poorer prognosis than nonsecreting germ cell tumors GCTs.<ref name="pmid9046301">{{cite journal| author=Matsutani M, Sano K, Takakura K, Fujimaki T, Nakamura O, Funata N et al.| title=Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases. | journal=J Neurosurg | year= 1997 | volume= 86 | issue= 3 | pages= 446-55 | pmid=9046301 | doi=10.3171/jns.1997.86.3.0446 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9046301  }} </ref><ref name="pmid11767296">{{cite journal| author=Matsutani M, Japanese Pediatric Brain Tumor Study Group| title=Combined chemotherapy and radiation therapy for CNS germ cell tumors--the Japanese experience. | journal=J Neurooncol | year= 2001 | volume= 54 | issue= 3 | pages= 311-6 | pmid=11767296 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11767296  }} </ref>
{| style="border: 0px; font-size: 90%; margin: 3px; width: 600px" align="center"
| valign="top" |
|+
! style="background: #4479BA; width: 10%;" | {{fontcolor|#FFF|Prognosis}}
! style="background: #4479BA; width: 5%;" | {{fontcolor|#FFF|Type of intracranial germ cell tumors}}
! style="background: #4479BA; width: 5%;" | {{fontcolor|#FFF|Survival %}}
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
:Good prognosis
| style="padding: 5px 5px; background: #F5F5F5;" |
*Pure germinomas
*Mature teratomas
| style="padding: 5px 5px; background: #F5F5F5;" | 90 %
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
:Poor prognosis
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Choriocarcinoma]]
*[[Yolk sac tumor]]
*[[Embryonal carcinoma]]
*Mixed nongerminomatous germ cell tumors composed of choriocarcinoma, yolk sac tumor, and embryonal carcinoma
| style="padding: 5px 5px; background: #F5F5F5;" |40 %
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
:Intermediate prognosis
| style="padding: 5px 5px; background: #F5F5F5;" |
*Germinoma with syncytiotrophoblastic giant cells
*Immature teratoma
*Mixed tumors mainly composed of germinoma or teratoma
*Teratoma with [[malignant]] transformation
| style="padding: 5px 5px; background: #F5F5F5;" |70 %
|-
|}


The prognosis of various germ cell tumors is shown below in a tabular form:
The prognosis of various germ cell tumors is shown below in a tabular form:
{| style="border: 0px; font-size: 90%; margin: 3px; width: 600px" align=center
{| style="border: 0px; font-size: 90%; margin: 3px; width: 600px" align="center"
|valign=top|
| valign="top" |
|+
|+
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Type of tumor}}
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Type of tumor}}
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{{reflist|2}}
{{reflist|2}}


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Latest revision as of 20:28, 18 April 2019

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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

If left untreated, more than 50% of patients may continue to suffer from endocrine abnormalities such as growth hormone deficiency, growth retardation, hypopituitarism, and hypothyroidism, and may require lifelong hormonal replacement therapy. Common complications of germinoma include secondary cancers such as acute myeloid leukemia and radiation-induced brain neoplasms. Prognosis is generally excellent for germinomas, and the 5-year survival rate of patients with germinoma is approximately 70-90%. However, prognosis in general depends on the histological diagnosis and staging of the extent of disease.

Natural History

  • Usually, patients with tumors in the pineal region tend to present with a brief history of symptoms compared with tumors of the basal ganglionic or suprasellar region, which presents a long history of symptoms including double vision related to tectal and aqueductal compression.[1]
  • Approximately 35% of patients with suprasellar tumors will be asymptomatic for more than six months, and in these patients, the time between first symptom and diagnosis may be prolonged. Tumors arising in the suprasellar region often present with overt or subtle hormonal deficiencies and a prolonged prodrome often lasting months to years. A wide majority of patients may present with diabetes insipidus as the presenting finding; patients can usually compensate for antidiuretic hormone deficiency for months to years by drinking excessive amounts of fluid. This consequently leads to development of hormonal and visual changes as the tumor expands dorsally and compresses the optic chiasm. Symptoms related to endocrinopathy (delayed vertical growth, diabetes insipidus, etc.) are associated with delays in diagnosis of greater than 12 months, and are associated with higher incidences of disseminated disease.[2][3]
  • In approximately 22% of cases metastasis has been noted at time of diagnosis.
  • The presentation is often delayed with the tumors of the thalami and basal ganglia, with a larger tumor at diagnosis.
  • Although germ cell tumors may be disseminated at the time of diagnosis, signs and symptoms of spinal cord or cerebral cortical involvement are uncommon, except for some infrequent cases of germinomas which arise in the basal ganglionic region or thalamus.[4][5][6][7][8][9][10]

Complications

  • Patients with intracranial tumors located in the basal ganglia perform poorly compared with those who have tumors in the suprasellar and pineal regions; they have lower short-term retention of visual and verbal stimuli and full-scale IQs.
  • Larger irradiation volume and dose effect the following functions of the brain adversely:[11]
    • Intellectual functions
    • Concept
    • Executive function
    • Memory
    • Decline in neurocognitive function, and performance IQs
  • Approximately more than 50% of patients may continue to suffer from endocrine abnormalities such as growth hormone deficiency, growth retardation, hypopituitarism, and hypothyroidism, and may require lifelong hormonal replacement therapy[12][13]
  • Due to surgical resection of tumor or due surgical biopsies the following complications may occur:
    • Poor performance in psychosocial skills
    • Behavioral dysfunction
    • Financial difficulties
    • Lower KPS scores following surgery have been associated with impaired neurocognitive function
  • Complications related to chemotherapy may develop
  • The surgical morbidity associated with pineal-region tumors is approximately 2-5%. Patients may suffer from the following:
    • Transient movement abnormalities of eyes
    • Ataxia
    • Cognitive dysfunction
  • The other complications that may present in patients with intracranial germ cell tumors are following:
  • The incidence of secondary cancer is approximately 6%, in patients with intracranial tumors. The risk of death due to malignancy is approximately 16%. Radiation therapy and chemotherapy may both promote the development of secondary cancers such as acute myeloid leukemia and radiation-induced brain neoplasms.[14][15][16][17][18][19][20][21]

Prognosis

The following two factors are of prognostic significance:

  • Histological diagnosis
    • Germinomas were associated with significantly longer survival than nongerminomatous GCT's
  • Staging of the extent of disease
  • Generally, germinomas are associated with an excellent prognosis. Intracranial germinomas have a reported 90% survival to five years after diagnosis.[23] The 10-year survival of germinomas is 70%.

Based on the prognosis of the tumor, intracranial germ cell tumors may be classified into either good, intermediate, or poor prognosis. Pure germinomas carry a better prognosis than non germinomatous germ cell tumors (NGGCTs). Secreting germ cell tumors (GCTs) are generally considered to behave more aggressively and carry a poorer prognosis than nonsecreting germ cell tumors GCTs.[24][25]

Prognosis Type of intracranial germ cell tumors Survival %
Good prognosis
  • Pure germinomas
  • Mature teratomas
 90 %
Poor prognosis
 40 %
Intermediate prognosis
  • Germinoma with syncytiotrophoblastic giant cells
  • Immature teratoma
  • Mixed tumors mainly composed of germinoma or teratoma
  • Teratoma with malignant transformation
 70 %

The prognosis of various germ cell tumors is shown below in a tabular form:

Type of tumor 5-year survival rate
Germinoma
  • 70-90%
Mixed germ cell tumors
  • 60-80%
Nongerminomatous germ cell tumors
  • 30-50%

References

  1. ↑ "Childhood Central Nervous System Germ Cell Tumors Treatment (PDQ®) - PDQ Cancer Information Summaries - NCBI Bookshelf".
  2. ↑ Kilday JP, Laughlin S, Urbach S, Bouffet E, Bartels U (January 2015). "Diabetes insipidus in pediatric germinomas of the suprasellar region: characteristic features and significance of the pituitary bright spot". J. Neurooncol. 121 (1): 167–75. doi:10.1007/s11060-014-1619-7. PMID 25266413.
  3. ↑ Frappaz D, Pedone C, Thiesse P, Szathmari A, Conter CF, Mottolese C, Carrie C (October 2017). "Visual complaints in intracranial germinomas". Pediatr Blood Cancer. 64 (10). doi:10.1002/pbc.26543. PMID 28436607.
  4. ↑ Germ cell tumors. National Cancer Institute(2015) http://www.cancer.gov/types/brain/hp/child-cns-germ-cell-treatment-pdq#link/_60_toc Accessed on February 16, 2016
  5. ↑ Afzal S, Wherrett D, Bartels U, Tabori U, Huang A, Stephens D; et al. (2010). "Challenges in management of patients with intracranial germ cell tumor and diabetes insipidus treated with cisplatin and/or ifosfamide based chemotherapy". J Neurooncol. 97 (3): 393–9. doi:10.1007/s11060-009-0033-z. PMID 19820898.
  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.
  7. ↑ Packer, Roger J., Bruce H. Cohen, and Kathleen Cooney. "Intracranial germ cell tumors." The Oncologist 5.4 (2000): 312-320.
  8. ↑ Germ cell tumors. Radiopedia(2015) http://radiopaedia.org/articles/central-nervous-system-germinoma Accessed on January 25, 2016
  9. ↑ Germinoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Germinoma Accessed on January 26, 2016
  10. ↑ 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.
  11. ↑ Giglio P, Gilbert MR (January 2010). "Neurologic complications of cancer and its treatment". Curr Oncol Rep. 12 (1): 50–9. doi:10.1007/s11912-009-0071-x. PMC 3637950. PMID 20425608.
  12. ↑ Hale GA, Marina NM, Jones-Wallace D, Greenwald CA, Jenkins JJ, Rao BN, Luo X, Hudson MM (1999). "Late effects of treatment for germ cell tumors during childhood and adolescence". J. Pediatr. Hematol. Oncol. 21 (2): 115–22. PMID 10206457.
  13. ↑ Barnes N, Chemaitilly W (2014). "Endocrinopathies in survivors of childhood neoplasia". Front Pediatr. 2: 101. doi:10.3389/fped.2014.00101. PMC 4172013. PMID 25295241.
  14. ↑ Sugiyama K, Yamasaki F, Kurisu K, Kenjo M (2009). "Quality of life of extremely long-time germinoma survivors mainly treated with radiotherapy". Prog Neurol Surg. 23: 130–9. doi:10.1159/000210059. PMID 19329867.
  15. ↑ Sutton LN, Radcliffe J, Goldwein JW, Phillips P, Janss AJ, Packer RJ; et al. (1999). "Quality of life of adult survivors of germinomas treated with craniospinal irradiation". Neurosurgery. 45 (6): 1292–7, discussion 1297-8. PMID 10598695.
  16. ↑ Jinguji S, Yoshimura J, Nishiyama K, Aoki H, Nagasaki K, Natsumeda M; et al. (2013). "Factors affecting functional outcomes in long-term survivors of intracranial germinomas: a 20-year experience in a single institution". J Neurosurg Pediatr. 11 (4): 454–63. doi:10.3171/2012.12.PEDS12336. PMID 23373627.
  17. ↑ Acharya S, DeWees T, Shinohara ET, Perkins SM (2015). "Long-term outcomes and late effects for childhood and young adulthood intracranial germinomas". Neuro Oncol. 17 (5): 741–6. doi:10.1093/neuonc/nou311. PMC 4482856. PMID 25422317.
  18. ↑ Martens T, Rotermund R, Zu Eulenburg C, Westphal M, Flitsch J (2014). "Long-term follow-up and quality of life in patients with intracranial germinoma". Neurosurg Rev. 37 (3): 445–50, discussion 451. doi:10.1007/s10143-014-0544-8. PMID 24715277.
  19. ↑ Odagiri K, Omura M, Hata M, Aida N, Niwa T, Ogino I; et al. (2012). "Treatment outcomes, growth height, and neuroendocrine functions in patients with intracranial germ cell tumors treated with chemoradiation therapy". Int J Radiat Oncol Biol Phys. 84 (3): 632–8. doi:10.1016/j.ijrobp.2011.12.084. PMID 22420962.
  20. ↑ Ogawa K, Shikama N, Toita T, Nakamura K, Uno T, Onishi H; et al. (2004). "Long-term results of radiotherapy for intracranial germinoma: a multi-institutional retrospective review of 126 patients". Int J Radiat Oncol Biol Phys. 58 (3): 705–13. doi:10.1016/j.ijrobp.2003.07.001. PMID 14967424.
  21. ↑ Liang SY, Yang TF, Chen YW, Liang ML, Chen HH, Chang KP; et al. (2013). "Neuropsychological functions and quality of life in survived patients with intracranial germ cell tumors after treatment". Neuro Oncol. 15 (11): 1543–51. doi:10.1093/neuonc/not127. PMC 3813422. PMID 24101738.
  22. ↑ Jennings MT, Gelman R, Hochberg F (1985). "Intracranial germ-cell tumors: natural history and pathogenesis". J Neurosurg. 63 (2): 155–67. doi:10.3171/jns.1985.63.2.0155. PMID 2991485.
  23. ↑ Packer RJ, Cohen BH, Cooney K, Coney K (2000). "Intracranial germ cell tumors". Oncologist. 5 (4): 312–20. PMID 10964999.
  24. ↑ Matsutani M, Sano K, Takakura K, Fujimaki T, Nakamura O, Funata N; et al. (1997). "Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases". J Neurosurg. 86 (3): 446–55. doi:10.3171/jns.1997.86.3.0446. PMID 9046301.
  25. ↑ Matsutani M, Japanese Pediatric Brain Tumor Study Group (2001). "Combined chemotherapy and radiation therapy for CNS germ cell tumors--the Japanese experience". J Neurooncol. 54 (3): 311–6. PMID 11767296.


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