Germinoma natural history
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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
- 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:
- Brain atrophy
- Multifocal encephalomalacia
- Leukoencephalopathy
- Focal necrosis
- 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.[12][13][14][15][16][17][18][19]
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%).[20]
- Generally, germinomas are associated with an excellent prognosis. Intracranial germinomas have a reported 90% survival to five years after diagnosis.[21] 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.[22][23]
| Prognosis | Type of intracranial germ cell tumors | Survival % |
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90 % |
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40 % |
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70 % |
The prognosis of various germ cell tumors is shown below in a tabular form:
| Type of tumor | 5-year survival rate |
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References
- ↑ "Childhood Central Nervous System Germ Cell Tumors Treatment (PDQ®) - PDQ Cancer Information Summaries - NCBI Bookshelf".
- ↑ 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.
- ↑ 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.
- ↑ 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
- ↑ 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.
- ↑ 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.
- ↑ Packer, Roger J., Bruce H. Cohen, and Kathleen Cooney. "Intracranial germ cell tumors." The Oncologist 5.4 (2000): 312-320.
- ↑ Germ cell tumors. Radiopedia(2015) http://radiopaedia.org/articles/central-nervous-system-germinoma Accessed on January 25, 2016
- ↑ Germinoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Germinoma Accessed on January 26, 2016
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Packer RJ, Cohen BH, Cooney K, Coney K (2000). "Intracranial germ cell tumors". Oncologist. 5 (4): 312–20. PMID 10964999.
- ↑ 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.
- ↑ 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.