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==Natural history== | ==Natural history== | ||
Clinical presentations of germ cell tumors are dependent on the location of the tumor in the CNS, the size of the lesion and age of the patient. The anatomic relationship between the pineal gland and the quadrigeminal plate, third ventricle and deep venous structures accounts for most of the symptoms associated with pineal region tumors [1, 14].Pineal region. Patients with tumors in the pineal region usually have a shorter history of symptoms than do patients with tumors of the suprasellar or basal ganglionic region, with weeks to months of symptoms that include raised intracranial pressure and diplopia related to tectal and aqueductal compression. Symptoms and signs unique to masses in the pineal and posterior third ventricular region include Parinaud syndrome (vertical gaze impairment, convergence nystagmus, and light-near pupillary response dissociation), headache, and nausea and vomiting. | |||
Suprasellar germinomas usually present with evidence of hypothalamic/pituitary dysfunction, which most commonly includes diabetes insipidus but may also include delayed sexual development, hypopituitarism and/or isolated growth failure [1, 15]. Up to 35% of patients with suprasellar tumors will be asymptomatic for more than six months, and in this subgroup of patients, the time between first symptom and diagnosis may be prolonged. Suprasellar region. Tumors arising in the suprasellar region often present with subtle or overt hormonal deficiencies and a protracted prodrome often lasting months to years. Diabetes insipidus caused by antidiuretic hormone deficiency occurs in 70% to 90% of patients and is the most common sentinel symptom; patients can usually compensate for this deficiency for months to years by drinking excessive amounts of fluid. Eventually, other hormonal symptoms and visual deficits emerge as the tumor expands dorsally and compresses or invades the optic chiasm.[6,11] | |||
Although germ cell tumors may be disseminated at the time of diagnosis, symptoms and signs of spinal cord or cerebral cortical involvement are uncommon, except for those infrequent cases of germinomas which arise in the thalamus or basal ganglionic region. | |||
Presenting symptoms of patients with intracranial GCTs depend upon the location of the tumor. Delays in diagnosis are common, especially symptoms related to endocrinopathy (delayed vertical growth, diabetes insipidus, etc.) are associated with delays of greater than 12 months, and are associated with higher incidences of disseminated disease [19]. | |||
Multifocal or bifocal tumors. Patients with multifocal or bifocal primaries may present with both suprasellar and pineal region syndromes.[6] | |||
Nonspecific symptoms such as enuresis, anorexia, and psychiatric complaints can lead to delays in diagnosis, whereas signs of increased intracranial pressure or visual changes tend to result in earlier diagnosis.[12]Many patients with unrecognized CNS GCTs may have had a long history of symptoms such as movement disorders, enuresis, anorexia, and psychiatric complaints. Diagnosis in such cases has been delayed from 7 months to 3 years.[7] | |||
==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. | ||
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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
Natural history
Clinical presentations of germ cell tumors are dependent on the location of the tumor in the CNS, the size of the lesion and age of the patient. The anatomic relationship between the pineal gland and the quadrigeminal plate, third ventricle and deep venous structures accounts for most of the symptoms associated with pineal region tumors [1, 14].Pineal region. Patients with tumors in the pineal region usually have a shorter history of symptoms than do patients with tumors of the suprasellar or basal ganglionic region, with weeks to months of symptoms that include raised intracranial pressure and diplopia related to tectal and aqueductal compression. Symptoms and signs unique to masses in the pineal and posterior third ventricular region include Parinaud syndrome (vertical gaze impairment, convergence nystagmus, and light-near pupillary response dissociation), headache, and nausea and vomiting.
Suprasellar germinomas usually present with evidence of hypothalamic/pituitary dysfunction, which most commonly includes diabetes insipidus but may also include delayed sexual development, hypopituitarism and/or isolated growth failure [1, 15]. Up to 35% of patients with suprasellar tumors will be asymptomatic for more than six months, and in this subgroup of patients, the time between first symptom and diagnosis may be prolonged. Suprasellar region. Tumors arising in the suprasellar region often present with subtle or overt hormonal deficiencies and a protracted prodrome often lasting months to years. Diabetes insipidus caused by antidiuretic hormone deficiency occurs in 70% to 90% of patients and is the most common sentinel symptom; patients can usually compensate for this deficiency for months to years by drinking excessive amounts of fluid. Eventually, other hormonal symptoms and visual deficits emerge as the tumor expands dorsally and compresses or invades the optic chiasm.[6,11]
Although germ cell tumors may be disseminated at the time of diagnosis, symptoms and signs of spinal cord or cerebral cortical involvement are uncommon, except for those infrequent cases of germinomas which arise in the thalamus or basal ganglionic region. Presenting symptoms of patients with intracranial GCTs depend upon the location of the tumor. Delays in diagnosis are common, especially symptoms related to endocrinopathy (delayed vertical growth, diabetes insipidus, etc.) are associated with delays of greater than 12 months, and are associated with higher incidences of disseminated disease [19]. Multifocal or bifocal tumors. Patients with multifocal or bifocal primaries may present with both suprasellar and pineal region syndromes.[6] Nonspecific symptoms such as enuresis, anorexia, and psychiatric complaints can lead to delays in diagnosis, whereas signs of increased intracranial pressure or visual changes tend to result in earlier diagnosis.[12]Many patients with unrecognized CNS GCTs may have had a long history of symptoms such as movement disorders, enuresis, anorexia, and psychiatric complaints. Diagnosis in such cases has been delayed from 7 months to 3 years.[7]
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:
- 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.[1][2][3][4][5][6][7][8]
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%).[9]
- Generally, germinomas are associated with an excellent prognosis. Intracranial germinomas have a reported 90% survival to five years after diagnosis.[10] The 10-year survival of germinomas is 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
- ↑ 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.