✓ The natural history of primary intracranial germ-cell tumors (GCT's) is defined from 389 previously published cases, of which 65% were germinomas, 18% teratomas, 5% embryonal carcinomas, 7% endodermal sinus tumors, and 5% choriocarcinomas. Intracranial GCT's display specificity in site of origin. Ninety-five percent arise along the midline from the suprasellar cistern (37%) to the pineal gland (48%), and an additional 6% involve both sites. The majority of germinomas (57%) arise in the suprasellar cistern, while most nongerminomatous GCT's (68%) preferentially involve the pineal gland (p < 0.0001). The age distribution of afflicted patients is unimodal, centering with an abrupt surge in frequency in the early pubertal years; 68% of patients are diagnosed between 10 and 21 years of age. Nongerminomatous GCT's demonstrate an earlier age of onset than do germinomas (p < 0.0001). Prolonged symptomatic intervals prior to diagnosis are common in germinomas (p = 0.0007), in suprasellar GCT's (p = 0.001), and a...

Intracranial germ-cell tumors: natural history and pathogenesis.

Objective To describe the emergence of pediatric psycho-oncology and to summarize research on psychosocial aspects of childhood cancer and survivorship. Methods To review research into illness communication and informed consent, procedural pain, late effects, psychological distress, coping and adjustment, and special risk populations. Methodological challenges, appropriate methodology, and directions for future research are discussed. Results The past 30 years have seen change from avoidance of communication about cancer to an emphasis on straightforward discussion of diagnosis and prognosis. Behavioral research has led to interventions to reduce procedural distress. Late effects have been observed in social functioning. Although average levels of distress in survivors of pediatric cancer are typical, subsets of more vulnerable patients and family members exist. Factors predicting positive and negative coping have been identified. Conclusions As the numbers of pediatric cancer survivors increase, psychosocial researchers will be better able to conduct longitudinal studies not only of adjustment and its predictors but also of the impact of the emerging medical treatments and interventions to ameliorate late effects of treatment. Additional funding, improving methodology, and multi-institutional cooperation will aid future pediatric psycho-oncology investigators.

/pdf/psychosocial-functioning-in-pediatric-cancer-4eczwbukll.pdf

Psychosocial Functioning in Pediatric Cancer

Objective To evaluate and compare psychological outcomes in long-term survivors of pediatric leukemia, Hodgkin’s disease, and non-Hodgkin’s lymphoma and sibling controls Methods Adult survivors of childhood leukemia, Hodgkin’s disease, and non-Hodgkin’s lymphoma (N = 5736) and sibling controls (N = 2565) were administered a long-term follow-up questionnaire allowing assessment of symptoms associated with depression and somatic distress Results The majority of respondents in this study did not demonstrate symptomatology indicative of depression or somatic distress Survivors, however, were significantly more likely than sibling controls to report symptoms of depression and somatic distress Women were significantly more likely to indicate symptoms of depression and somatic distress than were men; however, this difference did not vary by survivor/sibling status Similarly, socioeconomic (SES) variables predicted symptomatic levels of depression and somatic distress for both survivors and siblings, and these effects did not vary by survivor/sibling status Among leukemia, Hodgkin’s disease, and non-Hodgkin’s lymphoma survivors, in addition to gender and SES, the only treatment variable that predicted scores indicating depressive symptomatology was exposure to intensive chemotherapy Exposure to intensive chemotherapy also predicted scores indicative of somatic distress symptoms No other medical variables, including diagnostic category, age at diagnosis, time since diagnosis, and duration of treatment, predicted symptomatic scores for depression and somatic distress Conclusions This large, sibling-controlled, multisite study of young adult survivors of childhood leukemia, Hodgkin’s disease, and non-Hodgkin’s lymphoma found that survivors had significant increased risk for reporting symptoms of depression and somatic distress and that intensive chemotherapy added to this risk However, being a cancer survivor did not compound the effects of gender and SES variables on the 2 outcomes measured The ability of SES, gender, and treatment-related variables to predict psychological symptoms in this cohort of childhood survivors and sibling controls calls for future research into varied biological and psychosocial pathways by which cancer influences future psychosocial functioning

Psychological outcomes in long-term survivors of childhood leukemia, Hodgkin's disease, and non-Hodgkin's lymphoma: a report from the Childhood Cancer Survivor Study.

Intracranial germ cell tumors are a heterogeneous group of lesions which occur in children and adults. Within the classification of intracranial germ cell tumors, there are a variety of different tumor types which carry different prognoses. The diagnosis of an intracranial germ cell tumor usually requires histological information, but a subgroup of tumors will secrete specific tumor markers, including α-fetoprotein and β-human chorionic gonadotropin, which may obviate the need for surgical intervention. The management of intracranial germ cell tumors in both children and adults remains unsettled. Germinomas have a good prognosis, as over 90% of patients can be effectively treated with radiation therapy. The dose and volume of radiation therapy needed for disease control is not well established, and controversy exists concerning the need for whole brain or craniospinal radiation therapy for localized tumors. Germinomas are also chemosensitive and recent reports suggest that the dose and volume of radiation therapy required for disease control can be lessened with the addition of adjuvant chemotherapy. The outcome for patients with nongerminomatous germ cell tumors is less favorable. Radiation therapy alone will result in disease control in 40%-60% of patients. The addition of chemotherapy to radiation therapy may improve the rate of survival. The Oncologist 2000;5:312-320

Intracranial Germ Cell Tumors

As survival rates have risen for children with malignant primary brain tumors, so has the concern that many survivors have significant permanent cognitive deficits. Cranial irradiation (CRT) has been implicated as the major cause for cognitive dysfunction. To clarify the etiology, incidence, and severity of intellectual compromise in children with brain tumors after CRT, a prospective study was undertaken comparing the neuropsychological outcome in 18 consecutive children with malignant brain tumors treated with CRT to outcome in 14 children harboring brain tumors in similar sites in the nervous system who had not received CRT. Children with cortical or subcortical brain tumors were not eligible for study. Neuropsychological testing was performed after surgery prior to radiotherapy, after radiotherapy, and at 1- and 2-year intervals thereafter. Children who had received CRT had a mean full-scale intelligence quotient (FSIQ) of 105 at diagnosis which fell to 91 by Year 2. Similar declines were noted in their performance intelligence quotient (IQ) and verbal IQ. After CRT, patients demonstrated a statistically significant decline from baseline in FSIQ (p less than 0.02) and verbal IQ (p less than 0.04). Children who had not received CRT did not demonstrate a fall in any cognitive parameter over time. The decline between baseline testing and testing performed at Year 2 in patients who had CRT was inversely correlated with age (p less than 0.02), as younger children demonstrated the greatest loss of intelligence. Children less than 7 years of age at diagnosis had a mean decline in FSIQ of 25 points 2 years posttreatment. No other clinical parameter correlated with the overall IQ or decline in IQ. After CRT, children demonstrated a wide range of dysfunction including deficits in fine motor, visual-motor, and visual-spatial skills and memory difficulties. After CRT, children with brain tumors also demonstrated a fall in a wide range of achievement scores and an increased need, over time, for special help in school. The 2-year results of this study suggest that children with brain tumors treated with CRT are cognitively impaired and that these deficits worsen over time. The younger the child is at the time of treatment, the greater is the likelihood and severity of damage. These children, although not retarded, have a multitude of neurocognitive deficits which detrimentally affects school performance. New treatment strategies are needed for children with malignant brain tumors.

A prospective study of cognitive function in children receiving whole-brain radiotherapy and chemotherapy: 2-year results.

Tumors of the pineal and suprasellar region form a rare and interesting group of lesions with germinomas accounting for over 50% of all lesions in this anatomic region. The Brain Tumor Committee of Childrens Cancer Study Group (CCSG) recently surveyed all CCSG member institutions to determine treatment parameters and assess the techniques. A total of 140 patients were seen during the period from 1960 to 1975; 118 patients were evaluable, having adequate treatment records. One hundred and one patients were less than 30 years of age with a 2:1 male predominance. Thirty-six of the 57 biopsied patients (63%) were found to have germinomas. The survival of patients in the germinoma group (72%) was comparable to that of the patients without biopsy (71%). The overall survival rate for all patients (biopsied and unbiopsied) was 65% with follow-up times ranging from 2 to 15 years. Nine patients developed spinal cord metastases (8%), two of whom also had simultaneous primary recurrence; none of these patients had received adjunctive spinal irradiation. Cancer 43:698–701, 1979.

https://onlinelibrary.wiley.com/doi/pdf/10.1002/1097-0142%28197902%2943%3A2%3C698%3A%3AAID-CNCR2820430243%3E3.0.CO%3B2-Y

Tumors of the pineal and suprasellar region: Childrens Cancer Study Group treatment results 1960--1975: a report from Childrens Cancer Study Group.

Brain tumors in children: current cooperative and institutional chemotherapy trials in newly diagnosed and recurrent disease.

When adult survivors of childhood cancer were compared with their peers, survivors were found to be at least as well adjusted. Indeed, some evidence was suggestive of survivors having adaptive advantages in everyday life. The survivors reported significantly more positive affect, less negative affect, higher intimacy motivation, more perceived personal control, and greater satisfaction with control in life situations. Despite these apparent strengths associated with surviving childhood cancer, several specific problems were documented. Survivors were more likely than peers to have repeated school grades, to be worried about issues of fertility, and to express dissatisfaction with important relationships. The latter finding was interpreted as reflecting the high expectations of survivors for relationships, based on their difficult yet interpersonally rewarding experiences during times of illness.

Psychologic adaptation of survivors of childhood cancer.

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Tumors of the pineal and suprasellar region: Childrens Cancer Study Group treatment results 1960--1975: a report from Childrens Cancer Study Group.

Brain tumors in children: current cooperative and institutional chemotherapy trials in newly diagnosed and recurrent disease.

Psychologic adaptation of survivors of childhood cancer.