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Bifocal Intracranial Germinoma: A Retrospective Analysis of Treatment Outcomes in 76 Patients
Proceedings of the 52nd Annual ASTRO Meeting
2858
Bifocal Intracranial Germinoma: A Retrospective Analysis of Treatment Outcomes in 76 Patients
D. C. Weksberg1, Y. Shibamoto2, A. C. Paulino1 1
The Methodist Hospital, Houston, TX, 2Nagoya City University, Graduate School of Medical Sciences, Nagoya, Japan
Purpose/Objective(s): Bifocal germinomas (BFG) are a rare subset of pediatric intracranial neoplasms comprising 6-25% of intracranial germinomas. Therapy for BFG is controversial - some believe that BFG are a manifestation of disseminated disease and advocate craniospinal irradiation (CSI), while others believe that BFG represents regional disease, and support the use of limited fields (whole brain or whole ventricular irradiation; WB, WVI). The purpose of our study is to review our own treatment experience in addition to the reported literature to help determine the appropriate radiotherapy fields for BFG. Materials/Methods/Results: We analyzed 20 BFG patients at our institutions; 95% were male, with a mean age of 21 years, and all had classic bifocal lesions (pineal gland and suprasellar region); 35% had positive CSF. 11 patients received CSI alone, while 7 received WB/WVI with chemotherapy; 2 received local or WB radiation without chemotherapy. Median follow-up was 120 months, with no recurrence observed. Additionally, we were able to identify 56 BFG patients from the literature. Patients with atypical bifocal tumors (involving sites other than the pineal and suprasellar regions) and those with disease outside the neuraxis were excluded, as were patients for whom treatment or outcome data were unavailable. Our combined data on 76 patients represents the largest reported series on BFG. We identified 51 patients with bifocal lesions only (Group I), and 25 with bifocal lesions and ventricular or CSF disease (Group II). Median follow-up was 61 months, and recurrence was observed in 8 patients (89% progression free survival). Overall PFS was 94% for Group I and 76% for Group II. We next analyzed patient outcome by treatment approach (radiation fields +/- chemotherapy). For patients in Group I there were no failures in patients receiving CSI (n = 11), 2 spinal failures in patients receiving WB/WVI or local treatment (GTV) without chemotherapy (n = 17), and 1 spinal failure in patients receiving WB/WVI/GTV plus chemotherapy (n = 23). For patients in Group II, there were no failures in patients receiving CSI (n = 11), but 4 spinal failures were observed in patients receiving WB/WVI/GTV plus chemotherapy (n = 13), and 1 patient who received WB treatment without chemotherapy (n = 1) failed in the spine and brain. Conclusions: CSI is associated with excellent PFS in BFG. In typical BFG patients (Group I), we find that treatment with chemotherapy and radiation that omits spinal fields is associated with greater than 95% PFS. Patients with ventricular dissemination or CSF positive disease (Group II) are best treated with CSI. Author Disclosure: D.C. Weksberg, None; Y. Shibamoto, None; A.C. Paulino, None.
2859
Risk Factors for Recurrence in Childhood and Adolescent Thyroid Cancer
S. Markovina1 P. W. Grigsby2 1
University of Wisconsin School of Medicine and Public Health, Madison, WI, 2Washington University School of Medicine, St. Louis, MO Purpose/Objective(s): The purpose of this study was to examine the correlation of clinical and treatment factors on local control, distant control, and survival in children and adolescents with well-differentiated thyroid cancer. Materials/Methods: A prospective cohort of 124 patients under the age of 22 with well-differentiated thyroid carcinoma was reviewed. There were 27 males and 97 females who ranged in age from 4 to 21.9 years. 111 patients were white, 7 patients were Black, 3 were Asian, and 3 were of Hispanic ethnicity. Tumor histology was papillary in 94 (76%), follicular variant in 25 (20%), pure follicular in 2 (1.6%), trabecular in 1 (0.8%), insular in 1 (0.8%), and Hurthle cell in 1 (0.8%). At diagnosis, 45 patients (36%) had disease confined to the thyroid, 66 patients (53%) had additional involvement of neck lymph nodes, and 13 patients (10%) also had lung metastases. 112 patients (90%) underwent total thyroidectomy, 3 underwent subtotal thyroidectomy, and the remaining 9 patients underwent lobectomy as initial surgery for their disease. Radioactive iodine following initial surgery was administered in 103. All patients were prescribed thyroid hormone suppressive therapy. Results: The overall survival rate with average follow-up of 15.8 years (range, 20 days to 51 years in our patient cohort was 98% (two deaths). One death was secondary to a congenital heart condition and the second death was due to thyroid cancer. The 10-year progression-free survival rate was 70%. At the time of analysis, 40 patients had experienced recurrence (32%). Time to first recurrence ranged from 0.69 to 36 years with a mean time to progression of 8.5 years with the majority of recurrences occurring within the first 5 years after diagnosis. Age at diagnosis (p = 0.033), extensive disease at the time of diagnosis (p = 0.012), histologic tumor size (p = 0.043), and no post-operative radioactive iodine treatment (p = 0.0013) were significant factors for developing recurrent disease. After an initial complete response to therapy, the recurrence-free survival of patients with one negative whole body 131I scintigraphy was found to be 70%. Recurrence-free survival of patients with two consecutive negative scans was 80%, and patients with three or more consecutive negative whole body 131I scans was 100%. Conclusions: Prognosis for well-differentiated thyroid carcinoma in children and adolescents is excellent. Patients diagnosed in the first two decades of life have very low risk of mortality despite having extensive disease at diagnosis and high recurrence rates. Younger age and limited disease at diagnosis, as well as treatment with 131I therapy are associated with a lower risk of recurrence. Three consecutive negative total body 131I scans has a strong negative predictive value for recurrent disease. Author Disclosure: S. Markovina, None; P.W. Grigsby, None.
2860
Prospective Study of Concurrent Erlotinib and Radiotherapy (RT): Pattern of Failure for Pediatric High-Grade Glioma
A. S. Pai Panandiker, T. E. Merchant, S. Sharma, I. Qaddoumi, A. Gajjar, A. Broniscer St. Jude Children’s Research Hospital, Memphis, TN Purpose/Objective(s): To prospectively analyze the pattern of failure with conformal radiotherapy (RT) and concurrent and postRT epidermal growth factor receptor inhibitor in pediatric high-grade glioma.
S587
2858
Bifocal Intracranial Germinoma: A Retrospective Analysis of Treatment Outcomes in 76 Patients
D. C. Weksberg1, Y. Shibamoto2, A. C. Paulino1 1
The Methodist Hospital, Houston, TX, 2Nagoya City University, Graduate School of Medical Sciences, Nagoya, Japan
Purpose/Objective(s): Bifocal germinomas (BFG) are a rare subset of pediatric intracranial neoplasms comprising 6-25% of intracranial germinomas. Therapy for BFG is controversial - some believe that BFG are a manifestation of disseminated disease and advocate craniospinal irradiation (CSI), while others believe that BFG represents regional disease, and support the use of limited fields (whole brain or whole ventricular irradiation; WB, WVI). The purpose of our study is to review our own treatment experience in addition to the reported literature to help determine the appropriate radiotherapy fields for BFG. Materials/Methods/Results: We analyzed 20 BFG patients at our institutions; 95% were male, with a mean age of 21 years, and all had classic bifocal lesions (pineal gland and suprasellar region); 35% had positive CSF. 11 patients received CSI alone, while 7 received WB/WVI with chemotherapy; 2 received local or WB radiation without chemotherapy. Median follow-up was 120 months, with no recurrence observed. Additionally, we were able to identify 56 BFG patients from the literature. Patients with atypical bifocal tumors (involving sites other than the pineal and suprasellar regions) and those with disease outside the neuraxis were excluded, as were patients for whom treatment or outcome data were unavailable. Our combined data on 76 patients represents the largest reported series on BFG. We identified 51 patients with bifocal lesions only (Group I), and 25 with bifocal lesions and ventricular or CSF disease (Group II). Median follow-up was 61 months, and recurrence was observed in 8 patients (89% progression free survival). Overall PFS was 94% for Group I and 76% for Group II. We next analyzed patient outcome by treatment approach (radiation fields +/- chemotherapy). For patients in Group I there were no failures in patients receiving CSI (n = 11), 2 spinal failures in patients receiving WB/WVI or local treatment (GTV) without chemotherapy (n = 17), and 1 spinal failure in patients receiving WB/WVI/GTV plus chemotherapy (n = 23). For patients in Group II, there were no failures in patients receiving CSI (n = 11), but 4 spinal failures were observed in patients receiving WB/WVI/GTV plus chemotherapy (n = 13), and 1 patient who received WB treatment without chemotherapy (n = 1) failed in the spine and brain. Conclusions: CSI is associated with excellent PFS in BFG. In typical BFG patients (Group I), we find that treatment with chemotherapy and radiation that omits spinal fields is associated with greater than 95% PFS. Patients with ventricular dissemination or CSF positive disease (Group II) are best treated with CSI. Author Disclosure: D.C. Weksberg, None; Y. Shibamoto, None; A.C. Paulino, None.
2859
Risk Factors for Recurrence in Childhood and Adolescent Thyroid Cancer
S. Markovina1 P. W. Grigsby2 1
University of Wisconsin School of Medicine and Public Health, Madison, WI, 2Washington University School of Medicine, St. Louis, MO Purpose/Objective(s): The purpose of this study was to examine the correlation of clinical and treatment factors on local control, distant control, and survival in children and adolescents with well-differentiated thyroid cancer. Materials/Methods: A prospective cohort of 124 patients under the age of 22 with well-differentiated thyroid carcinoma was reviewed. There were 27 males and 97 females who ranged in age from 4 to 21.9 years. 111 patients were white, 7 patients were Black, 3 were Asian, and 3 were of Hispanic ethnicity. Tumor histology was papillary in 94 (76%), follicular variant in 25 (20%), pure follicular in 2 (1.6%), trabecular in 1 (0.8%), insular in 1 (0.8%), and Hurthle cell in 1 (0.8%). At diagnosis, 45 patients (36%) had disease confined to the thyroid, 66 patients (53%) had additional involvement of neck lymph nodes, and 13 patients (10%) also had lung metastases. 112 patients (90%) underwent total thyroidectomy, 3 underwent subtotal thyroidectomy, and the remaining 9 patients underwent lobectomy as initial surgery for their disease. Radioactive iodine following initial surgery was administered in 103. All patients were prescribed thyroid hormone suppressive therapy. Results: The overall survival rate with average follow-up of 15.8 years (range, 20 days to 51 years in our patient cohort was 98% (two deaths). One death was secondary to a congenital heart condition and the second death was due to thyroid cancer. The 10-year progression-free survival rate was 70%. At the time of analysis, 40 patients had experienced recurrence (32%). Time to first recurrence ranged from 0.69 to 36 years with a mean time to progression of 8.5 years with the majority of recurrences occurring within the first 5 years after diagnosis. Age at diagnosis (p = 0.033), extensive disease at the time of diagnosis (p = 0.012), histologic tumor size (p = 0.043), and no post-operative radioactive iodine treatment (p = 0.0013) were significant factors for developing recurrent disease. After an initial complete response to therapy, the recurrence-free survival of patients with one negative whole body 131I scintigraphy was found to be 70%. Recurrence-free survival of patients with two consecutive negative scans was 80%, and patients with three or more consecutive negative whole body 131I scans was 100%. Conclusions: Prognosis for well-differentiated thyroid carcinoma in children and adolescents is excellent. Patients diagnosed in the first two decades of life have very low risk of mortality despite having extensive disease at diagnosis and high recurrence rates. Younger age and limited disease at diagnosis, as well as treatment with 131I therapy are associated with a lower risk of recurrence. Three consecutive negative total body 131I scans has a strong negative predictive value for recurrent disease. Author Disclosure: S. Markovina, None; P.W. Grigsby, None.
2860
Prospective Study of Concurrent Erlotinib and Radiotherapy (RT): Pattern of Failure for Pediatric High-Grade Glioma
A. S. Pai Panandiker, T. E. Merchant, S. Sharma, I. Qaddoumi, A. Gajjar, A. Broniscer St. Jude Children’s Research Hospital, Memphis, TN Purpose/Objective(s): To prospectively analyze the pattern of failure with conformal radiotherapy (RT) and concurrent and postRT epidermal growth factor receptor inhibitor in pediatric high-grade glioma.
S587