Background The purpose of this analysis is to report long-term health-related quality of life (HR... more Background The purpose of this analysis is to report long-term health-related quality of life (HRQoL) among brain tumor survivors treated with proton therapy (PRT) at a very young age. Methods Fifty-nine children <4 years old received PRT between 2000 and 2011. Forty families participated. HRQoL was assessed by child self-report (CSR; age ≥5) and parent proxy report (PPR; age 2+) using the PedsQL Core. Results The median age was 2.5 years (range, 0.3–3.8) at PRT and 9.1 years (5.5–18) at last follow-up. The most common diagnoses were ependymoma (n = 22) and medulloblastoma (n = 7). Median follow-up is 6.7 years (3–15.4). Follow-up mean CSR and PPR scores were: total core (78.4 and 72.9), physical (82.9 and 75.2), psychosocial (76.0 and 71.6), emotional (74.4 and 70.7), social (81.2 and 75.1), and school (72.4 and 69.9). Parent-reported HRQoL fell within a previously defined range for healthy children in 37.5% of patients, and for children with severe health conditions in 45% of p...
We evaluated the therapeutic efficacy of autologous bone marrow transplantation (ABMT) in 49 pati... more We evaluated the therapeutic efficacy of autologous bone marrow transplantation (ABMT) in 49 patients with acute leukemia, using ex-vivo-treated marrow with mafosfamide. This report summarizes data collected from 8 Italian centers, involving 27 cases with acute lymphoid leukemia (ALL) and 22 cases with acute nonlymphoid leukemia (ANLL). The analysis of the results shows that the disease-free survival of ALL and
The authors assessed whether the use of a topical anti-inflammatory agent at the radiation entry ... more The authors assessed whether the use of a topical anti-inflammatory agent at the radiation entry site would prevent radiation-induced skin damage and found that the use of a topical anti-inflammatory agent, such as one containing 10% trolamine salicylate, may reduce skin damage at the radiation entry site if used prophylactically.
Optic pathway gliomas represent approximately 5% of all pediatric intracranial tumors. While thes... more Optic pathway gliomas represent approximately 5% of all pediatric intracranial tumors. While these tumors are most frequently low grade astrocytomas, they follow a highly variable clinical course, and accordingly, there is much debate regarding their optimal management. Their propensity to occur in very young children and infants further complicates selection of therapy. Historically, surgery and radiotherapy have played a primary role in management, however, in the last 15 years, chemotherapy has evolved into the first-line treatment of choice. Nonetheless, chemotherapy frequently fails, but serves to delay implementation of radiotherapy or surgery until the child has progressed neuropsychologically. An overall favorable prognosis for this tumor emphasizes the need for careful selection of therapy. Herein, we review the major features of optic pathway glioma, including epidemiology, pathology, therapeutic interventions, outcome, and treatment sequelae.
To our knowledge, this is the first reported case of the use of stereotactic radiotherapy for an ... more To our knowledge, this is the first reported case of the use of stereotactic radiotherapy for an eosinophilic granuloma (EG) of the clivus. We report follow-up information on two previously reported cases and suggest a management plan for this rare lesion. We report the case of a 4.5-year-old boy who presented with a complete abducens palsy on the right with an associated head turn. A computed tomographic scan of his head revealed a lytic lesion on that side, and magnetic resonance imaging showed the mass to be of low intensity on T1-weighted images and of high intensity on T2-weighted images with heterogeneous enhancement. A transnasal stereotactic biopsy was performed, revealing an EG. The patient was treated with stereotactic radiotherapy, and he became symptom-free with radiographic resolution of his lesion. Reviewing the literature, we found 13 series with 87 cases of EG in the petrous portion of the temporal bone. EG in the cranial base occurring outside of the temporal bone or in the temporal bone and extending intracranially is, however, quite rare, with only nine other cases reported, two of them clival. These findings suggest a classification schema in which cranial base EG lesions be grouped with either the more common extracranial petrous temporal bone lesions or the very rare intracranial lesions. Although there are few cases in the literature, treatment results indicate that clival EG, and perhaps all intracranial cranial base EGs, be treated by a biopsy alone, followed by surgery or stereotactic radiotherapy if there is an incomplete resolution of the symptoms or if there is a recurrence.
Informed and could freely decide. It seems important to evaluate the quality of current Informati... more Informed and could freely decide. It seems important to evaluate the quality of current Information practice. Methodl: PIs undergoing bronchoscopy were investigated by standard• Ized Interviews about contents and circumstances of the information they got before they gave IC. Furthermore they were asked whether they would like more or rather less information and they were tested with the State• Trait-Anxiety-Inventory. Between Jul 94 and Feb 95 we evaluated 80 pts. Relult.: Generally the legal requirements were met, although out of administrative reasons some pts were informed shortly before the bron• choscopy. n pis (96%) were satisfied with the Information given and only 3 pis would have wanted more. However, 33 pts (41%) could not remember one single of nine mentioned risks of the procedure and 19 pts (24%) did not even remember that they were not allowed to eat and drink shortly after the investigation. Additional information with a video was perceived as Informative but inducing anXiety. Conclullon: Informing patients about medical procedures is a complex communicative challenge. We suggest that valid IC requires to prove that the information given actually reached the patient.
There is a substantial risk of a second cancer for persons with hereditary retinoblastoma, which ... more There is a substantial risk of a second cancer for persons with hereditary retinoblastoma, which is enhanced by radiotherapy. To examine long-term risk of new primary cancers in survivors of childhood retinoblastoma and quantify the role of radiotherapy in sarcoma development. Cohort incidence study of patients with retinoblastoma followed for a median of 20 years, and nested case-control study of a radiation dose-response relationship for bone and soft tissue sarcomas. A total of 1604 patients with retinoblastoma who survived at least 1 year after diagnosis, identified from hospital records in Massachusetts and New York during 1914 to 1984. Incidence of subsequent cancers was statistically significantly elevated only in the 961 patients with hereditary retinoblastoma, in whom 190 cancers were diagnosed, vs 6.3 expected in the general population (relative risk [RR], 30 [95% confidence interval, 26-47]). Cumulative incidence (+/-SE) of a second cancer at 50 years after diagnosis was 51.0% (+/-6.2%) for hereditary retinoblastoma, and 5.0% (+/-3.0%) for nonhereditary retinoblastoma. All 114 sarcomas of diverse histologic types occurred in patients with hereditary retinoblastoma. For soft tissue sarcomas, the RRs showed a stepwise increase at all dose categories, and were statistically significant at 10 to 29.9 Gy and 30 to 59.9 Gy. A radiation risk for all sarcomas combined was evident at doses above 5 Gy, rising to 10.7-fold at doses of 60 Gy or greater (P&amp;amp;lt;.05). Genetic predisposition has a substantial impact on risk of subsequent cancers in retinoblastoma patients, which is further increased by radiation treatment. A radiation dose-response relationship is demonstrated for all sarcomas and, for the first time in humans, for soft tissue sarcomas. Retinoblastoma patients should be examined for new cancers and followed into later life to determine whether their extraordinary cancer risk extends to common cancers of adulthood.
Infants with acute lymphoblastic leukemia (ALL) have a very poor prognosis. Since 1985, we have i... more Infants with acute lymphoblastic leukemia (ALL) have a very poor prognosis. Since 1985, we have intensified therapy for infants with ALL by including a month of high dose multiagent chemotherapy after remission induction. Between 1985 and 1995, we treated 23 infants (age &amp;amp;lt; 12 months). We compared the presenting characteristics and outcomes of these infants with the 11 infants treated on our protocols between 1973 and 1985, an era prior to the intensification of therapy. Available bone marrow samples from infants treated since 1985 were analyzed for the presence of MLL gene rearrangements by Southern blot analyses and for TEL-AML1 gene fusion by reverse transcriptase-polymerase chain reaction. With a median follow-up of 5.6 years, the 50-month event free survival (EFS) (+/- standard error) for the 23 infants was 54 +/- 11%, a significant improvement (P = 0.001) compared with the outcome for the 11 infants treated on our protocols prior to 1985 (EFS = 9 +/- 9%). Of the seven infants found to have a rearranged MLL gene, three (43%) remained in first complete remission. None of the nine infant bone marrow specimens tested had evidence of TEL-AML1 gene fusion. The intensified therapy was complicated by a high incidence of infections, including septicemia in 52% of patients and Pneumocystis carinii pneumonitis in 22% of patients. Late effects identified in the 13 long term survivors (median age, 6 years) included developmental delay and learning disabilities of varying severity (82% of evaluable patients), asymptomatic cataracts (67%), asymptomatic echocardiographic abnormalities (30%), obesity (27%), and short stature (18%). Intensification of therapy significantly improved the EFS of infants with ALL compared with previous, less intensive regimens and with the experience of other investigators. Future treatment for infants should attempt to improve efficacy while minimizing toxicity.
The following represents disclosure information provided by authors of this manuscript. All relat... more The following represents disclosure information provided by authors of this manuscript. All relationships are considered compensated. Relationships are self-held unless noted. I 5 Immediate Family Member, Inst 5 My Institution. Relationships may not relate to the subject matter of this manuscript. For more information about ASCO's conflict of interest policy, please refer to www.asco.org/rwc or ascopubs.org/jco/site/ifc.
Radiation therapy (RT) is integral in the treatment of pediatric brain tumors; however, photon RT... more Radiation therapy (RT) is integral in the treatment of pediatric brain tumors; however, photon RT (XRT) often results in intellectual decline, executive functioning (EF) deficits, academic underachievement/failure, and lower health-related quality of life (HRQoL). Proton RT (PRT) provides more targeted therapy, minimizing damage to the developing brain, yet few studies have examined its neuropsychological effects. This study investigated the role of EF in academic skills and HRQoL in a sample of children treated with PRT. A mediation model was proposed in which academic skills mediated relations between aspects of EF and school-based HRQoL (sHRQoL). Sixty-five children (x̅ = 12.4; 43.9% male) treated with PRT completed follow-up neuropsychological testing as part of routine care. Measures included assessment of intellectual functioning, EF, attention, and academic skills (reading, math, spelling). Parents reported on children's EF and attention problems. sHRQoL was assessed via ...
International journal of radiation oncology, biology, physics, 2018
Radiation therapy is integral in treatment of pediatric brain tumors, but it is associated with n... more Radiation therapy is integral in treatment of pediatric brain tumors, but it is associated with negative long-term sequelae. Proton beam radiation therapy (PRT), which enables better focusing of radiation on tumors, may entail fewer sequelae. This prospective study examined cognitive and adaptive functioning in children and young adults treated with PRT. A total of 155 patients were assessed using age-appropriate measures for cognitive and adaptive functioning at start of or during PRT (baseline) and at follow-up. Mean age at baseline was 8.9 years; mean follow-up interval was 3.6 years. Diagnoses included medulloblastoma, craniopharyngioma, ependymoma, glial tumors, germ cell tumors, and others. The sample was divided by age at baseline (<6 years [N = 57, or 37%] and ≥6 years [N = 98, or 63%]) and by PRT field (craniospinal irradiation [CSI; 39%] and focal irradiation [61%]). Scores for mean intelligence quotient (IQ) and adaptive functioning skills were in the average range at ...
There is currently much interest in identifying and mitigating gender inequity within medicine, t... more There is currently much interest in identifying and mitigating gender inequity within medicine, the greater workforce and society as a whole. We provide an evidence-based review of current and historical trends in gender diversity in the RO physician workforce and identify potential barriers to diversity and inclusion in training, professional development, and career advancement. Next, we move to actionable items, addressing methods to mitigate bias, harassment, and other impediments to professional productivity and characterizing leadership lessons and imperatives for departmental, institutional, and organizational leaders.
International journal of radiation oncology, biology, physics, 2018
While cranial irradiation is often essential to attain a cure for pediatric brain tumors, it is a... more While cranial irradiation is often essential to attain a cure for pediatric brain tumors, it is associated with adverse neurocognitive outcomes, primarily manifested as declines in full-scale IQ (FSIQ). This decline results primarily from a decline in processing speed, a component of FSIQ. However, estimated IQ (EIQ) is frequently used in research and clinical settings but does not incorporate processing speed. We hypothesized that EIQ systematically underestimates neurocognitive sequelae in irradiated pediatric brain tumor survivors. We treated 185 pediatric brain tumor patients with proton radiation therapy. All patients had at least 1 neuropsychological evaluation at baseline and/or 1 or more follow-up evaluations with sufficient data to calculate both FSIQ and EIQ. The Wechsler Intelligence Scales were used to calculate FSIQ and EIQ for each patient, and mixed linear models were used to assess disparities between FSIQ and EIQ. At baseline, EIQ was 2.2 points (95% confidence inte...
International journal of radiation oncology, biology, physics, 2018
Proton radiation therapy is commonly used in young children with brain tumors for its potential t... more Proton radiation therapy is commonly used in young children with brain tumors for its potential to reduce late effects. However, some proton series report higher rates of brainstem injury (0%-16%) than most photon series (2.2%-8.6%). We report the incidence of brainstem injury and a risk factor analysis in pediatric patients with posterior fossa primary tumors treated with proton radiation therapy at our institution. The study included 216 consecutive patients treated between 2000 and 2015. Dosimetry was available for all but 4 patients. Grade 2 to 5 late brainstem toxicity was assessed by the National Cancer Institute Common Terminology Criteria for Adverse Events version 4.0. The histologies include medulloblastoma (n=154, 71.3%), ependymoma (n=56, 25.9%), and atypical teratoid rhabdoid tumor (n=6, 2.8%). The median age at irradiation was 6.6 years (range, 0.5-23.1 years); median dose, 54 gray relative biological effectiveness (Gy RBE) (range, 46.8-59.4 Gy RBE); and median follow-...
European Journal of Cancer Part B: Oral Oncology, 1995
Sixty-eight children who were diagnosed with acute lymphoblastic leukaemia (ALL) prior to age 5 y... more Sixty-eight children who were diagnosed with acute lymphoblastic leukaemia (ALL) prior to age 5 years and treated with chemotherapy alone, chemotherapy plus 1800 cGy cranial irradiation (RT), or chemotherapy plus 2400 cGy RT were assessed clinically for overall dental health. All patients were at least 60 months in continuous remission. Dental caries were assessed by NIDR diagnostic criteria, oral hygiene was assessed by the modified Oral Hygiene Index, and gingival health was assessed by the modified gingival index of Loe and Silness. There was no significant difference in caries experience between the three groups nor with the normal population. Those patients that received 2400 cGy RT had significantly higher plaque and periodontal index scores than patients in the other treatment groups. The results of this study suggest that: (1) children with ALL treated with any of the described modalities are at no greater risk of developing dental caries than the normal population; and (2) patients receiving 2400 cGy prior to age 5 years are at greater risk of developing periodontal disease than patients treated with other central nervous system prophylaxis regimens examined in this study.
From 1968 to 1983, 19 patients were treated at the Joint Center for Radiation Therapy for symptom... more From 1968 to 1983, 19 patients were treated at the Joint Center for Radiation Therapy for symptomatic mediastinal masses before a biopsy was obtained. This study evaluates the impact of radiation on the ability to establish a pathologic diagnosis and the results of subsequent empirical therapy if no diagnosis was established. Eight of the 19 (42%) patients were not able to have a histologic diagnosis established at the time of biopsy. Seven of these eight patients went on to receive empiric therapy for what was thought to be the most likely diagnosis on clinical grounds. Four of the seven have not relapsed; three who have relapsed were found to have the diagnosis for which they were empirically treated. The untreated patient relapsed with seminoma. Thus, the use of emergency irradiation for mediastinal masses is sometimes associated with the loss of pathologic diagnosis. These patients likely have a radioresponsive disease (ie, lymphoma or seminoma) that may be treated successfully ...
Primary lymphoma of bone is an unusual extranodal presentation of pediatric non-Hodgkin's lym... more Primary lymphoma of bone is an unusual extranodal presentation of pediatric non-Hodgkin's lymphoma (NHL). Treatment with radiotherapy alone has resulted in a disease-free survival rate of approximately 50% in most adult series. Between January 1973 and April 1985, 11 children with biopsy-proven NHL of bone were seen and treated at our institutions. The minimal clinical staging included chest and bone radiographs, a radionuclide bone scan, complete blood cell counts and serum chemistries, and a bone marrow aspirate and biopsy. The age range was 9 to 17 years with a median age of 14 years. Histology included diffuse lymphoblastic lymphoma in four patients and diffuse histiocytic lymphoma in seven. Each patient was treated with the Adriamycin/prednisone/Oncovin (APO) protocol and ten patients received concomitant radiation to the whole bone when possible and a boost to the primary lesion(s). The median tumor dose was 5,000 rad (range, 3,600 to 5,600). The median follow-up was 8 yea...
Background The purpose of this analysis is to report long-term health-related quality of life (HR... more Background The purpose of this analysis is to report long-term health-related quality of life (HRQoL) among brain tumor survivors treated with proton therapy (PRT) at a very young age. Methods Fifty-nine children <4 years old received PRT between 2000 and 2011. Forty families participated. HRQoL was assessed by child self-report (CSR; age ≥5) and parent proxy report (PPR; age 2+) using the PedsQL Core. Results The median age was 2.5 years (range, 0.3–3.8) at PRT and 9.1 years (5.5–18) at last follow-up. The most common diagnoses were ependymoma (n = 22) and medulloblastoma (n = 7). Median follow-up is 6.7 years (3–15.4). Follow-up mean CSR and PPR scores were: total core (78.4 and 72.9), physical (82.9 and 75.2), psychosocial (76.0 and 71.6), emotional (74.4 and 70.7), social (81.2 and 75.1), and school (72.4 and 69.9). Parent-reported HRQoL fell within a previously defined range for healthy children in 37.5% of patients, and for children with severe health conditions in 45% of p...
We evaluated the therapeutic efficacy of autologous bone marrow transplantation (ABMT) in 49 pati... more We evaluated the therapeutic efficacy of autologous bone marrow transplantation (ABMT) in 49 patients with acute leukemia, using ex-vivo-treated marrow with mafosfamide. This report summarizes data collected from 8 Italian centers, involving 27 cases with acute lymphoid leukemia (ALL) and 22 cases with acute nonlymphoid leukemia (ANLL). The analysis of the results shows that the disease-free survival of ALL and
The authors assessed whether the use of a topical anti-inflammatory agent at the radiation entry ... more The authors assessed whether the use of a topical anti-inflammatory agent at the radiation entry site would prevent radiation-induced skin damage and found that the use of a topical anti-inflammatory agent, such as one containing 10% trolamine salicylate, may reduce skin damage at the radiation entry site if used prophylactically.
Optic pathway gliomas represent approximately 5% of all pediatric intracranial tumors. While thes... more Optic pathway gliomas represent approximately 5% of all pediatric intracranial tumors. While these tumors are most frequently low grade astrocytomas, they follow a highly variable clinical course, and accordingly, there is much debate regarding their optimal management. Their propensity to occur in very young children and infants further complicates selection of therapy. Historically, surgery and radiotherapy have played a primary role in management, however, in the last 15 years, chemotherapy has evolved into the first-line treatment of choice. Nonetheless, chemotherapy frequently fails, but serves to delay implementation of radiotherapy or surgery until the child has progressed neuropsychologically. An overall favorable prognosis for this tumor emphasizes the need for careful selection of therapy. Herein, we review the major features of optic pathway glioma, including epidemiology, pathology, therapeutic interventions, outcome, and treatment sequelae.
To our knowledge, this is the first reported case of the use of stereotactic radiotherapy for an ... more To our knowledge, this is the first reported case of the use of stereotactic radiotherapy for an eosinophilic granuloma (EG) of the clivus. We report follow-up information on two previously reported cases and suggest a management plan for this rare lesion. We report the case of a 4.5-year-old boy who presented with a complete abducens palsy on the right with an associated head turn. A computed tomographic scan of his head revealed a lytic lesion on that side, and magnetic resonance imaging showed the mass to be of low intensity on T1-weighted images and of high intensity on T2-weighted images with heterogeneous enhancement. A transnasal stereotactic biopsy was performed, revealing an EG. The patient was treated with stereotactic radiotherapy, and he became symptom-free with radiographic resolution of his lesion. Reviewing the literature, we found 13 series with 87 cases of EG in the petrous portion of the temporal bone. EG in the cranial base occurring outside of the temporal bone or in the temporal bone and extending intracranially is, however, quite rare, with only nine other cases reported, two of them clival. These findings suggest a classification schema in which cranial base EG lesions be grouped with either the more common extracranial petrous temporal bone lesions or the very rare intracranial lesions. Although there are few cases in the literature, treatment results indicate that clival EG, and perhaps all intracranial cranial base EGs, be treated by a biopsy alone, followed by surgery or stereotactic radiotherapy if there is an incomplete resolution of the symptoms or if there is a recurrence.
Informed and could freely decide. It seems important to evaluate the quality of current Informati... more Informed and could freely decide. It seems important to evaluate the quality of current Information practice. Methodl: PIs undergoing bronchoscopy were investigated by standard• Ized Interviews about contents and circumstances of the information they got before they gave IC. Furthermore they were asked whether they would like more or rather less information and they were tested with the State• Trait-Anxiety-Inventory. Between Jul 94 and Feb 95 we evaluated 80 pts. Relult.: Generally the legal requirements were met, although out of administrative reasons some pts were informed shortly before the bron• choscopy. n pis (96%) were satisfied with the Information given and only 3 pis would have wanted more. However, 33 pts (41%) could not remember one single of nine mentioned risks of the procedure and 19 pts (24%) did not even remember that they were not allowed to eat and drink shortly after the investigation. Additional information with a video was perceived as Informative but inducing anXiety. Conclullon: Informing patients about medical procedures is a complex communicative challenge. We suggest that valid IC requires to prove that the information given actually reached the patient.
There is a substantial risk of a second cancer for persons with hereditary retinoblastoma, which ... more There is a substantial risk of a second cancer for persons with hereditary retinoblastoma, which is enhanced by radiotherapy. To examine long-term risk of new primary cancers in survivors of childhood retinoblastoma and quantify the role of radiotherapy in sarcoma development. Cohort incidence study of patients with retinoblastoma followed for a median of 20 years, and nested case-control study of a radiation dose-response relationship for bone and soft tissue sarcomas. A total of 1604 patients with retinoblastoma who survived at least 1 year after diagnosis, identified from hospital records in Massachusetts and New York during 1914 to 1984. Incidence of subsequent cancers was statistically significantly elevated only in the 961 patients with hereditary retinoblastoma, in whom 190 cancers were diagnosed, vs 6.3 expected in the general population (relative risk [RR], 30 [95% confidence interval, 26-47]). Cumulative incidence (+/-SE) of a second cancer at 50 years after diagnosis was 51.0% (+/-6.2%) for hereditary retinoblastoma, and 5.0% (+/-3.0%) for nonhereditary retinoblastoma. All 114 sarcomas of diverse histologic types occurred in patients with hereditary retinoblastoma. For soft tissue sarcomas, the RRs showed a stepwise increase at all dose categories, and were statistically significant at 10 to 29.9 Gy and 30 to 59.9 Gy. A radiation risk for all sarcomas combined was evident at doses above 5 Gy, rising to 10.7-fold at doses of 60 Gy or greater (P&amp;amp;lt;.05). Genetic predisposition has a substantial impact on risk of subsequent cancers in retinoblastoma patients, which is further increased by radiation treatment. A radiation dose-response relationship is demonstrated for all sarcomas and, for the first time in humans, for soft tissue sarcomas. Retinoblastoma patients should be examined for new cancers and followed into later life to determine whether their extraordinary cancer risk extends to common cancers of adulthood.
Infants with acute lymphoblastic leukemia (ALL) have a very poor prognosis. Since 1985, we have i... more Infants with acute lymphoblastic leukemia (ALL) have a very poor prognosis. Since 1985, we have intensified therapy for infants with ALL by including a month of high dose multiagent chemotherapy after remission induction. Between 1985 and 1995, we treated 23 infants (age &amp;amp;lt; 12 months). We compared the presenting characteristics and outcomes of these infants with the 11 infants treated on our protocols between 1973 and 1985, an era prior to the intensification of therapy. Available bone marrow samples from infants treated since 1985 were analyzed for the presence of MLL gene rearrangements by Southern blot analyses and for TEL-AML1 gene fusion by reverse transcriptase-polymerase chain reaction. With a median follow-up of 5.6 years, the 50-month event free survival (EFS) (+/- standard error) for the 23 infants was 54 +/- 11%, a significant improvement (P = 0.001) compared with the outcome for the 11 infants treated on our protocols prior to 1985 (EFS = 9 +/- 9%). Of the seven infants found to have a rearranged MLL gene, three (43%) remained in first complete remission. None of the nine infant bone marrow specimens tested had evidence of TEL-AML1 gene fusion. The intensified therapy was complicated by a high incidence of infections, including septicemia in 52% of patients and Pneumocystis carinii pneumonitis in 22% of patients. Late effects identified in the 13 long term survivors (median age, 6 years) included developmental delay and learning disabilities of varying severity (82% of evaluable patients), asymptomatic cataracts (67%), asymptomatic echocardiographic abnormalities (30%), obesity (27%), and short stature (18%). Intensification of therapy significantly improved the EFS of infants with ALL compared with previous, less intensive regimens and with the experience of other investigators. Future treatment for infants should attempt to improve efficacy while minimizing toxicity.
The following represents disclosure information provided by authors of this manuscript. All relat... more The following represents disclosure information provided by authors of this manuscript. All relationships are considered compensated. Relationships are self-held unless noted. I 5 Immediate Family Member, Inst 5 My Institution. Relationships may not relate to the subject matter of this manuscript. For more information about ASCO's conflict of interest policy, please refer to www.asco.org/rwc or ascopubs.org/jco/site/ifc.
Radiation therapy (RT) is integral in the treatment of pediatric brain tumors; however, photon RT... more Radiation therapy (RT) is integral in the treatment of pediatric brain tumors; however, photon RT (XRT) often results in intellectual decline, executive functioning (EF) deficits, academic underachievement/failure, and lower health-related quality of life (HRQoL). Proton RT (PRT) provides more targeted therapy, minimizing damage to the developing brain, yet few studies have examined its neuropsychological effects. This study investigated the role of EF in academic skills and HRQoL in a sample of children treated with PRT. A mediation model was proposed in which academic skills mediated relations between aspects of EF and school-based HRQoL (sHRQoL). Sixty-five children (x̅ = 12.4; 43.9% male) treated with PRT completed follow-up neuropsychological testing as part of routine care. Measures included assessment of intellectual functioning, EF, attention, and academic skills (reading, math, spelling). Parents reported on children's EF and attention problems. sHRQoL was assessed via ...
International journal of radiation oncology, biology, physics, 2018
Radiation therapy is integral in treatment of pediatric brain tumors, but it is associated with n... more Radiation therapy is integral in treatment of pediatric brain tumors, but it is associated with negative long-term sequelae. Proton beam radiation therapy (PRT), which enables better focusing of radiation on tumors, may entail fewer sequelae. This prospective study examined cognitive and adaptive functioning in children and young adults treated with PRT. A total of 155 patients were assessed using age-appropriate measures for cognitive and adaptive functioning at start of or during PRT (baseline) and at follow-up. Mean age at baseline was 8.9 years; mean follow-up interval was 3.6 years. Diagnoses included medulloblastoma, craniopharyngioma, ependymoma, glial tumors, germ cell tumors, and others. The sample was divided by age at baseline (<6 years [N = 57, or 37%] and ≥6 years [N = 98, or 63%]) and by PRT field (craniospinal irradiation [CSI; 39%] and focal irradiation [61%]). Scores for mean intelligence quotient (IQ) and adaptive functioning skills were in the average range at ...
There is currently much interest in identifying and mitigating gender inequity within medicine, t... more There is currently much interest in identifying and mitigating gender inequity within medicine, the greater workforce and society as a whole. We provide an evidence-based review of current and historical trends in gender diversity in the RO physician workforce and identify potential barriers to diversity and inclusion in training, professional development, and career advancement. Next, we move to actionable items, addressing methods to mitigate bias, harassment, and other impediments to professional productivity and characterizing leadership lessons and imperatives for departmental, institutional, and organizational leaders.
International journal of radiation oncology, biology, physics, 2018
While cranial irradiation is often essential to attain a cure for pediatric brain tumors, it is a... more While cranial irradiation is often essential to attain a cure for pediatric brain tumors, it is associated with adverse neurocognitive outcomes, primarily manifested as declines in full-scale IQ (FSIQ). This decline results primarily from a decline in processing speed, a component of FSIQ. However, estimated IQ (EIQ) is frequently used in research and clinical settings but does not incorporate processing speed. We hypothesized that EIQ systematically underestimates neurocognitive sequelae in irradiated pediatric brain tumor survivors. We treated 185 pediatric brain tumor patients with proton radiation therapy. All patients had at least 1 neuropsychological evaluation at baseline and/or 1 or more follow-up evaluations with sufficient data to calculate both FSIQ and EIQ. The Wechsler Intelligence Scales were used to calculate FSIQ and EIQ for each patient, and mixed linear models were used to assess disparities between FSIQ and EIQ. At baseline, EIQ was 2.2 points (95% confidence inte...
International journal of radiation oncology, biology, physics, 2018
Proton radiation therapy is commonly used in young children with brain tumors for its potential t... more Proton radiation therapy is commonly used in young children with brain tumors for its potential to reduce late effects. However, some proton series report higher rates of brainstem injury (0%-16%) than most photon series (2.2%-8.6%). We report the incidence of brainstem injury and a risk factor analysis in pediatric patients with posterior fossa primary tumors treated with proton radiation therapy at our institution. The study included 216 consecutive patients treated between 2000 and 2015. Dosimetry was available for all but 4 patients. Grade 2 to 5 late brainstem toxicity was assessed by the National Cancer Institute Common Terminology Criteria for Adverse Events version 4.0. The histologies include medulloblastoma (n=154, 71.3%), ependymoma (n=56, 25.9%), and atypical teratoid rhabdoid tumor (n=6, 2.8%). The median age at irradiation was 6.6 years (range, 0.5-23.1 years); median dose, 54 gray relative biological effectiveness (Gy RBE) (range, 46.8-59.4 Gy RBE); and median follow-...
European Journal of Cancer Part B: Oral Oncology, 1995
Sixty-eight children who were diagnosed with acute lymphoblastic leukaemia (ALL) prior to age 5 y... more Sixty-eight children who were diagnosed with acute lymphoblastic leukaemia (ALL) prior to age 5 years and treated with chemotherapy alone, chemotherapy plus 1800 cGy cranial irradiation (RT), or chemotherapy plus 2400 cGy RT were assessed clinically for overall dental health. All patients were at least 60 months in continuous remission. Dental caries were assessed by NIDR diagnostic criteria, oral hygiene was assessed by the modified Oral Hygiene Index, and gingival health was assessed by the modified gingival index of Loe and Silness. There was no significant difference in caries experience between the three groups nor with the normal population. Those patients that received 2400 cGy RT had significantly higher plaque and periodontal index scores than patients in the other treatment groups. The results of this study suggest that: (1) children with ALL treated with any of the described modalities are at no greater risk of developing dental caries than the normal population; and (2) patients receiving 2400 cGy prior to age 5 years are at greater risk of developing periodontal disease than patients treated with other central nervous system prophylaxis regimens examined in this study.
From 1968 to 1983, 19 patients were treated at the Joint Center for Radiation Therapy for symptom... more From 1968 to 1983, 19 patients were treated at the Joint Center for Radiation Therapy for symptomatic mediastinal masses before a biopsy was obtained. This study evaluates the impact of radiation on the ability to establish a pathologic diagnosis and the results of subsequent empirical therapy if no diagnosis was established. Eight of the 19 (42%) patients were not able to have a histologic diagnosis established at the time of biopsy. Seven of these eight patients went on to receive empiric therapy for what was thought to be the most likely diagnosis on clinical grounds. Four of the seven have not relapsed; three who have relapsed were found to have the diagnosis for which they were empirically treated. The untreated patient relapsed with seminoma. Thus, the use of emergency irradiation for mediastinal masses is sometimes associated with the loss of pathologic diagnosis. These patients likely have a radioresponsive disease (ie, lymphoma or seminoma) that may be treated successfully ...
Primary lymphoma of bone is an unusual extranodal presentation of pediatric non-Hodgkin's lym... more Primary lymphoma of bone is an unusual extranodal presentation of pediatric non-Hodgkin's lymphoma (NHL). Treatment with radiotherapy alone has resulted in a disease-free survival rate of approximately 50% in most adult series. Between January 1973 and April 1985, 11 children with biopsy-proven NHL of bone were seen and treated at our institutions. The minimal clinical staging included chest and bone radiographs, a radionuclide bone scan, complete blood cell counts and serum chemistries, and a bone marrow aspirate and biopsy. The age range was 9 to 17 years with a median age of 14 years. Histology included diffuse lymphoblastic lymphoma in four patients and diffuse histiocytic lymphoma in seven. Each patient was treated with the Adriamycin/prednisone/Oncovin (APO) protocol and ten patients received concomitant radiation to the whole bone when possible and a boost to the primary lesion(s). The median tumor dose was 5,000 rad (range, 3,600 to 5,600). The median follow-up was 8 yea...
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Papers by Nancy Tarbell