Audiological profile of patients treated for childhood cancer / Perfil audiológico de pacientes tratados de câncer na infância

Abstract Objective: To characterize the hearing loss after cancer treatment, according to the type of treatment, with identification of predictive factors. Methods: Two hundred patients who had cancer in childhood were prospectively evaluated. The mean age at diagnosis was 6 years, and at the audiometric assessment, 21 years. The treatment of the participants included chemotherapy without using platinum derivatives or head and neck radiotherapy in 51 patients; chemotherapy using cisplatin without radiotherapy in 64 patients; head and neck radiotherapy without cisplatin in 75 patients; and a combined treatment of head and neck radiotherapy and chemotherapy with cisplatin in ten patients. Patients underwent audiological assessment, including pure tone audiometry, speech audiometry, and immittancemetry. Results: The treatment involving chemotherapy with cisplatin caused 41.9% and 47.3% hearing loss in the right and left ear, respectively, with a 11.7-fold higher risk of hearing loss in the right ear and 17.6-fold higher in the left ear versus patients not treated with cisplatin (p < 0.001 and p < 0.001, respectively). Children whose cancer diagnosis occurred after the age of 6 have shown an increased risk of hearing loss vs. children whose diagnosis occurred under 6 years of age (p = 0.02). Conclusion: The auditory feature found after the cancer treatment was a symmetrical bilateral sensorineural hearing loss. Chemotherapy with cisplatin proved to be a risk factor, while head and neck radiotherapy was not critical for the occurrence of hearing loss.

Resumo Objetivo: Caracterizar as alterações auditivas após o tratamento do câncer, segundo o tipo de tratamento identificando os fatores preditivos. Método: Foram avaliados prospectivamente duzentos pacientes que tiveram cancer na infância. A idade média ao diagnóstico foi de 6 anos e à avaliação audiométrica de 21 anos de idade. O tratamento incluiu quimioterapia sem uso de derivados de platina ou radioterapia em cabeça e pescoço em 51 pacientes; quimioterapia com uso de cisplatina sem radioterapia em 64 pacientes; radioterapia em cabeça e pescoço sem cisplatina em 75 pacientes; e 10 pacientes receberam o tratamento combinado de radioterapia em cabeça e pescoço e quimioterapia com cisplatina. Os pacientes foram submetidos à avaliação audiológica incluindo audiometria tonal, audiometria vocal e imitanciometria. Resultados: O tratamento envolvendo quimioterapia com cisplatina levou a 41,9% e 47,3% de perda auditiva na orelha direita e esquerda, respectivamente, apresentando risco 11,7 vezes maior de desenvolver perda auditiva na orelha direita e 17,6 vezes na orelha esquerda do que aqueles que não receberam cisplatina (p < 0,001 e p < 0,001; respectivamente). Crianças cujo diagnóstico do câncer ocorreu após os 6 anos de idade mostraram maior risco de apresentar perda auditiva do que crianças menores do que 6 anos de idade (p = 0,02). Conclusão: A característica audiológica encontrada após tratamento oncológico foi perda auditiva sensorioneural bilateral simétrica. A quimioterapia com cisplatina mostrou ser fator de risco, enquanto a radioterapia em cabeça e pescoço não foi determinante para aquisição da perda auditiva.

Audiological profile of patients treated for childhood cancer.

OBJECTIVE: To characterize the hearing loss after cancer treatment, according to the type of treatment, with identification of predictive factors. METHODS: Two hundred patients who had cancer in childhood were prospectively evaluated. The mean age at diagnosis was 6 years, and at the audiometric assessment, 21 years. The treatment of the participants included chemotherapy without using platinum derivatives or head and neck radiotherapy in 51 patients; chemotherapy using cisplatin without radiotherapy in 64 patients; head and neck radiotherapy without cisplatin in 75 patients; and a combined treatment of head and neck radiotherapy and chemotherapy with cisplatin in ten patients. Patients underwent audiological assessment, including pure tone audiometry, speech audiometry, and immittancemetry. RESULTS: The treatment involving chemotherapy with cisplatin caused 41.9% and 47.3% hearing loss in the right and left ear, respectively, with a 11.7-fold higher risk of hearing loss in the right ear and 17.6-fold higher in the left ear versus patients not treated with cisplatin (p<0.001 and p<0.001, respectively). Children whose cancer diagnosis occurred after the age of 6 have shown an increased risk of hearing loss vs. children whose diagnosis occurred under 6 years of age (p=0.02). CONCLUSION: The auditory feature found after the cancer treatment was a symmetrical bilateral sensorineural hearing loss. Chemotherapy with cisplatin proved to be a risk factor, while head and neck radiotherapy was not critical for the occurrence of hearing loss.

High dose rate and external beam radiotherapy in locally advanced prostate cancer.

PURPOSE: To report the outcomes and toxicity of high dose rate brachytherapy as a boost for localized prostate cancer. MATERIALS AND METHODS: Between 1997 and 2000, the medical records of 131 patients with prostate adenocarcinoma who were treated with external beam radiation therapy and high dose rate brachytherapy, were retrospectively analyzed. Furthermore, 55% of the patients received neoadjuvant/concurrent or adjuvant androgen deprivation therapy. Patients were stratified into 2 groups. Group 1 included 65 patients with Gleason score 7, pretreatment prostate specific antigen (PSA) between 10 and 20 ng/mL, and clinical stage T2b. Group 2 included 66 patients with Gleason score between 8 and 10, PSA greater than 20 ng/mL, and clinical stage greater than T2b. RESULTS: At a median follow-up of 62.8 months, the 5-year biochemical control (BC) rate, as defined by the American Society for Therapeutic Radiology and Oncology Phoenix Consensus panel statement, was 81% and overall survival was 91%. BC in Groups 1 and 2 were 87% and 71%, respectively. On univariate analysis risk group, pretreatment PSA and age were significant predictors of BC. However, on multivariate analysis only pretreatment PSA was significant. Using the Radiation therapist oncology group criteria, there were 2 (1.5%) cases of grade 3 acute urinary toxicity. Regarding late side effects (n = 5), 4% of patients had grade 3 genitourinary toxicity and no grade 4 complication was observed. CONCLUSIONS: External beam radiation therapy and high dose rate brachytherapy for prostate cancer resulted in excellent BC, and overall survival with minimal severe, acute, or late complications.

Unusual dissemination patterns of low-grade astrocytomas in childhood.

CONTEXT: Low-grade astrocytomas are intracerebral lesions of relatively high frequency in the under-18 pediatric population. They often present indolent behaviour, and complete surgical resection is the choice treatment. In cases where the surgery is not possible, chemotherapy and radiotherapy may be used. Medical reports do not recommend examination of the spinal cord at diagnosis or during treatment, since the risk of dissemination of the lesion to the spine is minimal according to medical experience. We describe here four cases of children with low-grade astrocytoma with aggressive dissemination to the neuroaxis.

Unusual dissemination patterns of low-grade astrocytomas in childhood

CONTEXT: Low-grade astrocytomas are intracerebral lesions of relatively high frequency in the under-18 pediatric population. They often present indolent behaviour, and complete surgical resection is the choice treatment. In cases where the surgery is not possible, chemotherapy and radiotherapy may be used. Medical reports do not recommend examination of the spinal cord at diagnosis or during treatment, since the risk of dissemination of the lesion to the spine is minimal according to medical experience. We describe here four cases of children with low-grade astrocytoma with aggressive dissemination to the neuroaxis.

CONTEXTO: Gliomas de baixo grau de malignidade são lesões intracerebrais relativamente freqüentes na população pediátrica menor de 18 anos de idade. Eles freqüentemente são indolentes em seu comportamento e a ressecção cirúrgica completa é o tratmento de eleição. Nos casos em que a cirurgia não é possível, a quimioterapia e a radioterapia podem ser utilizadas. Relatos da literatura não recomendam a avaliação radiológica da coluna espinhal ao diagnóstico ou durante o tratamento, desde que o risco de disseminação destas lesões para a coluna é considerado mínimo. Descrevemos aqui quatro casos de crianças com gliomas de baixo grau de malignidade com disseminação agressiva para o neuroeixo.

Retinoblastoma in children older than 5 years of age.

BACKGROUND: Retinoblastoma is a malignant tumor of the embryonic neural retina. About 80% of cases are diagnosed before age 4, with a median age at diagnosis of 2 years. OBJECTIVE: To determine characteristics and prognosis of retinoblastoma in children older than 5 years. PROCEDURES: From 1986 to 2002, medical records of 16 patients out of 453 cases referred to Hospital do Câncer AC Camargo, São Paulo, Brazil. RESULTS: Median age at diagnosis was 73.7 months (range 65-144) and there was an equal gender distribution. Fifteen patients presented with unilateral disease. The mean time between first symptoms and diagnosis was 9.6 months (range 0-48). Most cases were diagnosed in advanced stages and 15 eyes were enucleated. Eleven patients presented with intraocular tumor (1 Reese II and 10 Reese V) and five presented with extraocular disease (one CCG II and four CCG III). Twelve patients are still alive with a median follow-up of 92 months (range 65-199). CONCLUSIONS: Because of its low incidence at this age, diagnosis of retinoblastoma is usually delayed due to low level of suspicion. Therefore, it is important that physicians are aware of this disease in order to perform an earlier diagnosis, and decrease treatment-related morbidity.

Trilateral retinoblastoma.

BACKGROUND: Trilateral retinoblastoma (TRB) is a syndrome consisting of unilateral or bilateral hereditary retinoblastoma (Rb) associated with an intracranial neuroblastic tumor. Although its incidence is low, the prognosis is very poor. This article reports four cases of TRB and discusses the role of neuroimaging screening for early detection. PROCEDURE: From January 1986 to December 2003, 470 children with Rb were admitted to the Pediatrics and Ophthalmology Departments, A C Camargo Hospital, São Paulo, Brazil. RESULTS: There were four patients with pineoblastoma, two of whom had a positive familial history. The age at diagnosis of Rb was 4, 6, 10, and 24 months while the age of diagnosis of TRB was 10, 25, 57, and 72 months. One patient presented TRB at initial diagnosis of Rb. Three patients had bilateral disease and all of them had one eye enucleated, followed by chemotherapy and/or external beam radiation therapy (EBRT). One child with unilateral disease was only submitted to enucleation. In spite of intensive treatment, all patients died with progressive disease within 7, 8, 12, and 12 months after diagnosis of TRB. CONCLUSIONS: Early diagnosis as well as new therapeutic approaches are needed to achieve better results.

Crianças portadoras de neurofibromatose tipo 2: o pediatra geral pode reconhecer esta entidade? / Children with neurofibromatosis type 2: can the general pediatrician recognize this entity?

A neurofibromatose tipo 2 é uma desordem autossômica dominante causada pela inativação ou perda de ambos os alelos do gene supressor de tumor NF2. Neste artigo estão sumarizados a epidemiologia, genética características clínicas, e os critérios para avaliar e diagnosticar o quadro nas pessoas de risco...

Treatment of retinoblastoma patients with chemoreduction plus local therapy: experience of the AC Camargo Hospital, Brazil.

To evaluate the efficacy of conservative management of intraocular retinoblastoma with chemoreduction combined with local therapy with or without plaque radiation in the preservation of the eye, and avoidance of external beam radiation therapy (EBRT) (success rate). From 1995 to 2000, 84 newly diagnosed patients with intraocular retinoblastoma were admitted to the Pediatric Department of the Hospital do Cancer A.C. Camargo, São Paulo, Brazil. All children were treated with 2 to 6 cycles of chemotherapy (carboplatin, vincristine, and etoposide) plus local therapy (cryotherapy, laser photocoagulation, and thermotherapy), or plaque radiation therapy during and/or after the chemotherapy. The Mann-Whitney test was used to compare means of quantitative variables. The chi test or the Fisher exact test were employed to verify the association between the outcome and the independent variables. For all tests alpha=5% was adopted. Success rate was higher for patients with bilateral tumors (54%) than for children with unilateral tumors (19%) (P=0.003). For patients with Reese-Ellsworth stages I, II, and III, no statistically significant differences in the success rates were noted in the group of unilateral (50%) and bilateral tumors (79.1%) (P=0.179). Among children with Reese-Ellsworth stages IV and V, the success rate was significantly higher for patients with bilateral tumors (40.7%) than for those with unilateral (0%) (P=0.012). Chemoreduction combined with local therapy, with or without plaque radiotherapy, is efficacious in avoiding enucleation and the use of external beam radiation therapy for children with intraocular retinoblastoma.

Neoadjuvant radiochemotherapy in the treatment of fixed and semi-fixed rectal tumors. Analysis of results and prognostic factors.

PURPOSE: To report the retrospective analysis of patients with locally advanced rectal cancer treated with neodjuvant radiochemotherapy. METHODS AND MATERIALS: From January 1994 to December 2003, 101 patients with fixed (25%) or semi-fixed (75%) rectal adenocarcinoma were treated by preoperative radiotherapy with a dose of 45 Gy at the whole pelvis and 50.4 Gy at primary tumor, concomitant to four weekly chemotherapies with 5-Fluorouracil (425 mg/m2) and Leucovorin (20 mg/m2). In 71 patients (70.3%) the primary tumor was located up to 6 cm from the anal verge and in 30 (29.7%) from 6.5 cm to 10 cm. Age, gender, tumor fixation, tumor distance from the anal verge, clinical response, surgical technique, and postoperative TNM stage were the prognostic factors analyzed for overall survival (OS), disease-free survival (DFS), and local control (LC) at five years. RESULTS: Median follow-up time was 38 months (range, 2-141). Complete response was observed in eight patients (7.9%), partial in 54 (53.4%) and absence in 39 (38.7%). OS, DFS and LC were 52.6%, 53.8%, and 75.9%, respectively. Distant metastasis occurred in 40 (39.6%) patients, local recurrence in 20 (19.8%) and both in 16 (15.8%). Patients with fixed tumors had lower OS (17% Vs 65.6%; p < 0.001), DFS (31.2% Vs 60.9%; p = 0.005), and LC (58% Vs 82%; p = 0.004). Patients with tumors more than 6 cm above the anal verge had better LC (93% Vs 69%; p = 0.04). The postoperative TNM stage was a significant factor for DFS (I:64.1%, II:69.6%, III:35.2%, IV:11.1%; p < 0.001) and for LC (I:75.7%, II: 92.9%, III:54.1%, IV:100%; p = 0.005). Patients with positive lymph nodes had worse OS (37.9% Vs 70.4%, p = 0.006), DFS (32% Vs 72.7%, p < 0.001) and LC (56.2% Vs 93.4%; p < 0.001). CONCLUSION: This study suggests that the neoadjuvant treatment employed was effective for local control. Fixation of the lesion and lymph nodes metastasis were the main adverse prognostic factors. Distant failures were frequent, supporting the need of new drugs for adjuvant chemotherapy.

Biochemical Relapse for Prostate Cancer Following Radical Prostatectomy: Salvage Radiotherapy Without HormonalTherapy

Objectives: This paper aims to study biochemical control, hormonal therapy-free survival, and prognostic factors related tosalvage radiation for prostate cancer patients submitted to radical prostatectomy (RP) without hormonal therapy (HT) before orduring radiation. Materials and Methods: from August 2002 to July 2004, 39 prostate cancer patients submitted to RPpresented biochemical failure after achieving PSA nadir (<0.2ng/ml). All patients were submitted to three-dimensional conformalexternal beam radiation therapy (3DC-EBRT) and no patients had received HT. Median age was 62 years, median preoperativePSA was 9.4ng/ml, median Gleason Score was 7. We defined PSA rise above 0.2 as biochemical failure after surgery. Median3DC-EBRT dose was 70Gy, and biochemical failure after EBRT was defined as three consecutive rises in PSA or a single risesufficient to trigger HT. Results: Biochemical non-evidence of disease (BNED) in 3 years was 72%. PSA doubling time (PSADT)lower than 4 months (p=0.04), and delay to salvage EBRT (p=0.05) were associated to worse chance of successful salvagetherapy. Late morbidity was acceptable. Conclusion: Expressive PSA control (72% BNED / 3years) could be achieved withsalvage radiotherapy in well-selected patients. The importance of PSADT was confirmed, and radiotherapy should be started asearly as possible. Follow-up is somewhat short, but it is possible to conclude that it is possible to achieve a long interval freefrom hormonal therapy with low rate of toxicity, avoiding or at least delaying morbidity related to hormonal treatment.radiotherapy