Your browser doesn't support javascript.
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 242
Filtrar
1.
J Coll Physicians Surg Pak ; 29(6): 553-557, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31133156

RESUMO

OBJECTIVE: To describe different types of primary extraocular muscle (EOM) tumours based on the results of imaging studies, peroperative clinical picture and their histopathological diagnosis. STUDY DESIGN: Case series. PLACE AND DURATION OF STUDY: Al-Shifa Trust Eye Hospital, Rawalpindi, from July 2001 to January 2017. METHODOLOGY: A retrospective analysis of 640 diagnosed orbital tumours was carried out using non-randomised sampling technique, and the prevalence of primary EOM tumours was determined. Based on the results of imaging studies, the clinical picture observed during surgery (orbitotomy) and the histopathological diagnosis, primary EOM tumours were divided into different types, accordingly. RESULTS: Nineteen (n=19) primary EOM tumours (frequency of 2.96%) had 12 types of histopathological diagnoses, and were categorised into inflammatory tumours (n=8, 42%), vascular tumours (n=4, 21%), lymphoproliferative tumours (n=3, 16%), neurogenic tumours (n=2, 10.5%) and myogenic tumours (n=2, 10.5%). The recti were involved more frequently than obliques (n=15, 78.94% and n=4, 21.06%, respectively). All the patients presented with proptosis of varying degree with some degree of globe rotation and had surgical excision/appropriate management. Visual acuity was not affected in any of the patients. Four (n=4, 21.05%) tumours were malignant (NHL, ASPS, myeloid sarcoma and rhabdomyosarcoma) and these patients underwent chemotherapy and/or radiotherapy. CONCLUSION: Biopsy-proven primary EOM tumours were devisable into five broad categories. Patients with primary EOM tumours presented with proptosis and impaired ocular motiliy. The primary EOM tumours involved both the recti and the obliques and were excised surgically with favourable outcomes in most cases.


Assuntos
Neoplasias Musculares/patologia , Músculos Oculomotores/patologia , Neoplasias Orbitárias/patologia , Adolescente , Adulto , Biópsia , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Musculares/epidemiologia , Neoplasias Musculares/cirurgia , Músculos Oculomotores/cirurgia , Neoplasias Orbitárias/classificação , Neoplasias Orbitárias/epidemiologia , Neoplasias Orbitárias/cirurgia , Paquistão/epidemiologia , Prevalência , Rabdomiossarcoma/epidemiologia , Rabdomiossarcoma/patologia , Rabdomiossarcoma/cirurgia , Sarcoma Alveolar de Partes Moles/epidemiologia , Sarcoma Alveolar de Partes Moles/patologia , Sarcoma Alveolar de Partes Moles/cirurgia
2.
BMC Cancer ; 18(1): 1247, 2018 Dec 13.
Artigo em Inglês | MEDLINE | ID: mdl-30541504

RESUMO

BACKGROUND: Uterine sarcomas consist a heterogeneous group of mesenchymal gynecological malignancies with unclear therapeutic recommendations and unspecific but poor prognosis, since they usually metastasize and tend to recur very often, even in early stages. METHODS: We retrospectively analyzed all female patients with uterine sarcomas treated in our institution over the last 17 years. Clinico-pathological data, treatments and outcomes were recorded. Kaplan-Meier curves were plotted and time-to-event analyses were estimated using Cox regression. RESULTS: Data were retrieved from 61 women with a median age of 53 (range: 27-78) years, at diagnosis. Fifty-one patients were diagnosed with leiomyosarcoma (LMS), 3 with high grade endometrial stromal sarcoma (ESS), 5 with undifferentiated uterine sarcoma (UUS), 1 with Ewing sarcoma (ES) and 1 with Rhabdomyosarcoma (RS). 24 cases had stage I, 7 stage II, 14 stage III and 16 stage IV disease. Median disease-free survival (DFS) in adjuvant approach was 18.83 months, and median overall survival (OS) 31.07 months. High mitotic count (> 15 mitoses) was significantly associated with worse OS (P < 0.001) and worse DFS (P = 0.028). CONCLUSIONS: Mitotic count appears to be independent prognostic factor while further insights are needed to improve adjuvant and palliative treatment of uterine sarcomas.


Assuntos
Gerenciamento Clínico , Rabdomiossarcoma/diagnóstico , Sarcoma do Estroma Endometrial/diagnóstico , Sarcoma de Ewing/diagnóstico , Adulto , Idoso , Feminino , Grécia/epidemiologia , Humanos , Pessoa de Meia-Idade , Índice Mitótico/métodos , Índice Mitótico/tendências , Prognóstico , Estudos Retrospectivos , Rabdomiossarcoma/epidemiologia , Rabdomiossarcoma/terapia , Sarcoma do Estroma Endometrial/epidemiologia , Sarcoma do Estroma Endometrial/terapia , Sarcoma de Ewing/epidemiologia , Sarcoma de Ewing/terapia
3.
Int J Pediatr Otorhinolaryngol ; 112: 109-112, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30055718

RESUMO

OBJECTIVE: To examine the types of pediatric middle ear tumors and review the demographics, management, and survival of pediatric patients with rhabdomyosarcoma (RMS) of the middle ear. METHODS: Pediatric patients in the Surveillance, Epidemiology, and End Results (SEER) database were included from 1973 to 2014 based on a diagnosis of middle ear tumors using the ICD O-3 code: C30.1: Middle ear primary site. Patients were included from ages 0-18 years. RESULTS: Forty pediatric middle ear tumor cases were identified. Twenty patients were female (50%). Twenty-seven (67.5%) cases were rhabdomyosarcomas (RMS). Pediatric RMS patients tended to be diagnosed in early childhood (mean age 5.30 years, standard deviation 2.9, range 1.00-13.00, 59.3% of patients were ages 5 or below). Most pediatric RMS patients received chemotherapy and radiation therapy as part of the treatment regimen (88.8%). Finally, the 5-year overall and disease-specific survival rates were 59% and 63% respectively. CONCLUSIONS: Pediatric middle ear tumors are rare. Females and male pediatric patients are both at risk for middle ear tumors. RMS is the most common malignant middle ear tumor affecting pediatric patients. Despite the use of multimodality therapies, survival rates for pediatric patients with RMS of the middle ear are low. Physicians may consider including middle ear tumors on the differential diagnosis for pediatric patients with symptoms presenting similarly to non-resolving otitis media.


Assuntos
Neoplasias da Orelha/epidemiologia , Orelha Média , Rabdomiossarcoma Embrionário/epidemiologia , Adenocarcinoma Papilar/epidemiologia , Adolescente , Distribuição por Idade , Quimiorradioterapia , Criança , Pré-Escolar , Terapia Combinada , Gerenciamento Clínico , Neoplasias da Orelha/mortalidade , Neoplasias da Orelha/terapia , Feminino , Histiocitose de Células de Langerhans/epidemiologia , Humanos , Lactente , Estimativa de Kaplan-Meier , Masculino , Tumores Neuroectodérmicos Primitivos Periféricos/epidemiologia , Procedimentos Cirúrgicos Otorrinolaringológicos , Prognóstico , Rabdomiossarcoma/epidemiologia , Rabdomiossarcoma/mortalidade , Rabdomiossarcoma/terapia , Rabdomiossarcoma Embrionário/mortalidade , Rabdomiossarcoma Embrionário/terapia , Programa de SEER , Distribuição por Sexo , Taxa de Sobrevida , Estados Unidos/epidemiologia
4.
Indian Pediatr ; 54(9): 735-738, 2017 Sep 15.
Artigo em Inglês | MEDLINE | ID: mdl-28984250

RESUMO

OBJECTIVE: Analyze the profile and outcome of children with rhabdomyosarcoma from a pediatric-oncology unit. DESIGN: Retrospective analysis of case records over 23 years (1990-2012). Setting: Government-run, tertiary-care, university hospital in Northern India. PARTICIPANTS: 159 children (<12-years) with a diagnosis of rhabdomyosarcoma were enrolled. The median age was 4 years; 13% were infants. MAIN OUTCOME MEASURE: Five-year event free survival. RESULTS: The median symptom interval was 2-months. Head and neck region was the most frequent site (44%), followed by tumors in the extremity (15.7%). The majority (67%) of the tumors were located at 'unfavorable' sites; 68% were >5 cm in size. The most frequent (58%) pathological subtype was embryonal. Treatment was based on the 'Intergroup Rhabdomyosarcoma Study (IRS) Group' risk-stratification. 33% were 'low-risk' children, 11% were 'high-risk'. Treatment-refusal (18%) and abandonment (33%) were major impediments. The median ± SE five-year event free survival of those taking treatment was 43.6 ± 6%. Conclusion: Large sized tumors, tumors at unfavorable locations, and treatment refusal/abandonment contributed to inferior outcome in children with rhabdomyosarcoma.


Assuntos
Rabdomiossarcoma/epidemiologia , Criança , Pré-Escolar , Feminino , Hospitais Pediátricos , Humanos , Índia/epidemiologia , Estimativa de Kaplan-Meier , Masculino , Estudos Retrospectivos , Rabdomiossarcoma/mortalidade
6.
Curr Oncol Rep ; 19(7): 44, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28501984

RESUMO

PURPOSE OF REVIEW: Three primary categories of gynecologic cancer are found in pediatric and adolescent patients: stromal carcinomas including juvenile granulosa cell tumors and Sertoli-Leydig cell tumors, rhabdomyosarcomas arising from the vagina and cervix (sarcoma botryoides), and ovarian germ cell tumors which comprise a wide range of histologies. These entities are rare and treatment approaches have focused on decreasing late effects of chemotherapy treatment. Here, we review presentation, histologic classifications, diagnosis, and treatment recommendations for pediatric gynecologic cancers. RECENT FINDINGS: Event-free and overall survival for these cancers is high, and the goals of treatment are minimization of morbidity and preservation of fertility with unilateral salpingo-oophorectomies and limited staging. Surveillance of tumor markers after surgery is helpful in monitoring for disease progression and adjuvant chemotherapy is often reserved for patients at recurrence. Recent literature supports avoiding chemotherapy even in high-grade germ cell tumors in the pediatric population.


Assuntos
Neoplasias dos Genitais Femininos/epidemiologia , Tumor de Células da Granulosa/epidemiologia , Rabdomiossarcoma/epidemiologia , Tumor de Células de Sertoli-Leydig/epidemiologia , Adolescente , Biomarcadores Tumorais/genética , Criança , Intervalo Livre de Doença , Feminino , Neoplasias dos Genitais Femininos/tratamento farmacológico , Neoplasias dos Genitais Femininos/genética , Neoplasias dos Genitais Femininos/patologia , Tumor de Células da Granulosa/tratamento farmacológico , Tumor de Células da Granulosa/genética , Tumor de Células da Granulosa/patologia , Humanos , Rabdomiossarcoma/tratamento farmacológico , Rabdomiossarcoma/genética , Rabdomiossarcoma/patologia , Sarcoma/tratamento farmacológico , Sarcoma/epidemiologia , Sarcoma/patologia , Tumor de Células de Sertoli-Leydig/tratamento farmacológico , Tumor de Células de Sertoli-Leydig/genética , Tumor de Células de Sertoli-Leydig/patologia , Neoplasias Vaginais/tratamento farmacológico , Neoplasias Vaginais/epidemiologia , Neoplasias Vaginais/patologia
7.
Curr Opin Pediatr ; 29(3): 354-357, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-28319559

RESUMO

PURPOSE OF REVIEW: To review the recent literature regarding biologic characteristics of pediatric solid tumors in African children. RECENT FINDINGS: Data regarding pediatric solid tumors in Africa, while increasing, remain sparse when considering the ethnic and geographic diversity of the continent. Recent work, especially regarding nephroblastoma in Kenya, has identified some biologic variability among local tribes but also when compared with North American tumors. In general, reports from across the continent reveal markedly poorer survival for pediatric patients with solid tumors when compared with high-resourced regions. SUMMARY: Multiple resource-related and infrastructure-related challenges contribute to poorer outcomes, and these require systematic, multidisciplinary, and structured solutions. Socioeconomic factors and limited access to care currently seem to drive the survival outcomes in children with solid cancers in Africa.


Assuntos
Neoplasias Ósseas , Neoplasias Renais , Neoplasias Neuroepiteliomatosas , Neoplasias da Retina , Rabdomiossarcoma , Sarcoma de Ewing , Tumor de Wilms , África/epidemiologia , Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/epidemiologia , Neoplasias Ósseas/terapia , Criança , Países em Desenvolvimento , Acesso aos Serviços de Saúde , Disparidades em Assistência à Saúde , Humanos , Neoplasias Renais/diagnóstico , Neoplasias Renais/epidemiologia , Neoplasias Renais/terapia , Neoplasias Neuroepiteliomatosas/diagnóstico , Neoplasias Neuroepiteliomatosas/epidemiologia , Neoplasias Neuroepiteliomatosas/terapia , Pediatria , Neoplasias da Retina/diagnóstico , Neoplasias da Retina/epidemiologia , Neoplasias da Retina/terapia , Rabdomiossarcoma/diagnóstico , Rabdomiossarcoma/epidemiologia , Rabdomiossarcoma/terapia , Sarcoma de Ewing/diagnóstico , Sarcoma de Ewing/epidemiologia , Sarcoma de Ewing/terapia , Fatores Socioeconômicos , Tumor de Wilms/diagnóstico , Tumor de Wilms/epidemiologia , Tumor de Wilms/terapia
8.
J Pediatr Hematol Oncol ; 39(2): e62-e68, 2017 03.
Artigo em Inglês | MEDLINE | ID: mdl-28060113

RESUMO

The study was aimed at evaluating adherence to treatment protocol and outcome in pediatric parameningeal rhabdomyosarcoma (PM-RMS). We analyzed the characteristics, treatment administered, outcomes and patterns of failure of pediatric PM-RMS, who were treated with multimodality therapy between January 2005 and December 2013.Univariate and multivariate analysis (MVA) was completed to evaluate the impact of various prognostic factors. Thirty-seven patients were treated at our institution. Majority of them had the primary disease in paranasal sinuses (n=13). Majority of the patients belonged to group III (n=30) and stage III (n=24). The overall response rate to treatment was 52.5% (n=21). At a mean follow-up of 19.1 months, 23 patients developed disease progression. The actuarial rates of failure-free survival and overall survival (OS) at 2 years were 40% and 67.5%, respectively. Patients who received >20 weeks of intended chemotherapy schedule (P=0.02) and had complete response to first-line treatment (P=0.0004) were found to have superior failure-free survival on MVA. Complete response was the lone determinant of superior OS on MVA (P=0.006). Majority of patients with PM-RMS present with advanced stage disease. Response to first-line treatment is a significant predictor of superior progression-free survival and OS in these patients.


Assuntos
Neoplasias de Cabeça e Pescoço/epidemiologia , Cooperação do Paciente , Rabdomiossarcoma/epidemiologia , Adolescente , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Institutos de Câncer/estatística & dados numéricos , Quimiorradioterapia , Criança , Pré-Escolar , Terapia Combinada , Irradiação Craniana , Ciclofosfamida/administração & dosagem , Dactinomicina/administração & dosagem , Intervalo Livre de Doença , Feminino , Neoplasias de Cabeça e Pescoço/patologia , Neoplasias de Cabeça e Pescoço/cirurgia , Neoplasias de Cabeça e Pescoço/terapia , Humanos , Índia/epidemiologia , Lactente , Estimativa de Kaplan-Meier , Masculino , Cuidados Paliativos , Neoplasias dos Seios Paranasais/epidemiologia , Neoplasias dos Seios Paranasais/patologia , Neoplasias dos Seios Paranasais/cirurgia , Neoplasias dos Seios Paranasais/terapia , Prognóstico , Modelos de Riscos Proporcionais , Indução de Remissão , Estudos Retrospectivos , Rabdomiossarcoma/patologia , Rabdomiossarcoma/cirurgia , Rabdomiossarcoma/terapia , Terapia de Salvação , Centros de Atenção Terciária/estatística & dados numéricos , Resultado do Tratamento , Vincristina/administração & dosagem , Adulto Jovem
9.
Am J Emerg Med ; 35(1): 20-24, 2017 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-27765482

RESUMO

OBJECTIVE: To examine clinical characteristics associated with bacteremia in febrile nonneutropenic pediatric oncology patients with central venous catheters (CVCs) in the emergency department (ED). BACKGROUND: Fever is the primary reason pediatric oncology patients present to the ED. The literature states that 0.9% to 39% of febrile nonneutropenic oncology patients are bacteremic, yet few studies have investigated infectious risk factors in this population. METHODS: This was a retrospective cohort study in a pediatric ED, reviewing medical records from 2002 to 2014. Inclusion criteria were patients with cancer, temperature at least 38°C, presence of a CVC, absolute neutrophil count greater than 500 cells/µL, and age less than 22 years. Exclusion criteria were repeat ED visits within 72 hours, bloodwork results not reported by the laboratory, and patients without oncologic history documented at the study hospital. The primary outcome measure is a positive blood culture (+BC). Other variables include age, sex, CVC type, cancer diagnosis, absolute neutrophil count, vital signs, upper respiratory infection (URI) symptoms, and amount of intravenous (IV) normal saline (NS) administered in the ED. Data were analyzed using descriptive statistics and a multiple logistic regression model. RESULTS: A total of 1322 ED visits were sampled, with 534 enrolled, and 39 visits had +BC (7.3%). Variables associated with an increased risk of +BC included the following: absence of URI symptoms (odds ratio [OR], 2.30; 95% CI, 1.13-4.69), neuroblastoma (OR, 3.65; 95% CI, 1.47-9.09), "other" cancer diagnosis (OR, 4.56; 95% CI, 1.93-10.76), tunneled externalized CVC (OR, 5.04; 95% CI, 2.25-11.28), and receiving at least 20 mL/kg IV NS (OR, 2.34; 95% CI, 1.2-4.55). The results of a multiple logistic regression model also showed these variables to be associated with +BC. CONCLUSION: The absence of URI symptoms, presence of an externalized CVC, neuroblastoma or other cancer diagnosis, and receiving at least 20 mL/kg IV NS in the ED are associated with increased risk of bacteremia in nonneutropenic pediatric oncology patients with a CVC.


Assuntos
Bacteriemia/epidemiologia , Cateterismo Venoso Central/métodos , Cateteres Venosos Centrais , Febre/epidemiologia , Hidratação/estatística & dados numéricos , Neuroblastoma/epidemiologia , Neutrófilos , Infecções Respiratórias/epidemiologia , Bacteriemia/sangue , Cateterismo Periférico , Neoplasias do Sistema Nervoso Central/epidemiologia , Criança , Pré-Escolar , Estudos de Coortes , Serviço Hospitalar de Emergência , Feminino , Febre/sangue , Hospitais Pediátricos , Humanos , Leucemia Mieloide Aguda/epidemiologia , Contagem de Leucócitos , Modelos Logísticos , Los Angeles/epidemiologia , Masculino , Análise Multivariada , Neoplasias/epidemiologia , Razão de Chances , Osteossarcoma/epidemiologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/epidemiologia , Infecções Respiratórias/sangue , Estudos Retrospectivos , Rabdomiossarcoma/epidemiologia , Fatores de Risco , Sarcoma de Ewing/epidemiologia
10.
J Pediatr Hematol Oncol ; 39(1): 67-71, 2017 01.
Artigo em Inglês | MEDLINE | ID: mdl-27820124

RESUMO

INTRODUCTION: Breast metastasis is rare in childhood malignancies. Soft tissue sarcomas, especially rhabdomyosarcomas (RMS), and hematologic neoplasms, such as lymphomas, are the most common tumors that metastasize to the breast, albeit rare. MATERIALS AND METHODS: All cases with breast metastasis within a cohort of 200 RMS patients followed in our institution during 1990 to 2014 were assessed retrospectively and the literature was reviewed. RESULTS: There were 3 adolescent female patients with breast metastasis. All had alveolar histology. The primary tumors were in the parameningeal sites, extremities, and the perineum, respectively. Two patients had breast metastasis at diagnosis, and 1 during follow-up. In 1 breast lesion, there was a complete response to chemotherapy, and in another there was no response to chemotherapy, and the patient underwent radical mastectomy. In the third patient, there was partial response, and lesions progressed. All patients died with recurrent/progressive disease, 2 with no recurrence in the breast. In the English literature, there are 70 cases including our cases. All but 1 involve female patients, all adolescents, most have alveolar histology and poor prognosis. All had chemotherapy, whereas some had surgery and/or radiotherapy for local treatment. CONCLUSION: Breast metastasis should be considered in adolescent female patients with RMS. Optimal management is not clear. Besides chemotherapy, mastectomy and radiotherapy should be considered on a case basis.


Assuntos
Neoplasias da Mama/secundário , Rabdomiossarcoma/secundário , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias da Mama/epidemiologia , Neoplasias da Mama/terapia , Criança , Evolução Fatal , Feminino , Humanos , Mastectomia , Prognóstico , Radioterapia Adjuvante , Estudos Retrospectivos , Rabdomiossarcoma/epidemiologia , Rabdomiossarcoma/patologia , Rabdomiossarcoma/terapia , Centros de Atenção Terciária/estatística & dados numéricos , Turquia/epidemiologia
11.
J Pediatr Hematol Oncol ; 39(1): 62-66, 2017 01.
Artigo em Inglês | MEDLINE | ID: mdl-27879537

RESUMO

Soft tissue sarcoma constitutes 8% of all tumors in adolescent and young adults (AYA), with rhabdomyosarcoma (RMS) accounting for 5.2% to 6.5% of the soft tissue sarcoma total within this group. AYAs have a higher propensity for metastasis and inferior outcomes. Metastases to the breast have been reported in ∼3% to 6% of RMS cases. A review of our hospital's tumor registry identified cases of RMS diagnosed between January 1, 2004 and December 31, 2013. A total of 46 patients with RMS were identified, having a mean age of 12.5 years (range, 1 to 49 y). There were 26 males (57%) and 20 females (43%). Eighteen patients (39%) were AYAs, including 10 women. Four patients (8.7%) were identified with breast involvement, all of whom were AYA females. Treatment modalities included chemotherapy, surgical resection, and radiation. One patient is a long-term survivor. Although RMS is uncommon in AYAs, breast involvement occurs almost exclusively in AYA women and is associated with alveolar histology, metastatic disease, and poor outcomes. In total, 4/10 of all AYA females had breast involvement. Routine examination or imaging of the breasts in AYAs with RMS is not currently standard practice at diagnosis or follow-up, but this analysis suggests it should be considered in female AYA patients.


Assuntos
Neoplasias da Mama/secundário , Rabdomiossarcoma/secundário , Adolescente , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biomarcadores Tumorais/análise , Neoplasias da Mama/diagnóstico por imagem , Neoplasias da Mama/epidemiologia , Neoplasias da Mama/terapia , Neoplasias da Mama Masculina/epidemiologia , Neoplasias da Mama Masculina/secundário , Terapia Combinada , Evolução Fatal , Feminino , Humanos , Masculino , Mastectomia , Estudos Retrospectivos , Rabdomiossarcoma/diagnóstico por imagem , Rabdomiossarcoma/epidemiologia , Rabdomiossarcoma/terapia , Terapia de Salvação , Adulto Jovem
12.
Pediatr Blood Cancer ; 64(6)2017 06.
Artigo em Inglês | MEDLINE | ID: mdl-27882658

RESUMO

BACKGROUND: Adolescents with cancer are enrolled in clinical trials at far lower rates than children. This report compares the number of adolescents (15-19-year-olds) and children (0-14-year-olds) enrolled in the protocols of the European pediatric Soft tissue sarcoma Study Group (EpSSG) with the number of cases expected to occur. METHODS: The observed-to-expected (O/E) ratio was detected in the EpSSG countries contributing most of the cases, that is, Italy, France, Spain, the Netherlands, United Kingdom, and Ireland. The observed cases included patients enrolled in any of the EpSSG protocols from October 2008 to October 2015, when all EpSSG protocols were open in these countries. The number of expected cases was calculated from the incidence rates estimated throughout the RARECAREnet database in the countries' population-based cancer registries. RESULTS: In the countries considered, 2,118 cases aged 0-19 years were enrolled in the EpSSG trials from 2008 to 2015: 82.8% were children and 17.2% were adolescents. The O/E ratio was 0.30 among patients 15-19 years old, as opposed to 0.64 for those 0-14 years old. The O/E ratio differed for the different subtypes: in adolescents, it was 0.64 and 0.18 for rhabdomyosarcoma (RMS) and non-rhabdomyosarcoma soft tissue sarcomas (NRSTS), respectively; in children, it was 0.77 and 0.50, respectively. The O/E ratios differed across the countries considered. CONCLUSIONS: Adolescents were less well represented than children on the EpSSG protocols, with better enrolment for RMS than for NRSTS for all age groups.


Assuntos
Acesso aos Serviços de Saúde , Rabdomiossarcoma/epidemiologia , Rabdomiossarcoma/terapia , Adolescente , Adulto , Fatores Etários , Criança , Pré-Escolar , Ensaios Clínicos como Assunto , Europa (Continente)/epidemiologia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos
13.
J Gynecol Obstet Biol Reprod (Paris) ; 45(8): 821-826, 2016 Oct.
Artigo em Francês | MEDLINE | ID: mdl-27212613

RESUMO

Rhabdomyosarcoma, most common soft tissue tumor in children, represent 8% of solid tumors in children. Conversely, in adults, this histology is very rare and no consensual recommendation is supported. If gynecological localization is one of the most frequent in children, it is a minority in adults. The management of this type of tumor is based on treatment multimodality combining surgery, chemotherapy, radiotherapy and brachytherapy. This pathological separate entity differs from other sarcomas by its greater sensitivity to chemotherapy and radiotherapy. The aim of this study is to conduct a general review of diagnostic and treatment of genital tract rhabdomyosarcoma in adults, and to report pathological characteristics of this type of tumor.


Assuntos
Neoplasias dos Genitais Femininos/terapia , Rabdomiossarcoma/terapia , Adulto , Feminino , Neoplasias dos Genitais Femininos/diagnóstico , Neoplasias dos Genitais Femininos/epidemiologia , Humanos , Rabdomiossarcoma/diagnóstico , Rabdomiossarcoma/epidemiologia
14.
J Laryngol Otol ; 130(6): 571-4, 2016 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-27112730

RESUMO

BACKGROUND: Head and neck cancer emergency presentations are uncommon but persistent. However, there is little published literature on this aspect of cancer and patient demographics. This study aimed to assess the incidence, patient profile, tumour site and stage of emergency cancer presentations in our region. METHOD: Retrospective review of regional cancer database over a five-year period. RESULTS: Emergency presentations accounted for 7 per cent of all cases. There was no difference in patient age and risk factors between the emergency and non-emergency presentations. The emergency presentation group showed a greater proportion of female patients compared to the non-emergency presentation group (30 vs 15 per cent). In all emergency presentations, the cancer was at advanced stages. Oropharyngeal cancer was the commonest emergency presentation of cancer, but the third commonest in the non-emergency group. CONCLUSION: Emergency presentations are increasing annually. Female patients and oropharyngeal cancer showed greater representation compared to male patients and laryngeal cancer.


Assuntos
Carcinoma de Células Pequenas/epidemiologia , Carcinoma de Células Escamosas/epidemiologia , Emergências/epidemiologia , Neoplasias de Cabeça e Pescoço/epidemiologia , Melanoma/epidemiologia , Rabdomiossarcoma/epidemiologia , Carcinoma de Células Pequenas/complicações , Carcinoma de Células Pequenas/diagnóstico , Carcinoma de Células Pequenas/patologia , Carcinoma de Células Escamosas/complicações , Carcinoma de Células Escamosas/diagnóstico , Carcinoma de Células Escamosas/patologia , Bases de Dados Factuais , Transtornos de Deglutição/epidemiologia , Transtornos de Deglutição/etiologia , Serviço Hospitalar de Emergência , Feminino , Neoplasias de Cabeça e Pescoço/complicações , Neoplasias de Cabeça e Pescoço/diagnóstico , Neoplasias de Cabeça e Pescoço/patologia , Humanos , Neoplasias Laríngeas/complicações , Neoplasias Laríngeas/diagnóstico , Neoplasias Laríngeas/epidemiologia , Masculino , Melanoma/complicações , Melanoma/diagnóstico , Melanoma/patologia , Neoplasias Bucais/complicações , Neoplasias Bucais/diagnóstico , Neoplasias Bucais/epidemiologia , Estadiamento de Neoplasias , Neoplasias Orofaríngeas/complicações , Neoplasias Orofaríngeas/diagnóstico , Neoplasias Orofaríngeas/epidemiologia , Faringite/epidemiologia , Faringite/etiologia , Prognóstico , Encaminhamento e Consulta , Sons Respiratórios/etiologia , Estudos Retrospectivos , Rabdomiossarcoma/complicações , Rabdomiossarcoma/diagnóstico , Rabdomiossarcoma/patologia , Distribuição por Sexo , Carcinoma de Células Escamosas de Cabeça e Pescoço , Taxa de Sobrevida , Reino Unido/epidemiologia
15.
Ann Epidemiol ; 26(2): 141-145, 2016 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-26795698

RESUMO

PURPOSE: The purpose of the study was to examine whether birth characteristics affect the risk of rhabdomyosarcoma (RMS) in children and adolescents younger than 19 years. METHODS: A total of 722 RMS cases diagnosed at the age of 0-19 years during 1988-2011 were identified from the California Cancer Registry and matched by birth date, sex, and race to 2,888 controls using California birth records. Conditional logistic regression was used to estimate the risk of RMS associated with birth weight, gestational age, and size for gestational age. RESULTS: High birth weight (odds ratio [OR]: 1.00; 95% confidence interval [CI]: 0.78-1.29) and large for gestational age (LGA; OR: 0.94, 95% CI: 0.72-1.23) were not associated with RMS risk overall. Among non-Hispanic whites, the ORs were 1.33 for high birth weight (95% CI: 0.94-1.89) and 1.17 for LGA (95% CI: 0.78-1.75); no indications of association were observed for other racial or ethnic groups (P interaction <.10). Compared with normal gestational age, preterm (<37 weeks) and post-term (>40 weeks) babies had 16%-18% lower risks of RMS overall, after adjusting for birth weight. CONCLUSIONS: In the largest study to date, there was an indication of association between high birth weight, LGA, and increased RMS risk among non-Hispanic white children and adolescents, but not in other racial or ethnic groups.


Assuntos
Peso ao Nascer , Desenvolvimento Fetal , Idade Gestacional , Rabdomiossarcoma/epidemiologia , Adolescente , California/epidemiologia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Rabdomiossarcoma/etnologia , Adulto Jovem
16.
Acta Paediatr ; 105(1): 74-81, 2016 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-26331464

RESUMO

AIM: Positive outcomes for paediatric rhabdomyosarcoma (RMS) were high in Sweden during the 1990s, but the last decade has seen decreasing trends in overall survival rates. We investigated the incidence, patient and disease characteristics, treatment and outcome of RMS to see whether any reason could be found for this decline. METHODS: This study included 210 children under the age of 15 who were diagnosed with RMS and whose details were recorded in the population-based Swedish Childhood Cancer Registry from 1984 to 2010. RESULTS: The overall annual incidence of RMS was 4.9 per million, and the 5-year overall survival rates were 59 ± 7% in 1984-1989, 78 ± 5% in 1990-1999 and 71 ± 5% in 2000-2010. When patients with localised disease were analysed separately, there was no difference in the 5-year survival rates between 1990 and 1999 (82 ± 5%) and 2000-2010 (81 ± 5%), but the outcome in 1984-1989 (53 ± 8%) was significantly worse. The prevalence of metastatic disease was unexpectedly high during 2000-2010 (28%, p = 0.010), compared to an overall mean of 18% for the whole study period. CONCLUSION: Our results suggest that a higher rate of metastatic disease may explain the declining trend in overall survival rates in paediatric RMS in Sweden over the last decade.


Assuntos
Metástase Neoplásica , Rabdomiossarcoma/mortalidade , Adolescente , Criança , Pré-Escolar , Terapia Combinada , Feminino , Seguimentos , Humanos , Incidência , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Masculino , Sistema de Registros , Rabdomiossarcoma/epidemiologia , Rabdomiossarcoma/secundário , Rabdomiossarcoma/terapia , Taxa de Sobrevida , Suécia/epidemiologia
17.
BMC Res Notes ; 8: 474, 2015 Sep 24.
Artigo em Inglês | MEDLINE | ID: mdl-26404043

RESUMO

BACKGROUND: Childhood cancer becomes a public health problem in developing countries which aggravates the burden of childhood mortality by infectious diseases and malnutrition. In poor countries, the death rate for most pediatric cancers is almost 100%. This study attempts to determine the magnitude, patterns and trends of pediatric malignancies in the study area which is important in re-evaluating existing services and in improving facilities and patient care. METHODS: A retrospective study of 3 year period were carried out among all children aged below 15 years old admitted into the pediatric wards of Gondar University Hospital, Northwest Ethiopia. The charts of all children aged below 15 years old admitted in the pediatric wards due to cancer were reviewed by using the data collection format. Data were entered and analyzed using SPSS version 20 statistical package. RESULT: A total of 71 cancer cases were diagnosed and admitted to the pediatrics ward during the study period. More than two-third of the study subjects 50 (70.4%) were males. The mean age of study subjects was 7 ± 4 year where majority 26 (36.6%) of the study subjects were ≥10 years. Of all, 43 (60.6%) were hematological malignancy followed by Wilms tumor 13 (18.3%), Neuroblastoma 5 (7%), Rhabdomyosarcoma 3 (4.2%), Brain tumor 3 (4.2%), Hepatoblastoma 2 (2.8%). More than two-third of cases were found to be concomitantly malnourished being stunted, wasted and under weight. Nearly half of patients had not received chemotherapy and majority of those started chemotherapy did not complete all the treatment cycles. Shortage and absence of safe and affordable chemotherapy drugs were the major reasons for therapy interruption. CONCLUSION: The study shows increasing childhood cancer cases over the years. Hematological malignancy takes the leading prevalence followed by Wilms tumor and Neuroblastoma. The majority of cases were also discharged without any clinical change that had the only death option. Therefore, the government and the hospital should give emphasis to establish cancer therapy centers and insure accessibility and affordability of chemotherapy drugs.


Assuntos
Transtornos da Nutrição Infantil/epidemiologia , Neoplasias Hematológicas/epidemiologia , Hepatoblastoma/epidemiologia , Neuroblastoma/epidemiologia , Rabdomiossarcoma/epidemiologia , Tumor de Wilms/epidemiologia , Adolescente , Antineoplásicos/economia , Antineoplásicos/uso terapêutico , Criança , Transtornos da Nutrição Infantil/mortalidade , Transtornos da Nutrição Infantil/patologia , Pré-Escolar , Etiópia/epidemiologia , Feminino , Neoplasias Hematológicas/tratamento farmacológico , Neoplasias Hematológicas/mortalidade , Neoplasias Hematológicas/patologia , Hepatoblastoma/tratamento farmacológico , Hepatoblastoma/mortalidade , Hepatoblastoma/patologia , Hospitais Universitários , Humanos , Lactente , Masculino , Neuroblastoma/tratamento farmacológico , Neuroblastoma/mortalidade , Neuroblastoma/patologia , Pobreza , Prevalência , Estudos Retrospectivos , Rabdomiossarcoma/tratamento farmacológico , Rabdomiossarcoma/mortalidade , Rabdomiossarcoma/patologia , Análise de Sobrevida , Tumor de Wilms/tratamento farmacológico , Tumor de Wilms/mortalidade , Tumor de Wilms/patologia
18.
Niger J Med ; 24(1): 32-6, 2015 Jan-Mar.
Artigo em Inglês | MEDLINE | ID: mdl-25807671

RESUMO

BACKGROUND: Rhabdomyosarcoma is the most common soft tissue sarcoma in children ≤ 15 years of age. There is, however, a paucity of reports on the pattern of its occurrence in Nigeria and other parts of Africa. OBJECTIVE: The aim of this review is to highlight the age and sex distribution, anatomical location and morphological characteristics of rhabdomyosarcoma among children in Kano, northwestern Nigeria. PATIENTS AND METHOD: This is a 14 year retrospective study of all cases of rhabdomyosarcomas occurring in children diagnosed at the Pathology Department of Aminu Kano Teaching Hospital, Kano, Nigeria. Clinical data obtained included sex, age, tumour site and histologic subtype. RESULTS: Fifty two children were seen with a male to female ratio of 1 : 1.3. The ages of these Patients ranged from 4 months to 15 years with a mean (SD) age of 6.99 (4.1) years and peak age frequency in the 0 to 5 years age group. The embryonal variant was the most common histological subtype (69%). The others were alveolar (27%) and pleomorphic (4%) variants. The head and neck was the most affected region (44%) and the most common primary site was the orbit (19.2%). CONCLUSION: Rhabdomyosarcoma is the most common childhood soft tissue sarcoma in Kano. It occurs in females more frequently than males and the most common histologic subtype is embryonal rhabdomyosarcoma affecting predominantly the orbit.


Assuntos
Neoplasias de Cabeça e Pescoço/epidemiologia , Rabdomiossarcoma/epidemiologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Nigéria/epidemiologia , Neoplasias Orbitárias/epidemiologia , Estudos Retrospectivos , Rabdomiossarcoma Alveolar/epidemiologia , Rabdomiossarcoma Embrionário/epidemiologia
19.
Cancer Med ; 4(5): 781-90, 2015 May.
Artigo em Inglês | MEDLINE | ID: mdl-25809884

RESUMO

Relatively little is known about the epidemiology and factors underlying susceptibility to childhood rhabdomyosarcoma (RMS). To better characterize genetic susceptibility to childhood RMS, we evaluated the role of family history of cancer using data from the largest case-control study of RMS and the Utah Population Database (UPDB). RMS cases (n = 322) were obtained from the Children's Oncology Group (COG). Population-based controls (n = 322) were pair-matched to cases on race, sex, and age. Conditional logistic regression was used to evaluate the association between family history of cancer and childhood RMS. The results were validated using the UPDB, from which 130 RMS cases were identified and matched to controls (n = 1300) on sex and year of birth. The results were combined to generate summary odds ratios (OR(s) ) and 95% confidence intervals (CI). Having a first-degree relative with a cancer history was more common in RMS cases than controls (OR(s) = 1.39, 95% CI: 0.97-1.98). Notably, this association was stronger among those with embryonal RMS (OR(s) = 2.44, 95% CI: 1.54-3.86). Moreover, having a first-degree relative who was younger at diagnosis of cancer (<30 years) was associated with a greater risk of RMS (OR(s) = 2.37, 95% CI: 1.34-4.18). In the largest analysis of its kind, we found that most children diagnosed with RMS did not have a family history of cancer. However, our results indicate an increased risk of RMS (particularly embryonal RMS) in children who have a first-degree relative with cancer, and among those whose relatives were diagnosed with cancer at <30 years of age.


Assuntos
Família , Predisposição Genética para Doença , Rabdomiossarcoma/epidemiologia , Rabdomiossarcoma/etiologia , Adolescente , Fatores Etários , Estudos de Casos e Controles , Criança , Pré-Escolar , Bases de Dados Factuais , Feminino , Humanos , Masculino , Razão de Chances , Vigilância da População , Fatores de Risco , Utah/epidemiologia
20.
Asian Pac J Cancer Prev ; 16(2): 757-60, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-25684521

RESUMO

AIM: To present the epidemiologic data (age, gender, size etc) and histopathologic and immunohistochemical features of cases of rhabdomyosarcoma (RMS) reported in our department. SETTINGS: Section of Histopathology, Department of Pathology and Microbiology, Aga Khan University Hospital, Karachi, Pakistan. DURATION: All cases of RMS diagnosed between January 1, 2003 and December 31, 2012 were included in the study. RESULTS: A total of 277 cases were included. Embryonal rhabdomyosarcoma (ERMS) was by far the dominant histologic type (87.4%) followed by alveolar type (ARMS)(9.4%). ERMS was much more common in males (64.0%)and over 65% of cases occurred in the first decade of life (over 90% in the first two decades). Head and neck region was commonest site for ERMS (46.7%), followed by the genitourinary system (16.1%). Over 65% cases of ARMS occurred in the extremities. Over 80% cases of ARMS occurred in the first 3 decades of life. Immunohistochemical staining for Desmin and MyoD1 was positive in 96.7% and 85.4% respectively. CONCLUSIONS: The epidemiologic data and microscopic findings in our patients are similar to international published data on rhabdomyosarcoma.


Assuntos
Rabdomiossarcoma/epidemiologia , Rabdomiossarcoma/patologia , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Estudos Epidemiológicos , Feminino , Seguimentos , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Paquistão/epidemiologia , Prognóstico , Rabdomiossarcoma/classificação , Centros de Atenção Terciária , Adulto Jovem
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA