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OBJECTIVES: The aim of the study was to evaluate the long-term outcome and late effects in pediatric patients with nasopharyngeal carcinoma (NPC) treated with neoadjuvant chemotherapy (NACT), followed by radiotherapy (RT). METHODS: Ninety-two children (65 male, 27 female) diagnosed with NPC between 1989 and 2017 in the Istanbul University, Institute of Oncology were evaluated retrospectively. NACT consisted of three cycles of cisplatin-containing regimen every 3 weeks, followed by RT. RESULTS: The median age was 13 years (5-18 years). Most had locoregionally advanced disease (stage III/IVA/IVB) and five had distant metastases at presentation. At a median follow-up of 108 months (3-332 months), 5- and 10-year overall survival rates and event-free survival rates were 87.5%, 79.7% and 82.1%, 78.9%, respectively. Three patients with distant metastasis are long-term survivors. Thirteen patients relapsed at a median of 8 months (2-23 months). Hypothyroidism (36%) and xerostomia (25%) were the most frequent long-term treatment-related toxicities. Nine second malignancies developed in eight patients, eight in the irradiated field at a median of 14 years (range 5-26 years), five of whom are long-term survivors after curative surgery. CONCLUSIONS: Three courses of cisplatin-containing NACT, followed by RT lead to high survival and locoregional control rate in advanced stage NPC in children. Patients with distant metastasis should also be treated with curative intent by systemic chemotherapy and locoregional radiotherapy. Patients should be followed closely for recurrences and long-term morbidities including second malignancies, which may be treated with curative surgeries if diagnosed early.
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Neoplasias Nasofaríngeas , Neoplasias Primarias Secundarias , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Quimioradioterapia/efectos adversos , Niño , Cisplatino , Terapia Combinada , Supervivencia sin Enfermedad , Femenino , Humanos , Masculino , Carcinoma Nasofaríngeo/tratamiento farmacológico , Neoplasias Nasofaríngeas/patología , Estadificación de Neoplasias , Neoplasias Primarias Secundarias/etiología , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
BACKGROUND: The prognosis of children with diffuse intrinsic pontine gliomas (DIPG) is dismal. This study aims to evaluate the characteristics and treatment outcome of children with DIPG in a single center. METHODS: We reviewed the outcome of children with DIPG treated at the Oncology Institute of Istanbul University from February 1999 to May 2012. RESULTS: Fifty children (26 female, 24 male) with the median age of 7 years were analyzed. The median duration of symptoms was 30 days. All patients received radiotherapy (RT). Before the year 2000, 12 patients received only RT. Thirty-eight had concomitant and/or adjuvant chemotherapy with RT. Between 2000 and 2004, 17 patients received cis-platinum or vincristine as sensitizers during RT and CCNU + vincristine combination after RT. Since 2004, 21 patients received temozolomide (TMZ) concomitantly during RT and as adjuvant chemotherapy after RT. The median survival time of all patients was 13 months (1-160 months). Patients receiving RT + TMZ had a significantly higher overall survival than patients with only RT (p = 0.018). Patients receiving RT + chemotherapy other than TMZ also had a significantly higher overall survival than patients receiving only RT (p = 0.013). Patients receiving RT + TMZ + and chemotherapy other than TMZ had a significantly higher survival than patients receiving only RT (p = 0.005). CONCLUSION: In our series, patients receiving RT + TMZ and also patients receiving RT + chemotherapy other than TMZ had a significantly higher overall survival than patients treated with only RT. Hence, administering chemotherapy during and after RT seems to prolong survival in some DIPG patients.
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Antineoplásicos Fitogénicos/uso terapéutico , Neoplasias del Tronco Encefálico/terapia , Glioma/terapia , Puente/patología , Vincristina/uso terapéutico , Adolescente , Neoplasias del Tronco Encefálico/tratamiento farmacológico , Neoplasias del Tronco Encefálico/patología , Neoplasias del Tronco Encefálico/radioterapia , Quimioterapia Adyuvante , Niño , Preescolar , Terapia Combinada , Femenino , Glioma/tratamiento farmacológico , Glioma/patología , Glioma/radioterapia , Humanos , Lactante , Masculino , Pronóstico , Tasa de Supervivencia , Resultado del TratamientoRESUMEN
Neuroblastoma (NBL), a malignant embryonic tumor derived from neural crest cells, is the most common tumor worldwide among children less than 1 year of age. Metastasis to the mandible is uncommon. This article reports the case of a 15-month-old male diagnosed with NBL with bone metastasis including the mandible which resulted in severe tooth mobility. Dentists or pediatricians should consider the primary or metastatic tumors of the maxillofacial region in the differential diagnosis in children presenting with premature loss of teeth related to tooth mobility.
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Neoplasias de las Glándulas Suprarrenales/patología , Neoplasias Mandibulares/complicaciones , Neoplasias Mandibulares/secundario , Neuroblastoma/secundario , Movilidad Dentaria/etiología , Humanos , Lactante , MasculinoRESUMEN
AIM: To standardize diagnosis and treatment of childhood Wilms tumor (WT) in Turkey. METHODS AND PATIENTS: Between 1998 and 2006, WT patients were registered from 19 centers. Patients <16 years with unilateral WT whose treatment started in first postoperative 3 weeks were included. Treatments were stage I favorable (FH) and unfavorable histology (UH) patients, VCR + Act-D; stage IIA FH, VCR + Act-D; stage IIB FH, VCR + Act-D + radiotherapy (RT); stage III-IV FH, VCR + Act-D + adriamycin (ADR) + RT; stages II-IV UH tumors, VCR + Act-D + ADR + etoposide + RT. RESULTS: 165/254 registered cases were eligible (bilateral, 5.9%) [median age 3.0 years; M/F: 0.99; 50/165 cases < or =2 years]. 9.7% cases had UH tumors. Disease stages were stage I 23.6%; IIA 36.4%; IIB 5.5%; III 22.4%; IV 12.1%. Cases >2 years had significantly more advanced disease. 1/11 cases with recurrent disease died; 2/165 had progressive disease, 2/165 had secondary cancers, and all 4 died. In all cases 4-year OS and EFS were 92.8 and 86.5%, respectively. Both OS and EFS were significantly worse in stage IV. CONCLUSIONS: Despite problems in patient management and follow-up, treatment results were encouraging in this first national experience with a multicentric study in pediatric oncology. Revisions and modifications are planned to further improve results and minimize short- and long-term side effects.
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Neoplasias Renales/terapia , Tumor de Wilms/terapia , Adolescente , Niño , Preescolar , Terapia Combinada , Femenino , Humanos , Lactante , Recién Nacido , Neoplasias Renales/mortalidad , Masculino , Tumor de Wilms/mortalidadRESUMEN
Conjunctival papilloma is a benign tumor of the conjunctival mucosa. In childhood, papilloma represents 7-10% of conjunctival tumors. Human papillomavirus (HPV)-6 and HPV-11 are the major HPV types responsible for conjunctival lesions. A five-year-old boy with a two-year history of conjunctival papilloma caused by HPV type 11 treated with systemic interferon alpha is reported and the literature is reviewed.
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Antivirales/uso terapéutico , Neoplasias de la Conjuntiva/virología , Papillomavirus Humano 11 , Interferón-alfa/uso terapéutico , Papiloma/virología , Infecciones por Papillomavirus/virología , Preescolar , Neoplasias de la Conjuntiva/patología , Papillomavirus Humano 11/aislamiento & purificación , Humanos , Masculino , Papiloma/patología , Infecciones por Papillomavirus/patologíaRESUMEN
Between January 1990 and December 2006, 123 patients
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Neoplasias Óseas/terapia , Osteosarcoma/terapia , Adolescente , Neoplasias Óseas/mortalidad , Niño , Preescolar , Terapia Combinada , Femenino , Humanos , Masculino , Osteosarcoma/mortalidad , PronósticoRESUMEN
OBJECTIVES: Despite the developments in chemotherapy protocols, improvement in the survival rates of osteosarcoma has been limited. We evaluated the effect of certain prognosis-related proteins on survival of patients with osteosarcoma. METHODS: Data from 45 patients (24 males, 21 females) who were treated and followed-up for osteosarcoma were reviewed. Following neoadjuvant chemotherapy, 41 patients underwent extremity saving surgery, and four patients underwent amputation. The most frequent localization was the lower end of the femur (n=23, 51.1%), followed by the upper end of the tibia (n=10, 22.2%). Three patients had metastasis on admission. Surgical resection samples were retrieved from the pathology archive and analyzed immunohistochemically for the expression of p-glycoprotein p170, p53, heat-shock protein 27 (HSP27), HSP90, and nm23. The effect of these proteins on prognosis and survival was assessed with survival analysis using the Kaplan-Meier method. The mean follow-up was 49.7 months (range 6 to 185 months). RESULTS: Three patients with metastasis on admission died within five years due to pulmonary metastasis. New metastases developed in 29 patients. Total 5-year and 10-year survival rates were 60% and 43%, respectively. The corresponding disease-free survival rates were 41% and 24%. Five-year survival was 29% in patients who developed metastasis. Among clinical factors, survival was influenced only by the presence of metastasis on admission (p=0.044). Five-year and 10-year survival rates were significantly different between patients with and without p53 positivity (p=0.04), while the other proteins were not significantly associated with survival. CONCLUSION: Our data suggest that p53 may be used as a prognostic marker in osteosarcoma due to its significant association with survival.
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Neoplasias Óseas/mortalidad , Resistencia a Antineoplásicos , Inmunohistoquímica/métodos , Osteosarcoma/mortalidad , Proteína p53 Supresora de Tumor/metabolismo , Subfamilia B de Transportador de Casetes de Unión a ATP/metabolismo , Adolescente , Adulto , Biomarcadores de Tumor/metabolismo , Neoplasias Óseas/tratamiento farmacológico , Neoplasias Óseas/terapia , Niño , Terapia Combinada , Supervivencia sin Enfermedad , Femenino , Estudios de Seguimiento , Proteínas de Choque Térmico HSP27/metabolismo , Proteínas HSP90 de Choque Térmico/metabolismo , Humanos , Estimación de Kaplan-Meier , Masculino , Persona de Mediana Edad , Metástasis de la Neoplasia , Osteosarcoma/tratamiento farmacológico , Osteosarcoma/terapia , Pronóstico , Factores de Tiempo , Adulto JovenRESUMEN
OBJECTIVES: We evaluated long-term treatment results of patients with primary osteosarcoma and the effect of prognostic factors on overall survival and disease-free survival. METHODS: Between 1995 and 2005, 180 patients (111 males, 69 females; mean age 21+/-10 years; range 7 to 64 years) were treated for primary osteosarcoma. Overall and disease-free survival rates were analyzed for 165 patients with high-grade osteosarcoma with the Kaplan-Meier method. The effects of potential prognostic factors were assessed, including age, gender, localization, tumor size, primary metastasis on presentation, the presence of pathologic fractures, necrosis rate, and infection. All the patients received chemotherapy before and after surgery. The mean follow-up period was 49.7 months (range 6 to 185) months. RESULTS: Sixty-nine patients were below 16 years of age. The most frequent involvement was in the distal femur (47.2%), followed by the proximal tibia (25%). Sixteen patients presented with a pathologic fracture, and 12 patients with metastasis. The median tumor size was 10 cm. The overall five- and 10-year survival rates were 68% and 60%, and disease-free survival rates were 50% and 44%, respectively. Only the presence of a pathologic fracture and primary metastasis on presentation were found to affect prognosis. CONCLUSION: The two conditions, primary metastasis and a pathologic fracture, found as the most important prognostic factors in our study are mainly associated with late presentation. As in every malignant disease, early admission would provide better survival rates.
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Neoplasias Óseas/mortalidad , Osteosarcoma/mortalidad , Adolescente , Adulto , Neoplasias Óseas/diagnóstico por imagen , Neoplasias Óseas/patología , Neoplasias Óseas/terapia , Niño , Preescolar , Terapia Combinada , Supervivencia sin Enfermedad , Femenino , Humanos , Masculino , Persona de Mediana Edad , Metástasis de la Neoplasia , Osteosarcoma/diagnóstico por imagen , Osteosarcoma/patología , Osteosarcoma/terapia , Pronóstico , Radiografía , Análisis de Supervivencia , Turquía/epidemiologíaRESUMEN
Thrombocytopenia is a frequent finding in patients with solid tumors. It is usually caused by bone marrow infiltration or by myelosuppression due to anticancer therapy; however immune thrombocytopenia (ITP) associated with solid tumors is rare. Neuroblastoma is the most common extracranial solid tumor in children. Here we report the case of a two-year-nine-month-old patient with adrenal neuroblastoma who presented with ITP. Paraneoplastic ITP was considered in the differential diagnosis. Bone marrow infiltration and other causes of thrombocytopenia were excluded and the patient was treated with intravenous immunoglobulin and tumor resection. Platelet count increased rapidly after surgery and complete remission of ITP was achieved.
RESUMEN
BACKGROUND: Hypertrophic osteoarthropathy (HOA) is a syndrome characterized by clubbing of the fingers and toes, periosteal new bone formation of the long bones and polyarthritis. CASE REPORT: In this report, two children with intrathoracic Hodgkin's disease and HOA are presented. CONCLUSIONS: Intrathoracic neoplasms are one of the major causes of HOA in adults; however HOA is rarely associated with intrathoracic malignancies in children. HOA associated with intrathoracic Hodgkin's disease is even more rare, but should be kept in mind.
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Enfermedad de Hodgkin/complicaciones , Osteoartropatía Hipertrófica Primaria/complicaciones , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/administración & dosificación , Biopsia con Aguja Fina , Niño , Enfermedad de Hodgkin/diagnóstico , Enfermedad de Hodgkin/terapia , Humanos , Inyecciones Intravenosas , Masculino , Osteoartropatía Hipertrófica Primaria/diagnóstico , Osteoartropatía Hipertrófica Primaria/terapia , Síndromes Paraneoplásicos , Resultado del TratamientoRESUMEN
AIMS: There is a subgroup of patients with Wilms' tumour (WT) having favourable clinicopathological features but adverse outcome. We aimed to investigate the prognostic significance of angiogenesis and whether it can be used for predicting which patients will fall into this category, and the possible role of vascular endothelial growth factor (VEGF) on angiogenesis in WT. METHODS: Tumours in nephrectomy specimens from 63 WT patients were investigated for neovascularisation and VEGF expression by immunohistochemistry. The endothelial cells were highlighted by anti-CD34 and anti-CD31, and the microvessels in the hot-spots were counted. Correlations between the microvessel density (MVD), VEGF expression, clinicopathological features and prognosis were studied. RESULTS: Among 21 patients with follow-up data, favourable histology was detected in 17, seven of which died of disease. Patients with highly vascular tumours showed significantly poorer prognosis than those with low vascular tumours. There was no significant relationship between angiogenesis and VEGF expression. VEGF immunostaining revealed various patterns in different components of WT. CONCLUSIONS: We suggest that high MVD can be used as an indicator of poor prognosis with WT patients displaying favourable histology and there might be some additional growth factors other than VEGF which may also be responsible for angiogenesis in WTs.
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Neoplasias Renales/metabolismo , Neovascularización Patológica/metabolismo , Factor A de Crecimiento Endotelial Vascular/metabolismo , Tumor de Wilms/metabolismo , Antígenos CD34/metabolismo , Niño , Preescolar , Femenino , Humanos , Técnicas para Inmunoenzimas , Lactante , Riñón/irrigación sanguínea , Neoplasias Renales/irrigación sanguínea , Neoplasias Renales/mortalidad , Masculino , Microcirculación/patología , Nefrectomía , Molécula-1 de Adhesión Celular Endotelial de Plaqueta/metabolismo , Pronóstico , Tasa de Supervivencia , Tumor de Wilms/irrigación sanguínea , Tumor de Wilms/mortalidadRESUMEN
BACKGROUND: Clear cell sarcoma of the kidney (CCSK) in adults is extremely rare. Optimal treatment of adult patients with CCSK remains unclear. CASE PRESENTATION: A 22-year-old man presented with a 2-month history of left flank pain. A color duplex sonography revealed a hypervascular, heterogeneous renal mass. Abdominal and pelvic computerized tomography showed a heterogeneous mass originating from the lower pole of the left kidney and infiltrating to the psoas muscle. Further evaluation including bone scan did not demonstrate any evidence of metastases. A left radical nephrectomy with hilar lymphadenectomy through an intraperitoneal approach with an anterior subcostal incision was performed. The histopathological diagnosis of the mass was a clear cell sarcoma of the kidney. No lymph node metastases were found. Concomitant chemo-radiotherapy was performed. Therapy-related serious side effects were not observed. There was no evidence of local recurrence or metastases during the following twenty-four months after therapy. CONCLUSION: We believe that the combination therapy is efficacious for preventing the local recurrence and distant metastases. Accurate diagnosis is very important and therapy must also include doxorubicin regardless of the disease stage in adult patients with CCSK.
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Neoplasias Renales/terapia , Sarcoma de Células Claras/terapia , Adulto , Terapia Combinada , Humanos , MasculinoRESUMEN
OBJECTIVES: We evaluated pediatric patients who underwent surgery for Langerhans cell histiocytosis with mono- or polyostatic involvement and an extensive soft tissue component and/or a high risk for pathologic fractures. METHODS: The study included 13 children (6 boys, 7 girls; mean age 6 years; range 2 to 11 years) who were treated surgically for bone and soft tissue involvement of Langerhans cell histiocytosis with a high risk for fractures of the long bones and the pelvis. Eleven patients had only skeletal involvement while three patients had multisystem involvement. There were 17 bone lesions in the following localizations: acetabulum (n=7), femur neck and subtrochanteric region-proximal femur (n=5), and proximal (n=2) and distal (n=2) humerus. One patient had multifocal bone involvement. The risk for pathologic fractures was assessed using the scoring system of Mirels. Treatment consisted of surgery followed by chemotherapy. The results of treatment were evaluated according to the criteria by the Study Group of Langerhans Cell Histiocytosis. The mean follow-up after orthopedic surgery was 67 months (range 34 to 172 months). RESULTS: Only one patient developed recurrence, in which case bone marrow involvement responded well to chemotherapy. Radiographically, graft consolidation was observed in all the patients in the third postoperative month. No bone sequelae such as shortness and deformity were detected throughout the follow-up period. CONCLUSION: Surgical treatment combined with chemotherapy is effective in the treatment of aggressive histiocytic bone lesions associated with extensive soft tissue involvement and high risk for fractures.
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Huesos/cirugía , Histiocitosis de Células de Langerhans/complicaciones , Enfermedades Óseas/complicaciones , Enfermedades Óseas/diagnóstico por imagen , Enfermedades Óseas/patología , Enfermedades Óseas/cirugía , Huesos/patología , Niño , Protección a la Infancia , Preescolar , Terapia Combinada , Femenino , Humanos , Masculino , Espasticidad Muscular/complicaciones , Espasticidad Muscular/diagnóstico por imagen , Espasticidad Muscular/patología , Espasticidad Muscular/cirugía , Radiografía , Recurrencia , Índice de Severidad de la Enfermedad , Resultado del TratamientoRESUMEN
Nasopharyngeal carcinoma (NPC) is a characteristic tumor displaying epidemiological, genetic and regional distribution properties and is unique by its natural behavior and therapy. Investigation of the molecular and biological changes, gene amplifications and activations that occur during carcinogenesis and progression can provide new insight into the pathology of the disease and may add biological factors that can be used as new prognostic markers. The p53 tumor suppressor gene is the most frequently mutated gene in human cancer. Although point mutations in the p53 gene are observed in nasopharyngeal cancer, the mutation rate is lower than in other tumors. Immunohistochemical studies have shown significant p53 overexpression in NPC material. In this study, p53 protein immunoreactivity was investigated in paraffin sections of primary nasopharyngeal tumors and metastatic cervical lymph nodes and association with clinical and histopathological characteristics was evaluated. Ninety-seven paraffin sections from 81 patients with NPC treated from 1990 to 1996 were examined by immunohistochemistry and were correlated with clinical features and treatment outcome. Among a total of 97 samples, positive staining for p53 protein was observed in 83 (85.5%) samples while no staining was found in 14 (14.5%) cases. Immunoreactivity was observed in 62 (81.5%) of the primary nasopharyngeal biopsy specimens. The correlation between p53 expression and histological type, stage, age and sex distributions was tested. After statistical analysis according to Chi-square test and Yates' correction, no significant difference was demonstrated (p>0.05). There was no statistically significant correlation with p53 immunoreactivity and overall and disease-free survival. Although the association between NPC and p53 is not clear, our study confirms that p53 overexpression is present in a considerable subset of patients with NPC.
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Carcinoma/metabolismo , Neoplasias Nasofaríngeas/metabolismo , Proteína p53 Supresora de Tumor/metabolismo , Adolescente , Adulto , Anciano , Biomarcadores de Tumor , Carcinoma/mortalidad , Carcinoma/secundario , Núcleo Celular/metabolismo , Núcleo Celular/patología , Niño , Femenino , Humanos , Técnicas para Inmunoenzimas , Ganglios Linfáticos/metabolismo , Ganglios Linfáticos/patología , Metástasis Linfática , Masculino , Persona de Mediana Edad , Neoplasias Nasofaríngeas/mortalidad , Neoplasias Nasofaríngeas/patología , Estadificación de Neoplasias , Tasa de Supervivencia , Proteína p53 Supresora de Tumor/genéticaRESUMEN
OBJECTIVE: To evaluate the clinicopathologic prognostic factors in malignant ovarian germ cell tumors. METHODS: We reviewed the medical records of 70 patients treated from 1990 to 2006 at our center. Clinical data including demographics, stage, surgery, chemotherapy, survival, menses status, and fertility were collected from patients' charts. RESULTS: Median age was 22 years (range, 9-68). The histologic subtypes included 36 dysgerminomas, 11 yolk sac tumors, 3 immature teratomas, 1 embryonal carcinomas, and 19 mixed types. The most striking clinicopathologic finding was a history of concomitant immunosuppressant therapy, which was observed in 2 patients. Two patients had contralateral sex-cord tumors at presentation and follow-up. During a median follow-up period of 4.6 years, 11 patients had recurrence. The median time to recurrence was 8 months (6-28 months). Recurrences appeared in the abdominopelvic cavity in 9 out of 11 patients. Only one could be salvaged with second-line chemotherapy. Cumulative survival rate was 97% and 60% in patients with dysgerminoma and nondysgerminoma, respectively. Nondysgerminoma histology and residual tumor after surgery were unfavorable prognostic factors (P < 0.001 and P = 0.015). Fertility-sparing surgery was as effective as radical surgery among all eligible patients. Of patients with known menstrual status, 96% had regular menses. Of the 8 patients who opted for conception among these patients, 7 delivered healthy infants. CONCLUSIONS: Nondysgerminomas have an aggressive clinical course. New treatment strategies are needed for eradication of abdominopelvic disease at initial diagnosis and recurrent setting. Occurrence of malignant ovarian germ cell tumors may be associated with immunosuppression in some patients. Sex-cord stromal tumors may present with bilateral involvement. It is possible to maintain fertility after fertility-sparing surgery followed by chemotherapy.
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Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias de Células Germinales y Embrionarias/patología , Neoplasias Ováricas/patología , Adolescente , Adulto , Anciano , Niño , Femenino , Estudios de Seguimiento , Humanos , Registros Médicos , Persona de Mediana Edad , Estadificación de Neoplasias , Neoplasias de Células Germinales y Embrionarias/tratamiento farmacológico , Neoplasias de Células Germinales y Embrionarias/cirugía , Neoplasias Ováricas/tratamiento farmacológico , Neoplasias Ováricas/cirugía , Pronóstico , Tasa de Supervivencia , Resultado del Tratamiento , Adulto JovenRESUMEN
OBJECTIVE: To evaluate the clinicopathological prognostic features, factors and outcomes of chemotherapy in ovarian yolk sac tumours (YST). STUDY DESIGN: We reviewed the medical records of 32 women with ovarian YST treated from 1990 to 2006 at two centres. RESULTS: The median follow-up was 36 months. The median age was 22 (range, 9-68). Two patients were postmenopausal. The most common symptoms at diagnosis included abdominal swelling or mass (72%) and abdominopelvic pain (62%). The location of the tumour was bilateral in 2 cases. Eight patients were in stage I, 4 patients in stage II, 17 patients in stage III, and 3 patients in stage IV. Eighteen patients underwent unilateral salpingo-oophorectomy, two bilateral salpingo-oophorectomy and two cystectomy, while 10 patients had total abdominal hysterectomy and two bilateral salpingo-oophorectomy. Of 32 patients who received postoperative chemotherapy, 27 were treated with a bleomycin/etoposide/cisplatin (BEP) regimen. Seventy-two percent of patients were alive at the last follow-up visit. Ten (31%) patients suffered from a recurrence of the disease with a median time to recurrence of 8 months (range, 6-28 months). The most common site of recurrence was the intra-abdominal space, with 8 patients. Only one patient who had recurrence could be salvaged. Fertility-sparing surgery was found at least as effective as radical surgery. While age, histology (mixed vs. pure), stage, tumour size, ascites, and marker levels were not found as prognostic factors, the presence of residual tumour (P=0.014) and BEP chemotherapy (P=0.016) were significant prognostic factors in univariate analysis. CONCLUSIONS: In patients with ovarian YST, fertility-sparing surgery is as effective as radical surgery. Optimal cytoreductive surgery and standard BEP regimen are the most decisive prognostic factors. In these tumours, adjunctive therapeutic modalities to eradicate intra-abdominal disease and effective salvage therapy strategies are needed.
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Tumor del Seno Endodérmico/diagnóstico , Tumor del Seno Endodérmico/patología , Neoplasias Ováricas/diagnóstico , Neoplasias Ováricas/patología , Adolescente , Adulto , Anciano , Protocolos de Quimioterapia Combinada Antineoplásica/administración & dosificación , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Bleomicina/administración & dosificación , Niño , Cisplatino/administración & dosificación , Terapia Combinada , Tumor del Seno Endodérmico/terapia , Etopósido/administración & dosificación , Femenino , Estudios de Seguimiento , Humanos , Histerectomía , Persona de Mediana Edad , Recurrencia Local de Neoplasia/epidemiología , Neoplasias Ováricas/terapia , Ovariectomía , Pronóstico , Estudios Retrospectivos , Factores de Riesgo , Resultado del Tratamiento , Adulto JovenRESUMEN
POEMS syndrome is a rare multisystem disorder, which is characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. We report a 15-year-old girl with POEMS syndrome, who developed growth retardation, delayed puberty, gradually increasing abdominal distention, brown skin pigmentation, hypogonadism, hepatosplenomegaly, lympadenomegaly, monoclonal gammopathy, and anemia. The patient dramatically responded to pulse steroid therapy. To our knowledge, this patient is one of the youngest reported cases of POEMS syndrome.
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Trasplante de Células Madre Hematopoyéticas , Síndrome POEMS/diagnóstico , Síndrome POEMS/terapia , Esteroides/uso terapéutico , Abdomen/patología , Adolescente , Anemia/diagnóstico , Diagnóstico Diferencial , Femenino , Trastornos del Crecimiento/diagnóstico , Hepatomegalia/diagnóstico , Humanos , Hipogonadismo/diagnóstico , Paraproteinemias/diagnóstico , Trastornos de la Pigmentación/diagnóstico , Trastornos de la Pigmentación/terapia , Valor Predictivo de las Pruebas , Pubertad Tardía/diagnóstico , Resultado del TratamientoRESUMEN
BACKGROUND: Variation in serum levels of CD44, which acts as an adhesion receptor involved in lymphocyte migration and binding, have been reported in some malignancies. The aim of this study is to compare serum levels of CD44 in children with sarcomas with those in healthy children. PROCEDURE: CD44 levels were measured by enzyme-linked immunosorbent assay (ELISA) in serum samples taken at diagnosis from 55 children with sarcomas and from 27 healthy children of similar age, sex, and socioeconomic status. RESULTS: There was no statistically significant difference between CD44 serum levels of children with sarcomas and those of healthy children. No significant difference was observed between CD44 serum levels of each patient group and those of control group (P > 0.05). There was no significant difference among CD44 serum levels of patient groups according to stage or outcome. CONCLUSIONS: In this study, serum CD44 levels were not found to be of value in the diagnosis or prognosis in children with sarcomas.
Asunto(s)
Biomarcadores de Tumor/sangre , Receptores de Hialuranos/sangre , Osteosarcoma/diagnóstico , Rabdomiosarcoma/diagnóstico , Sarcoma de Ewing/diagnóstico , Adolescente , Análisis de Varianza , Estudios de Casos y Controles , Niño , Preescolar , Ensayo de Inmunoadsorción Enzimática , Femenino , Humanos , Lactante , Masculino , Osteosarcoma/sangre , Valor Predictivo de las Pruebas , Pronóstico , Rabdomiosarcoma/sangre , Sarcoma de Ewing/sangre , Estadísticas no Paramétricas , TurquíaRESUMEN
BACKGROUND: Ependymal tumors are classified as ependymoma (benign or low grade) versus anaplastic ependymoma (malignant or high grade). Ependymomas represent 5-10% of intracranial neoplasm in children. In this study, demographic data and the treatment results of pediatric patients with ependymal tumors, treated in a single institute, is reported. PATIENTS AND METHODS: Between 1989 and 2001, 40 (22 M/18 F) previously untreated patients with a median age of 5.5 years (3 months-15 years), of histologically proven ependymal tumors (except ependymoblastomas) were referred to the Institute of Oncology, University of Istanbul. The localization was supratentorial in 18, infratentorial in 20, both supra and infratentorial in two patients. Histologic subgroups were 18 ependymomas (43.6%), and 22 anaplastic ependymomas (56.4%). Total tumor resection was performed in 20 patients (50%), subtotal in 18 patients (45%), and biopsy only in 2 patients (5%). Postoperative treatment consisted of regional (8 patients) or craniospinal (CSI) (9 patients) radiotherapy (RT) in patients with ependymoma; regional (7 patients) or CSI RT (14 patients) with chemotherapy (ChT) in patients with anaplastic ependymoma; ChT only (1 patient) in patients less than 3 years of age. The standard technique for posterior fossa irradiation was parallel-opposed lateral fields and total dose was 45-54 Gy. Between September 1989 and May 1991 patients received regimen A, which consisted of RT followed by eight-in-one ChT, given every 4 weeks for eight courses. Patients who were treated between June 1991 and July 1994, received regimen B, which included two courses of postoperative "VEC" (vincristine, etoposide, cisplatin) ChT, administered every 3 weeks, followed by RT applied with low dose concomitant cisplatin used as a radiosensitizer. Patients with objective response to postoperative "VEC" continued to have "VEC" after completion of RT for six more courses. From August 1994 on, patients received regimen C, consisting of RT and concomitant infusion of cisplatin followed by "VCPCU" (vincristine, cyclophosphamide, procarbazine, lomustine) administered every 4 weeks for eight courses. RESULTS: A total of 40 patients were included in the outcome and survival data. The 5-year overall survival (OS) rate was 64.9%, and the 5-year progression-free survival rate was 50.8% for the whole series. Median time for progression or relapse was 24.3 months and there were 19 patients (43.6%) with relapse or progression. Non-metastatic patients (P = 0.0008, 5-year OS rate was 82% vs. 29%), and totally resected patients (P = 0.01, 5-year OS rate was 80% vs. 55%), and > or =3 years of age (P = 0.04, 5-year OS rate was 75% vs. 38%) had significantly better outcome. CONCLUSIONS: The majority of complete responders were patients who had total tumor removal. Treatment failure occurred mainly within the first 2 years, and outcome was dismal for patients who relapsed or had progressive disease. The median age at diagnosis is 6 years in our patient group; younger children (less than 3 years old) have less favorable outcome. There was no significant difference in survival or progression-free survival between the two histologic subtypes.
Asunto(s)
Neoplasias Encefálicas , Ependimoma , Adolescente , Neoplasias Encefálicas/mortalidad , Neoplasias Encefálicas/patología , Neoplasias Encefálicas/terapia , Niño , Preescolar , Supervivencia sin Enfermedad , Ependimoma/mortalidad , Ependimoma/patología , Ependimoma/terapia , Femenino , Humanos , Lactante , Masculino , Análisis Multivariante , Factores de Riesgo , Tasa de Supervivencia , Turquía/epidemiologíaRESUMEN
OBJECTIVES: Brain is a rare site of metastasis in most extracranial pediatric solid tumors. The aim of this study is to investigate the incidence, treatment, prognosis of brain metastasis in extracranial pediatric malignant tumors in a single institution and to review the literature. METHODS: From September 1989 to December 2002, 1100 children Asunto(s)
Neoplasias Encefálicas/epidemiología
, Neoplasias Encefálicas/secundario
, Sarcoma/epidemiología
, Sarcoma/secundario
, Adolescente
, Antineoplásicos/uso terapéutico
, Neoplasias Óseas/epidemiología
, Neoplasias Óseas/secundario
, Neoplasias Óseas/terapia
, Neoplasias Encefálicas/terapia
, Niño
, Preescolar
, Femenino
, Humanos
, Lactante
, Neoplasias Pulmonares/epidemiología
, Neoplasias Pulmonares/secundario
, Neoplasias Pulmonares/terapia
, Masculino
, Neoplasias del Mediastino/epidemiología
, Neoplasias del Mediastino/secundario
, Neoplasias del Mediastino/terapia
, Neoplasias de Células Germinales y Embrionarias/epidemiología
, Neoplasias de Células Germinales y Embrionarias/secundario
, Neoplasias de Células Germinales y Embrionarias/terapia
, Pronóstico
, Radioterapia Adyuvante
, Sarcoma/terapia
, Análisis de Supervivencia
, Tumor de Wilms/epidemiología
, Tumor de Wilms/secundario
, Tumor de Wilms/terapia